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

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          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.

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          Calretinin, a more sensitive but less specific marker than alpha-inhibin for ovarian sex cord-stromal neoplasms: an immunohistochemical study of 215 cases.

          Although inhibin has been shown to be a sensitive marker for ovarian sex cord-stromal and fibrous neoplasms, it may be negative in some cases. Calretinin, a mesothelial marker, has shown promise as a marker for sex cord-stromal neoplasms. The aim of this study was to evaluate and compare calretinin and inhibin as immunohistochemical markers for sex cord-stromal and fibrous neoplasms. A total of 215 ovarian neoplasms were immunostained with commercially available antibodies to calretinin and inhibin. These tumors included 87 sex cord-stromal (39 granulosa cell, 13 Sertoli-Leydig, 4 Sertoli, 9 thecomas, 14 fibrothecomas, and 8 other stromal tumors), 37 fibrous (20 fibromas, 9 adenofibromas, and 8 fibrosarcomas), 65 epithelial, 22 germ cell, and 4 miscellaneous neoplasms. The staining was evaluated on a 0-4 scale based on percentage of neoplastic cells labeling: 0 = none; 1+ = 1-25%; 2+ = 26-50%; 3+ = 51-75%; 4+ = 76-100%. Calretinin reactivity was detected in 100% of sex cord-stromal and 90% of fibrous neoplasms, including 32 that were inhibin negative (2 granulosa cell tumors, 1 Sertoli-Leydig cell tumor, 1 thecoma, 3 fibrothecomas, 16 fibromas, 6 adenofibromas, and 3 fibrosarcomas). All four calretinin-negative fibrous neoplasms were inhibin negative. Calretinin staining was also detected in 22% of epithelial neoplasms but none of the germ cell and miscellaneous neoplasms tested. Inhibin staining was detected in 92% of sex cord-stromal neoplasms, 22% of fibrous neoplasms, 2% of epithelial neoplasms, and none of the germ cell and miscellaneous neoplasms tested. Calretinin has a 97% sensitivity and 85% specificity for sex cord-stromal and fibrous neoplasms, whereas inhibin has a 71% sensitivity and 99% specificity. This study shows that both calretinin and inhibin are useful in the diagnosis of ovarian sex cord-stromal and fibrous neoplasms. Calretinin is a more sensitive but less specific immunohistochemical marker than inhibin. Calretinin is particularly useful in the diagnosis of sex cord-stromal and fibrous neoplasms that are inhibin negative. The high frequency of calretinin in fibrous neoplasms suggests that a subgroup of these neoplasms may be derived from specialized gonadal stromal cells, perhaps thecal cells.
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            Ovarian stromal tumors containing lutein or Leydig cells (luteinized thecomas and stromal Leydig cell tumors)--a clinicopathological analysis of fifty cases.

            Fifty ovarian stromal tumors that had a predominant pattern of fibroma or thecoma but also contained cells typical of steroid hormone-secreting cells are reported. Forty-six tumors were classified as luteinized thecomas because the steroid cells resembled lutein cells and lacked crystalloids of Reinke. Four were classified as stromal Leydig cell tumors because crystalloids were identified in the steroid cells. The luteinized thecomas occurred at an average age of 46 years and were associated with estrogenic manifestations in 50% and androgenic changes in 11% of the cases. In the remaining cases there was no clinical or pathological evidence of steroid hormone production at the time of diagnosis. Six patients, two of whom were virilized, were pregnant. Four tumors appeared malignant on histologic examination. One of these tumors was rapidly fatal, the outcome is unknown in a second case, the third patient is alive and well at 5 years, and the fourth tumor was diagnosed too recently for evaluation of its behavior. The stromal Leydig cell tumors occurred at an average age of 61 years and were associated with virilization in one case, endometrial hyperplasia in another case, and endometrial hyperplasia with carcinoma in a third case. The fourth tumor was unassociated with endocrine manifestations. Luteinized thecomas and stromal Leydig cell tumors are indistinguishable except for the presence of crystalloids of Reinke in the latter. In view of the prolonged search that is necessary to find these structures in some stromal Leydig cell tumors and their well-known absence in the majority of testicular Leydig cell tumors, it is reasonable to assume that an unknown proportion of tumors in the luteinized thecoma category are unrecognized stromal Leydig cell tumors.
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              Fibrosarcoma of the ovary arising in a fibrothecomatous tumor with minor sex cord elements. A Case report and review of the literature.

              We report the case of a 69-year-old woman who presented with postmenopausal bleeding. Endometrial curettings showed complex atypical hyperplasia with focal well-differentiated adenocarcinoma. A computed tomographic scan of the abdomen revealed a right ovarian mass. Histologically, the right ovarian tumor was a fibrothecoma with minor sex cord elements showing focal fibrosarcomatous change. Fibrosarcoma of the ovary is a rare tumor that is considered to arise de novo or secondary to benign fibromatous tumors. Fibrothecoma of the ovary with minor sex cord elements is also a rare entity. To the best of our knowledge, this is the first reported case of a fibrosarcoma arising in a fibrothecoma with minor sex cord elements.

                Author and article information

                Pediatr Rep
                Pediatr Rep
                Pediatric Reports
                PAGEPress Publications, Pavia, Italy
                12 August 2014
                12 August 2014
                : 6
                : 3
                [1 ]Department of Pathology, Deen Dayal Upadhyay Hospital , Hari Nagar, New Delhi
                [2 ]Department of Pathology, Saraswathi Institute of Medical Sciences , Anwarpur, Hapur, UP
                [3 ]Department of Obstetric And Gynecology, Deen Dayal Upadhyay Hospital , Hari Nagar, New Delhi, India
                Author notes
                Department of Pathology, Saraswathi Institute of Medical Sciences, Anwarpur, Hapur, UP, India. +91.996.852.6588. nigamjs@

                Contributions: the authors contributed equally.

                Conflict of interests: the authors declare no potential conflict of interests.

                ©Copyright N. Sood et al.

                This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License ( which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

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                Figures: 2, Tables: 0, Equations: 0, References: 11, Pages: 3
                Case Report


                calretenin, inhibin, immunohistochemistry, estrogenic


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