Tumores en ovario en pediatría / Ovarian tumors in pediatrics

Los tumores de ovario en la edad pediátrica son raros, representan 1-5 % de los tumores infantiles, con una incidencia anual de 2,6 casos por cada 100.000 pacientes. La mayoría son benignos y se tratan de quistes funcionales, sin embargo, entre 10-20 % son malignos y generalmente se presentan en adolescentes; estos últimos, se dividen en 3 grupos: tumores epiteliales, germinales, y estromales o de células sexuales. Método: Estudio retrospectivo de tipo transversal, observacional, no experimental. Se analizaron los pacientes con diagnóstico de tumor de ovario, ingresados en el Servicio de Cirugía Pediátrica del Hospital de Niños "Dr. José Manuel de los Ríos", entre el 1 de enero de 2017 y 01 de julio de 2022. Resultados: 18 pacientes incluidos en el estudio, con edad media de 8,23 años (DE 4,77); los síntomas más frecuentes presentados al momento del ingreso fueron: aumento de volumen abdominal (52,94 %, 9 pacientes), y dolor abdominal (35,29 %, 6 pacientes), entre otros. Reporte patológico: 2 pacientes con quistes de ovario funcional (11,76 %) y 16 pacientes con tumor neoplásico (88,23 %), de los cuales 8 fueron germinales (53,33 %), 5 tumores epiteliales (33,33 %) y 2 pacientes con linfoma (13,33 %). Conclusión: Los tumores de ovario en general tienen una edad promedio de presentación de 8 años y los tumores neoplásicos se presentaron principalmente en adolescentes, siendo el tipo histológico más frecuente el tumor germinal y dentro de este grupo el teratoma quístico maduro. (AU)

Ovarian tumors in pediatric age are rare, representing 1-5 % of childhood tumors, with an annual incidence of 2.6 cases per 100,000 patients. Most of them are benign and functional cysts; however, between 10-20 % are malignant and generally occur in teenagers; the latter are divided into 3 groups: epithelial, germinal, and stromal or sex cell tumors. Methods: Retrospective, cross-sectional, observational, non-experimental study. Patients with a diagnosis of ovarian tumor, admitted to the Pediatric Surgery Service of the Children's Hospital "Dr. José Manuel de los Ríos", between January 01, 2017 and July 01, 2022, were analyzed. Results: 18 patients included in the study, with mean age 8.23 years (SD 4.77); the most frequent symptoms presented at admission were: increased abdominal volume (52.94 %, 9 patients), and abdominal pain (35.29 %, 6 patients), among others. Pathological report: 2 patients with functional ovarian cysts (11.76 %) and 16 patients with neoplastic tumor (88.23 %), of which 8 were germinal (53.33 %), 5 epithelial tumors (33.33 %) and 2 patients with lymphoma (13.33 %). Conclusion: Ovarian tumors in general have an average age of presentation of 8 years and neoplastic tumors occurred mainly in teenagers, the most common histological type being the germ cell tumor and within this group the mature cystic teratoma. (AU)

Expert consensus: Profiling and management of advanced or metastatic epithelial ovarian cancer / Consenso de expertos basado en la evidencia: perfilamiento y manejo del carcinoma epitelial de ovario de alto grado avanzado.

Introduction and objective: The approach to patients with advanced or metastatic high-grade epithelial ovarian cancer (EOC) has evolved over time with the advent of new therapies and multimodal strategies. The objective of this consensus of experts is to generate national recommendations for the profiling and management of advanced or metastatic high-grade OEC, defined as stages III and IV of the "The International Federation of Gynecology and Obstetrics (FIGO) classification at the time of diagnosis to base on the literature review that included international evidence-based clinical practice guidelines (CPG). Material and methods: Eleven panelists (oncologists and gynecological oncologists) answered 8 questions about the profiling and management of advanced or metastatic ovarian epithelial carcinoma. The panelists were chosen for their academic profile and influence in national health institutions. Guidelines from the "ESMO Standardized Operating Procedures Consensus Conference" were used to develop the consensus. It was agreed that the level of agreement to accept a recommendation should be ≥ 80%. The document was peer reviewed. Results: Eight general recommendations are made, which are presented into five domains. Some of these recommendations are subdivided into specific recommendations. Initial treatment Recommendation 1.1 Complete primary cytoreduction (PCS) surgery is suggested as the initial therapy of choice for patients with high-grade or metastatic EOC, which should ideally be carried out in centers with experience, followed by adjuvant therapy. 1.2 Neoadjuvant chemotherapy followed by interval cytoreduction surgery (ICS) is suggested in those who are unlikely to achieve a complete cytoreduction in PCS either due to unresectable metastatic disease or who present unresectability criteria (imaging, laparoscopic and/or by laparotomy) and that have been defined by a gynecological oncologist and patients with poor functional status and comorbidities according to the criteria of the multidisciplinary team (clinical oncology, gynecological oncology, radiology, etc.). Recommendation 2. In patients with high-grade epithelial ovarian cancer (EOC), in stage III locally advanced or metastatic, who received neoadjuvant chemotherapy and achieved a complete or partial response (cytoreduction with tumor residue < 2.5 mm), the use of Hyperthermic IntraPeritoneal Chemotherapy (HIPEC) could be considered as an alternative to standard platinum-based adjuvant intravenous chemotherapy during interval cytoreductive surgery, after discussion in a multidisciplinary tumor board, at a center experienced in treating this type of patients. Use of genetic testing. Recommendation 3. It is suggested at the time of diagnosis to offer molecular genetic testing to all patients with high-grade advanced or metastatic EOC regardless of family history. Recommendation 4. It is suggested to offer genetic counseling, by qualified personnel, to all patients with high-grade advanced or metastatic EOC who are ordered genetic testing. Recommendation 5. It is suggested that all patients with advanced or metastatic high-grade EOC undergo a germ panel that includes the Breast Cancer Susceptibility Genes 1/2 genes (BRCA 1/2) and the other susceptibility genes according to with institutional protocols and the availability of genetic testing panels; If it is negative, then somatic testing should be performed that includes the homologous recombination deficiency (HRD) status, regardless of family history. Adjuvant Therapy Recommendation 6. 6.1. It is suggested that all patients with advanced stage III/IV EOC, with PSC of (0-2), got adjuvant intravenous chemotherapy as standard treatment within six weeks after Prc. It is suggested paclitaxel/carboplatin. Recommendation 6.2. It is suggested to use standard chemotherapy base on platinum plus Bevacizumab as adjuvant chemotherapy to patients with high-risk disease (EOC stage IV or stage III with suboptimal tumor cytoreduction), following by bevacizumab as maintenance. The use of bevacizumab as maintenance therapy is not recommended if bevacizumab was not included in the first line of treatment. We suggested the dose used in GOG-0218 and ICON7 trials. Recommendation 6.3 It is suggested combined intravenous/intraperitoneal chemotherapy only for selected patients, with optimal cytoreduction (residual lesions < 1 cm), especially those without residual disease (R0) and who are evaluated in a multidisciplinary meeting. It is not considered standard treatment. Recommendation 6.4. 6.4.1 It is suggested to use Poly ADP ribose polymerase (PARP) inhibitors such as olaparib or niraparib as maintenance after receiving first-line chemotherapy in patients with stage III/IV BRCA1/2 positive EOC who received platinumbased chemotherapy and obtained complete response/partial response (CR/PR), 6.4.2 It is suggested to use olaparib alone or in combination with bevacizumab or niraparib in patients with stage III/IV BRCA1/2 positive EOC who received platinum-based chemotherapy plus bevacizumab and achieved CR/PR. 6.4.3 It is suggested to use niraparibin patients with stage III/IV BRCA1/2 negative or unknown EOC who received platinum-based chemotherapy and achieved CR/PR. 6.4.4 It is suggested to use bevacizumab or olaparib plus bevacizumab in patients with EOC stage III/IV BRCA1/2 negative or unknown (HRD positive) who received platinum-based chemotherapy plus bevacizumab and obtained CR/PR. Treatment of disease relapse Recommendation 7. Secondary cytoreductive surgery followed by chemotherapy is suggested for selected patients with high-grade advanced EOC in first relapse, platinum-sensitive (platinum-free interval ≥ 6 months), positive "Arbeitsgemeinschaft Gynäkologische Onkologie ­ AGO" score or "I-model" positive (< 4.7) with a potential resection to R0 in centers with access to optimal surgical and postoperative support. Note: Platinum-free interval and AGO score have only been developed as positive predictors of complete resection and not to exclude patients from surgery. Recommendation 8. 8.1 For patients with relapse advanced high-grade EOC platinum-sensitive, the following is suggested: Platinum-based combination chemotherapy: carboplatin/liposomal doxorubicin or carboplatin/paclitaxel or carboplatin/nab-paclitaxel or carboplatin/docetaxel or carboplatin/gemcitabine) for six cycles. If combination therapy is not tolerated, give carboplatin or cisplatin alone. Combination chemotherapy (carboplatin/gemcitabine or carboplatin/paclitaxel or carboplatin/doxorubicin liposomal) plus bevacizumab followed by bevacizumab as maintenance (until progression or toxicity). Recommendation 8.2 For patients with relapsed advanced high-grade EOC platinum-resistant, it is suggested: Sequential treatment with chemotherapy, preferably with a non-platinum single agent (weekly paclitaxel or pegylated liposomal doxorubicin or docetaxel or oral etoposide or gemcitabine or trabectidine or, topotecan). Weekly paclitaxel or pegylated liposomal doxorubicin or topotecan could be administrate with or without bevacizumab. Other agents are considered potentially active (capecitabine, cyclophosphamide, ifosfamide, irinotecan, oxaliplatin, pemetrexed, vinorelbine, cyclophosphamide) could be recommended for later lines. Hormone receptor-positive patients who do not tolerate or have no response to cytotoxic regimens may receive hormone therapy with tamoxifen or other agents, including aromatase inhibitors (anastrozole and letrozole) or leuprolide acetate, or megestrol acetate. Patients with a performance score ≥ 3 should be considered only for best supportive care. Recommendation 8.3 Maintenance therapy with PARP inhibitors: It is suggested in patients with relapse advanced high-grade EOC stage III/IV BRCA1/2 (positive, negative or unknown) who have received two or more lines of platinum-based chemotherapy and have achieved CR/PR, use olaparib, niraparib or rucaparib. Niraparib could be useful in BRCA 1/2 +/-/unknown patients, as rucaparib, however, the latter does not yet have approval from the regulatory office in Colombia. Conclusions: It is expected that the recommendations issued in this consensus will contribute to improving clinical care, oncological impact, and quality of life of these women.

Introducción y objetivo: el abordaje de pacientes con cáncer epitelial de ovario (CEO) de alto grado avanzado o metastásico ha ido evolucionando a través del tiempo con el advenimiento de nuevas terapias y estrategias multimodales. El objetivo de este consenso de expertos es generar recomendaciones nacionales para el perfilamiento y manejo del CEO de alto grado avanzado o metastásico, definido como estadios III y IV de la clasificación de la Federación Internacional de Ginecología y Obstetricia (FIGO) al momento del diagnóstico, a partir de la revisión de la literatura que incluyó guías de práctica clínica (GPC) internacionales basadas en la evidencia. Materiales y métodos: once panelistas (oncólogos y ginecólogos oncólogos) respondieron ocho preguntas sobre el perfilamiento y manejo del carcinoma epitelial de ovario avanzado o metastásico. Los panelistas fueron escogidos por su perfil académico e influencia en instituciones de salud nacionales. Para el desarrollo del consenso se utilizaron los lineamientos de la "Conferencia de consenso de procedimientos operativos estandarizados de ESMO". Se definió que el nivel de acuerdo para aceptar una recomendación debía ser ≥ 80%. El documento fue revisado por pares. Resultados: Se hacen 8 recomendaciones generales, presentadas en cinco dominios; algunas de ellas se subdividen en recomendaciones específicas. Tratamiento inicial Recomendación 1 1.1. Como terapia inicial de elección para pacientes con CEO de alto grado o metastásico se sugiere la cirugía de citorreducción primaria (Cpr) completa que, idealmente, debe realizarse en centros con experiencia, seguida de terapia adyuvante. 1.2. Se sugiere quimioterapia neoadyuvante seguida de cirugía de citorreducción de intervalo (Cint) en quienes sea improbable alcanzar una citorreducción completa en la Cpr, bien sea por enfermedad metastásica no resecable o que presenten criterios de irresecabilidad (imagenológicos, laparoscópicos o por laparotomía) que hayan sido definidos por un ginecólogo oncólogo. También en pacientes con un pobre estado funcional y comorbilidades de acuerdo con el criterio del equipo multidisciplinario (oncología clínica, ginecología oncológica, radiología, etc.). Recomendación 2. En pacientes con CEO de alto grado, en estadio III localmente avanzado o metastásico, que recibieron quimioterapia neoadyuvante y alcanzaron respuesta completa o parcial (citorreducción con residuo tumoral < 2,5 mm), se podría evaluar el uso de la quimioterapia intraperitoneal hipertérmica (Hyperthermic IntraPeritoneal Chemotherapy - HIPEC) como alternativa a la quimioterapia IV adyuvante estándar basada en platinos durante la Cint, previa discusión en junta multidisciplinaria, en un centro de experiencia en este tipo de pacientes. Uso de pruebas genéticas Recomendación 3. Al momento del diagnóstico, se sugiere ofrecer testeo molecular genético a toda paciente con CEO de alto grado avanzado o metastásico, independientemente de la historia familiar. Recomendación 4. Se sugiere ofrecer asesoramiento genético, por parte de personal calificado, a toda paciente con CEO de alto grado avanzado o metastásico a quien se le ordene un testeo genético. Recomendación 5. Se sugiere que a toda paciente con CEO de alto grado avanzado o metastásico se le realice panel germinal que incluya los genes de susceptibilidad al cáncer de mama 1/2 (BRCA 1/2) y los otros genes de susceptibilidad de acuerdo con los protocolos institucionales y la disponibilidad de paneles de testeo genético; si es negativo entonces se debería realizar testeo somático que incluya el estatus de deficiencia de la recombinación homóloga (homologous recombination deficiency - HRD), independientemente de la historia familiar. Terapia adyuvante Recomendación 6 6.1. Se sugiere que a toda paciente con CEO estadios III/IV avanzado o metastásico, con estatus de desempeño (performance score care - PSC) de 0-2 se le administre como tratamiento estándar quimioterapia intravenosa (IV) adyuvante dentro de las seis semanas posteriores a la Cpr. Se sugiere administrar paclitaxel/carboplatino. 6.2. Se sugiere utilizar quimioterapia estándar basada en platino más bevacizumab como adyuvancia en pacientes con enfermedad de alto riesgo (CEO estadios IV o III con citorreducción tumoral subóptima), continuando con bevacizumab como mantenimiento. No se recomienda el uso de bevacizumab como terapia de mantenimiento si no se incluyó en la primera línea de tratamiento. Se sugiere seguir los esquemas de los estudios Gynecologic Oncology Group Study (GOG-0218) e International Collaborative Ovarian Neoplasm (ICON7). 6.3. Se sugiere la quimioterapia combinada IV/intraperitoneal (IP) solo para pacientes seleccionadas, con una citorreducción óptima (lesiones residuales < 1 cm), en especial aquellas sin enfermedad residual (R0) y que sean evaluadas en junta multidisciplinaria. La quimioterapia combinada IV/IP no se considera como tratamiento estándar. 6.4. 6.4.1. Se sugiere utilizar inhibidores de poli(ADP-ribosa) polimerasa (PARP) tales como olaparib o niraparib como mantenimiento después de recibir una primera línea de quimioterapia en pacientes con CEO estadios III/IV BRCA1/2 positivo que recibieron quimioterapia basada en platino y obtuvieron respuesta completa/respuesta parcial (RC/RP). 6.4.2. Se sugiere utilizar olaparib solo o en combinación con bevacizumab o niraparib en pacientes con CEO estadios III/IV BRCA1/2 positivo que recibieron quimioterapia basada en platino más bevacizumab y obtuvieron RC/RP. 6.4.3. Se sugiere utilizar niraparib en pacientes con CEO estadio III/IV BRCA1/2 negativo o desconocido que recibieron quimioterapia basada en platino y obtuvieron RC/RP. 6.4.4. Se sugiere utilizar bevacizumab u olaparib más bevacizumab en pacientes con CEO estadios III/IV BRCA1/2 negativo o desconocido (HRD positivo) que recibieron quimioterapia basada en platino más bevacizumab y obtuvieron RC/RP. Tratamiento de la recaída de la enfermedad Recomendación 7. Se sugiere la realización de la cirugía de citorreducción secundaria (Csec), seguida de quimioterapia, a pacientes seleccionadas con CEO de alto grado avanzado o metastásico en primera recaída, platino-sensibles (intervalo libre de platinos ≥ 6 meses), puntuación Arbeitsgemeinschaft Gynäkologische Onkologie (AGO) positiva o Integrate model (I-Model) positivo (< 4,7), y con una potencial resección a R0, en centros con acceso a soporte quirúrgico y posoperatorio óptimo. Nota: el intervalo libre de tratamiento con platinos y la puntuación AGO solo se han desarrollado como predictores positivos de resección completa y no para excluir a las pacientes de la cirugía. Recomendación 8 8.1. Para pacientes con CEO de alto grado avanzado o metastásico en recaída platino-sensibles se sugiere: Quimioterapia combinada basada en platino: carboplatino/doxorrubicina liposomal o carboplatino/paclitaxel o carboplatino/ nab-paclitaxel o carboplatino/docetaxel o carboplatino/gemcitabina, por seis ciclos. Si no se tolera la terapia combinada, dar carboplatino o cisplatino solo. Quimioterapia combinada: carboplatino/gemcitabina o carboplatino/paclitaxel o carboplatino/doxorubicina liposomal, más bevacizumab, seguida de bevacizumab como mantenimiento (hasta progresión o toxicidad). 8.2. Para pacientes con CEO de alto grado avanzado o metastásico en recaída, platino-resistentes, se sugiere: Tratamiento secuencial con quimioterapia, preferiblemente con un agente único que no sea un platino (paclitaxel semanal o doxorrubicina liposomal pegilada o docetaxel o etopósido oral o gemcitabina o trabectidina o topotecan). El paclitaxel semanal o la doxorrubicina liposomal pegilada o el topotecan pueden ser administrados con o sin bevacizumab. Existen otros agentes que se consideran potencialmente act ivos (capecitabina, ciclofosfamida, ifosfamida, irinotecán, oxaliplatino, pemetrexed, vinorelbina, ciclofosfamida), que se podrían recomendar para líneas posteriores. Las pacientes con receptores hormonales positivos que no toleran o no tienen respuesta a los regímenes citotóxicos pueden recibir terapia hormonal con tamoxifeno u otros agentes, incluidos los inhibidores de la aromatasa (anastrozol y letrozol) o acetato de leuprolide o acetato de megestrol. Pacientes con PSC ≥ 3 deberían ser consideradas solo para el mejor cuidado de soporte. 8.3. Terapia de mantenimiento con inhibidores PARP. Para pacientes con CEO de alto grado avanzado o metastásico en recaída estadios III/IV BRCA1/2 (positivo, negativo o desconocido), que hayan recibido dos o más líneas de quimioterapia basada en platino y hayan alcanzado RC/RP, se sugiere utilizar olaparib, niraparib o rucaparib. El niraparib podría ser útil en pacientes BRCA 1/2 +/-/desconocido, al igual que el rucaparib, sin embargo, este último no tiene aún aprobación del ente regulador en Colombia. Conclusiones: se espera que las recomendaciones emitidas en este consenso contribuyan a mejorar la atención clínica, el impacto oncológico y la calidad de vida de estas mujeres.

Preoperative neutrophil/lymphocyte ratio as prognostic factor in epithelial ovarian cancer / Cociente neutrófilos/linfocitos preoperatorio como factor pronóstico en el cáncer epitelial de ovario.

Objectives: To determine if there is an association between the neutrophil to lymphocyte ratio (NLR) and prognosis in patients with epithelial ovarian cancer (EOC) diagnosed and treated in a Spanish population. Material and methods: Retrospective cohort of patients with epithelial ovarian cancer who had neutrophil and lymphocyte values in complete blood count before the histopathological diagnosis and survival of at least three months, in an intermediate complexity hospital. Convenience sampling. Measured variables included age, menopausal stage, parity, International Federation of Gynecology and Obstetrics (FIGO) stage, treatment type, residual tumor, lymph node involvement, presence of ascites, cytology, histologic type, differentiation grade, and CA-125 values. Additionally, outcomes, overall survival, disease/progression-free survival were also measured. Bivariate inferential and Cox regression analyses were performed. Results: Out of 78 candidates, 60 women with EOC were included. Of them, 24 (40%) had a low NLR (≤ 2,9) while 36 (60%) had a high NLR (> 2,9). An association was found between high NLR levels and suboptimal cytoreductive surgery. High NLR ratios were associated with lower overall survival (Hazard ratio (HR): 4.1; 95% CI: 1.4-11.8) and lower 5-year disease-free survival (HR: 2.6; 95% CI: 1.2-5.7). Conclusions: A plasma neutrophil to lymphocyte ratio of more than 2.9 was associated with poor prognosis in patients with epithelial ovarian cancer in our setting. There is a need to establish the optimal cut-off point and conduct prospective studies with larger patient numbers in order to support this information.

Objetivos: evaluar si hay asociación entre los valores del cociente plasmático neutrófilos/ linfocitos (NLR) y el pronóstico en pacientes con cáncer epitelial de ovario (CEO) diagnosticadas y tratadas en una población española. Materiales y métodos: cohorte retrospectiva de pacientes con cáncer epitelial de ovario que tuvieran un recuento de neutrófilos y linfocitos en hemograma previo al diagnóstico histopatológico en un hospital de nivel medio de complejidad y posterior sobrevida de, al menos, 3 meses. Muestreo por conveniencia. Se midieron: edad, estado menopáusico, paridad, estadio Federación International de Ginecología y Obstetricia (FIGO), tipo de tratamiento, tumor residual, afectación ganglionar, presencia de ascitis, citología, tipo histológico, grado de diferenciación y cifras de CA-125; como desenlaces, sobrevida global y sobrevida libre de enfermedad o progresión. Análisis inferencial bivariado y por regresión de Cox. Resultados: de 78 candidatas, ingresaron 60 mujeres con CEO. De ellas, 24 (40%) presentaron un NLR bajo (≤ 2,9) y 36 (60 %) elevado (> 2,9). Se encontró asociación entre los niveles altos de NLR y cirugía citoreductora subóptima. Los niveles altos de NLR se asociaron a menor sobrevida global (Hazard ratio (HR): 4,1; IC 95%: 1,4-11,8) y menor sobrevida libre de enfermedad a los 5 años (HR:2,6; IC 95 %: 1,2-5,7). Conclusiones: un cociente plasmático neutrófilos/linfocitos mayor de 2,9 se asoció a un mal pronóstico en pacientes con cáncer epitelial de ovario en nuestro medio. Se necesita determinar el punto de corte óptimo y realizar estudios prospectivos con mayor número de pacientes que avalen esta información.

SEOM-GEICO clinical guideline on epithelial ovarian cancer (2023).

In recent years, the incorporation of new strategies to the therapeutic armamentarium has completely changed the outcomes of epithelial ovarian cancer (EOC). The identification of new predictive and prognostic biomarkers has also enabled the selection of those patients more likely to respond to targeted agents. Nevertheless, EOC is still a highly lethal disease and resistance to many of these new agents is common. The objective of this guideline is to summarize the most relevant strategies to manage EOC, to help the clinician throughout the challenging diagnostic and therapeutic processes and to provide evidence-based recommendations.

The every woman study™ low- and middle-income countries edition protocol: A multi-country observational study to assess opportunities and challenges to improving survival and quality of life for women with ovarian cancer.

BACKGROUND: Ovarian cancer is a challenging disease to diagnose and treat effectively with five-year survival rates below 50%. Previous patient experience research in high-income countries highlighted common challenges and opportunities to improve survival and quality of life for women affected by ovarian cancer. However, no comparable data exist for low-and middle-income countries, where 70% of women with the disease live. This study aims to address this evidence gap. METHODS: This is an observational multi-country study set in low- and middle-income countries. We aim to recruit over 2000 women diagnosed with ovarian cancer across multiple hospitals in 24 countries in Asia, Africa and South America. Country sample sizes have been calculated (n = 70-96 participants /country), taking account of varying national five-year disease prevalence rates. Women within five years of their diagnosis, who are in contact with participating hospitals, are invited to take part in the study. A questionnaire has been adapted from a tool previously used in high-income countries. It comprises 57 multiple choice and two open-ended questions designed to collect information on demographics, women's knowledge of ovarian cancer, route to diagnosis, access to treatments, surgery and genetic testing, support needs, the impact of the disease on women and their families, and their priorities for action. The questionnaire has been designed in English, translated into local languages and tested according to local ethics requirements. Questionnaires will be administered by a trained member of the clinical team. CONCLUSION: This study will inform further research, advocacy, and action in low- and middle-income countries based on tailored approaches to the national, regional and global challenges and opportunities. In addition, participating countries can choose to repeat the study to track progress and the protocol can be adapted for other countries and other diseases.

Granulosa cell tumor of the ovary: a series of 6 cases.

Granulosa cell tumor (GCT) is a rare ovarian malignancy that represents only 2-3% of all cases. There are two subtypes of GCT: juvenile/JGCT (5% of cases) and adult/AGCT (95% of cases). This study aimed to describe a series of 6 GCT cases. The 6 study patients were managed from June 2011 to November 2022 in a private oncology clinic located in Teresina (PI), Brazil. At diagnosis, the mean patient age was 47 years, and symptoms in 5 patients (83%) were pelvic pain and/or increased abdominal volume. The majority of the patients (N=4/67%) had no comorbidities or findings related to GCT on physical examination. The mean tumor size was 11 cm. Five (83%) tumors were stage Ia and one tumor (17%) was stage III. Regarding tumor subtype, 5 (83%) were AGCT and 1 (17%) was JGCT. Surgical treatment consisted of unilateral salpingo-ophorectomy in 2 patients (33%), total hysterectomy and bilateral salpingo-ophorectomy in 3 patients (50%), and cytoreduction (suboptimal) in 1 patient (17%). After a mean follow-up period of 62.7 months, 5 patients (83%) are still alive and free of disease. One (17%) died from disease progression after 126 months. In the current study, disease-free overall survival was 83%, in a mean follow-up period of 62.7 months.

Delivery of hereditary cancer genetics services to patients newly diagnosed with ovarian and endometrial cancers at three gynecologic oncology clinics in the USA, Brazil, and Mexico.

OBJECTIVE: Three gynecologic oncology clinics located in the USA, Brazil, and Mexico collaborated to evaluate their delivery of hereditary cancer genetics services. This descriptive retrospective review study aimed to establish baseline rates and timeliness of guideline-recommended genetics service delivery to patients with ovarian, fallopian tube, primary peritoneal (ovarian), and endometrial cancers at each clinic. METHODS: Patients who were newly diagnosed with ovarian and endometrial cancers between September 1, 2018 and December 31, 2020 were identified from the medical records of the clinics. Genetics service delivery metrics included the rates of mismatch repair deficiency tumor testing for patients with endometrial cancer (microsatellite instability/immunohistochemistry, MSI/IHC), referral to genetics services for patients with ovarian cancer, completed genetics consultations, and germline genetic testing for patients with ovarian and endometrial cancers. Timeliness was calculated as the average number of days between diagnosis and the relevant delivery metric. Descriptive statistics were used to analyze data. RESULTS: In total, 1195 patients (596 with ovarian cancer, 599 with endometrial cancer) were included in the analysis, and rates of genetics service delivery varied by clinic. For patients with ovarian cancer, referral rates ranged by clinic from 32.6% to 89.5%; 30.4-65.1% of patients completed genetics consultation and 32.6-68.7% completed genetic testing. The timeliness to genetic testing for patients with ovarian cancer ranged by clinic from 107 to 595 days. A smaller proportion of patients with endometrial cancer completed MSI/IHC testing (10.0-69.2%), with the average time to MSI/IHC ranging from 15 to 282 days. Rates of genetics consultation among patients with endometrial cancer ranged by clinic from 10.8% to 26.0% and 12.5-16.6% completed genetic testing. CONCLUSIONS: All clinics successfully established baseline rates and timeliness of delivering hereditary cancer genetics services to patients with ovarian and endometrial cancers. Lower rates of delivering genetics services to patients with endometrial cancer warrant additional research and quality improvement efforts.

[Pseudoascitis by giant ovaric cyst] / Pseudoascitis por quiste ovárico gigante.

The term pseudoascitis is used in patients who give the false impression of ascites, with abdominal distension but without peritoneal free fluid. The case of a 66-year-old woman, hypertensive and hypothyroid with occasional alcohol consumption, who consults due to progressive abdominal distension of 6 months of evolution and diffuse percussion dullness is presented, in whom a paracentesis is performed with the wrong endorsement of examination ultrasound that reports abundant intrabdominal free fluid (Fig. 1), later finding in the CT scan of the abdomen and pelvis an expansive process of cystic appearance of 295mm x 208mm x 250mm. Left anexectomy is programmed (Fig. 2) with pathological report of mucinous ovarian cystadenoma. The case report refers to the availability of the giant ovarian cyst within the differential diagnosis of ascites. If no symptoms or obvious signs of liver, kidney, heart or malignant disease are found and / or ultrasound does not reveal typical signs of intra-abdominal free fluid (fluid in the bottom of the Morrison or Douglas sac, presence of floating free intestinal handles), a CT scan and / or an RMI should be requested before performing paracentesis, which could have potentially serious consequences.

El término pseudoascitis, se utiliza en los pacientes que dan la falsa impresión de ascitis, con distensión abdominal pero sin líquido libre peritoneal. Se presenta el caso de una mujer de 66 años, hipertensa e hipotiroidea con consumo ocasional de alcohol, que consulta por distensión abdominal progresiva de 6 meses de evolución y matidez difusa a la percusión, en quien se realiza una paracentesis con el aval equivoco de examen ecográfico que informa abundante líquido libre  intrabdominal (Fig. 1), hallando posteriormente en TAC de abdomen y pelvis un proceso expansivo de aspecto quístico de 295mm x 208mm x 250mm. Se programa anexectomia izquierda (Fig. 2) con informe anatomopatológico de cistoadenoma mucinoso de ovario. La comunicación del caso remite a tener disponible el quiste ovárico gigante dentro de los diagnósticos diferenciales de ascitis. Si no se hallan síntomas o signos evidentes de insuficiencia hepática, renal, cardiaca o enfermedad maligna y/o la ecografía no revela signos típicos de líquido libre intrabdominal  (líquido en el fondo de saco de Morrison o de Douglas, presencia de asas intestinales libres flotantes), se debería solicitar una TAC y/o una RMI antes de realizar una paracentesis, la cual podría tener consecuencias potencialmente graves.

Rare virilizing tumor: ovarian steroid cell tumor, not otherwise specified: a case report.

BACKGROUND: Ovarian steroid cell tumors, not otherwise specified is a rare sex cord-stromal tumor. Almost 60% of all steroid cell tumors are categorized as not otherwise specified and represent less than 0.1% of all ovarian neoplasm. Some of them are endocrinologically active, producing virilization signs in young women. The recommended treatment is primarily surgical. CASE PRESENTATION: We present the case of a 20-year-old Mexican woman with secondary amenorrhea and virilization signs. She was treated with combined oral contraceptives from 13 years old, due to a misdiagnosis of polycystic ovarian syndrome. However, 4 months after stopping medication, amenorrhea and virilization signs worsened. Biochemically, she had high serum total testosterone and free testosterone levels, and a pelvic and transvaginal ultrasound followed by a pelvic tomography scan demonstrated a right adnexal tumor. She underwent right salpingo-oophorectomy and the histopathological and immunochemistry exams confirmed the diagnosis. The patient was followed for a year after surgery and until then, her menses were regular and she had no recurrence of virilization signs. CONCLUSION: The purpose of this case report is to alert physicians to rule out ovarian steroid cell tumor, not otherwise specified diagnosis in young women with increased testosterone after discarding common causes such as polycystic ovarian syndrome. A multidisciplinary team including a gynecologist, endocrinologist, radiologist, and pathologist should be involved for correct diagnosis at the proper time.

Familial history and prevalence of BRCA1, BRCA2 and TP53 pathogenic variants in HBOC Brazilian patients from a public healthcare service.

Several studies have demonstrated the cost-effectiveness of genetic testing for surveillance and treatment of carriers of germline pathogenic variants associated with hereditary breast/ovarian cancer syndrome (HBOC). In Brazil, seventy percent of the population is assisted by the public Unified Health System (SUS), where genetic testing is still unavailable. And few studies were performed regarding the prevalence of HBOC pathogenic variants in this context. Here, we estimated the prevalence of germline pathogenic variants in BRCA1, BRCA2 and TP53 genes in Brazilian patients suspected of HBOC and referred to public healthcare service. Predictive power of risk prediction models for detecting mutation carriers was also evaluated. We found that 41 out of 257 tested patients (15.9%) were carriers of pathogenic variants in the analyzed genes. Most frequent pathogenic variant was the founder Brazilian mutation TP53 c.1010G > A (p.Arg337His), adding to the accumulated evidence that supports inclusion of TP53 in routine testing of Brazilian HBOC patients. Surprisingly, BRCA1 c.5266dupC (p.Gln1756fs), a frequently reported pathogenic variant in Brazilian HBOC patients, was not observed. Regarding the use of predictive models, we found that familial history of cancer might be used to improve selection or prioritization of patients for genetic testing, especially in a context of limited resources.

Clinical characteristics of patients with Breast and / or Ovarian Cancer with mutations in the BRCA1 and BRCA2 genes in Córdoba, Argentina / [Características clínicas de pacientes con Cáncer de Mama y / o Cáncer de Ovario con mutaciones en los genes BRCA1 y BRCA2 en Córdoba, Argentina.]

Introduction: Hereditary predisposition syndromes to cancer represent 5-10% of cancer cases, the most studied being HBOC produced by mutations in the BRCA1/2 genes. Objectives: To describe clinical, histopathological and PV characteristics in patients with HBOC in Córdoba, Argentina and compare it with those without BRCA1/2 mutations. Methods: Cross-sectional, correlational and observational analysis of patients from Córdoba. The ANOVA, Student's t test contingency tables and Fisher exact test were used the significance level was α = 0.05. Results: 155 women with BC, OC and BC/OC were studied. 40 BRCA1 / 2 mutations were identified. No differences were found in the age of diagnosis between patients with and without BRCA1/2 mutations. A significant association was found between VP in BRCA1/2 and the type of cancer (p = 0.003); all cases with BC/OC presented mutations in BRCA1/2. No significant association was found between mutated/non-mutated and personal history, family background, and ER-PR-HER2. 23.1% and 38.1% of BC cases were TN in individuals with VP in BRCA 1 and 2, respectively. The prevalence of mutations was 25.8% and the prevalence of novel PV was 10.0%. Conclusions: Patients with BC-VP BRCA1/2 are associated with ductal histology, and younger age of presentation with VP BRCA1. We did not find significant differences in the age at diagnosis of BC between patients with BRCA1 and BRCA2 mutations, a higher proportion of BC TN is observed than in the general population. In our sample, the prevalence of BRCA1/2 mutations among patients who meet criteria for HBOC is 25.8%, with 10% new pathogenic variant.

Introducción: Los síndromes de predisposición hereditaria al cáncer representan un 5-10% de los casos de cáncer, el más estudiado es HBOC producido por mutaciones en los genes BRCA1/2. Objetivos: Describir características clínicas, histopatológicas y VP en pacientes con HBOC en Córdoba, Argentina y compararla con aquellas sin mutaciones en BRCA1/2. Métodos: Análisis transversal, correlacional y observacional de pacientes de Córdoba. Se utilizó la prueba ANOVA, t de Student, tablas de contingencia y prueba exacta de Fisher, el nivel de significancia fue α=0,05. Resultados: Se estudiaron 155 mujeres con CM, CO y CM/CO. Se identificaron 40 mutaciones en BRCA1/2. No se encontraron diferencias en edad de diagnóstico entre pacientes con y sin mutaciones en BRCA1/2. Se encontró asociación significativa entre VP en BRCA1/2 y el tipo de cáncer (p=0,003); todos los casos con CM/CO presentaron mutaciones en BRCA1/2. No se encontró asociación significativa entre mutados/no mutados y AP, AF, RE-RP-HER2. El 23.1% y 38.1% de los casos de CM fueron TN en individuos con VP en BRCA 1 y 2 respectivamente. La prevalencia de mutaciones fue 25,8% y la prevalencia de VP noveles del 10,0%. Conclusiones: Las pacientes con CM-VP BRCA1/2 están asociadas con histología ductal, y menor edad de presentación con VP BRCA1. No encontramos diferencias significativas en edad de diagnóstico del CM entre pacientes con mutaciones BRCA1 y BRCA2, se observa una mayor proporción CM TN que en la población en general. En nuestra muestra, la prevalencia de mutaciones en BRCA1/2 entre los pacientes que reúnen criterios para HBOC es del 25,8%, con 10% de VP noveles.

Tumor de ovario de los cordones sexuales con túbulos anulares: reporte de caso y revisión de la literatura / Ovarian sex cord tumor with annular tubules: case report and review of the literature

RESUMEN Objetivos: Reportar un caso de Tumor de Ovario de los Cordones Sexuales con Túbulos Anulares (TCSTA), hacer una revisión de la literatura acerca del diagnóstico, tratamiento y pronóstico de esta condición. Materiales y métodos: Se informa el caso de una mujer que consultó al Instituto Nacional de Cancerología, Bogotá (Colombia), con diagnóstico final de TCSTA avanzado, quien recibió tratamiento quirúrgico y quimioterapia con evolución satisfactoria a los 6 meses. Se realizó una búsqueda de la literatura en las bases de datos Medline vía PubMed, LILACS y Scielo, que incluía reportes y series de caso de mujeres con diagnóstico de TCSTA, publicados desde 1990, sin incluir rango de edad. Se extrajo información sobre el diagnóstico, tratamiento y pronóstico reportado. Se realizó resumen narrativo de los hallazgos. Resultados: Se incluyeron 14 publicaciones que incluían 26 pacientes. La edad media al diagnóstico fue de 22,5 años. Los síntomas principales fueron alteraciones de la menstruación y dolor pélvico. La tomografía computarizada fue la tecnología de imágenes más frecuentemente utilizada. El tratamiento fue quirúrgico, siempre usado, acompañado de quimioterapia (29 %); 2 casos recibieron radioterapia. Hubo recaída en el 20 % de los casos. La mortalidad fue del 12,5 %, toda en el primer año. Conclusiones: La información sobre la utilidad diagnóstica de las imágenes, marcadores tumorales y estudios de histoquímica es escasa, como también los datos sobre el pronóstico de la entidad. El tratamiento quirúrgico es el de elección, teniendo en cuenta el deseo de fertilidad de la paciente y el estadio tumoral. Se requieren más estudios que documenten de manera más detallada el manejo de esta condición.

ABSTRACT Objectives: To report a case of ovarian sex cord tumor with annular tubules (SCTAT) and conduct a literature review on diagnosis, treatment and prognosis of this condition. Material and methods: Case report of a woman with a final diagnosis of advanced SCTAT seen at the National Cancer Institute in Bogota (Colombia) who received surgical treatment and chemotherapy with a satisfactory course after 6 months. A literature search was conducted in the Medline via PubMed, LILACS and Scielo databases, including case reports and series of women diagnosed with SCTAT published since 1990, not using age ranges. Information about diagnosis, treatment and reported prognosis was retrieved. A narrative summary of the findings was prepared. Results: Fourteen publications with 26 patients were included. Mean age at diagnosis was 22.5 years. The main symptoms were menstruation abnormalities and pelvic pain. Computed tomography (CT) was the imaging technology most frequently used. Surgical treatment was used in all cases, together with chemotherapy in 29 %; 2 patients received radiotherapy. Recurrence occurred in 20 % of cases. Mortality was 12.5 %, with all deaths occurring within the first year. Conclusions: There is a paucity of information about the diagnostic utility of imaging, tumor markers and histochemical studies, as well as prognosis of this disease condition. Surgery is the treatment of choice, taking into consideration the patient's wishes regarding fertility, as well as the stage of the tumor. Further studies are needed to provide more detailed information about this condition.

Germline Pathogenic Variant Prevalence Among Latin American and US Hispanic Individuals Undergoing Testing for Hereditary Breast and Ovarian Cancer: A Cross-Sectional Study.

PURPOSE: To report on pathogenic germline variants detected among individuals undergoing genetic testing for hereditary breast and/or ovarian cancer (HBOC) from Latin America and compare them with self-reported Hispanic individuals from the United States. METHODS: In this cross-sectional study, unrelated individuals with a personal/family history suggestive of HBOC who received clinician-ordered germline multigene sequencing were grouped according to the location of the ordering physician: group A, Mexico, Central America, and the Caribbean; group B, South America; and group C, United States with individuals who self-reported Hispanic ethnicity. Relatives who underwent cascade testing were analyzed separately. RESULTS: Among 24,075 unrelated probands across all regions, most were female (94.9%) and reported a personal history suggestive of HBOC (range, 65.0%-80.6%); the mean age at testing was 49.1 ± 13.1 years. The average number of genes analyzed per patient was highest in group A (A 63 ± 28, B 56 ± 29, and C 40 ± 28). Between 9.1% and 18.7% of patients had pathogenic germline variants in HBOC genes (highest yield in group A), with the majority associated with high HBOC risk. Compared with US Hispanics individuals the overall yield was significantly higher in both Latin American regions (A v C P = 1.64×10-9, B v C P < 2.2×10-16). Rates of variants of uncertain significance were similar across all three regions (33.7%-42.6%). Cascade testing uptake was low in all regions (A 6.6%, B 4.5%, and C 1.9%). CONCLUSION: This study highlights the importance of multigene panel testing in Latin American individuals with newly diagnosed or history of HBOC, who can benefit from medical management changes including targeted therapies, eligibility to clinical trials, risk-reducing surgeries, surveillance and prevention of secondary malignancy, and genetic counseling and subsequent cascade testing of at-risk relatives.

Abdominal tuberculosis mimicking ovarian cancer. A case series report.

INTRODUCTION: Peritoneal tuberculosis (abdominal tuberculosis) can be confused with a malignant neoplasm. OBJECTIVE: To describe clinical and demographic characteristics of patients with abdominal tuberculosis mimicking advanced ovarian cancer, diagnosed in a national reference cancer center. METHODS: Clinical and pathological characteristics of nine patients with abdominal tuberculosis that clinically resembled advanced ovarian cancer are described. RESULTS: Median age was 47 years; the most common socioeconomic status was low (44%). Abdominal pain and weight loss occurred in 77.7%; ascites, in 55.5%; 22.2% had a positive COMBE test, and 100% had no history of pulmonary tuberculosis. CA-125 elevation was reported in 77.7%, with levels > 500 U/mL in 57.1%. Tomography reported carcinomatosis in 50% and pelvic tumor and ascites in 37.5%. All patients underwent surgery, where 62.5% were diagnosed by intraoperative pathology study as neoplastic disease. CONCLUSION: Tuberculosis is considered the great imitator, which is why abdominal tuberculosis diagnosis should be borne in mind when faced with a suspicious case, even when clinical presentation, imaging studies, and even intraoperative examination suggest ovarian cancer.

INTRODUCCIÓN: La tuberculosis peritoneal (tuberculosis abdominal) puede ser confundida con una neoplasia maligna. OBJETIVO: Describir características clínicas y demográficas de pacientes con tuberculosis abdominal que semeja cáncer de ovario avanzado, diagnosticados en un centro oncológico de referencia nacional. MÉTODOS: Se describen las características clínicas y patológicas de nueve pacientes con tuberculosis abdominal que clínicamente semejaba cáncer de ovario avanzado. RESULTADOS: La mediana de edad fue de 47 años, el estrato socioeconómico más común fue bajo (44 %). El dolor abdominal y la pérdida ponderal se presentaron en 77.7 %, ascitis en 55.5 %, prueba COMBE positiva en 22.2 % y ausencia de antecedente de tuberculosis pulmonar en 100 %. La elevación de CA-125 se reportó en 77.7 %, con > 500 U/mL en 57.1 %. La tomografía indicó carcinomatosis en 50 % y tumor pélvico y ascitis en 37.5 %. Todas las pacientes fueron sometidas a cirugía; 62.5 % fueron diagnosticadas mediante estudio patológico transoperatorio como enfermedad neoplásica. CONCLUSIÓN: La tuberculosis es considerada como la gran imitadora, por ello se debe tener en mente el diagnóstico de tuberculosis abdominal ante un caso sospechoso, aun cuando la presentación clínica, estudios de imagen e, incluso, el examen transoperatorio sugieran cáncer de ovario.

[Neonatal bilateral ovarian cystadenoma: Conservative or surgical treatment?] / Cistoadenoma ovárico bilateral neonatal, ¿tratamiento conservador o quirúrgico?

INTRODUCTION: Bilateral complex ovarian cysts in newborns are rare and their reporting becomes imperative to in crease knowledge about the best therapeutic management. OBJECTIVE: To describe the clinical case of a newborn with a diagnosis of bilateral complex ovarian cysts and to discuss the conditions for conservative or surgical management according to the ultrasound characteristics of the cyst. CLINICAL CASE: At 35 weeks of gestational age, prenatal ultrasound identified the presence of cystic masses in both adnexa, so it was decided to interrupt the pregnancy by cesarean section at 37 weeks. After birth, bilateral ovarian cysts of 3.5 x 4.4 x 2.7 and 3.4 x 2.4 x 3.3 cm, right and left, respectively, were corro borated. The right cyst had a septum of 1.4 mm thick and thickened wall of 3 mm which was com patible with complex cysts. On the 4th day of extrauterine life, laparoscopic vacuum aspiration and deroofing with electrocautery of the upper wall of both cysts was performed, without complications. The diagnosis of ovarian serous cystadenoma was made by pathological anatomy. CONCLUSIONS: We describe a case with adequate prenatal diagnosis and laparoscopic surgical intervention of a bilateral ovarian cyst > 4 cm. Prenatal diagnosis is essential for choosing the best therapy management (con servative or surgical) depending on the echography characteristics of the cyst. Neonatal surgery is recommended for simple ovarian cysts >4 cm, complex cysts regardless of their size, and those that become complex cysts during conservative management.

Tumor de ovario borderline: concordancia entre biopsia contemporánea y biopsia definitiva / Borderline tumor of the ovary: accuracy of intraoperative frozen section and definitive biopsy

OBJETIVO: Los tumores de ovario borderline (BOT) son un grupo de lesiones neoplásicas de origen epitelial del ovario que presentan características de tumores malignos, pero sin invasión del estroma, y se caracterizan por tener un buen pronóstico. El objetivo del estudio es determinar la concordancia diagnóstica entre biopsia contemporánea y definitiva de los BOT en nuestro centro hospitalario. MÉTODO: Se realizó un estudio analítico retrospectivo de corte transversal de las biopsias contemporáneas y definitivas de BOT en la base de datos de anatomía patológica del Hospital Padre Hurtado, entre los años 2010 y 2019. El análisis estadístico de concordancia se realizó mediante test de kappa. RESULTADOS: Se revisaron 4546 informes de biopsias entre los años 2010 y 2019. Se pesquisaron 163 tumores malignos de ovario, de los cuales 69 (42,33%) correspondieron a BOT. De estos, 39 fueron serosos (56,2%), 28 mucinosos (40,57%) y 2 (2,8%) de tipo endometrioide. El resultado de concordancia diagnóstica de BOT seroso es moderada, del 75,71% con un índice de kappa de 0,5143 (p = 0,000), y el de BOT mucinoso es débil, del 65,71% con un índice de kappa de 0,2398 (p = 0,0222). CONCLUSIONES: Los BOT corresponden a un gran porcentaje dentro de los tumores malignos del ovario, siendo el subtipo seroso el más común. La concordancia entre biopsia contemporánea y definitiva es de débil a moderada.

OBJECTIVE: Borderline ovarian tumors (BOT) are a group of neoplastic lesions of epithelial origin of the ovary that present characteristics of malignant tumors but without stromal invasion and are characterized by having a good prognosis. The objective of the study is to determine the concordance between frozen section and definitive biopsy of BOT in our hospital center. METHOD: A retrospective, cross-sectional analytical study of the frozen section and definitive BOT biopsies was performed in the pathological anatomy database of the Padre Hurtado Hospital during the years 2010 and 2019. The statistical and concordance analysis was performed using kappa tests. RESULTS: 4546 biopsy reports were reviewed during 2010 and 2018. A total of 163 malignant ovarian tumors were investigated, of which 69 (42%) corresponded to BOT. Of these, the most common subtypes were 39 (56.2%) serous, 28 (40.57%) mucinous and 2 (2.8%) endometroid. The concordance results of serous BOT is moderate, 75.71% with a kappa index of 0.5143 (p = 0.000), and mucinous BOT have fair concordance, 65.71% with a kappa index of 0.2398 (p = 0.0222). CONCLUSIONS: BOT correspond to a large percentage of malignant tumors of the ovary, with the serous subtype being the most common. The concordance between contemporary and definitive biopsy is between fair and moderate.

Encefalitis por anticuerpos contra el receptor N-metil-D-aspartato secundaria a teratoma ovárico en una paciente pediátrica / Encephalitis due to antibodies against the N-methyl-D-aspartate receptor secondary to ovarian teratoma in a pediatric patient

La encefalitis por anticuerpos contra el receptor N-metilD-aspartato es un proceso inmunomediado en el que autoanticuerpos se dirigen contra la subunidad GluN1 del receptor de glutamato del sistema nervioso central. Se caracteriza por la aparición aguda o subaguda de síntomas psiquiátricos, como confusión, pérdida de la memoria a corto plazo, cambios de conducta, catatonía, seguidos por manifestaciones neurológicas, tales como convulsiones, alteraciones del movimiento, disfunciones autonómicas, coma y depresión respiratoria. Es grave y potencialmente mortal. Su asociación con teratoma de ovario como síndrome paraneoplásico fue descrita en mujeres jóvenes. En la población pediátrica, es mucho menos frecuente y se reporta en comunicaciones de 1 o 2 pacientes y en series de pocos casos. Se presenta una paciente de 13 años con encefalitis paraneoplásica por anticuerpos contra el receptor N-metil-Daspartato, secundaria a un teratoma ovárico maduro.

The encephalitis due to antibodies against the N-methylD-aspartate receptor is a process immune-mediated in which antibodies are directed against the GluN1 subunit of the glutamate receptor in the central nervous system. It is characterized by an acute or subacute onset of psychiatric symptoms such as confusion, short-term memory loss, behavioral changes, catatonia followed by neurological manifestations such as seizures, movement disturbances, autonomic dysfunctions, coma, and respiratory depression. It is serious and life threatening. Its association with ovarian teratoma as a paraneoplastic syndrome was described in youngwomen. In the pediatric population it is much less frequent and is reported in publications of one or two patients and in series of few cases. We present a 13-year-old patient with encephalitis paraneoplastic due to antibodies against the N-methyl-Daspartate receptor, secondary to a mature ovarian teratoma.

[Encephalitis due to antibodies against the N-methyl-D-aspartate receptor secondary to ovarian teratoma in a pediatric patient]. / Encefalitis por anticuerpos contra el receptor N-metil-D-aspartato secundaria a teratoma ovárico en una paciente pediátrica

The encephalitis due to antibodies against the N-methylD-aspartate receptor is a process immune-mediated in which antibodies are directed against the GluN1 subunit of the glutamate receptor in the central nervous system. It is characterized by an acute or subacute onset of psychiatric symptoms such as confusion, short-term memory loss, behavioral changes, catatonia followed by neurological manifestations such as seizures, movement disturbances, autonomic dysfunctions, coma, and respiratory depression. It is serious and life threatening. Its association with ovarian teratoma as a paraneoplastic syndrome was described in young women. In the pediatric population it is much less frequent and is reported in publications of one or two patients and in series of few cases. We present a 13-year-old patient with encephalitis paraneoplastic due to antibodies against the N-methyl-Daspartate receptor, secondary to a mature ovarian teratoma

La encefalitis por anticuerpos contra el receptor N-metil-D-aspartato es un proceso inmunomediado en el que autoanticuerpos se dirigen contra la subunidad GluN1 del receptor de glutamato del sistema nervioso central. Se caracteriza por la aparición aguda o subaguda de síntomas psiquiátricos, como confusión, pérdida de la memoria a corto plazo, cambios de conducta, catatonía, seguidos por manifestaciones neurológicas, tales como convulsiones, alteraciones del movimiento, disfunciones autonómicas, coma y depresión respiratoria. Es grave y potencialmente mortal. Su asociación con teratoma de ovario como síndrome paraneoplásico fue descrita en mujeres jóvenes. En la población pediátrica, es mucho menos frecuente y se reporta en comunicaciones de 1 o 2 pacientes y en series de pocos casos. Se presenta una paciente de 13 años con encefalitis paraneoplásica por anticuerpos contra el receptor N-metil-Daspartato, secundaria a un teratoma ovárico maduro.

Teratoma ovariano como causa de dor abdominal em criança na idade pré-escolar / Ovarian teratoma as a cause of abdominal pain in preschool children

Introdução: Os tumores ovarianos, raros na infância, apresentam maior incidência entre 8 e 9 anos, tendo pico aos 19 anos. A sintomatologia principal manifesta-se por dor abdominal, massa palpável, febre, constipação e mais raramente polaciúria e disúria. Os teratomas ovarianos maduros embora sejam neoplasias majoritariamente benignas, se malconduzidas, podem evoluir para emergências cirúrgicas. Objetivo: Analisar o teratoma ovariano maduro como possível etiologia de dor abdominal crônica em pacientes pediátricos. Relato de Caso: Criança, 2 anos e 9 meses, levada ao atendimento médico com relato de dor abdominal crônica, 3 meses de evolução, com períodos de agudização, de forte intensidade, associada à disúria e polaciúria conduzida como principal suspeita infecção do trato urinário (ITU). A partir da clínica compatível, associado a leucocitúrias, mesmo não havendo crescimento bacteriano nas culturas, devido à recorrência do quadro, seguiu-se a propedêutica adequada de investigação de ITU de repetição, através do rastreio de má formações das vias urinárias. O ultrassom abdominal total com dinâmica do trato urinário constatou presença de volumosa formação expansiva originada provavelmente em ovário esquerdo. Estendida a pesquisa diagnóstica foi realizada tomografia computadorizada do abdome e pelve com achados sugestivos de teratoma ovariano esquerdo, exercendo comportamento expansivo local com desvio e compressão de estruturas anatômicas circunvizinhas. Com a hipótese diagnóstica evidenciada foi encaminhada à cirurgia pediátrica e oncologia para tratamento adequado, com realização de ooforectomia e seguimento clínico. Conclusão: Importância da propedêutica adequada para casos de dor abdominal crônica em pacientes pediátricos devido à extensão de possíveis diagnósticos diferenciais.

Introduction: Ovarian tumors, rare in childhood, have a higher incidence between 8 and 9 years, peaking at 19 years. The main symptomatology is manifested by abdominal pain, palpable mass, fever, constipation, and more rarely polaciuria and dysuria. Mature ovarian teratomas although they are mostly benign neoplasms, if poorly conducted, they can progress to surgical emergencies. Objective: To analyze mature ovarian teratoma as a possible etiology of chronic abdominal pain in pediatric patients. Case Report: Child, 2 years and 9 months, taken to medical care with report of chronic abdominal pain, three months of evolution, with periods of sharpness, of strong intensity, associated with dysuria and polaciuria and primary suspected urinary tract infection (UTI). From the compatible clinic, associated with leukocytes, though there is no bacterial growth in the cultures, due to the recurrence of the condition, appropriate propaedeutic investigation of repeat UTI was followed by screening for urinary pathway malformations. Total abdominal ultrasound with urinary tract dynamics was requested, which that found the presence of a large expansive formation probably originating in the left ovary. Extended diagnostic research, the computed tomography of the abdomen and pelvis was performed with findings suggestive of left ovarian teratoma exerting local expansive behavior with deviation and compression of surrounding anatomical structures. With the diagnostic hypothesis evidenced, she was referred to pediatric surgery and oncology for appropriate treatment with oophorectomy and clinical follow-up. Conclusion: Importance of adequate propaedeutics for cases of chronic abdominal pain in pediatric patients due to the extent of possible differential diagnoses.

Cistoadenoma volumoso associado a teratoma ovariano maduro, com evolução para pseudomixoma peritoneal: relato de caso / Giant cystadenoma associated with mature ovarian teratoma with progression to pseudomyxoma peritonei: a case report

Massas anexiais representam grande desafio diagnóstico, devido a seu caráter geralmente assintomático¹. Traz-se caso peculiar de paciente que evoluiu com sintomas exuberantes de rápida evolução, situação característica de cistoadenomas, tumores benignos de origem epitelial²,³. Realizada laparotomia, com achados macroscópicos que revelaram conteúdo mucinoso e presença de pelos e neovascularização em seu interior. O anatomopatológico confirmou o diagnóstico de cistoadenoma associado a teratoma ovariano, sendo assim considerado um tumor de colisão ovariana.

Adnexal masses represent a major diagnostic challenge due to its usually asymptomatic characteritcs¹. We present a peculiar case of a patient who evolved with exuberant symptoms of rapid evolution, characteristic of cystadenomas, benign tumors of epithelial origin²,³. Laparotomy was performed, with macroscopic findings that revealed mucinous content and the presence of hairs and neovascularization inside. Pathology confirmed the diagnosis of cystadenoma associated with ovarian teratoma, thus being considered an ovarian collision tumor.