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.

Tumor de células de la granulosa tipo juvenil tras la menopausia / Juvenile granulosa cell tumor in menopausal woman

Los tumores de células de la granulosa son tumoraciones ováricas infrecuentes. Hay dos tipos histológicos: adulto y juvenil. Los tumores de células de la granulosa juvenil representan el 5 %, y solamente un 3 % ocurre en mayores de 30 años. Ante la sospecha, el diagnóstico definitivo intraoperatorio es complejo dada su rareza y su fácil confusión con otras neoplasias ováricas. El patrón quístico con células de la granulosa inmaduras, con frecuentes mitosis, la ausencia de cuerpos de Call-Exner y el estudio inmunoquístico lo confirman. Su baja prevalencia dificulta su diagnóstico. El estadio de la enfermedad es el factor pronóstico más importante, y resulta imprescindible una completa resección. El papel de la terapia complementaria no está bien establecido, además los estudios disponibles solamente incluyen un número mínimo de casos, que no diferencian mujeres adultas. El adecuado seguimiento para la detección precoz de una posible recidiva tardía supone un reto clínico(AU)

Granulosa cell tumors are rare ovarian tumors. There are two histological types: adult and juvenile. Juvenile granulosa cell tumors account for 5%, with only 3% occurring in people over 30 years of age. Given the suspicion, the definitive intraoperative diagnosis is complex given its rarity and its easy confusion with other ovarian neoplasms. The cystic pattern with immature granulosa cells, with frequent mitosis, the absence of Call-Exner bodies and the immunocystic study confirm this. Its low prevalence makes it difficult to diagnose. The stage of the disease is the most important prognostic factor, and complete resection is essential. The role of complementary therapy is not well established, and the available studies include only a minimal number of cases, which do not differentiate between adult women. Adequate follow-up for the early detection of a possible late recurrence is a clinical challenge(AU)

Complete restoration of fertility in a patient treated for androgen-secreting granulosa cell tumor - Case report.

CASE REPORT: A 35-yr-old patient suffering from secondary amenorrhea for two years before she was diagnosed. Secondary amenorrhea occurred after the first normal vaginal delivery, and it was initially associated with breastfeeding and a formerly diagnosed thyroid disease. Transvaginal ultrasound confirmed a tumorous mass of the right ovary. Blood hormone tests detected high serum inhibin B and Anti-Müllerian hormone levels and high androgen level with no signs of virilization. Surgical treatment was indicated for a definitive diagnosis of suspected sex cord-stromal tumor. Right-sided laparoscopic salpingo-oophorectomy was performed, and the histopathological analysis confirmed the diagnosis of granulosa cell tumor adult type. The oncological team recommended adjuvant chemotherapy after the operation, but the patient did not give an informed consent. One month after surgical treatment, spontaneous menstrual bleeding occurred with normalization of sex hormone levels and the menstrual cycle. Nine months after surgical treatment, the patient was examined again due to secondary amenorrhea. Ultrasound confirmed a vital intrauterine pregnancy. The pregnancy course was normal, and the patient had a full-term spontaneous vaginal delivery of her second child. CONCLUSION: Restoration of fertility after a temporary loss due to hormone-secreting granulosa cell tumor is possible after sparing surgical treatment. The role of adjuvant chemotherapy is controversial, particularly in patients with stage I-II disease because of the rarity of this tumor and the absence of prospective randomized studies.

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.

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.

Cáncer sincrónico de ovario y cúpula vaginal. Reporte de un caso y revisión de la bibliografía / Synchronous ovarian and vaginal vault cancer. Report of a case and review of the literature

Resumen INTRODUCCIÓN: El cáncer sincrónico es poco frecuente y de origen incierto. Se caracteriza por diferentes lesiones malignas que afectan dos o más zonas del aparato reproductor al mismo tiempo o en un lapso menor a un año; la combinación ovario y endometrio es la más frecuente. OBJETIVO: Reportar un caso de cáncer ginecológico sincrónico, tumor maligno de células de la granulosa de ovario y carcinoma de células escamosas en la cúpula vaginal; además, revisar la bibliografía al respecto. CASO CLÍNICO: Paciente de 47 años, con histerectomía total por miomatosis y una lesión de gran tamaño que ocupaba el espacio pélvico y comprimía el techo vesical. Acudió a consulta debido a un sangrado genital abundante, pérdida de peso y de orina en forma involuntaria. En el examen físico se evidenció una lesión friable en la cúpula vaginal de 3 cm. Se decidieron la ooforectomía izquierda y la extirpación tumoral. La biopsia y la inmunohistoquímica de las lesiones reportó un tumor maligno de células de la granulosa del adulto localizado en el ovario, y un carcinoma de células escamosas en la cúpula vaginal. Se indicó quimioterapia y en la actualidad la paciente permanece sin recidiva de la enfermedad y en seguimiento oncológico. CONCLUSIÓN: El cáncer sincrónico es poco frecuente, de ahí que se disponga de poca información. Gracias al acierto del diagnóstico y de la conducta terapéutica a seguir, luego de 24 meses la paciente del caso no ha tenido signos de recidiva de la enfermedad; continúa en seguimiento oncológico conforme a los protocolos establecidos.

Abstract INTRODUCTION: Synchronous cancer in gynecology is a rare entity, of uncertain etiology, consisting of different malignant lesions affecting two or more areas of the female reproductive system, at the same time or within a period of less than 1 year, the combination of ovary and endometrium being the most frequent. OBJECTIVE: to report a case of synchronous gynecological cancer, malignant granulosa cell tumor of the ovary and squamous cell carcinoma of the vaginal vault and in view of the infrequency of the case to review the literature. CLINICAL CASE: 47 years old patient, who underwent total hysterectomy for uterine myomatosis plus right oophorectomy for showing ovarian tumor during surgery, the histopathological report reported malignant granulosa cell tumor, abdomino-pelvic tomography was requested, which reported a large pelvic space occupying lesion (LOE) with compression of the bladder roof. She came to our office for the first time due to abundant genital bleeding, weight loss and involuntary urine loss, physical examination revealed a friable lesion in the vaginal vault of 3cm, surgical intervention was decided where a left oophorectomy and tumor pruning were performed, Biopsy and immunohistochemistry of lesions with report of malignant granulosa cell tumor of adult granulosa cells in the ovary and squamous cell carcinoma in the vaginal vault, referred to medical oncology who applied chemotherapy and currently the patient is without recurrence of the disease and in oncological follow-up. CONCLUSION: Synchronous cancer is an infrequent pathology, where much information remains to be investigated to better understand this entity. We present the first reported case of synchronous ovarian granulosa cell tumor cancer with vaginal vault cancer. Fortunately our patient 24 months after completing her treatment shows no signs of recurrence of the disease and will be followed up according to the established oncologic protocols.

Tumor de las células de la granulosa del adulto / Adult granulosa cell tumor

Resumen ANTECEDENTES: El tumor de células de la granulosa representa del 2 al 5% de las neoplasias del ovario. Su manifestación clínica no siempre es específica. OBJETIVO: Analizar el comportamiento del tumor de las células de la granulosa y aportar experiencia para su tratamiento. CASO CLÍNICO: Paciente de 52 años, con proliferación de células de la granulosa con escaso citoplasma y núcleos ovoides, algunos de ellos con surcos prominentes, con patrón de crecimiento trabecular y difuso. La manifestación inicial fue un episodio de sangrado posmenopáusico que hizo sospechar la patología endometrial. La inmunohistoquímica reportó positividad para inhibina y débilmente positivo para alfa-fetoproteína, negativo para citoqueratinas de amplio espectro, EMA y cromogranina; ki-67: 5-10%. Se indicó histerectomía y doble anexectomía por laparoscopia y omentectomía. Con el diagnóstico de tumor de células de la granulosa estadio IC se indicó tratamiento coadyuvante con quimioterapia, 3 ciclos de bleomicina, etopósido y cisplatino. El seguimiento se efectuó con ecografía y concentraciones de inhibina B, que han permanecido en límites de normalidad en el control periódico. CONCLUSION: El tumor de células de la granulosa es de bajo grado de malignidad y diseminación preferentemente local. Su pronóstico es excelente, aunque debido a su recurrencia, años después del diagnóstico inicial parece razonable prolongar la vigilancia con exámenes físicos y el estudio de marcadores tumorales.

Abstract BACKGROUND: Granulosa cell tumor represents 2 to 5% of ovarian neoplasms. Its clinical manifestation is not always specific. OBJECTIVE: To analyze the behavior of granulosa cell tumor and to provide experience for its treatment. CLINICAL CASE: A 52-year-old patient with granulosa cell proliferation with scant cytoplasm and ovoid nuclei, some of them with prominent grooves, with trabecular and diffuse growth pattern. The initial manifestation was an episode of postmenopausal bleeding that raised suspicion of endometrial pathology. Immunohistochemistry was positive for inhibin and weakly positive for alpha-fetoprotein, negative for broad-spectrum cytokeratins, EMA and chromogranin; ki-67: 5-10%. Hysterectomy and double adnexectomy by laparoscopy and omentectomy were indicated. With the diagnosis of granulosa cell tumor stage IC, adjuvant treatment with chemotherapy was indicated, 3 cycles of bleomycin, etoposide and cisplatin. Follow-up was carried out with ultrasound and inhibin B concentrations, which have remained within normal limits in the periodic control. CONCLUSION: Granulosa cell tumor is of low malignancy grade and preferably local dissemination. Its prognosis is excellent, although due to its recurrence, years after the initial diagnosis it seems reasonable to prolong surveillance with physical examinations and the study of tumor markers.

Síndrome de pseudo-Meigs por tumor de células de la granulosa / Pseudo-Meigs syndrome due to granulosa cell tumor / Pseudo-Meigs syndrome due to granulosa cell tumor

RESUMEN Definimos síndrome de Meigs como la triada de tumor ovárico benigno, derrame pleural y ascitis, una condición clínica rara que se resuelve con la resección del tumor. Estas mismas características pueden presentarse en el síndrome de pseudoMeigs que se asocia a tumores malignos, que agregan un aumento importante de los niveles del marcador CA-125. Es conocido por muchos años, pero su fisiopatología aún no está clara. Se presenta un caso de síndrome pseudo-Meigs y se hace una breve revisión bibliográfica de sus características más importantes.

ABSTRACT Meigs' syndrome is defined as the triad of benign ovarian tumor, pleural effusion and ascites, a rare clinical condition that is treated with tumor resection. Same characteristics may occur in cases of malignant tumors, that add a notable increase in antigen CA-125 serum levels, constituting the pseudo-Meigs syndrome. They have been known for many years, but their pathophysiology remains unclear. We report the case of a pseudo-Meigs syndrome, and a brief bibliography review of the most important characteristics of these syndromes is performed.

Ovarian granulosa cell tumor and clomiphene citrate resistance. A case report and review of the literature.

Granulosa cell tumors (GCTs) account for less than 5% of all ovarian malignancies, occur in younger ages, are usually diagnosed in their early stages, and have a good prognosis. GCTs usually present with features of hyperestrogenism. This paper reports the unusual case of an adult with a GCT with manifestations including amenorrhea, mild hirsutism, infertility, clomiphene citrate (CC) resistance (CC), mildly elevated testosterone, high anti-Müllerian hormone (AMH) and normal estrogen levels. The patient was initially diagnosed with polycystic ovarian syndrome (PCOS), and after four attempts at ovarian stimulation she was diagnosed with CC resistance. The patient later underwent laparoscopic evaluation on account of a solid mass on her left ovary. The pathology report described it as a borderline adult type GCT and four weeks after surgery she had a positive pregnancy test. Twelve months after delivery, the patient had no obvious symptoms of disease and her menstrual cycle was normal. Serial measurements of serum inhibin B, AMH, estrogen, and testosterone levels were within normal range. In conclusion, the resistance to clomiphene manifested by the patient might be explained by a potential mechanism implicating inhibin B and AMH due to the presence of a GCT. Further studies are required to evaluate the role of AMH and Inhibin B in the mechanism of CC resistance in women with PCOS.

Tumor de células granulosas del esófago / Granulosa cell tumor in the esophagus

Los tumores de células granulosas afectan raramente al esófago. Presentamos el caso de tumores de células granulosas del esófago y revisar la literatura. Se presenta una paciente de 21 años de edad y piel negra con síntomas de reflujo gastroesofágico. La endoscopia digestiva demostró un tumor de 2 cm que protruía hacia la luz del órgano, localizado en el tercio inferior, con mucosa sana. El esofagograma corroboró el hallazgo en la porción abdominal del órgano y la biopsia endoscópica informó que se trataba de tumores de células granulosas. El acceso videolaparoscópico fue convertido a cirugía convencional debido a la abertura mucosa y a que quedaba un pequeño fragmento del tumor. El periodo posoperatorio transcurrió sin complicaciones. Un año después de la operación no existía evidencia de recidiva. Los tumores de células granulosas son raros en el esófago. Los tumores pequeños (< 1 cm) pueden ser tratados conservadoramente, mientras que los de mayor volumen deben ser resecados mediante diferentes técnicas: endoscópicas y cirugía de mínimo acceso o convencional(AU)

Granulosa cell tumors rarely affect the esophagus. We present the case of granulosa cell tumors of the esophagus and literature review. A 21-year-old patient of black skin presents with symptoms of gastroesophageal reflux. The digestive endoscopy showed a 2-cm tumor that protruded towards the organ lumen, located in the lower third, with healthy mucosa. The esophagogram corroborated the finding in the abdominal portion of the organ, and the endoscopic biopsy reported that there were granulosa cell tumors. Videolaparoscopic access was converted to conventional surgery due to the mucosal opening and because a small fragment of the tumor was remaining. The postoperative period had no complications. One year after the operation, there was no evidence of recurrence. Granulosa cell tumors are rare in the esophagus. Small tumors (smaller than 1 cm) can be treated conservatively, while larger tumors should be resected using different techniques: endoscopic and minimal access or conventional surgery(AU)

Tumor de células granulosas del esófago / Granulosa cell tumor in the esophagus

Los tumores de células granulosas afectan raramente al esófago. Presentamos el caso de tumores de células granulosas del esófago y revisar la literatura. Se presenta una paciente de 21 años de edad y piel negra con síntomas de reflujo gastroesofágico. La endoscopia digestiva demostró un tumor de 2 cm que protruía hacia la luz del órgano, localizado en el tercio inferior, con mucosa sana. El esofagograma corroboró el hallazgo en la porción abdominal del órgano y la biopsia endoscópica informó que se trataba de tumores de células granulosas. El acceso videolaparoscópico fue convertido a cirugía convencional debido a la abertura mucosa y a que quedaba un pequeño fragmento del tumor. El periodo posoperatorio transcurrió sin complicaciones. Un año después de la operación no existía evidencia de recidiva. Los tumores de células granulosas son raros en el esófago. Los tumores pequeños (< 1 cm) pueden ser tratados conservadoramente, mientras que los de mayor volumen deben ser resecados mediante diferentes técnicas: endoscópicas y cirugía de mínimo acceso o convencional(AU)

Granulosa cell tumors rarely affect the esophagus. We present the case of granulosa cell tumors of the esophagus and literature review. A 21-year-old patient of black skin presents with symptoms of gastroesophageal reflux. The digestive endoscopy showed a 2-cm tumor that protruded towards the organ lumen, located in the lower third, with healthy mucosa. The esophagogram corroborated the finding in the abdominal portion of the organ, and the endoscopic biopsy reported that there were granulosa cell tumors. Videolaparoscopic access was converted to conventional surgery due to the mucosal opening and because a small fragment of the tumor was remaining. The postoperative period had no complications. One year after the operation, there was no evidence of recurrence. Granulosa cell tumors are rare in the esophagus. Small tumors (smaller than 1 cm) can be treated conservatively, while larger tumors should be resected using different techniques: endoscopic and minimal access or conventional surgery(AU)

Tumor da célula da granulosa associado à piometra em uma gata de sete meses / Granulosa-cell tumor associated with pyometra in a seven months old cat

As neoplasias ovarianas em gatas são raras e, quando relatadas, estão associadas a animais senis, assim como a piometra de causa não iatrogênica. O objetivo deste trabalho foi relatar o caso de uma gata jovem com neoplasia ovariana, tumor de células da granulosa associado ao complexo hiperplasia endometrial cística/piometra (HECP). O animal foi atendido no setor de Reprodução Animal e Obstetrícia Veterinária (RAOV) de um Hospital Veterinário Escola, com histórico de monta natural recente, anorexia, êmese, polidipsia, poliúria e descarga vaginal purulenta. Ao exame físico, observou-se apatia, mucosas ictéricas, aumento de volume da região abdominal e presença de secreção vaginal purulenta. À ultrassonografia, foi visibilizado conteúdo ecogênico no útero diagnóstico de HECP e estrutura ecogênica na cavidade abdominal, na região ovariana, confirmado pelo exame histopatológico como sendo tumor das células da granulosa. A conduta terapêutica adotada foi a cirurgia de ovariossalpingo-histerectomia (OSH).(AU)

Ovarian neoplasias in cats are rare, and are associated with advanced ages, as well as non-iatrogenic pyometra. The objective of the present study was to report a case of a young cat showing signs of a rare neoplasia, the granulosa-cell tumor, associated with complex cystic endometrial hyperplasia/pyometra (HECP). The animal was attended in the Animal Reproduction and Veterinary Obstetrics sector of a Teaching Veterinary Hospital with a history of natural breeding, anorexia, emesis, polydipsia, polyuria, and purulent vaginal discharge. During clinical examination, apathy, icterus, swelling of the abdomen, and purulent vaginal discharge were observed. On the ultrasonographic exam, an echogenic content inside the uterus was observed, leading to diagnosis of HECP and an echogenic structure in the abdominal cavity, in ovarian region, confirmed by histopathology as being of granulosa cells. The therapeutic conduct adopted was salpingo-oophorectomy and hysterectomy surgery (OSH).(AU)

Tumor da célula da granulosa associado à piometra em uma gata de sete meses / Granulosa-cell tumor associated with pyometra in a seven months old cat

As neoplasias ovarianas em gatas são raras e, quando relatadas, estão associadas a animais senis, assim como a piometra de causa não iatrogênica. O objetivo deste trabalho foi relatar o caso de uma gata jovem com neoplasia ovariana, tumor de células da granulosa associado ao complexo hiperplasia endometrial cística/piometra (HECP). O animal foi atendido no setor de Reprodução Animal e Obstetrícia Veterinária (RAOV) de um Hospital Veterinário Escola, com histórico de monta natural recente, anorexia, êmese, polidipsia, poliúria e descarga vaginal purulenta. Ao exame físico, observou-se apatia, mucosas ictéricas, aumento de volume da região abdominal e presença de secreção vaginal purulenta. À ultrassonografia, foi visibilizado conteúdo ecogênico no útero diagnóstico de HECP e estrutura ecogênica na cavidade abdominal, na região ovariana, confirmado pelo exame histopatológico como sendo tumor das células da granulosa. A conduta terapêutica adotada foi a cirurgia de ovariossalpingo-histerectomia (OSH).(AU)

Ovarian neoplasias in cats are rare, and are associated with advanced ages, as well as non-iatrogenic pyometra. The objective of the present study was to report a case of a young cat showing signs of a rare neoplasia, the granulosa-cell tumor, associated with complex cystic endometrial hyperplasia/pyometra (HECP). The animal was attended in the Animal Reproduction and Veterinary Obstetrics sector of a Teaching Veterinary Hospital with a history of natural breeding, anorexia, emesis, polydipsia, polyuria, and purulent vaginal discharge. During clinical examination, apathy, icterus, swelling of the abdomen, and purulent vaginal discharge were observed. On the ultrasonographic exam, an echogenic content inside the uterus was observed, leading to diagnosis of HECP and an echogenic structure in the abdominal cavity, in ovarian region, confirmed by histopathology as being of granulosa cells. The therapeutic conduct adopted was salpingo-oophorectomy and hysterectomy surgery (OSH).(AU)

Tumor de células de la granulosa del adulto: reporte de un caso con manifestación inusual / Adult granulosa cell tumor: a case report with unusual presentation

Resumen OBJETIVO: describir un caso clínico de tumor de células de la granulosa de tipo adulto, en forma bilateral, con diagnóstico inicial erróneo de carcinoma de vejiga urinaria. CASO CLÍNICO: paciente de 43 años de edad, con antecedente de hiperplasia endometrial sin atipia. El padecimiento se inició en marzo de 2015, con dolor en la fosa renal izquierda. En la exploración física se documentó distensión abdominal, dolor ocasional y pérdida de peso (3 kg) en cuatro meses; masa abdominal, sólida, dolorosa, tacto vaginal con abultamiento y desplazamiento de la pared lateral izquierda de la vagina. La tomografía axial computada evidenció tres masas en el hueco pélvico. En la laparotomía se resecaron ambos tumores ováricos. Se inició tratamiento con quimioterapia coadyuvante, con cisplatino-etopósido (CDDP/VP-16). CONCLUSIÓN: la manifestación bilateral del tumor de células de la granulosa del adulto aparece en sólo 3% de los casos. Luego de practicar estudios radiológicos y de inmunohistoquímica se estableció el diagnóstico correcto. La quimioterapia coadyuvante con cisplatino-etopósido, ha demostrado resultados satisfactorios. Actualmente no existen datos de actividad tumoral y la paciente permanece en seguimiento.

Abstract OBJETIVO: Describes a clinical case of adult granulosa cell tumor, bilaterally, with initial misdiagnosis of urinary bladder carcinoma. CLINICAL CASE: 43 year old female with antecedent of endometrial hyperplasia without atypia. Starts condition in March 2015 with pain in renal fossa. At the time of consultation presented abdominal distention, occasional pain, weight loss (3 kg) in 4 months. On physical exploration an abdominal mass was found, it was solid and painful. Vaginal touch with presence of a lump and displacement of the left side wall of the vagina. In the computerized axial tomography 3 masses were seen in pelvic hollow. The patient underwent a laparotomy and both tumors were resected. Adjuvant chemotherapy was initiated. CONCLUSION: The bilateral presentation of this type occurs only in 3% of these tumors. After radiological and immunohistochemistry studies a correct diagnosis was established. Adjuvant chemotherapy based in cisplatin/etoposide has shown satisfactory results. Currently these are no data of tumor activity. Continuous surveillance is been performed.

Tumor de las células de la granulosa: importancia de la inmunohistoquímica en el diagnóstico

Se presenta un caso de una paciente joven de 30 años quien consultó por trastornos menstruales y dolor abdominal de evolución de 1 año. Se le diagnóstico una tumoración de ovario poco frecuente como son los tumores de las células de la granulosa por medio de marcadores de inmunohistoquímica.

It is presented a case of a young woman who had menstrual problems and abdominal pain since one year ago. It was diagnosed an uncommon ovarian tumor such as tumor of granulosa cells using immunohistochemical markers.

Metástasis pulmonar de tumor de células de la granulosa como recurrencia del tumor primario después de diez años del tratamiento quirúrgico / Pulmonary metastasis of granulosa cell tumor as recurrence of primary tumor after 10 years of surgical treatment

Los tumores de células de la granulosa son neoplasias de bajo grado, que corresponden al 2-5% de los tumores malignos del ovario y presentan una baja incidencia. Las manifestaciones clínicas dependerán del tamaño tumoral y de la exposición prolongada a estrógenos producidos por las células neoplásicas. Habitualmente, son tumores unilaterales, sólido-quísticos con focos de hemorragia, constituidos por células pálidas con su característico pliegue nuclear, en un trasfondo fibrotecomatoso, con cuerpos de Call Exner sólo en el 30-60% de los casos. El diagnóstico se realiza con los niveles séricos de estradiol y con exámenes imagenológicos como ecotomografía ginecológica, tomografía axial computarizada o resonancia nuclear magnética. El tratamiento quirúrgico es la elección. Las recurrencias pueden ocurrir años posteriores al diagnóstico inicial y en general son pélvicas. El factor pronóstico más determinante en la evolución de la enfermedad es el estadio clínico al momento del diagnóstico. Se presenta el caso clínico de una paciente postmenopáusica con metástasis pulmonar de tumor de células de la granulosa después de diez años del tratamiento quirúrgico.

The granulosa cell tumors are low grade neoplasias that correspond to 2-5% of all malignant tumors of the ovary with a low incidence in the population. The clinical presentation depends on the tumor size and the long term exposition to estrogens produced by neoplastic cells. They are usually unilateral tumors exhibiting a mixture of cystic and solid areas with bleeding, constituted by pale cells with their characteristic nuclear groove in a fibrotecomatous background, with Call Exner bodies only in 30-60% of cases. The diagnosis is done with serum levels of estradiol and gynecological imaging analysis such as echotomography, Computerized axial tomography and magnetic nuclear resonance. The surgical treatment is the choice. The recurrences could happen years after the first diagnosis and usually are in pelvic area. The most important prognostic factor in the disease progression is the clinical stage at diagnosis. It is presented a case report of a postmenopausal patient with lung metastasis of granulosa cell tumor after ten years of surgical treatment.

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.

Tumor adulto de células de la granulosa del ovario: presentación de un caso / Adult granulosa cell tumor of ovary: a case presentation

Introducción: el tumor de células de la granulosa ovárica representa del 2 al 5 % de todos los tumores de ovario. Producen hormonas esteroideas con acciones estrogénicas o androgénicas, por lo que esto facilita su diagnóstico precoz. Se reconocen dos subtipos histopatológicos, uno adulto y otro juvenil. Objetivo: describir el cuadro clínico y hallazgo histopatológico de una paciente con tumor de células de la granulosa. Caso clínico: se muestra el caso de una paciente de 49 años de edad que clínicamente presentó signos de virilización progresiva. Se le diagnosticó por estudio de imagen de ultrasonido transvaginal y tomografía axial computarizada un tumor en el ovario derecho, se realizó su intervención quirúrgica y biopsia que dio como resultado tumor de células de la granulosa. Conclusiones: los tumores que producen estrógenos son los que se encuentran con mayor frecuencia, representando las tres cuartas partes de los tumores de la granulosa. La virilización en la mujer post menopaúsica por un tumor de células de la granulosa es infrecuente, sin embargo, fue el diagnóstico histopatológico en esta paciente.

Background: ovary granulosa cell tumors represent the 2 to 5 % of all ovary tumors. It produces steroid hormones with estrogenic or androgynous actions which makes easier its early diagnosis. Two histopathological subtypes are recognized: adult and juvenile. Objective: to describe the clinical manifestation and histopathological founds in a patient with a granulosa cell tumor. Clinical case: the case of a forty-nine-year-old patient, who clinically presented signs of progressively acquiring male characteristics, is presented. She was diagnosed a tumor in the right ovary by means of a transvaginal ultrasound and a computerized axial tomography. A surgical procedure and a biopsy were conducted; the results of the biopsy confirmed the presence of a granulosa cell tumor. Conclusions: tumors that produce estrogens are more frequently found and represent the three quarters of the granulosa tumors. Acquiring male characteristics by a granulosa cell tumor in postmenopausal women is infrequent; nevertheless, it was the histopathological diagnosis for this patient.

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.

Granulosa cell tumor of the testis in a newborn.

Testicular neoplasms are uncommon tumors of childhood. These tumors comprise the germ cell tumors, and other tumors that may originate from histological testicular components, which are unrelated to the germinal lineage. Among the latter are the sex cord-stromal tumors (SCST), an important entity in newborns. SCSTs comprise, among others, granulosa cell tumors, which are more common in the ovary, but in rare cases may develop in the testis. The prognosis is excellent since it is universally benign. Diagnosis, which is sometimes challenging, is usually made after orchiectomy and pathological examination, which is characterized by morphological features and positive expression of inhibin, calretinin, and vimentin, and negative for alpha-fetoprotein. The authors present the case of a newborn with a right enlarged testis detected during the first examination after birth. Ultrasonography showed a heterogeneous solid/cystic mass in the right testis, without retroperitoneal lymphadenopathy. A right inguinal orchiectomy was performed 21 hours after birth. Pathologic examination revealed a juvenile granulosa cell tumor of the right testicle. After 4 years of follow-up, as expected, the child presented an uneventful outcome.