A sclerosing stromal tumor of the ovary is an extremely rare benign tumor; it usually is found during the second and third decades of life. Patients present with pelvic pain or a palpable abdominal mass. Hormonal effects such as masculinization are uncommon. Here, an 11-year old premenarchal girl presented with deepening of the voice. In addition, clitoromegaly and hirsutism with a male suprapubic hair pattern were observed. The laboratory findings showed that the testosterone level was elevated to 3.67 ng/mL, andostenedione to above 10 ng/mL, dehydroepiandrosterone-sulfate to 346 µg/dL and 17-hydroxy progesterone (17-OHP) to 11.28 ng/mL. The chromosome evaluation revealed a 46,XX female karyotype. An adrenocorticotropic hormone stimulation test was performed. The 17-OHP to cortisol ratio in 30 minutes was 0.045, which suggested a heterozygote for the 21-hydroxylase deficiency. However, the
A sclerosing stromal tumor (SST) of the ovary is a rare benign subtype of a sex cord stromal tumor. Since its first description in 1973 by Chalvardjian and Scully
An 11-year-old premenarchal girl presented to the hospital with deepening of the voice and hirsutism, which was reported to have started about one year ago. Physical examination showed a male suprapubic hair pattern and 1 cm sized enlarged clitoris. The Tanner stage was II for breast and V for pubic hair development. The bone age was 13 years of age, advanced compared to the chronological age. The laboratory findings showed that the testosterone level was elevated to 3.67 ng/mL (normal range, 0.2 to 0.38 ng/mL), andostenedione to above 10 ng/mL (maximum level 1.7 ng/mL), dehydroepiandrosterone-sulfate (DHEA-S) to 346 µg/dL (normal range, 34 to 129 µg/dL) and 17-hydroxy progesterone (17-OHP) to 11.28 ng/mL (normal range, 0.2 to 2.65 ng/mL). The chromosome evaluation revealed a 46,XX female karyotype. The impression was non-classical congenital adrenal hyperplasia. An adrenocorticotropic hormone stimulation test (15 µg/kg bolus i.v.) was performed. The 17-OHP to cortisol ratio in 30 minutes was 0.045, which suggested a heterozygote for the 21-hydroxylase deficiency. However, the
The pelvic ultrasound showed an 8.5×5.9 cm mass in the left pelvic cavity. For further evaluation of the mass and adnexa, magnetic resonance imaging (MRI) was carried out and revealed an 8.9×6.2×6.6 cm well-defined mass of the left ovary (
Children and adolescents account for only 1% of all tumors in girls younger than 17 years, and sex cord stromal tumors constitute 10% of pediatric ovarian neoplasms
Most SSTs are hormonally inactive. Chalvardjian and Scully
The case reported here is a unique case of an SST presenting with masculinization in an 11 year-old premenarchal girl. The patient had Tanner II breast and Tanner V male suprapubic hair development. In addition, there were elevated hormonal levels of testosterone, andostenedione, and DHEA-S. After resection of the tumor, the hormone levels returned to the normal range and the hirsutism resolved. Three months after surgery, the patient had spontaneous menarche. Ultrasonography is a useful initial tool for differentiating between cystic and solid masses and determining the organ of origin. However, computed tomography and MRI are both more sensitive for delineating the nature of the mass and tumor extension. On MRI, a diagnosis of SST can be strongly suggested, when typical signal patterns such as hypointense nodules, hyperintense stroma, lobulation, strong enhancement with gadolinium and a peripheral hypointense rim are present
The differential diagnosis of SSTs of the ovary includes other sex cord stromal tumors such as fibromas and thecomas. These may be differentiated from the SST on the basis of histopathology and immunohistochemical findings
The SST presented here was atypical as it showed virilization in a premenarchal girl. Additional cases, especially those studied hormonally, may help detailed understanding for clinical manifestations of SST of the ovary.
Magnetic resonance imaging of a large ovarian tumor (arrows). A) Axial T2-weighted image shows about a 9×6 cm well-circumscribed hypointense mass in the pelvic cavity, arised from left ovary. B) Axial Gd-enhanced T1-weighted image demonstrates strong contrast enhancement of the tumor.
Histologic findings of sclerosing stromal tumor. A) The tumor shows an alternative pseudolobular pattern constiting of cellular and hypocellular areas (H&E, ×100). B) The cellular area is composed of vacuolated cells and spindled fibroblast-like cells (H&E, ×400). Immunohistochemically, the tumor cells are positive for smooth muscle actin (C, ×200) and α-inhibin (D, ×200).