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.

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.

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.

Juvenile granulosa cell tumor of the ovary presenting as isosexual precocious puberty: A case report / Philippine Journal of Obstetrics and Gynecology

lsosexual precocious puberty is rare and a thorough investigation must be done in order to identify the cause of the precocity. This paper presents the case of a 4 year-old girl who was brought to the emergency room due to vaginal bleeding associated with onset of secondary sexual characteristics. Estradiol and anti-mullerian hormone levels were elevated. Abdominal ultrasound revealed an abdominopelvic mass probably an ovarian new growth with benign sonologic features. Computer tomography of the brain with contrast showed normal findings. Elective surgery was planned after correction of the anemia and other causes of precocious puberty were excluded. She underwent an exploratory laparotomy and left salpingooophorectomy with frozen section. Final histopathology report showed juvenile granulosa cell tumor of the left ovary.

Granulosa cell tumor of testis: Clinicopathological correlation of a rare tumor.

Background: Granulosa cell tumor of testis is a rare tumor accounting for less than 4% of adult testicular tumors though they account for nearly 30% of childhood testicular tumors. Due to the rarity of these tumors, exact etiology, pathogenesis, prognostic factors and best treatment approach are not well known. The molecular events in pathogenesis of these stromal tumors have begun to unravel and these developments put forth a reasonable and scientifi c explanation for the association of these tumors with developmental anomalies like undescended testis. However, many questions remain unanswered. Materials and Methods: We performed a retrospective analysis of clinicopathological features of all Granulosa Cell Tumors of testis from our archives in addition to an extensive literature search using PUBMED with the key words “Granulosa Cell Tumor, testis”. Results: We found six cases in our archives, two of which were of juvenile type and four of adult type. One out of these six cases presented with metastases. All cases underwent radical orchidectomy. Morphology and immunohistochemistry were classical in all cases and there was no diagnostic dilemma. Literature search revealed 63 cases of testicular Granulosa Cell Tumor in addition to highlighting the similarities in the biology and the dissimilarities in the clinical behavior as compared to ovarian Granulosa Cell Tumor. Conclusion: Testicular Granulosa Cell Tumor is a rare tumor, which although histologically similar to its ovarian counterpart, differs in clinical behavior. Further detailed investigations are needed to reveal the mystery behind the differing clinical behavior despite histological and immunohistochemical similarity between the testicular and ovarian Granulosa Cell Tumors.

Precocious Pseudopuberty due to Ovarian Causes.

Background: It is important to differentiate central from peripheral causes of precocious puberty because of distinct management options. Case Characteristics: 4 girls with discordant pubertal development. Observations: All had low basal and GnRHa stimulated FSH & LH level with high estradiol level. Abdominal ultrasonogram helped in diagnosing precocious pseudopuberty- ovarian cyst in 3 children and juvenile granulosa cell tumour in one. Outcome: Case 1 and 4 underwent surgery in view of persistent cyst and tumor, respectively. Rest were managed conservatively. Regression of pubertal signs observed in all children during follow-up. Conclusion: Precocious pseudopuberty can be differentiated from central precocious puberty by GnRHa Stimulation test, bone age and abdominal ultrasound.

Ovarian juvenile granulosa cell tumor: a clinicopathological study of 8 cases / 临床与实验病理学杂志

Purpose To investigate the clinicopathologic features, diagnosis and differential diagnosis of ovarian juvenile granulosa cell tumor (JGCT).Methods The history records, pathologic features and immunophenotype of 8 cases of JGCT were retrospectively evaluated and their prognosis was achieved by follow-up.Results The age of patients ranged from 6～21 years old,with an average age of 15.1 years.The main clinical manifestations included an abdominal mass, ascites and isosexual pseudoprecocity. Cut surface of the tumor was typically solid with cysts formed. The histopathological changes displayed solid nests, diffuse sheet, multiple round or ovoid follicles in variable size.Macrofollicles could be seen in some cases.The follicular pattern consisted of small cystic cavities containing eosinophilic secretions. The tumor cells were round or polygonal, medium in size. The tumor cells had abundant pale or slightly eosinophilic cytoplasm, round nuclei with fine chromatin. Nuclear grooves were inconspicuous.Mitosis figures could be found. Immunohistochemical results showed that the tumor cells expressed inhibin-α,CD99,vimentin; while Melan-A,calretinin and S-100 were positive staining in part of the cases.CKpan,EMA,PLAP,Syn and CgA were negative in all the cases.Conclusions Ovarian juvenile granulosa cell tumor is a rather rare, low malignant tumor with good prognosis. Its diagnosis depends on the histologic and immunohistochemical findings and clinical features. Its differential diagnosis includes adult granulose cell tumor, hypercalcaemic type small cell carcinoma, carcinoid and dysgerminoma.

Simultaneous occurrence of a granulosa cell tumor and a serous cystadenoma in the same ovary: A case report / 대한산부인과학회지

This is the first case report of a patient with a large serous cystadenoma accompanied by a juvenile granulosa cell tumor that was discovered in the remaining ovarian tissue. A 25-year-old female was presented with constipation and amenorrhea. Ultrasonography revealed a large cystic mass in the left ovary with a normal uterus. The remaining ovarian tissue seemed normal at first look after cystectomy, but showed abnormal consistency on palpation. The remaining ovarian tissue was removed and granulosa cell tumor was confirmed by pathologic examination. The patient has been followed up for 30 months without evidence of recurrence. We would like to emphasize the importance of inspection, and palpation of remaining ovarian tissue during operation to avoid risks of remaining ovarian pathology.

5 Cases of Granulosa Cell Tumors of the Ovary / 대한산부인과학회잡지

Sex cord stromal tumors (GCT) of the ovary compose just 5% of ovarian tumors. Most of them are granulosa cell tumors (GCT). There are two types of tumors, juvenile (JGCT) and adult type (AGCT), which have different clinical and histopathological features. JGCT represents only 5% of GCT. GCT is characterized by secretion of estrogen. Patients may present with vaginal bleeding in adult type, and sexual pseudoprecocity in juvenile type, as results of prolonged exposure to tumor-derived estrogen. Surgery is a principle of treatment and required for definite tissue diagnosis, staging, and tumor debulking. Survival of patients with GCT is generally excellent because most patients present with early stage disease. Because of the propensity of GCT to recur years after initial diagnosis, prolonged surveillance such as physical examination and serum tumor markers such as estradiol and inhibin is reasonable. We present 5 cases GCT, 4 AGCT and 1 JGCT, with brief review of literature.

A Case of Juvenile Granulosa Cell Tumor / 대한산부인과학회잡지

Granulosa cell tumors costitute 1% to 2% of all ovarian tumors and occur mostly in adult women. Juvenile granulosa cell tumor (JGCT) differs from the adult type in clinical and pathologic features as well as biological behavior. Usually associated with estrogen production, the tumor leads to endocrinologic symptoms such as isosexual precocity in premenarcheal girl and menstrual irregularities or amenorrhea in postpubertal woman. JGCT is diagnosed in early stage with favorable prognosis in most cases while in more advanced stage with grave outcome. The clinical stage at the time of diagnosis is considered the most important prognostic factor. If fertility is desired in the absence of contralateral or pelvic involvement, unilateral salpingo- oophorectomy is justifiable. We experienced a case of JGCT in 22 years old woman who presented with amenorrhea and low abdominal pain, and report this case with a brief review of literatures.

A Case of Juvenile Granulosa Cell Tumor of the Ovary / 대한산부인과학회잡지

Juvenile granulosa cell tumor is a well-known sex-cord stromal ovarian neoplasm different in appearance and behavior from the adult granulosa cell tumor and occurs most (80%) frequently in the first two decades. The prognosis of juvenile granulosa cell tumor in children is good in most cases, but correlated with stage, size, the presence of ruptures, the grade of nuclear atypia, the degree of mitotic activity. The surgery remains the principal line of treatment for low stage juvenile granulosa cell tumor in children, but for those patient with high stage juvenile granulosa cell tumor or recurrence, the best treatment has yet to be determined and tumor sensitivity to radiation therapy and chemotherapy is not clearly. We present a case of juvenile granulosa cell tumor with brief review of literatures.

Juvenile Granulosa Cell Tumor of Ovary in a Premenarcheal Girl / 대한산부인과학회잡지

Granulosa cell tumors are rare in children, and less than 5% of all cases occur before puberty. In premenarcheal girls, the juvenile type of this tumor usually elicits the signs of sexual precocity. We have recently experienced a case of juvenile granulosa cell tumor of the ovary presented with sexual precocity. It is different from the adult granulosa cell tumor with regard to clinical and pathological features as well as biological behaviour as previously described by Scully. Only the clinical stage at the time of diagnosis is considered to be related to the prognosis. About 90% are diagnosed in early stage with a favorable prognosis. More advanced stage have a poor clinical outcome.

A Case of Ruptured Juvenile Granulosa Cell Tumor / 대한산부인과학회잡지

Sex cord-stromal tumors of the ovary are the third most common types of neoplasms that develop in the ovary and account for about 5-8% of all ovarian malignancies. Juvenile granulosa cell tumor (JGCT) is one of the sex cord-stromal tumors of the ovary has distinct differences from adult granulosa cell tumor (AJCT) with regard to clinical and pathological features as well as biological behavior most frequently occuring in the first two decades of life. Usually Call-exner bodies are rare, and luteinization is frequent in JGCT. The tumor may be solid, cystic, or both. In premenarcheal girls, juvenile granulosa cell tumor usually (82%) elicits the signs of sexual precocity. The tumor should removed as soon as the diagnosis is established. Surgery is the best treatment choice for low stage juvenile granulosa cell tumor in children, but for those with high stage juvenile granulosa cell tumor or recurrent tumor, the best treatment and sensitivity of tumor to radiation therapy and chemotherapy have not yet been determined clearly. About 90% are diagnosed in early stage so, prognosis of juvenile granulosa cell tumor in children is good in most cases, but tumor with more advanced stage has worse clinical outcome correlated with its stage, presence of ruptures, grade of nuclear atypia, degree of mitotic activity. And the clinical stage at the time of diagnosis is considered most important prognostic factor. We experienced a case of ruptured juvenile granulosa cell tumor so, we present a case with brief review of literature.

Two Cases of Juvenile Granulosa Cell Tumor / 대한소아혈액종양학회지

Juvenile granulosa cell tumor (JGCT) is one of the rare sex cord stromal tumors of the ovary. The majority of JGCT are found in prepubertal girls and young women. Unlike adult granulosa cell tumor (AGCT), JGCT is characterized by high frequency of mitosis and early relapse after treatment. Patients with JGCT in FIGO stage Ia have an excellent outcome with an event-free survival of about 90% following surgical resection alone, while those in advanced stages have an unfavorable outcome. We report two cases of JGCT in stage Ic, who showed a quite different outcome. One was managed initially with surgical resection alone and died of subsequent relapse. Another was managed with multimodality treatments including surgical resection, adjuvant chemotherapy and radiotherapy, and didn't show any evidence of relapse during 16 months of follow-up period. Further studies to evaluate the beneficial effects of chemotherapy and/or radiotherapy are required in patients with JGCT in stage Ic.

Juvenile Granulosa Cell Tumor Arising in Undescended Testis: A case report

Juvenile granulosa cell tumor is rare but one of the common congenital testicular neoplasms. Although histological features are typical of its ovarian counterpart, testicular juvenile granulosa cell tumor has a distinctly different clinical presentation. We report a case of juvenile granulosa cell tumor arising in the cryptochid testis of a 4-day-old newborn. A 6 5 5 cm sized multilocular cyst containing thick, mucinous fluid was found in the peritoneal cavity. The external surface of the cyst was smooth and the septae were relatively thin. The cyst consisted of numerous mucin-filled, cystic follicles lined by cells having vacuolated cytoplasm and round to oval dark nuclei without grooves. Cells resembling granulosa cells of an ovarian follicle were also observed in the intervening stroma forming irregular solid nests.

A Case of Juvenile Granulosa Cell Tumor of the Ovary

Juvenile granulosa cell tumor (JGCT) is one of the sex cord stromal tumors of the ovary ocurring in the first two decades of life. These tumors are different from adult granulosa cell tumor (AJCT) with regard to clinical and pathological fetures. Follicles are often irregular, Call-exner bodies are rare, and luteinization is frequent. The tumor may be solid, cystic, or both. The most common presenting symptoms are abnormal uterine bleeding and pain. Breast swelling, pain and tenderness may also be associated with unopposed estrogen secretion by granulosa cell tumors. The tumor should be removed as soon as the diagnosis is estabilished. The juvenile granulosa cell tumor has a good overall prognosis because fewer than 5% of these tumors in children are malignant.

Juvenile Granulosa Cell Tumor of the Ovary: Report of a Case of Malignant Form with Unusual Pleomorphism

A case of Juvenile Granulosa Cell Tumor (JGCT) of the ovary with unusual pleomorphic histologic and malignant biologic behaviour is described. The tumor occurred in a 10-year-old girl and was associated with clinical features of isosexual pseudoprecosity and a marked elevation of serum estradiol. The mass manifested initially in the right ovary and subsequently involved the contralateral ovary. A multi-organ metastasis developed during a 6-month-interval despite chemotherapy. She received two operations at 6-month interval, and tissues were obtained from the tumor mass. A marked histologic difference was observed between these two samples. The second biopsy showed profound cellular pleomorphism with numerous multinucleated tumor giant cell formation and hyaline bodies. The differential diagnosis from germ cell tumor and the possible factors for the pleomorphism are discussed.