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.

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 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.

A clinicopathological study of granulosa cell tumors of the ovary: Can morphology predict prognosis?

Objective: Granulosa cell tumors (GCT) are low-grade malignant sex cord-stromal tumors (SCST) with late metastasis/recurrences and long disease-free periods. We performed a clinicopathological evaluation of GCT to ascertain features having prognostic impact. Materials and Methods: All cases of GCT of ovary from January 2006 to December 2018 were assessed for architectural patterns, nuclear grooves, and Call-Exner bodies. Each feature was graded on frequency of occurrence: not present (0)–very frequent (3). Anisonucleosis, necrosis, and inflammation were noted. Cases were grouped on mitotic count; <10 mitosis/10 High power field (HPF) or >=11 mitoses/10 HPF and Ki-67 index; <10% Ki-67 and >=11% Ki-67. Results: GCT formed 60.1% of SCST. Sixty cases' ages were in the range of 15–78 years (median 45). Clinical details were available in 37. Commonest presentation was abnormal uterine bleeding. Serum CA125 was raised in 16.1% and Inhibin in 58.8%. Seventy percent were in stage I. Disease recurrence was associated with higher stage (P = 0.007). The most frequent pattern was diffuse sheets (47%). Call-Exner bodies were absent in 22.2%. Grooves with score 1, 2, and 3 were seen in 35.8%, 23.5%, and 13.6%, respectively. Anisonucleosis was present in 26.7%, necrosis in 11.1%, and lympho-plasmacytic infiltrate in 43%. Out of total, 93.3% had <10 mitosis/10 HPF and 43.2% had recurrence, most with high Ki-67 (P = 0.064). Conclusion: Our study outlines histomorphological spectrum of GCT and emphasizes its frequent occurrence in lower stages with late recurrences. The presence of grooves may indicate granulosa-cell origin. Call-Exner bodies are not a necessity. Histomorphological features are not prognostically important. However, prognostic value of Ki-67 cannot be excluded. Limitation of the study was a small number of cases with follow-up.

Synchronous occurrence of lobular breast carcinoma and granulosa cell tumor of ovary mimicking metastatic lobular breast carcinoma

Breast cancer is one of the main sources of ovarian metastasis. Diffuse sheet of lobular carcinoma cells can strongly mimic granulosa cell tumor (GCT) which itself is a rare ovarian neoplasm constituting <5% of all the ovarian neoplasms. A 55-year-old female presented with a painful lump in the right breast associated with nipple discharge for 4 months, which on radiological and cytological findings was suspicious of an epithelial malignancy. During routine work-up, a solid-cystic lesion in the left ovarian adnexa was identified. The patient underwent right modified radical mastectomy along with left salpingo-oophorectomy. Histopathological and immunohistochemical features confirmed the diagnosis of a synchronous lobular carcinoma breast with GCT ovary. Simultaneous occurrence of Lobular carcinoma breast (LCB) and GCT ovary is extremely rare. Morphologically, these can look similar, raising a suspicion of LCB metastasis to ovary. This is important to differentiate the two for a proper patient management and prognosis

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)

Ovarian Gynandroblastoma with a Juvenile Granulosa Cell Tumor Component in a Postmenopausal Woman: A Case Report and Literature Review

Gynandroblastoma is an extremely rare sex cord-stromal tumor with both female (granulosa cell tumor) and male (Sertoli-Leydig cell tumor) elements. Juvenile granulosa cell tumors are also very rare and are so named because they usually occur in children and adolescents. A 71-year-old woman with right upper quadrant abdominal pain visited our hospital. Pelvic computed tomography showed a large multilocular cystic mass, suspected to be of ovarian origin. We performed a total abdominal hysterectomy (total abdominal hysterectomy was performed) with bilateral salpingo-oophorectomy. A 13-cm multilocular cystic mass with serous fluid was observed in her right ovary. Upon microscopic examination, the solid component of the mass showed both Sertoli-Leydig cell and juvenile granulosa cell differentiation, which we diagnosed as gynandroblastoma. Gynandroblastoma with a juvenile granulosa cell tumor component is extremely rare and, until now, only six cases have been reported in the English literature. We report the first gynandroblastoma with a juvenile granulosa cell tumor component diagnosed in an elderly patient, along with a literature review.

The value of MRI in differentiating diagnosis of ovarian thecoma-fibroma and adult-type ovarian granulosa cell tumor / 实用放射学杂志

Objective To compare the MRI features of ovarian thecoma-fibroma and adult-type ovarian granulosa cell tumor (OGCT),and to investigate the differential value of MRI.Methods 13 patients (8 cases of ovarian thecoma-fibroma,5 cases of adult-type OGCT)confirmed by pathology were collected,in which MRI findings were analyzed retrospectively.Results 13 lesions were all unilateral.The shape of more lesions appeared round or oval,and huge lesions were lobulated.Most tumors were dominant solid and well-defined borders.The length-diameter of ovarian thecoma-fibromas ranged from 23 to 268 mm,with the mean diameter of (124±84)mm;on T1WI the solid part of tumors showed equal signal in 5 cases,slight lower signal in 3 cases,on T2WI all tumors were heterogeneous signal,6 lesions appeared equal signal with obscure nebulous high signal;all of 8 lesions were enhanced slightly;4 cases were accompanied with pelvic effusion and other type tumors of the ovarian.The length-diameter of adult-type OGCT tumors ranged from 27 to 110 mm, with the mean diameter of (84±33)mm;the solid part of lesions showed slight lower signal on T1WI and high signal on T2WI,the honeycomb and fissure cystic areas were found in all tumors,4 cases were accompanied with hemorrhage;all tumors were enhanced from moderately to highly,and delay contrast enhancement;the cases were accompanied with endometrial hyperplasia in 3 cases,endometrial carcinoma in 1 case.The non-enhanced cystic areas of two groups of tumors showed lower signal on T1WI and high signal on T2WI. Conclusion There are different characteristics between ovarian thecoma-fibroma and adult-type OGCT on MRI findings,which are helpful to the differential diagnosis.

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.

CT and MRI findings of ovarian granulosa cell tumor / 实用放射学杂志

Objective To study the CT and MRI features of ovarian granulosa cell tumor(OGCT).Methods The clinical and imaging data of 12 cases with OGCT proved by pathology were analyzed retrospectively(10 cases were performed with CT,5 cases were performed with MRI,and 3 cases were performed with both CT and MRI).Results All lesions were solitary.The tumors presented round,oval, or lobulated with clear boundary.In 12 cases, 3 cases were multiple cystic masses with uneven thickness of cyst walls and septation.Mild enhancement of the cyst walls and septation was showed after contrast enhancement.7 cases were cystic and solid masses with multiple cysts, typically with sponge-like pattern,and most cases showed slightly to moderately enhancement.2 cases were solid mass with septation,1 case showed significant enhancement,and 1 case showed slightly enhancement.6 of 12 cases presented enlargement of the utreus and endometrium thickening,1 case was associated with endometrial carcinoma.Conclusion OGCT has characteristic CT and MRI features, and CT and MRI examinations combined with estrogen abnormality are helpful for diagnosis.

Clinicopathological study of 24 cases of ovarian cellular fibromas / 中国肿瘤临床

Objective:To investigate the clinical and pathological characteristics of ovarian cellular fibromas.Methods:The sample con-sisted of 24 cases of ovarian cellular fibromas from February 2008 to March 2017 in the Obstetrics and Gynecology Hospital of Fudan University.Clinical histories were retrieved,and pathological slides were reviewed.Results:The age of the patients ranged from 17 to 70 years old,with a mean age of 46.5 years.Clinical symptoms included ovarian masses,abdominal pain,or pleuroperitoneal fluid.Se-rum CA125 notably increased in two patients.Of the 24 cases,13 and 10 occurred in the right and left ovaries,respectively,and one case occurred bilaterally.Tumor cells were densely cellular in all cases and were mitotically active in three cases(5-7/10 high power fields).A minor component of sex cord elements(<10% area of the tumor)was present in three cases,and luteinized cells were ob-served in four cases.None of the cases manifested recurrence during follow-up ranging from 1 month to 109 months.Conclusion:Ovarian cellular fibromas are pure ovarian stromal tumors that may manifest mitotic activity, sex cord elements, and luteinization. Some patients present with pleuroperitoneal fluid and increased CA125.Thus,the pathological features of cellular fibromas must be mastered to avoid misdiagnosis for other benign or malignant tumors and improper treatment.

Clinicopathological study of 24 cases of ovarian cellular fibromas / 中国肿瘤临床

Objective:To investigate the clinical and pathological characteristics of ovarian cellular fibromas.Methods:The sample con-sisted of 24 cases of ovarian cellular fibromas from February 2008 to March 2017 in the Obstetrics and Gynecology Hospital of Fudan University.Clinical histories were retrieved,and pathological slides were reviewed.Results:The age of the patients ranged from 17 to 70 years old,with a mean age of 46.5 years.Clinical symptoms included ovarian masses,abdominal pain,or pleuroperitoneal fluid.Se-rum CA125 notably increased in two patients.Of the 24 cases,13 and 10 occurred in the right and left ovaries,respectively,and one case occurred bilaterally.Tumor cells were densely cellular in all cases and were mitotically active in three cases(5-7/10 high power fields).A minor component of sex cord elements(<10% area of the tumor)was present in three cases,and luteinized cells were ob-served in four cases.None of the cases manifested recurrence during follow-up ranging from 1 month to 109 months.Conclusion:Ovarian cellular fibromas are pure ovarian stromal tumors that may manifest mitotic activity, sex cord elements, and luteinization. Some patients present with pleuroperitoneal fluid and increased CA125.Thus,the pathological features of cellular fibromas must be mastered to avoid misdiagnosis for other benign or malignant tumors and improper treatment.

Adult granulosa cell tumors of the ovary: a retrospective study of 30 cases with respect to the expression of steroid synthesis enzymes / 부인종양

OBJECTIVE: Some, but not all, granulosa cell tumors are characterized by estrogen production. This study was designed to determine whether there are clinical or pathological variations in granulosa cell tumors in relation to the expression of sex steroid synthesis enzymes. METHODS: Clinical symptoms, serum hormonal values, and histology of 30 granulosa cell tumor patients who underwent surgery between 2002 and 2014 were retrospectively reviewed. RESULTS: Most patients presented with abnormal genital bleeding including abnormal menstrual cycles. Eight of 16 patients older than 50 years had endometrial hyperplasia and one had endometrial cancer. Serum 17β-estradiol (E2) levels tended to be higher in patients over 50 years of age (p=0.081). Serum follicle-stimulating hormone (FSH) levels were low in all patients irrespective of serum E2 levels. Magnetic resonance imaging revealed a thicker endometrium in older as compared to younger patients (p<0.05). Tumor cells in the majority of cases were positive for inhibin α and P450 aromatase, irrespective of age and serum E2 levels. P450 17α-hydroxylase (P450c17) expression varied among cases. P450c17 was strongly positive in luteinized tumor cells and weakly positive in theca cells and fibroblasts. High E2 levels were associated with P450c17-positive cells in the tumor (p<0.05). CONCLUSION: The expression of hormone-synthesizing enzymes divides granulosa cell tumors into 2 distinct types; tumors with P450c17-positive cells show elevated serum E2 and related clinical symptoms, while tumors without these cells show symptoms related to FSH suppression by inhibin.

Surgical Staging and Recurrence Management of Ovarian Granulosa Cell Tumor.

Introduction: Ovarian Granulosa Cell Tumors (GCT) is a potentially malignant tumor. Accurate diagnosis requires histological and immunohistochemical study. The treatment of choice is surgical staging and debulking staging according to International Federation of Gynecology and Obstetrics (FIGO) criteria, preferred by laparoscopy. The therapeutic role of adjuvant therapy is unclear. Relapses arefrequent, usually late and insidious. Case Report: we report two cases of Ovarian Granulosa Cell Tumor. First case, a laparoscopic oophorectomy for complex adnexal cyst, intraoperative study does not differentiate between Granulosa Cell Tumor or clear cell carcinoma of the ovary, so we performed a complete staging surgery by laparoscopy. Second case aparaortic recurrence twenty years after first surgery. Non-steroidal anti-androgen therapy was useful to control progression of the disease, finally laparoscopic excision of the lesion. Conclusion: A complete surgery in the management of Granulosa Cell Tumor is recommended. Relapses can occur very late and response to chemotherapy is poor, so it is important to perform a careful initial staging or debulkingprocedure because intraoperative pathological diagnosis is difficult and confusing.

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.

Insights into granulosa cell tumors using spontaneous or genetically engineered mouse models / 대한생식의학회지

Granulosa cell tumors (GCTs) are rare sex cord-stromal tumors that have been studied for decades. However, their infrequency has delayed efforts to research their etiology. Recently, mutations in human GCTs have been discovered, which has led to further research aimed at determining the molecular mechanisms underlying the disease. Mouse models have been important tools for studying GCTs, and have provided means to develop and improve diagnostics and therapeutics. Thus far, several genetically modified mouse models, along with one spontaneous mouse model, have been reported. This review summarizes the phenotypes of these mouse models and their applicability in elucidating the mechanisms of granulosa cell tumor development.

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.