Ollier disease (enchondromatosis) associated with ovarian juvenile granulosa cell tumor and precocious pseudopuberty

Granulose cell tumors represent 2% of ovarian tumors. They are classified as adult type and juvenile type. The juvenile type is associated with precocious pseudopuberty, irregular menstruation or nonspecific symptoms such as abdominal pain and palpable mass. More than 95% are confined to the ovary. A case of a 31-year-old patient is described. The patient consulted for 6-month evolution of increased abdominal volume and weight loss. Right ovary tumor was diagnosed. Tumor excision by gynecological laparotomy with positive frozen cut biopsy and surgical staging were performed. Pathological anatomy reported stromal tumor, and sexual cords suggestive of malignant juvenile granulose cell tumor. Immunohistochemistry reported positive inhibin B. It was concluded as stage IIIA2. This is a rare neoplasm with variable behavior. Accurate diagnosis is based on histological and immunohistochemical studies. Key words: Granulosa, Cell tumor, Juvenile granulosa cell tumor, Ascites, Diagnosis.

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A rare case of ovarian juvenile granulosa cell tumor without precocious pseudopuberty in an 11-month-old infant

Journal of Pediatric Surgery ◽

10.1016/j.jpedsurg.2011.10.061 ◽

2012 ◽

Vol 47 (1) ◽

pp. 269-270 ◽

Cited By ~ 1

Author(s):

Mohamed Moncef Hamdane ◽

Rym Dhouib ◽

Lamia Charfi ◽

Raoudha Doghri ◽

Karima Mrad ◽

...

Keyword(s):

Granulosa Cell ◽

Rare Case ◽

Granulosa Cell Tumor ◽

Cell Tumor ◽

Juvenile Granulosa Cell Tumor ◽

Precocious Pseudopuberty

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Juvenile granulosa cell tumor of the ovary in children: a clinical study of 15 cases.

Journal of Clinical Oncology ◽

10.1200/jco.1988.6.6.990 ◽

1988 ◽

Vol 6 (6) ◽

pp. 990-995 ◽

Cited By ~ 65

Author(s):

G Vassal ◽

F Flamant ◽

J M Caillaud ◽

F Demeocq ◽

C Nihoul-Fekete ◽

...

Keyword(s):

Granulosa Cell ◽

Recurrent Disease ◽

Stage Iv ◽

Granulosa Cell Tumor ◽

Cell Tumor ◽

Pediatric Department ◽

One Stage ◽

Juvenile Granulosa Cell Tumor ◽

Precocious Pseudopuberty

Juvenile granulosa cell tumor (JGCT) in children accounted for 12% of all ovarian tumors treated in the Institut Gustave-Roussy (IGR) Pediatric Department from 1967 to 1985. The median age of the 15 girls was 8 years 7 months (range, 22 months to 15 years 7 months). Precocious pseudopuberty was present in six of the seven girls under 8 years. Of the other seven girls, one developed virilization symptoms. Surgery was the first treatment in each case. According to the Wollner classification, there were six stage I, one stage II, six stage III (including four ruptured tumors), and one stage IV JGCT cases. One patient was not available for staging. An adjuvant treatment (five chemotherapy and one radiotherapy combined with chemotherapy) was administered to six patients. Eleven girls are alive and free of disease, with a median follow-up of 6 years (range, 2 to 18 years). Four girls relapsed 6 to 17 months after surgery and died. Two of these relapses occurred in bone. The prognosis for JGCT in children is favorable for the lower stages when treated with surgery, but the best treatment for extensive and recurrent disease has yet to be determined.

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Juvenile granulosa cell tumor

10.1055/s-0039-1685332 ◽

2016 ◽

Author(s):

Geetanjali Tuteja ◽

S. Unmesh ◽

S. Shree ◽

S. Rudra ◽

Keyword(s):

Granulosa Cell ◽

Precocious Puberty ◽

Early Stage ◽

Pubertal Development ◽

Granulosa Cell Tumor ◽

Cell Tumor ◽

Cell Tumour ◽

Tumour Resection ◽

Granulosa Cell Tumour ◽

Juvenile Granulosa Cell Tumor

The differential diagnosis for precocious puberty in a young female includes peripheral causes. This case report documents a rare cause of isosexual precocious puberty, a juvenile granulosa cell tumour of the ovary–and a brief literature review. A one year-old baby girl presented with mass abdomen, vaginal discharge and rapid onset of pubertal development. She underwent an exploratory laparotomy for tumour resection. Pathology reported a juvenile granulosa cell tumour of the ovary. Early stage granulosa cell tumor surgically treated has good prognosis. Adjuvant chemotherapy is not indicated in this setting.

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