Thecoma

Abstract: 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.

Abstract: 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.

Abstract: The clinical and pathologic findings of 203 patients with granulosa-theca tumors were analyzed to determine the features related to subsequent behavior of the tumor. The neoplasms were divided into three groups—granulosa tumors, mixed granulosa-theca tumors and thecomas. The actuarial survival rates of patients with granulosa tumors and mixed granulosa-theca tumors were nearly identical—both 97% at 5 years and 93% at 10 years. Only one of the 106 patients with a thecoma died of tumor, the other lesions being clinically and histologically benign. Capsular invasion and lymphatic invasion by the neoplasm were features associated with persistence of the tumor but the type of treatment, the degree of cellular atypism and mitotic activity were not. Unilateral oophorectomy is adequate therapy for granulosa-theca tumors confined to the ovary if biopsy and frozen-section examination are done on any suspicious lesion of the opposite ovary or other pelvic structure.

Abstract: Six cases of a distinctive ovarian lesion associated with sclerosing peritonitis were found in patients 13 to 76 years of age. Five patients presented with abdominal swelling; on examination, three were found to have unilateral or bilateral pelvic masses, one had ascites and evidence of small bowel obstruction, and one had ascites alone. The sixth patient presented with small bowel obstruction. At operation, 1 to 9 L of ascitic fluid were found in five patients. Five patients with bilateral ovarian enlargement underwent bilateral oophorectomy or, in one case, bilateral ovarian wedge resection. The sixth patient had unilateral ovarian enlargement treated by unilateral oophorectomy. All had fibrotic thickening of the peritoneum, most prominently involving the omentum and small bowel, four at initial operation and two at a second operation performed shortly thereafter. One or more episodes of small bowel obstruction subsequently occurred in three of the five patients with follow-up. One woman died of pulmonary embolism 2 months after her initial operation; four were alive without clinical evidence of disease 8 months to 6 years postoperatively. Gross examination revealed unilateral (one case) or bilateral (two cases), predominantly solid ovarian tumors 12 to 31 cm in maximum dimension in three cases and slight enlargement and nodularity of both ovaries in the other three cases. Microscopic examination of the ovaries revealed a cellular proliferation of spindle cells with focal differentiation into nests of luteinized or partially luteinized cells; one case also had occasional foci of sex cord elements. The process was confined to the ovarian cortex in the three cases with slight ovarian enlargement. Residual ovarian follicles were entrapped by the proliferation in three cases, but were prominent in only one of them. Additional features included brisk mitotic activity, predominantly in the spindle cells, in three cases, and striking edema in the three cases with significant ovarian enlargement. The ovarian lesions were interpreted as luteinized thecomas, although a closely related hyperplasia with luteinization, instead of early neoplasia, cannot be excluded in the three cases with only slight ovarian enlargement (and in one, prominent entrapment of follicles). The peritoneal process consisted of a variably cellular proliferation of fibroblasts and myofibroblasts separated by collagen, fibrin, and occasional chronic inflammatory cells. These cases and two similar cases from the literature suggest a unique association between some luteinized thecomas (or a closely related proliferative lesion of the ovary) and sclerosing peritonitis, although the relation between the ovarian and peritoneal processes is presently enigmatic.