Virilizing Ovarian Fibrothecoma with Minor Sex Cord Elements in a 13 Year Old Girl: A Rare Case

Abstract

Fibrothecoma accounts for 3-4% of all ovarian neoplasms; it is usually hormonally inactive, but can be estrogenic or sometimes androgenic (11%); it is rare under 30 years. In a very few cases, minor sex cord elements (less than 10% of the tumor area) are present; therefore, it is considered as a separate subgroup of stromal tumors. The importance of immunohistochemistry in recognizing this kind of tumor has been fully documented, with variable results on inhibin staining, but specific positivity for calretenin in such cases. We report here the rare case of a 13-year-old child with ovarian fibrothecoma and minor sex cord stromal elements, who showed negativity for inhibin and positivity for calretenin.

A Rare Benign Tumor and an Associated Malignancy: Case Report of Ovarian Fibroma with Minor Sex Cord Elements and Uterine Endometrioid Adenocarcinoma

“…In these two cases, the thecoma cells were identified by light microscopy. Androgenic manifestation of fibrothecoma with minor sex cord elements was also reported by Sood et al which was seen in a 13-year-old patient [4]. In all of these cases, the endocrine function was due to luteinized thecoma cells or Leydig cells.…”

“…But in some cases, the indifferent sex cord cells may be seen intermingling with the stromal component [1,2]. The sex cord elements will be immunoreactive to inhibin, calretinin, CD99, CD 56, antikeratin antibody KL1, and MIC, while negative for smooth muscle actin, cytokeratin, and epithelial membrane antigen [4].…”

Ovarian fibrothecoma with minor sex cord elements: a case report

“…Most of the tumours reported in literature showed estrogenic features but only 2 cases presented with signs of virilization (Sood et al, and Sharma et al,). 12,13 This case also showed estrogenic features with postmenopausal bleeding.…”

Extraovarian Fibroma With Minor Sex Cord Elements: A Case Report and Literature Review

“…To date, 17 cases have been reported in the English-language literature. 24-33 The major components of the tumor were fibroma (8 cases including the present case), fibrothecoma (3 cases), thecoma (1 case), Leydig cell tumor (3 cases), and others. The sex cord elements were composed of fully differentiated granulosa cells, undifferentiated sex cord–type cells, or tubular structures resembling immature Sertoli cells, and these elements occupied less than 10% of the total tumor area.…”

“…1-23 Furthermore, ovarian stromal tumor with minor sex cord elements is also a rare tumor that was first described by Young and Scully in 1982. 24-33 This tumor may pose a diagnostic dilemma on histological examination.…”