Sclerosing stromal tumor of the ovary presenting as precocious puberty: Unusual presentation of the rare neoplasm Sclerosing stromal tumor (SST) is a rare sex cord stromal neoplasm of the ovary. Precocious puberty resulting from this tumor is extremely rare. We report a case of SST of the ovary in a 7-year-old Indian girl, who presented with the complaints of excessive development of breasts, early menarche, growth of pubic hairs, accelerated growth and abdominal distension. On clinical examination, a large rightsided abdomino-pelvic mass was palpable. Magnetic resonance imaging revealed a large, heterogeneous, solid and cystic tumor in the right adenexal region, suggestive of an ovarian neoplasm. Hormonal profi le showed markedly elevated estradiol and low follicle stimulating hormone levels. Clinical diagnosis of precocious puberty with right-sided ovarian mass was thus concluded. Right-sided salpingo-oophorectomy was then performed. Histopathology showed features consistent with SST of the ovary. Postoperatively, signs and symptoms of precocity gradually regressed and her serum estradiol level came down to normal.
Key words: Precocious, puberty, sclerosing stromal tumorCase Report
INTRODUCTIONSclerosing stromal tumor (SST) is a rare benign sex cord stromal neoplasm of the ovary, which usually occurs in the second and third decades of life.[1] Patients with this tumor often present with the complaints of pelvic mass, pain, abdominal distention, and menstrual irregularities. Anovulation, virilization and infertility may be present in these patients because SST is associated occasionally with estrogen and rarely androgen secretion.[2] Precocious puberty resulting from this tumor is extremely rare. [3,4] This case is being reported due to unique and unusual presentation of rare SST of the ovary. To the best of our knowledge, this is the fi rst reported case of SST resulting in precocious puberty from India.
CASE REPORTA 7-year-old Indian girl was presented by her mother to Gynecology Outpatient Department with the complaints of excessive development of her breasts for the last 1 year. She had attained menarche 8 months back. Her mother noticed growth of pubic hairs, accelerated body growth and abdominal distension for the last 4 months. Family history, antenatal, perinatal and developmental history was unremarkable. History of local trauma, sexual abuse, drug intake and any other systemic illness were absent. On clinical examination, a right-sided, large abdomino-pelvic mass was palpable. The mass was fi rm in consistency, immobile, measuring about 8 cm × 5 cm × 3 cm with well demarcated margins. Pelvic magnetic resonance imaging revealed a large, well-defi ned, heterogeneous solid and cystic mass measuring 8.5 cm × 5.5 cm × 4 cm in the right adenexal region [ Figure 1a]. Right ovary was not separately defi nable from the mass. The left ovary appeared normal. Patient's height was 127 cm and weight was 27 kg. She was Tanner stage IV for breast development and Tanner stage III for pubic hair. There were no signs of...