Proximal epithelioid sarcoma of the vulva. A rare neoplasm. A clinicopathological case / Sarcoma epitelioide proximal de la vulva. Una neoplasia rara. Un caso clínico-patológico

Epithelioid sarcoma is a rare aggressive soft tissue sarcoma, which can be distal or proximal types. The classic form (distal-type) of epithelioid sarcoma mainly occurs in teenagers and young adults. A rarer form, called large-cell (proximal-type) epithelioid sarcoma, tends to be more aggressive and mainly affects adults. The proximal subtype mostly arises from the proximal pelvis, limbs, and genital tract. We report a case of a 59 -year-old female, presented with a progressively growing mass in the left labia majora. Gynecologic examination revealed a 2 cm mobile and painless mass that was not attached to deep planes. The histological study showed a multinodular tumor was seen comprising sheets of oval to polygonal cells with moderate amount of cytoplasm. Interspersed were larger, rhabdoid cells with abundant eosinophilic cytoplasm and prominent nucleoli. On IHC, the tumor cells showed positivity for EMA and CKAE1/AE3 and do not expressed INI-1 in the nucleus. All tumor cells were negative for S-100 protein and CD34. The histopathological diagnosis was soft tissue of the vulvar region with proximal epithelioid sarcoma. The patient received adjuvant external pelvic radiotherapy and brachytherapy in the vulvar bed. Currently, 3 years after diagnosis, the patient does not present signs of tumor recurrence in her controls. Due to its low incidence, there are no evidence-based diagnostic algorithms or published recommendations for treatment. The prognosis is generally poor. A wide excision with clear margins is imperative with options of post-operative CT/RT in individual cases during a close follow-upbehavior, as seen in our case. (AU)

El sarcoma epitelioide es un sarcoma de tejido blando agresivo poco frecuente, que puede ser de tipo distal o proximal. La forma clásica (tipo distal) de sarcoma epitelioide se presenta principalmente en adolescentes y adultos jóvenes. Una forma más rara, llamada sarcoma epitelioide de células grandes (tipo proximal), tiende a ser más agresiva y afecta principalmente a adultos. El subtipo proximal surge principalmente de la pelvis proximal, las extremidades y el tracto genital. Presentamos el caso de una mujer de 59 años, que presentó una masa de crecimiento progresivo en labios mayores izquierdos. El examen ginecológico reveló una masa móvil e indolora de 2 cm que no estaba adherida a planos profundos. El estudio histológico mostró un tumor multinodular compuesto por láminas de células de forma ovalada a poligonal con moderada cantidad de citoplasma. Intercaladas había células rabdoides más grandes con abundante citoplasma eosinófilo y nucléolos prominentes. En IHC, las células tumorales mostraron positividad para EMA y CKAE1/AE3 y no expresaron INI-1 en el núcleo. Todas las células tumorales fueron negativas para la proteína S-100 y CD34. El diagnóstico histopatológico fue tejido blando de la región vulvar con sarcoma epitelioide proximal. La paciente recibió radioterapia pélvica externa adyuvante y braquiterapia en el lecho vulvar. Actualmente, a 3 años del diagnóstico, la paciente no presenta signos de recurrencia tumoral en sus controles. Debido a su baja incidencia, no existen algoritmos de diagnóstico basados en evidencia ni recomendaciones de tratamiento publicadas. El pronóstico es generalmente malo. Es imperativa una escisión amplia con márgenes claros con opciones de CT/RT postoperatoria en casos individuales durante un seguimiento cercano, como se observa en nuestro caso. (AU)

Cistoadenofibroma seroso de ovario: experiencia de 12 años en el Hospital General Universitario de Albacete / Ovarian serous cystadenofibroma: 12-year experience at the Hospital General Universitario de Albacete

ANTECEDENTES: El cistoadenofibroma seroso de ovario es una neoplasia epitelial benigna, relativamente infrecuente, de crecimiento lento y de causa desconocida. El diagnóstico preoperatorio es complejo debido a la ausencia de síntomas y signos específicos que permitan descartar la malignidad. OBJETIVO: Analizar retrospectivamente las características clínicas, radiológicas, histopatológicas, y la atención médica de las pacientes. MATERIALES Y MÉTODOS: Análisis retrospectivo y descriptivo de pacientes con diagnóstico histopatológico de cistoadenofibroma seroso de ovario atendidas en el Hospital General de Albacete entre los años 2010 a 2022. RESULTADOS: Se analizaron 635 piezas quirúrgicas, de las que el 57.74% correspondieron a neoplasias serosas benignas, el 17.41% a neoplasias serosas fronterizas y un 24.85% a neoplasias serosas malignas. Se identificaron 20 casos de pacientes con diagnóstico de cistoadenofibroma seroso de ovario. La edad media de esas pacientes fue de 47 años, con límites de 9 y 74 años. Un caso se asoció con un tumor proliferativo seroso atípico contralateral. El tratamiento fue quirúrgico y no se identificaron recurrencias en ninguno de los casos. CONCLUSIONES: El cistoadenofibroma seroso suele manifestarse como un quiste ovárico complejo, con componentes sólidos-quísticos y tabiques irregulares; por esto a menudo se diagnostica erróneamente como tumor maligno antes de la intervención. La biopsia por congelación ayuda a confirmar su naturaleza benigna y evita una cirugía extensa innecesaria. El tratamiento consiste, principalmente, en la extirpación quirúrgica del quiste con o sin ooforectomía. El pronóstico suele ser excelente.

Abstract BACKGROUND: Serous ovarian cystoadenofibroma is a relatively rare, slow-growing, benign epithelial neoplasm of unknown cause. Preoperative diagnosis is complex due to the absence of specific symptoms and signs to rule out malignancy. OBJECTIVE: To retrospectively analyze the clinical, radiologic, histopathologic features, and medical care of the patients. MATERIALS AND METHODS: Retrospective and descriptive analysis of patients with histopathological diagnosis of ovarian serous cystoadenofibroma attended at the General Hospital of Albacete between the years 2010 to 2022. RESULTS: 635 surgical specimens were analyzed, of which 57.74% corresponded to benign serous neoplasms, 17.41% to borderline serous neoplasms and 24.85% to malignant serous neoplasms. Twenty cases of patients with a diagnosis of ovarian serous cystoadenofibroma were identified. The mean age of these patients was 47 years, with limits of 9 and 74 years. One case was associated with a contralateral atypical serous proliferative atypical tumor. Treatment was surgical and no recurrences were identified in any of the cases. CONCLUSIONS: Serous cystoadenofibroma usually manifests as a complex ovarian cyst, with solid-cystic components and irregular septa; this is why it is often misdiagnosed as a malignant tumor before surgery. Freeze biopsy helps to confirm its benign nature and avoids unnecessary extensive surgery. Treatment consists mainly of surgical removal of the cyst with or without oophorectomy. The prognosis is usually excellent.

Progesterone elevation on the day of oocyte retrieval and live birth rate after in vitro fertilisation treatment.

The objective of this study was to evaluate if progesterone elevation (PE) on the day of oocyte retrieval is associated with IVF outcome. A prospective cohort study of 400 IVF-ICSI cycles, with fresh embryo transfer on day 2-3 was performed. We proposed a serum progesterone (P) level on percentile (p) 90 as a threshold.Pregnancy rates were not affected, however there were more miscarriages (25.7% vs 43.8%) and lower live birth rate (LBR) (28% vs 23.1%) in the PE group (not statistically significant). We also found a positive correlation between P levels and retrieved and mature oocytes, total embryos, and good quality embryos. This is the first study to analyse LBR based on P levels on the day of oocyte retrieval. PE is not associated with the IVF outcome, but there is a trend to lower ongoing pregnancy rate and LBR and more miscarriages. Our results also show that P levels have no negative effects on oocyte and embryo quality.Impact statementWhat is already known on this subject? The influence of PE during IVF cycle on pregnancy rates remains controversial.What do the results of this study add? This is the first study to analyse LBR based on P levels on the day of oocyte retrieval.What are the implications of these findings for clinical practice and/or further research? We demonstrated that pregnancy rates were not affected by PE at oocyte retrieval, but there is a trend to lower ongoing pregnancy rate and LBR and more miscarriages. Randomised controlled trials are needed to offer more evidence of these relationships.

Uterine necrosis. A rare complication of embolisation due to post-partum haemorrhage.

Post-partum haemorrhage (PPH) is a major cause of maternal mortality, according to the WHO. Early PPH occurs in 5% to 15% of births. In 80% of cases, uterine atonia is the cause. We describe a case of uterine necrosis following uterine artery embolization (UAE). due to PPH. The patient was a 37-year-old woman who had a normal delivery after her second pregnancy, but experienced haemorrhage due to uterine atonia that failed to respond to drugs. She underwent UAE with Spongostan® with no complications. The peri-partum period was normal. At 16 days, she consulted due to recurrence of fever. Magnetic resonance imaging confirm the diagnosis of uterine necrosis. Abdominal hysterectomy was performed.

Parto a través de rotura uterina posterior en una paciente con pesario de Arabin / Vaginal birth through posterior uterine breaking in Arabin pessary carrier patient

Resumen ANTECEDENTES: El parto pretérmino es una causa importante de morbilidad y mortalidad materno-fetal. El pesario cervical es un método eficaz para prevenir el parto pretérmino en pacientes con cuello uterino corto. CASO CLÍNICO: Paciente de 38 años, de 34.3 semanas de embarazo, que acudió a consulta por hidrorrea y dolor abdominal. Entre sus antecedentes ginecoobstétricos refirió la colocación de un pesario de Arabin a partir de la semana 20 del embarazo, por diagnóstico de cuello uterino corto (14 mm). La exploración médica reveló: cuello uterino cerrado y formado, con desgarro de 2 cm en la cara posterior uterina, indicándose cesárea de urgencia. Cinco minutos después tuvo aumento importante de dolor abdominal, objetivándose la cabeza fetal en IV plano de Hodge, por lo que se decidió la asistencia mediante parto en el área quirúrgica. Nació un varón de 2045 g, con Apgar 9/10, que ingresó al área de neonatología. Posteriormente se comprobó el desgarro ístmico-cervical, de aproximadamente 7 cm, con prolongación ascendente medial de 4 cm, que se suturó sin contratiempos. El puerperio inmediato y tardío transcurrieron con normalidad. Un año después del parto la paciente se encuentra en excelente estado de salud. CONCLUSIÓN: La rotura uterina en pacientes con pesario es una complicación extremadamente rara. Hasta la fecha no existe un esquema de tratamiento óptimo. Se prefiere una conducta conservadora, sobre todo si existe deseo reproductivo, e individualizar cada caso.

Abstract BACKGROUND: Preterm delivery is an important cause of maternal-fetal morbimortality. The cervical pessary is an effective method to prevent preterm birth in patients with short cervix. CLINICAL CASE: A 38-year-old patient, 34.3 weeks pregnant, attended the clinic due to hidrhorea and abdominal pain. Among his gynecological and obstetric history he referred to the placement of Arabin pessary from week 20 of pregnancy, by diagnosis of short cervix (14 mm). The medical examination revealed: Cervix closed and formed, with a 2 cm tear in the posterior uterine side, indicating an emergency caesarean section. Five minutes later, there was a significant increase in abdominal pain, with the fetal head being seen in the IV plane of Hodge. Therefore, it was decided to assist with delivery in the surgical area. A newborn male, 2045 g, was obtained with Apgar 9 / 10, who entered the neonatology area. Subsequently, the isthmic-cervical tear, of approximately 7 cm, with a medial ascending extension of 4 cm, which was sutured without incident, was confirmed. The immediate and delayed puerperium proceeded normally. One year after the obstetric event, the patient is in excellent health. CONCLUSIONS: Uterine rupture associated to pessary is an extremely rare complication. To date there is no optimal treatment scheme. A conservative behavior is preferred, especially if there is a reproductive desire, and each case is individualized.

Tumor neuroectodérmico recidivante. A propósito de un caso y revisión bibliográfica / Recurrent neuroectodermal tumor. A case report and literatura review

Resumen ANTECEDENTES: La relación entre cáncer y embarazo supone 0.07% de las complicaciones gestacionales. Cuando estas situaciones coinciden el tratamiento del tumor se dificulta. El tumor neuroectodérmico primitivo es una neoplasia relacionada con el sarcoma de Ewing y su incidencia es excepcional durante el embarazo. CASO CLÍNICO: Paciente de 34 años, con 36.3 semanas de embarazo, que ingresó a la unidad hospitalaria por dolor abdominal irradiado al miembro inferior derecho. A la exploración física se palpó una tumoración de gran dimensión en la fosa iliaca derecha. La ecografía abdominal objetivó una imagen compatible con un mioma. La resonancia magnética reportó una masa de 16 x 13 x 17 cm, retroperitoneal, paravertebral, coincidente con tumor neuroectodérmico, sarcoma y tumor neurogénico. La paciente tuvo parto eutócico, sin administración de analgesia epidural, del que nació una niña de 2950 g, con Apgar 8-9. Se efectuó una biopsia por aspiración con aguja gruesa, que reportó un tumor neuroectodérmico primitivo. El tratamiento consistió en quimioterapia con protocolo VAC (vincristina, dactinomicina y ciclofosfamida [14 ciclos]) y adriamicina (6 a 8 ciclos de inducción). Actualmente padece dolor neuropático en la pierna derecha y permanece en rehabilitación, con tratamiento médico. CONCLUSIONES: Los tumores neuroectodérmicos primitivos son neoplasias excepcionales durante el embarazo. Se requieren estudios complementarios para conocer la relación exacta entre este tipo de tumores y el embarazo, y de esta forma establecer el protocolo de tratamiento adecuado.

Abstract BACKGROUND: The relationship between cancer and pregnancy accounts for 0.07% of gestational complications. This aspect makes treatment difficult and has a negative impact on pregnant patients. The primitive neuroectodermal tumor is a neoplasm related to Ewing's sarcoma and its incidence is exceptional during pregnancy. CLINICAL CASE: A 34-year-old patient, 36.3 weeks pregnant, who was admitted to the hospital unit due to abdominal pain radiating to the right lower limb. Physical examination revealed a large tumor in the right iliac fossa. The abdominal ultrasound showed an image compatible with a myoma. Magnetic resonance imaging revealed a mass of 16 x 13 x 17 cm, retroperitoneal, paravertebral, coinciding with neuroectodermal tumor, sarcoma and neurogenic tumor. The patient had eutocic delivery, without administration of epidural analgesia, from which a girl of 2950 g was born, and Apgar 8/9. An aspiration biopsy was performed with a thick needle, which reported a primitive neuroectodermal tumor. The treatment consisted of chemotherapy with VAC protocol (vincristine, dactinomycin and cyclophosphamide [14 cycles]) and adriamycin (6 to 8 induction cycles). He currently suffers from neuropathic pain in the right leg and remains in rehabilitation, with medical treatment. CONCLUSIONS: Primitive neuroectodermal tumors are exceptional neoplasms during pregnancy. Complementary studies are required to know the exact relationship between this type of tumors and pregnancy, and in this way establish the appropriate treatment strategy.

Muerte fetal anteparto. Duelo perinatal / Antepartum stillbirth. Perinatal bereavement

Introducción. La muerte fetal intrauterina es una de las situaciones más difíciles de enfrentar en la práctica obstétrica diaria. Esta condición es bien caracterizada como un duelo. Especial énfasis debe ser otorgado al apoyo psicológico brindado especialmente a la madre. Sujetos y métodos. Se presenta el caso clínico de una primigesta de 40 semanas diagnosticada de muerte fetal anteparto. Ilustramos las etapas del duelo y su manejo clínico. Conclusiones. Consideramos interesante establecer protocolos y directrices bien planificadas sobre el cuidado de la madre y de la pareja durante la pérdida gestacional (AU)

Introduction. Intrauterine fetal death is one of the most difficult situations to face in daily obstetric practice. This condition is well characterized as a bereavement. Special emphasis should be given to the provision of psychological support, especially to the mother. Subjects and methods. We report the case of a primigravida at 40 weeks of pregnancy who was diagnosed with an antepartum stillbirth. We illustrate the stages of grief and their clinical management. Conclusions. Protocols and guidelines should be designed for the care of the mother and her partner during pregnancy loss (AU)

Pronóstico y resultados en pacientes diagnosticadas de endometriosis a las que se les ha realizado por primera vez una cirugía conservadora por dolor o esterilidad / Outcomes in patients with endometriosis undergoing a first conservative surgical intervention for pain or infertility

Objetivo: Analizar resultados clínicos en pacientes con sospecha de endometriosis sometidas a cirugía conservadora. Se estudia la disminución del dolor y el logro de gestación, así como las reintervenciones. Material y métodos: Se incluyó a las pacientes con sospecha de endometriosis con clínica de dolor o esterilidad en las que se llevó a cabo una cirugía conservadora por primera vez (n = 128). Se excluyeron las historias incompletas y las intervenciones cuya indicación no fue dolor o esterilidad. Resultados: El 70,3% se interviene por dolor y 29,7%, por esterilidad. En la primera visita posquirúrgica presenta dolor el 32,5%; a los 6 meses, el 42,5%, y al año, el 58,3%. Se reinterviene al 11,29%. Entre las reintervenidas por dolor hay un mayor porcentaje de clínica intestinal previa a la cirugía (p = 0,07), así como de episodios de dolor abdominal, que precisaron atención hospitalaria (p = 0,08) y presentaron dolor en la primera visita (p = 0,05) y al año (p = 0,03) en un porcentaje mayor que las no reintervenidas; recibieron tratamiento médico posquirúrgico en un porcentaje menor (p = 0,11). El 65,8% de las pacientes intervenidas por esterilidad consiguieron gestación. Conclusiones: En la primera visita, se observó un menor porcentaje de pacientes con dolor que en las siguientes. Entre las pacientes reintervenidas por dolor existe un porcentaje mayor de clínica intestinal y de episodios de dolor abdominal previos a la intervención que requirieron atención hospitalaria. El hallazgo de dolor en la primera visita y al año son factores de mal pronóstico para ser reintervenidas. Las pacientes reintervenidas por dolor presentan un menor porcentaje de tratamiento médico posquirúrgico. Más de la mitad de las pacientes con esterilidad y endometriosis han quedado gestantes espontáneamente tras la cirugía (AU)

Objective: To analyze clinical outcomes in patients with suspected endometriosis undergoing conservative surgery. Pain reduction, pregnancy rates, and reinterventions were analyzed. Material and methods: Patients with suspected endometriosis and symptoms of pain or infertility undergoing conservative surgery for the first time were included (n = 128). Exclusion criteria consisted of incomplete medical histories and interventions indicated for reasons other than pain or infertility. Results: Surgery was indicated for pain in 70.3% and for infertility in 29.7%. A total of 32.5% of the patients had pain at the first postsurgical visit, 42.5% at 6 months and 58.3% at 1 year. Reintervention was performed in 11.29%. Among reinterventions for pain, there was a higher percentage of intestinal symptoms before surgery (P=.07), as well as episodes of abdominal pain requiring hospital care (P=.08); a higher proportion of these patients had pain in the first visit (P=.05) and at 1 year (P=.03) than patients not undergoing reintervention. Postsurgical medical treatment was less frequent in patients undergoing reintervention (P=.11). Among patients undergoing surgery for infertility, pregnancy was achieved in 65.8%. Conclusions: Pain was less frequent in the first postsurgical visit than in subsequent visits. Among patients undergoing reintervention for pain, there was a higher percentage of intestinal symptoms and episodes of abdominal pain requiring hospital care prior to the intervention. Pain at the first visit and at 1 year are factors of poor prognosis for reintervention. Patients undergoing reintervention for pain less frequently required postsurgical medical treatment. More than half of patients with interfertility and endometriosis achieved spontaneous pregnancy after surgery (AU)

Recidiva de melanoma en gestación / Melanoma recurrence and pregnancy

La incidencia de melanoma durante el embarazo es del 0,1 al 2,8/1.000 partos. En mujeres con antecedente de melanoma, la gestación no parece aumentar el riesgo de recurrencia ni tener efectos adversos sobre la supervivencia; sin embargo, las pacientes con enfermedad recurrente o que precisan tratamiento durante la gestación tienen una supervivencia acortada de aproximadamente 6 meses. El melanoma es el tumor maligno que más frecuentemente metastatiza en la placenta y el feto. Presentamos el caso clínico de una gestante que, con antecedente de melanoma, presentó una recurrencia en el cerebro durante la gestación y falleció a los 21 días del parto sin presentar afectación placentaria ni fetal

The incidence of malignant melanoma during pregnancy has been estimated to be 0.1 to 2.8 per 1,000 pregnancies. In women with a history of melanoma, pregnancy does not seem to increase the risk of recurrence or to negatively influence survival. However, survival is approximately 6 months shorter in women with recurrent disease and those requiring treatment during pregnancy. Malignant melanoma is the most common type of cancer to metastasize to the placenta and fetus. We report the case of a pregnant woman with a history of melanoma who showed cerebral recurrence and died 21 days post-partum, without placental or fetal metastases (AU)

Obstrucción intestinal durante la gestación / Intestinal obstruction during pregnancy

Este manuscrito expone, por orden cronológico,3 casos de obstrucción intestinal tratados en nuestroservicio. Todos tuvieron lugar en el último año.A pesar de que las manifestaciones clínicas fueroncomparables en los 3 casos, su evolución fue muydiferente. El interés de publicar estos casos surgede la escasa incidencia y la gran morbimortalidadde esta afección; es fundamental el diagnósticotemprano de éste para mejorar la supervivencia

The present study describes three cases ofintestinal obstruction in chronological order treatedin our service in the last year. Although thereported symptoms were highly similar in all threepatients, outcomes were very different. The interestof these cases lies in the low incidence of thiscomplication during pregnancy and its highmorbidity and mortality. Early diagnosis is essential to improve survivalm (AU)