Juvenile granulosa cell tumor presenting as isosexual precocious puberty: A case report and review of literature
A 7-year-old girl presented with 18 months history of progressive breast development. She had attained menarche 1 year back and had bled 3-4 times. There was no evidence of local trauma or sexual abuse. Mother noticed hair growth over pubic area for 3-4 months and accelerated growth for 6 months. There was no history of meningitis, cranial irradiation, head trauma, headache, vomiting, gelastic seizures, medication intake, hypothyroidism, or systemic illness. Antenatal, perinatal, and developmental history was unremarkable. Her height (125.2 cms) and weight (26 kilograms) were above the 75th centile for age.[] Vital signs were normal. She was Tanner stage IV for breast development and Tanner III for pubic hair. There were no bony deformities, café-au-lait spots, or signs of virilization. Systemic examination was normal. Her abdomen was soft, non-tender with no obvious mass palpable or ascites. She was investigated to determine the cause for precocity. She had normal blood counts, liver function tests, kidney function tests, and TSH level. Serum estradiol level was elevated (414 pg/ml, prepubertal <20 pg/ml) with a suppressed FSH level (<1 IU/L). At a chronological age of 7 years 2 months, her bone age was advanced to 8 ½ years. A pelvic ultrasonographic scan showed a well-defined heterogeneous solid mass lesion in the right adnexa measuring 6 × 7 × 9 cms. There were few hypoechoic areas suggestive of necrosis. There was increased perilesional and intralesional vascularity. Right ovary could not be seen separately from the mass. Left ovary was normal. Uterus, measured 6 × 2 × 2 cms, was anteverted and had an endometrial thickness of 3.4 mm. Tumor markers including beta-HCG, CEA, and AFP were negative. Inhibin levels were not measured as testing is not easily available at our center. A diagnosis of isosexual precocity due to estrogen-secreting ovarian tumor was made. Elective excision of the mass was planned. But, a week later, she presented to the emergency department with abdominal pain necessitating emergency laparotomy. The right ovary was enlarged and twisted on its pedicle, and right salpingo oophorectomy was done. The resected tumor was encapsulated without any capsular breach, measured 450 grams and had 10 × 8 × 5 cms dimension. The left ovary was normal. There was no fluid in the cul-de-sac and no lymphadenopathy. Histologic examination revealed micro follicular and nodular patterns of tumor cells with round to oval nuclei, nuclear grooving, inconspicuous nucleoli, moderate cytoplasm, and extensive luteinisation. Fallopian tube was tumor-free. Ascitic fluid was negative for malignant cells. A final diagnosis of a benign feminizing JGCTO (FIGO Stage IA) was established.\nPostoperative course was uneventful, and her breast size regressed, menstrual bleeding stopped, and serum estradiol levels came down to normal. Even after 3 and half years, she has not developed any signs of recurrence.