Study of an Ovarian Sclerosing Stromal Tumor Presenting as Vaginal Bleeding in a 7-Month-Old

Abstract

This communication describes the histological, immunohistochemical, ultrastructural, and cytogenetic study of an ovarian sclerosing stromal tumor resected from a 7-month-old girl who presented with vaginal bleeding. The tumor is very rare, its pathogenesis is not clear, and its hormonal activity has been subject to debate. In addition, it has been rarely seen in children and never in infants, with the youngest patient reported being 10 years of age. Histological study of the tumor showed a process of multinodular asynchronous growth followed by gradual loss of cells, hyalinization, and eventual transformation into corpora albicantia-like structures, thus indicating that the process may be more akin to an ovarian nodular follicular hyperplasia than to a classical neoplasm. The study also documented an elevated proliferative MIB-1 index in the process, which had not been investigated in earlier reports, and illustrated the immunohistochemical reactivity of some of its stromal cells to progesterone receptors.

A 10-Month-Old Infant Presenting With Signs of Precocious Puberty Secondary to a Sclerosing Stromal Tumor of the Ovary in the Absence of Hormonal Elevation

“…A previous study showed that SST might undergo a process of multinodular asynchronous growth followed by gradual loss of cells, hyalinization, and eventual transformation into corpora albicantia-like structures. 11 The histology of this tumor supports the aforementioned observation. It appears that this child’s tumor was at the end stage of the process with mainly corpora albicantia-like structures and scattered degenerative vacuolated cells.…”

On the Pathogenesis of Sclerosing Stromal Tumor of the Ovary

“…Consistent numerical chromosomal aberrations were described in 21 of 29 ovarian tumors in the thecoma-fibroma group, and 71.5% of the cases with chromosomal abnormalities had trisomy 12 (11). Trisomy 12 was found in 4 of 5 cases of SST studied indicating a close relationship to other ovarian stromal tumors (12)(13)(14). Likewise, trisomy 12 has been reported in 5 of 6 cases of ovarian JGCT examined (15,16); however, monosomy 22 has been found in adult-type granulosa cell tumor (17,18).…”

Laparoscopic Management of Sclerosing Stromal Tumors of the Ovary Combined with Ectopic Pregnancy

“…These tumors occur most frequently in the second and third decades of life; patients aged <30 years account for 78.8% of all cases. Since it was first described by Chalvardjian and Scully [3], around 200 cases have been reported [4,5,6,7,8]. SSTO occurs with similar frequency in the left and right ovaries; two bilateral cases have been reported [6].…”

“…For instance, a low-grade malignant SSTO has been reported in Portugal [5]. The youngest reported patient with an SSTO has been a 7-month-old girl with vaginal bleeding [6]. Another case report has described a huge SSTO weighing 10 kg in a 71-year-old postmenopausal woman [7].…”