Primary retroperitoneal extraovarian granulosa cell tumor

Extraovarian granulosa cell tumors (GCTs) develop from ectopic gonadal tissue situated along the embryonal route of the genital ridge. Primary retroperitoneal tumors are extremely rare, with an incidence of 02% -06% and 80-85% probability of malignancy. Only eight such case reports have been published previously. We herein, report a rare case of extraovarian retroperitoneal GCT in a 55-year-old woman who presented with intermittent left lumbar region pain of one-year duration. She had a history of hysterectomy and bilateral salpingo-oophorectomy 8 years ago for uterine leiomyoma. Laparotomy revealed a retroperitoneal mass measuring 8cm x 10cm x 20cm in size, solid cystic with areas of necrosis and hemorrhage. The gross features, classical histopathology, and positive immunostaining of the retroperitoneal mass with inhibin, calretinin, PR, WT1 and immunonegativity for EMA were characteristic of adult-type GCT. Excluding any previous history of primary ovarian GCT in this patient, a de-novo retroperitoneal diagnosis was established.

Tumor de células granulares de la vulva: caso clínico / Granular cell tumor of the vulva: a clinical case

El tumor de células granulares es una neoplasia de la piel y los tejidos blandos muy poco frecuente, benigna y de crecimiento lento, pero con altas tasa de recurrencia. La localización más frecuente en el aparato genital es en la vulva. Se debe realizar diagnóstico diferencial con otras lesiones vulvares, debido al manejo distinto de esta tumoración. El diagnóstico generalmente es histológico, ya que clínicamente es muy difícil diferenciarlo de otras tumoraciones a nivel vulvar. El tratamiento recomendado es la exéresis quirúrgica, con márgenes de seguridad. En caso de bordes afectos, se recomienda realizar una reescisión, por la frecuencia de recurrencia local y porque ésta puede ser el primer indicador de una conducta agresiva. Presentamos un caso clínico a nivel vulvar, con el objetivo de destacar la importancia de realizar un diagnóstico correcto, para un buen manejo clínico y seguimiento. Las formas malignas suponen menos del 3% de estos tumores y son muy agresivas

The granular cell tumor is a neoplasm of the skin and soft tissue very rare, benign and slow growing, but with high recurrence rate. The most frequent location in the genital tract is in the vulva. Differential diagnosis should be made with other vulvar lesions, due to differences in the management of this tumor. The diagnosis is usually histological, since it is clinically difficult to differentiate it from other tumors at the vulvar level. The recommended treatment is surgical excision, with safety margins. In case of affected borders, it is recommended to perform a resection, due to the local recurrence and this may be the first indicator of aggressive behavior. We present a clinical case at the vulvar level, in order to emphasize the importance of making a correct diagnosis, for a good clinical management and follow-up. Malignant forms account for less than 3% of these tumors and are very aggressive.

Adult granulosa cell tumor of the testis masquerading as hydrocele

Adult testicular granulosa cell tumor is a rare, potentially malignant sex cord-stromal tumor, of which 30 cases have been described to date. We report the case of a 43-year-old male who complained of a left testicular swelling. Scrotal ultrasound showed a cystic lesion, suggestive of hydrocele. However, due to a clinical suspicion of a solid-cystic neoplasm, a high inguinal orchidectomy was performed, which, on pathological examination, was diagnosed as adult granulosa cell tumor. Adult testicular granulosa cell tumors have aggressive behaviour as compared to their ovarian counterparts. They may rarely be predominantly cystic and present as hydrocele. Lymph node and distant metastases have been reported in few cases. Role of MIB-1 labelling index in prognostication is not well defined. Therefore, their recognition and documentation of their behaviour is important from a diagnostic, prognostic and therapeutic point of view.

Diagnóstico prenatal de tumor de células de la granulosa de tipo juvenil / Prenatal diagnosis of juvenile granulosa cell tumor

Presentamos un caso clínico de diagnóstico prenatal de una masa testicular. Tras el nacimiento, se realizó la exéresis del tumor y el análisis anatomopatológico determinó que se trataba de un tumor de células de la granulosa juvenil. Los tumores testiculares son raros y deben considerarse en el diagnóstico diferencial de las masas escrotales en los neonatos. El tumor de células de la granulosa juvenil es una entidad clínico-patológica poco frecuente, que representa el 5% de los tumores testiculares prepuberales. Se considera una neoplasia benigna y la orquiectomía es una técnica quirúrgica curativa.

We report a case of a prenatally diagnosed testis tumor. After delivery, it was decided to perform right radical orchiectomy which was subsequently diagnosed as a juvenile granulosa cell tumor. Neonatal testicular tumors are rare and should be considered in the differential diagnosis of newborn scrotal masses. Juvenile granulosa cell tumor is a rare benign neoplasm of the testicular stroma that accounts for 5% of all prepuberal testis tumors. As a benign neoplasm, orchiectomy is sufficient for treatment.

Tumor de células de la granulosa asociado a hiperandrogenismo femenino rápidamente progresivo / Granulosa cell tumour associated with rapidly progressive female hyperandrogenism

Reportamos el caso de una mujer de 21 años con hiperandrogenismo rápidamente progresivo de origen tumoral ovárico. La biopsia informó tumor de células de la granulosa y la resección fue curativa. Se analizan los posibles mecanismos por los que un tumor de origen en células de la granulosa pueda sintetizar andrógenos.

We report a 21 year old woman with rapidly progressive hyperandrogenism of ovaric tumoral origin. The biopsy of the tumor reported a granulosa cell tumor and the surgery was curative. We analyze the possible mechanisms implied in the androgen production in the granulosa cells of the tumor.

Tumor de células de la granulosa del testículo, tipo juvenil: reporte de un caso y revisión de la literatura / Granulosa cell tumor of testis, juvenile type: case report and review of literature

El Tumor de Células de la Granulosa Juvenil (TCGJ) del testículo es una entidad clínico-patológica poco frecuente, que típicamente se presenta en la edad pediátrica representando el 15 por ciento de los tumores del estroma gonadal. Su comportamiento biológico es considerado benigno y la orquiectomía considerada curativa en estos pacientes. Se presenta el caso de un lactante de sexo masculino de 3 meses de edad con un TCGJ del testículo diagnosticado en la Unidad de Anatomía Patológica del Hospital Hernán Henríquez Aravena de Temuco.

Juvenile granulosa cell tumor of the testis (JGCT) is a uncommon clinical and pathological entity, typically present in children accounting for 15 percent of gonadal stromal tumors. Its biological behavior is deemed benign and orchiectomy considered curative in these patients. We present a case of a male infant 3 months of age with a JGCT diagnosed in the Pathology Unit of Hospital Hernán Henríquez Aravena, Temuco.

Clinicopathological aspects and their relation to prognosis in adult-type granulosa cell tumor of the ovary / Aspectos clinicopatológicos e sua relação com o prognóstico em tumor de células da granulosa do tipo adulto do ovário

INTRODUCTION AND OBJECTIVE: The adult granulosa cell tumors (AGCT) correspond to less than 5 percent of ovarian neoplasias. They are considered low malignant potential tumors and may recur after many years. The differential diagnosis must be made with other primary or metastatic ovarian neoplasias. The aim was to analyze clinical and pathological aspects of AGCT and relate them to its evolution. METHOD: in a 10- year (1995-2004) review of the files from University of Campinas Clinical Hospital, Brazil, 20 AGCT cases were found. The clinical records and slides were reviewed and age, symptoms, macro and microscopic aspects, diagnostic staging and recurrence were considered. When there was intraoperative biopsy, its accuracy was evaluated. RESULTS: Age ranged from 27 to 79 years (mean: 53) and the follow-up from 12 to 96 months (mean: 42). The main symptoms were post-menopause bleeding (45 percent), abdominal pain (35 percent) and palpable mass (25 percent). Most tumors were yellowish (60 percent) and the solid aspect (40 percent) was more common than the cystic or solid-cystic. The histological patterns were 40 percent solid, 15 percent macrofollicular and 45 percent combined forms. All of them with low mitotic index. Only three out of nine intraoperative frozen sections were accurately diagnosed. The clinical staging was 13 cases in Ia (65 percent), one case Ic and 6 IIIc. In three out of 14 hysterectomies there was simple endometrial hyperplasia with no atypia. Only the disease staging was significantly associated with recurrence (p < 0.0001). CONCLUSION: ACGT generally occurs after menopause and intraoperative biopsies are commonly inconclusive. Only advanced staging was related to the worst prognosis.

INTRODUÇÃO E OBJETIVO: O tumor de células da granulosa tipo adulto (TCGA) corresponde a menos de 5 por cento das neoplasias ovarianas. São de baixo potencial de malignidade, podem recorrer depois de muitos anos, e o diferencial deve ser feito com outras neoplasias primárias ou metastáticas. Analisamos os aspectos clínicos e patológicos do tumor, relacionando-os à evolução. MÉTODOS: Na revisão de 10 anos dos arquivos do laboratório de Anatomia Patológica do Hospital das Clínicas da Universidade de Campinas (UNICAMP), 20 casos de TCGA foram encontrados. Os prontuários e as lâminas foram revisados e considerados: idade, sintomas, aspectos macro e microscópicos, estádio ao diagnóstico e à recidiva. Quando houve biópsia intraoperatória, sua acurácia foi avaliada. RESULTADOS: A idade variou de 27 a 79 anos (média: 53); o seguimento de 12 a 96 meses (média: 42). Os sintomas principais: sangramento pós-menopausa (45 por cento), dor abdominal (35 por cento) e massa palpável (25 por cento). A maioria era amarelada (60 por cento), o aspecto sólido mais comum (40 por cento) que o cístico ou sólido-cístico. Os padrões histológicos foram: 40 por cento sólido, 15 por cento macrofolicular e 45 por cento de formas combinadas, todos com baixo índice mitótico. Apenas três de nove casos submetidos à biópsia intraoperatória foram diagnosticados corretamente. O estádio clínico foi: 13 casos Ia (65 por cento), um caso Ic e seis, IIIc. Em três de 14 histerectomias analisadas, havia hiperplasia endometrial simples sem atipia. Apenas o estádio da doença foi significativamente associado à recidiva (p < 0,0001). CONCLUSÃO: TCGA geralmente ocorre após a menopausa, as biópsias intraoperatórias são mais comumente inconclusivas e apenas o estádio avançado esteve relacionado com o pior prognóstico.

Tumor de células de la granulosa ovárico con metástasis pulmonares 26 años después / Granulosa cell tumor of the ovary with late (26-year) lung metastasis

Antecedentes: El tumor de células de la granulosa (TCG) ovárico es considerado una neoplasia de la cuerda sexual, de baja frecuencia, en su mayoría debuta en etapas tempranas, con patrón de crecimiento lento y puede presentar metástasis tardías. Objetivo: Presentar un caso clínico de metástasis pulmonares de TCG ovárico 26 años después del diagnóstico. Caso clínico: Mujer de 70 años, con antecedentes de TCG ovárico diagnosticado en 1976, tratado con histerectomía, salpingooforectomía bilateral y quimioterapia triasociada. En buenas condiciones generales hasta el 2002, detectándose múltiples nodulos tumorales pulmonares bilaterales correspondientes a metástasis de TCG. Recibió quimioterapia con platino y etopó-sido. Actualmente la paciente se encuentra viva, con enfermedad tumoral pulmonar de lento crecimiento. Conclusión: A lo mejor de nuestro conocimiento, se trata del tercer caso a nivel mundial de un TCG ovárico con metástasis pulmonares tardías, 26 años posdiagnóstico del tumor primario. Se señala el curso clínico de lenta evolución de esta neoplasia y la relevancia de los controles a largo plazo incluyendo niveles de estradiol o inhibina.

Background: Granulosa cell tumor of the ovary (GCT) of the ovary is a sex cord related tumor. It is a low frequency neoplasm with low clinical progression but it may show late metastases. Objective: To present a 70 year old female with a GCT of the ovary with multiple pulmonary metastases after 26 years from the initial diagnosis of the primary tumor. Clinic case: The patient had an ovarian GCT diagnosed by histology in 1976 and underwent hysterectomy, bilateral salpingoophorectomy and chemotherapy. The clinical course was satisfactory until 2002 when she presented with respiratory symptoms and images showing multiple bilateral pulmonary nodules that were histologically confirmed to be GCT metastases. The patient underwent chemotherapy with platinum and ethoposide. Actually the patient is alive with metastatic disease. Conclusion: To the best of our knowledge, this is the third case in medical literature of a GCT of the ovary with late pulmonary metastases 26 years after the initial diagnosis. This case illustrates the protracted clinical course of the neoplasm and the importance of the long term follow up including levels of estradiol and inhibin.

Unicystic granulosa cell tumor: a case report.

Granulosa Cell Tumors (GCT) constitutes 1.5% to 3.5% of all primary ovarian neoplasms. They may be solid, cystic or both. Unilocular cystic GCT are very rare. We report here a case of 32 years female with unicystic GCT in which the distinction from follicular cyst was difficult and was made by carefully examining the lining of the cyst,which showed occasional multiple layers of granulosa cells with a few Call-Exner bodies.

Granulosa cell ovarian tumor as cause of primary amenorrhoea

An 18-year old single girl presented in gynecological outpatients department in April 2004 as a case of primary amenorrhoea and abdominal mass. Laparotomy was performed and tumor removed. Diagnosis of granulosa cell tumor was confirmed on histopathology. Granulosa cell ovarian tumor usually presents with menstrual problem in the form of precocious puberty, irregular bleeding or postmenopausal bleeding. Our patient presented with primary amenorrhoea that is extremely rare presentation

Tumores cutáneos dolorosos: segunda parte / Painful cutaneous tumors: second part

Se describe una serie clínico-patológica de tumores cutáneos habitualmente dolorosos y se revisan las características de cada uno de ellos, lipomatosis dolorosa, neurilemoma, neuroma, nevo azul en tetina de goma, sinovialoma, tumor de células granulosas y tumor glómico

Tumor de células granulosas doble localización en lengua / Granular cell tumor: double localization on the tongue

El tumor de células granulosas es una neoformación de curso habitualmente benigno. Entidad clínica infrecuente y de histología definida. Su localización principal es en mucosa bucal, sobre todo en lengua, piel y tejido celular subcutáneo; menos frecuente en distintos órganos. Se presenta como un tumor único, noduloide, asintomático,duro y pequeño. Presentamos un caso de sexo masculino, caracterizado por tener dos tumores aislados, localizados en ambos bordes de la lengua; uno de aspecto noduloide ulcerado, y el otro tipo placa indurada, de dos años y medio de evolución, doloroso al roce y a la palpación. Ambos fueron extirpados quirúrgicamente, sin recaídas

Tumor de células da granulosa do ovário: estudo de casos com utilizaçäo da imuno-histoquímica no diagnóstico diferencial / Granulosa cell tumor of ovary: study of cases with utilization of the immunohistochemistry in differential diagnosis

Um estudo retrospectivo de 23 casos de tumor de células da granulosa do ovário foi realizado. Inicialmente foi feito um estudo imuno-histoquímico empregando anticorpos antiantígeno de membrana epitelial e citoceratina. Nenhum tumor com histologia característica mostrou positividade para estes anticorpos, enquanto três com histologia näo característica apresentaram reaçöes positivas. Estes tumores foram classificados como tumores epiteliais indiferenciados, pois tanto o prognóstico desfavorável como a baixa taza de sobrevida indicaram näo se tratar de tumor de células da granulosa. Os autores prosseguiram o estdo clínico e anatomopatológico somente os casos que confirmaram , pela histologia e imuno-histoquímica, ser tumor de células de granulosa. A dor abdominal foi o sintoma mais frequênte e somente 50 porcento apresentaram sintomas ligados à açäo estrogênica. Características histológicas näo diferiram das járelatadas. Näo se evidenciou o tamanho do tumor como fator prognóstico. Dos 12 casos em que foi realizada avaliaçäo do endométrio, o achado de hiperplasia foi o mais comum. O tratamento cirúrgico foi o de escolha como conduta inicial. O tratamento adjuvante ou complementar foi discutido.

Tumor de células granulares da laringe: relato de um caso / Granular cell tumor of the larynx: report of a case

O autor apresenta um caso de tumor de células granulares da laringe (tumor de Abrikosoff). A lesäo é rara, de acordo com a bibliografia apresentada, e tem interesse para alertar o especialista a pensar no seu diagnóstico

Tumor ovariano de células da granulosa e gravidez: relato de um caso / Granulosa cell tumor of the ovary and pregnancy: report of a case

É apresentada uma revisäo sobre os tumores das células da granulosa e descrito um caso de ocorrência em associaçäo a gravidez