Padrão histopatológico e incidência de carcinomatose peritoneal ao diagnóstico em mulheres com câncer de ovário / Histopathological pattern and incidence of peritoneal carcinomatosis at diagnosis in women with ovarian cancer

Objetivo: Descrever o padrão histopatológico e identificar a incidência de carcinomatose peritoneal no momento do diagnóstico de mulheres diagnosticadas com neoplasia de ovário. Métodos: Trata-se de um estudo transversal e descritivo, baseado na análise secundária de dados correspondentes aos prontuários de mulheres adultas com diagnóstico de neoplasia de ovário de um serviço de referência em oncologia clínica. Foram analisados 40 prontuários entre janeiro de 2007 e janeiro de 2017. Resultados: Ao estadiamento segundo o sistema da International Federation of Gynecology and Obstetrics, três mulheres (7,5%) apresentavam estadiamento clínico (EC) II, três (77,5%) estágio ECIII com carcinomatose peritoneal/invasão da pelve e seis (15%) estágio ECIV com metástases à distância, especialmente para pulmão e fígado. Em relação ao padrão histopatológico, 20 mulheres apresentaram adenocarcinoma seroso papilífero de alto grau (50%), 4 (10%) adenocarcinoma seroso papilífero de baixo grau, 3 (7,5%) adenocarcinoma endometrioide, 3 (7,5%) tumor de teca/granulosa, 3 (7,5%) carcinoma de células claras, 3 (7,5%) tumores não classificados, 2 (5%) disgerminoma e 2 (5%) com cistoadenocarcinoma mucinosos. Conclusão: É nítida a necessidade de mais estudos envolvendo essa patologia, de modo a favorecer o diagnóstico e a intervenção em estágios mais precoces e reduzir desfechos desfavoráveis. (AU)

Objective: To describe the histopathological pattern, and to identify the incidence of peritoneal carcinomatosis at the time of the diagnosis of women diagnosed with ovarian neoplasm. Methods: This is a cross-sectional and descriptive study, based on the secondary analysis of data corresponding to the medical records of adult women diagnosed with ovarian neoplasm in a reference service of clinical oncology. A total of 40 medical records were analyzed between January 2007 and January 2017. Results: At the staging (FIGO system) of the International Federation of Gynecology and Obstetrics, three women (7.5%) had clinical staging (EC) II staging, 31 (77.5%) were in the ECIII stage, with peritoneal carcinomatosis/pelvic invasion, six (15%) were in the ECIV stage, with metastases at a distance, especially to lung and liver. Regarding the histopathological pattern, twenty women had high-grade papillary serous adenocarcinoma (50%), 4 (10%) with low-grade papillary serous adenocarcinoma, 3 (7.5%) with endometrioid adenocarcinoma, 3 (7.5%) with granulosa-theca tumor, 3 (7.5%) with clear cell carcinoma, 3 (7.5%) with unclassified tumors, 2 (5%) with dysgerminoma, two (5%) with mucinous cystadenocarcinoma. Conclusions: There is a clear need for further studies involving this pathology, in order to favor diagnosis and intervention at earlier stages and to reduce unfavorable outcomes. (AU)

Cistoadenocarcinoma mucinoso de apéndice cecal / Mucinous cystoadenocarcinoma of the appendix

Objetivo Exposición de 3 casos tratados quirúrgicamente en nuestra clínica. Casos clínicos Caso 1: paciente mujer de 78 años con diagnóstico de quiste complejo de ovario derecho, se realizó la cirugía y se evidencia tumoración de apéndice cecal. Caso 2: paciente varón de 38 años con dolor abdominal crónico. Presenta marcador tumoral antígeno carcinoembrionario elevado, laparoscopia exploratoria evidencia líquido mucinoso peritoneal y tumoración apendicular. Caso 3: paciente mujer de 42 años con dolor crónico en fosa iliaca derecha con marcadores tumorales normales. Laparoscopia exploratoria evidencia tumoración dependiente del apéndice cecal con localización retrocecal ascendente. Discusión Se debe considerar a los mucoceles apendiculares dentro del diagnóstico diferencial de dolor en cuadrante inferior derecho del abdomen. Es preferible realizar una hemicolectomia radical para lesiones grandes y/o perforadas por el riesgo de existir cistoadenocarcinoma. El abordaje laparoscópico es una buena alternativa de manejo.

Objective We present three surgically treated cases in our hospital Case Reports Case 1: A 78 years old woman with a diagnosis of complex ovarian cyst. During surgery, an appendicular tumor was found. Case 2: A 38 years old male with chronic abdominal pain with high levels of carcinoembryonic antigen. During exploratory laparoscopy an appendicular tumor and mucinous peritoneal fluid were found. Case 3: A 42 years old woman with chronic pain in the right lower abdomen, negative tumor markers. During exploratory laparoscopy a retrocecal appendicular tumor was found. Discussion Appendiceal mucoceles should be considered in the differential diagnosis of right lower abdominal pain. If the lesions are big or perforated, a radical hemicolectomy should be performed due to the risk of a cystadenocarcinoma. Laparoscopic approach is a good alternative for management.

Expression of c-myc and mutation of the KRAS gene in patients with ovarian mucinous tumors.

We examined the expression of c-myc and mutations in the KRAS gene in ovarian mucinous tumors to explore the pathogenesis of these tumors and the feasibility of targeted gene therapy. Expression of c-myc protein and mutations in the KRAS gene in 24 cases of ovarian mucinous cystadenoma, 46 cases of ovarian borderline mucinous cystadenoma, and 46 cases of ovarian mucinous cystadenocarcinoma were detected using the immunohistochemistry PV-9000 2-step method and polymerase chain reaction-restriction fragment length polymorphism. The positive expression rates of c-myc in ovarian mucinous cystadenoma, borderline mucinous cystadenoma, and cystadenocarcinoma were 0, 39.1, and 65.2%, respectively (P < 0.01), while the mutation rates in KRAS were 0, 39.1 and 13.0%, respectively. The mutation rate of the borderline group was significantly higher, while rates in the other 2 groups were similar (P > 0.05). c-myc was not correlated with clinical stage, pathological grade, or age of patients with ovarian mucinous cystadenocarcinoma or borderline mucinous cystadenoma (P > 0.05), but was correlated with tumor size (P < 0.05). Mutations in KRAS were not correlated with clinical stage or tumor size in patients with borderline mucinous cystadenoma (P > 0.05), whereas it was correlated with age (P < 0.05). In borderline mucinous cystadenoma, c-myc expression and KRAS mutations were not correlated (P > 0.05). c-myc is involved in the formation of ovarian borderline mucinous cystadenoma and mucinous cystadenocarcinoma, and the KRAS gene may contribute to the formation of borderline mucinous cystadenoma.

Adenocarcinoma mucinoso de tipo endocervical e intestinal a partir de un teratoma quístico maduro del ovario / Endocervical and intestinal-type mucinous adenocarcinoma from a Mature Cystic Teratoma

Los tumores derivados de células germinales representan el 20-25% de tumores ováricos, siendo sólo 3% malignos. La degeneración maligna se presenta únicamente en 1-2% de los casos. La edad de presentación promedio de la transformación maligna es a los 50 años, siendo los más frecuentes el carcinoma de células escamosas y el adenocarcinoma (1). Dado que la supervivencia a largo plazo se encuentra en relación con la detección temprana y la completa resección quirúrgica, se hace necesario determinar las características clínico-patológicas que sugieran una posible transformación maligna de estos tumores. A continuación, se presenta el caso de una paciente perimenopáusica, con hallazgos histopatológicos indicativos de transformación maligna de un teratoma quístico maduro a cistoadenocarcinoma mucinoso bien diferenciado de tipo endocervical e intestinal.

The germ cell-derived tumors account for 20-25% of ovarian tumors, with 3% being malignant. The degeneration occurs in 1-2% of cases. The age of presentation is menopause, around the age 50, and the most common malignant histological types in this context are squamous cell carcinoma and adenocarcinoma. It is important to determine the clinical-pathological characteristics of malignant transformation (although currently not well established), as because early detection and complete surgical resection are important for long-term survival. A case is presented on a peri-menopausal woman with histopathological findings of malignant transformation of a mature cystic teratoma to well-differentiated mucinous cystadenocarcinoma endocervical and intestinal type.

Analysis of microsatellite instability and loss of heterozygosity in ovarian cancer: a study in the population of Espírito Santo, Brazil.

Ovarian cancer is currently the most lethal gynecological malignancy in women. It is a heterogeneous and cytogenetically complex disease previously associated with genomic instability. Our purpose was to analyze microsatellite markers to determine patterns and levels of instability as well as possible correlations with histopathological parameters. Polymerase chain reaction was used to characterize microsatellite instability (MSI) and loss of heterozygosity (LOH) in 24 ovarian tumors at 12 microsatellite loci. A total of 11 samples displayed MSI or LOH. Only low-level MSI was found. Markers D5S346 and CYP11 showed the highest MSI and LOH frequencies. D17S250 LOH was significantly associated with tumor histological type (P = 0.0003), and estrogen receptor α was also associated with tumor histological type (P = 0.048) when a combined analysis of LOH and MSI was performed. Furthermore, LOH was observed in a greater number of markers compared with those displaying MSI. Thus, our results support that MSI is less common than LOH in ovarian cancers.

Cistadenoma mucinoso del apéndice cecal como causa de gangrena intestinal / Appendiceal mucocele causing large bowel gangrene

Introduction: Appendiceal mucocele are lesions located in the cecum, which are found in 0.2 to 0.3 percent of appendectomies and correspond to 8-10 percent of all appendiceal tumors. Clinical case: We report a 83 years old female consulting for abdominal pain lasting 24 hours. On physical examination, signs of peritoneal irritation were found. The patient was operated, finding an appendiceal tumor that was coiled up in the mesentery with large bowel gangrene. An intestinal resection, terminal ileostomy, appendectomy and surgical lavage were performed. The pathological study of the surgical piece reported a mucinous cystadenoma.

Introducción: Los tumores del Apéndice Cecal corresponden al 0,5 por ciento de las neoplasias gastrointestinales y se encuentran entre el 0,8 por ciento y el 1 por ciento de las apendicectomías. El término Mucocele Apendicular (AM), se refiere a un grupo de lesiones localizados a nivel del ciego cuyo rango de incidencia está entre el 0,2 por ciento y el 0,3 por ciento de todas las apendicectomías, y entre el 8-10 por ciento de todos los tumores apendiculares. Las presentaciones clínicas más frecuentes son: cuadro clínico de apendicitis aguda, como hallazgo incidental como en el caso de esta paciente, o como masa abdominal en fosa ilíaca derecha. Caso clínico: Se presenta un caso de Cistade-noma Mucinoso Apendicular (CAM) encontrado como hallazgo incidental en un abdomen agudo quirúrgico con la particularidad de presentarse con una necrosis intestinal.

Cistoadenocarcinoma primario: excepcional etiología de un tumor quístico retroperitoneal. Primera comunicación nacional / Primary cystoadenocarcinoma exceptional etiology of a cystic retroperitoneal tumor

El cistoadenocarcinoma primario retroperitoneal es una enfermedad excepcional, siendo este el primer caso publicado en la literatura nacional. Su diagnóstico, en la totalidad de los casoscomunicados, es anatomopatológico luego del estudio definitivo de la pieza. Es debatida la extensión de la resección quirúrgica en caso de no existir compromiso de estructuras vecinas. Eltratamiento adyuvante se justifica cuando se produce la rotura de la tumoración o existe compromiso locorregional. Se presenta el caso clínico de una paciente de 29 años que consultó por dolor en flanco derecho,en la cual se constató una tumoración a dicho nivel. Tomográficamente se confirmó la existencia de un proceso quístico, al cual se le realizó la excéresis quirúrgica completa junto a un sector depared abdominal. El informe de anatomía patológica confirmó un cistoadenocarcinoma primario retroperitoneal. No se realizó tratamiento adyuvante, presentando buena evolución a 11 meses de la cirugía, sin evidencias de enfermedad.

Primary retroperitoneal cystoadenocarcinoma is an exceptionaldisease, being this the first case Publisher in the national literature.Diagnosis, in all of the cases reported is anatomopathological, anatomopathological, subsequent to the final study of the sample. Surgicalresection extension is debated if there is no compromise of neighboring structures. Adjuvant treatment is justified when the tumor is broken or there is locoregional compromise.We present the clinical case of a 29 year old patient who consulted for right lank pain, where a tumor was identified.Ultrasound confirmed the existence of a cystic process, and complete surgical abscission/exeresis was performednext to an area in the in the abdominal wall. Anatomopathological report confirmed a primaruy retroperitonealcistoadenocarcinoma. No adjuvant treatment was applied, evolution was good 11 months after surgery, no evidence of the disease.

O cistoadenocarcinoma primário retroperitoneal é uma doença rara, sendo este o primeiro caso publicado na literatura nacional. Em todos os casos publicados seu diagnóstico foi realizado por anatomia patológica do examedefinitivo do material. A extensão da resecção cirúrgica quando não se observa compromisso das estruturas próximas é motivo de debate. O tratamento adjuvante é justificadoquando há ruptura do tumor ou se registra compromisso regional.Descreve-se o caso clínico de uma paciente de 29 anos que consultou por dor no flanco direito onde se constatoua presença de uma tumoração. Uma tomografia computada confirmou a existência de um processo cístico, que foi ressecado completamentejuntamente com um setor da parede abdominal. O exame de anatomia patológica confirmou um cistoadenocarcinoma primário retroperitoneal. A paciente não recebeu tratamento adjuvante apresentadoboa evolução 11 meses depois da cirurgia, sem evidências de patologia.

Cistoadenocarcinoma primario: excepcional etiología de un tumor quístico retroperitoneal. Primera comunicación nacional / Primary cystoadenocarcinoma exceptional etiology of a cystic retroperitoneal tumor

El cistoadenocarcinoma primario retroperitoneal es una enfermedad excepcional, siendo este el primer caso publicado en la literatura nacional. Su diagnóstico, en la totalidad de los casoscomunicados, es anatomopatológico luego del estudio definitivo de la pieza. Es debatida la extensión de la resección quirúrgica en caso de no existir compromiso de estructuras vecinas. Eltratamiento adyuvante se justifica cuando se produce la rotura de la tumoración o existe compromiso locorregional. Se presenta el caso clínico de una paciente de 29 años que consultó por dolor en flanco derecho,en la cual se constató una tumoración a dicho nivel. Tomográficamente se confirmó la existencia de un proceso quístico, al cual se le realizó la excéresis quirúrgica completa junto a un sector depared abdominal. El informe de anatomía patológica confirmó un cistoadenocarcinoma primario retroperitoneal. No se realizó tratamiento adyuvante, presentando buena evolución a 11 meses de la cirugía, sin evidencias de enfermedad. (AU)

Primary retroperitoneal cystoadenocarcinoma is an exceptionaldisease, being this the first case Publisher in the national literature.Diagnosis, in all of the cases reported is anatomopathological, anatomopathological, subsequent to the final study of the sample. Surgicalresection extension is debated if there is no compromise of neighboring structures. Adjuvant treatment is justified when the tumor is broken or there is locoregional compromise.We present the clinical case of a 29 year old patient who consulted for right lank pain, where a tumor was identified.Ultrasound confirmed the existence of a cystic process, and complete surgical abscission/exeresis was performednext to an area in the in the abdominal wall. Anatomopathological report confirmed a primaruy retroperitonealcistoadenocarcinoma. No adjuvant treatment was applied, evolution was good 11 months after surgery, no evidence of the disease. (AU)

O cistoadenocarcinoma primário retroperitoneal é uma doença rara, sendo este o primeiro caso publicado na literatura nacional. Em todos os casos publicados seu diagnóstico foi realizado por anatomia patológica do examedefinitivo do material. A extensão da resecção cirúrgica quando não se observa compromisso das estruturas próximas é motivo de debate. O tratamento adjuvante é justificadoquando há ruptura do tumor ou se registra compromisso regional.Descreve-se o caso clínico de uma paciente de 29 anos que consultou por dor no flanco direito onde se constatoua presença de uma tumoração. Uma tomografia computada confirmou a existência de um processo cístico, que foi ressecado completamentejuntamente com um setor da parede abdominal. O exame de anatomia patológica confirmou um cistoadenocarcinoma primário retroperitoneal. A paciente não recebeu tratamento adjuvante apresentadoboa evolução 11 meses depois da cirurgia, sem evidências de patologia. (AU)

[Mucinous cystadenocarcinoma of pancreas]. / Cistoadenocarcinoma mucinoso de páncreas.

Mucinous cystadenocarcinoma of the pancreas represents around 6-36% of mucinous cystic neoplasm. The lesions are usually found in the body and tail of the pancreas and are generally solitary with a size range of 6-36 cm. We present a clinical case of a 63 years old patient with abdominal pain and weight loss. We used radiographic imaging studies. It was treated with surgery by distal pancreatectomy with splenectomy and transverse colectomy. Patient was not post operative complications.

Pseudomyxoma peritonei secondary to epithelial appendicular neoplasms. Experience in a non-specialised centre.

INTRODUCTION: Pseudomyxoma peritonei is an infrequent entity, defined by collections of gelatinous material in the abdomen and pelvis and mucinous implants on peritoneum, secondary to the rupture of a mucinous lesion, usually of ovarian or appendiceal origin. MATERIALS AND METHODS: We present our experience of 11 cases (6 males and 5 females) diagnosed with pseudomyxoma peritonei secondary to epithelial appendicular neoplasms over 27 years. The mean age of the patients was 68 years. Clinical manifestations were abdominal distension (55%), right lower quadrant pain (45%) suggesting acute appendicitis and constitutional syndrome (36%). An abdominal mass was detected at physical examination in 4 patients. CT scan revealed a tumour in right iliac fossa in 4 patients, peritoneal enlargement in 1 and a liquid collection in 1. Preoperative diagnosis was acute abdomen in 5 patients, peritoneal carcinomatosis in 3 and undetermined abdominal mass in 3. RESULTS: Surgical findings suggested pseudomyxoma peritonei in 8 patients and peritoneal carcinomatosis in 3. Appendicectomy was performed in 9 patients, and in 3 of them bilateral anexectomy was also performed. One patient underwent ileocaecal resection and another a right hemicolectomy. In all the cases, mucinous material was eliminated as much as possible. Pathology revealed mucinous cystoadenoma in 6 cases, mucinous cystoadenocarcinoma in 3 and epithelial hyperplasia in 2 patients. Median survival was 54 months, with a 5- year survival rate of 40%. The last case we treated was sent to a reference centre for the treatment of pseudomyxoma peritonei. CONCLUSIONS: There is no consensus on the best treatment for pseudomyxoma peritonei. We recommend avoiding incomplete surgical resections in non-reference centres and submitting patients to a reference centre to undergo adequate treatment.

[Elevated Ca 19.9 tumor marker without evidence of malignancy]. / Marcador tumoral Ca 19.9 aumentado sin evidencia de malignidad.

Tumor markers are a useful tool for surveillance of oncologic patients, whereas their role in the diagnosis of a malignancy is controversial. We present the case of a woman with a benign ovarian cyst with an unexpected elevation of Ca 19.9 after laparoscopic bilateral anexectomy.

Primary retroperitoneal mucinous cystadenocarcinoma: report of two cases.

BACKGROUND: Retroperitoneal cystadenocarcinomas are rare lesions, the majority of cases presented as one-patient reports. METHODS: We present two cases of retroperitoneal cystadenocarcinoma, both in women of reproductive age: one with aggressive behavior, and the remaining case, with a more indolent clinical evolution. RESULTS: One case presented as pelvic tumor, was treated with surgical resection of the disease, but manifested with recurrent disease a few months later despite use of chemotherapy. The second case involved a patient with diagnosis of abdominal tumor; during laparotomy, a retroperitoneal tumor was found and was totally removed. At follow-up, the patient is disease-free with no other treatment. CONCLUSION: The behavior and treatment of retroperitoneal cystadenocarcinoma are controversial. We suggest aggressive surgery including radical hysterectomy and bilateral salpingoopherectomy with adjuvant chemotherapy in these cases.

Marcador tumoral ca 19.9 aumentado sin evidencia de malignidad / Elevated Ca 19.9 tumor marker without evidence of malignancy

Los marcadores tumorales son de gran utilidad clínica en el seguimiento de los pacientes oncológicos. Su papel en el diagnóstico de tumoraciones malignas es controvertido. Presentamos un caso de una mujer con un quiste ovárico benigno en la cual los marcadores aumentan de forma inexplicable después de la extirpación del mismo.(AU)

Tumor markers are a useful tool for surveillance of oncologic patients, whereas their role in the diagnosis of a malignancy is controversial. We present the case of a woman with a benign ovarian cyst with an unexpected elevation of Ca 19.9 after laparoscopic bilateral anexectomy.(AU)

Marcador tumoral ca 19.9 aumentado sin evidencia de malignidad / Elevated Ca 19.9 tumor marker without evidence of malignancy

Los marcadores tumorales son de gran utilidad clínica en el seguimiento de los pacientes oncológicos. Su papel en el diagnóstico de tumoraciones malignas es controvertido. Presentamos un caso de una mujer con un quiste ovárico benigno en la cual los marcadores aumentan de forma inexplicable después de la extirpación del mismo.

Tumor markers are a useful tool for surveillance of oncologic patients, whereas their role in the diagnosis of a malignancy is controversial. We present the case of a woman with a benign ovarian cyst with an unexpected elevation of Ca 19.9 after laparoscopic bilateral anexectomy.

Cararcterização fenotípica dos tumores mucinosos do ovário / Phenotype characterization of the mucinous ovarian tumors

Os tumores mucinosos primários de ovário são classificados como benignos, borderline e malignos, com diferentes fenótipos, recentemente designados como gastrointestinal e seromucinoso. O propósito desse estudo retrospectivo foi analisar os diferentes padrões morfológicos de apresentação dos tumores mucinosos ovarianos e sua associação com o potencial de malignidade e perfil imunoistoquímico. Marcação imunoistoquímica para produtos de gene de mucina (MUC1, MUC2, MUC5AC e MUC6), RE, RP, CK7, CK20, CA19.9 e CA125 foram realizados em tissue microarrays. Os resultados sugerem que os fenótipos pilórico e intestinal puros são diferentes em relação ao potencial de malignidade e perfil imunoistoquímico. Provavelmente os tumores borderline de padrão intestinal, mesmo aqueles sem pseudomixoma peritoneal associado, devem ser considerados como tumores secundários, muitos destes com origem em apêndice cecal...

Primary mucinous ovarian tumors are classified as benign, borderline and malignant, with different phenotypes, recently called as gastrointestinal and seromucinous. The purpose of this retrospective study was to analyze the different morphological patterns of presentation of mucinous ovarian tumors and their association with malignant potential and immunohistochemical profile. Immunohistochemical staining for mucin gene product (MUC1, MUC2, MUC5AC and MUC6), ER, PR, CK7, CK20, CA19.9 and CA125 were performed in tissue microarrays. The results suggest that the pure pyloric and intestinal phenotypes differ regarding malignant potential and immunoprofile. Probably most intestinal borderline tumors, even those without pseudomixoma peritonei, should be considered as secondary tumor, most of them with appendiceal oring...