Sclerosing stromal tumor of the ovary associated with Meigs' syndrome: a case report

Sclerosing stromal tumors of the ovary are distinct, but rare benign neoplasms. These tumors appear solid and are very vascular giving the impression of malignant tumors. They occur mostly in young women. Morphologically they have distinct characteristics which differentiate them from other stromal tumors. Benign ovarian tumors associated with Meigs' syndrome are rare. In this article a case of ovarian sclerosing stromal tumor associated with Meigs' syndrome in a 17-year-old women is described and the differential diagnosis is also discussed.     

Lack of expression of c-kit protein (CD117) in mesenchymal tumors of the uterus and ovary.

c-kit is a proto-oncogene that codes for a transmembrane tyrosine kinase receptor (CD117). The gene product KIT is constitutively overexpressed in mastocytosis and gastrointestinal stromal tumors. Recently the use of the tyrosine kinase inhibitors, such as STI-571, has resulted in the successful treatment of bcr-abl-positive leukemias and gastrointestinal stromal tumors. In gastrointestinal stromal tumors, immunostaining for c-kit is diffusely positive. Because the expression of c-kit in mesenchymal tumors of the uterus and ovary has not been previously studied, we evaluated its expression in 38 of these tumors by immunohistochemistry. The number of positive labeled/total tumors were as follows: 0/8 malignant mullerian mixed tumors, 4/7 ovarian fibrosarcomas, 0/1 clear-cell ovarian sarcoma, 0/4 uterine leiomyosarcomas, 1/10 low-grade endometrial stromal sarcomas, 0/2 high-grade endometrial stromal sarcomas, and 3/6 endometrial stromal nodules. In all positive cases, no more than 5% of the cells were labeled. In conclusion, unlike gastrointestinal stromal tumors, mesenchymal tumors of the uterus and ovary rarely express c-kit. Therefore, it is unlikely that patients with these tumors will benefit from treatment with the currently available tyrosine kinase inhibitors.     

Bilateral ovarian stromal hyperplasia concealing a nonhilar, pure stromal-Leydig cell tumor. A case report.

BACKGROUND: Of ovarian stromal tumors containing Leydig cells, nonhilar, pure stromal-Leydig cell tumor is rare. CASE: An obese, diabetic, borderline hypertensive 41-year-old woman with a five-year history of oligomenorrhea and amenorrhea presented with complaints of masculinization. Physical examination revealed hirsutism and an enlarged clitoris. The only abnormal serum marker was elevated testosterone. At laparotomy both ovaries were enlarged and suspected to have bilateral stromal hyperthecosis. Histology revealed stromal hyperplasia along with a 1.5-cm, testosterone-producing pure stromal-Leydig cell tumor of the right ovary. CONCLUSION: Bilateral ovarian enlargement secondary to stromal hyperplasia in patients with masculinizing signs can conceal a small, unilateral pure stromal-Leydig cell tumor.     

Virilization in pregnancy due to a borderline mucinous ovarian tumor

Virilization in pregnancy due to borderline mucinous ovarian tumors is very rare. A case of a 28-year-old patient who was noted at 28 weeks' gestation to have marked virilization with raised serum androgens, ascites and a large complex right adnexal mass is presented. Delivery was carried out by cesarean section and at surgery a large tumor was noted in the right ovary. Histology revealed a borderline mucinous ovarian tumor with stromal luteinization, but there was no evidence of stromal invasion. Serum androgens returned to normal levels following surgery and the maternal virilization had resolved at the 6-week postnatal visit. Stromal changes in borderline mucinous ovarian tumors may result in virilization due to androgen production; surgical removal is associated with an excellent clinical outcome.     

Ovarian hemangioma presenting with hyperandrogenism and endometrial cancer: a case report

BACKGROUND: Hemangiomas are very rare tumors of the ovary. Here, we report a case of a mixed capillary and cavernous ovarian hemangioma and endometrial carcinoma presenting with postmenopausal bleeding, male pattern receding frontal hairline, and high serum androgen and estradiol levels. CASE: A 70-year-old White female underwent laparotomy for endometrial carcinoma. Intraoperative frozen-section examination of the uterus revealed a 3.5 x 3 cm, grade 1 endometrioid adenocarcinoma of the endometrium with more than 50% myometrial invasion. The left ovary contained a 1.5 x 1 x 1 cm, well-circumscribed hemorrhagic nodule on the cut surface. Final histopathological examination of the small nodule demonstrated multiple, enlarged, blood-filled vascular channels lined by a single layer of flattened regular endothelial cells with no atypical features. Vascular spaces within the tumor were of different sizes, ranging from small to large, and were separated by connective tissue. The surrounding ovarian stroma was hyperplastic and contained clusters of luteinized stromal cells. Microscopy of the right ovary showed minimal stromal proliferation and no luteinization of the ovarian stroma. CONCLUSION: This is the first case of an ovarian hemangioma synchronous with a well-differentiated endometrial carcinoma. Absence of estrogen and progesterone receptors in the endothelial cells of the hemangioma suggests that ovarian hemangiomas may occur independent of stimulation by estrogen and progesterone.     

Hormonaktive Ovarialtumoren

Hormone-producing tumors of the ovary are rare and account for less than 10% of ovarian tumors. Sex cord stromal tumors, notably granulosa cell tumors and Sertoli-Leydig cell tumors, are the most important histopathologic entities of these conditions. This article summarizes the most important information on treatment and diagnosis and prognosis of sex cord stromal tumors.     

Youngest Reported Patient Presenting with an Androgen Producing Sclerosing Stromal Ovarian Tumor

BackgroundSclerosing stromal tumors are extremely rare sex cord stromal tumors of the ovary, with approximately 100 cases reported since first described in 1973. These tumors present predominantly in the 2nd and 3rd decades of life, typically present with pelvic/abdominal pain and tenderness, mass, and/or abnormal menses, and with hormonal activity reported predominantly in postmenarchal females. Only 5 cases of these tumors have been reported in premenarchal girls, with age ranging from 7 months to 12 years. Only 2 demonstrated hormonal manifestations, with vaginal bleeding due to hyperestrogenism in the 7 month old, and virilization in an 11-year-old female.CaseWe report a 9-year-old female who was diagnosed with this ovarian tumor, and who presented with virilization.Summary and ConclusionThis report is remarkable as our patient not only was diagnosed with an ovarian tumor that is extremely rare in this age group but is the youngest reported patient with this tumor who presented with virilization.     

Ossifying luteinized thecoma of the ovary

A 51-year-old woman presented with lumbar backache leading to the preoperative diagnosis of a right solid adnexal mass with calcification on computed tomographic scan. Histological examination revealed a right ovarian luteinized thecoma characterized by extensive calcification and metaplastic ossification. Osteoblasts and osteoclasts surrounded the surface of the heterotopic bone. Haversian canals were occasionally identified in the bony trabeculae. Ossifying ovarian neoplasms are extremely rare and this case is the first to demonstrate the osseous metaplasia in ovarian luteinized thecoma. Received: 20 November 2001 / Accepted: 3 December 2001 Correspondence to N. Ohara     

Ovarian stromal luteoma in a patient with endometrial carcinoma

A 65-year-old patient with endometrial adenocarcinoma underwent hysterectomy. Pathological examination of the right ovary revealed a coexisting stromal luteoma. The possible relationship between the two neoplasms as well as the intriguing pathogenesis and controversial nomenclature of ovarian steroid cell tumors are discussed. The incidental discovery of a stromal luteoma in a total hysterectomy specimen removed for endometrial carcinoma raises interesting questions about the perceived innocuous nature of these rare lipid cell tumors.     

Sclerosing stromal ovarial tumor--a case report and review

Sclerosing stromal tumor (SST) is a rare benign ovarian neoplasm of stromal origin with less than 100 cases reported in the literature. Unlike the other stromal tumors, the comas and fibromas, which tend to occur in the fifth and sixth decades, sclerosing stromal tumors predominantly affect females in the second and third decades. Several unique histologic features including pseudolobulation, sclerosis and prominent vascularity of the tumor are clearly reflected at ultrasonography. We present a case of SST of the ovary in a 26-year-old female, and describe the ultrasound findings with pathologic correlation.     

Initial endometriosis showing direct morphologic evidence of metaplasia in the pathogenesis of ovarian endometriosis.

It is believed that ovarian endometriosis may be generated by a celomic metaplastic process from existing epithelium in the ovary. However, no morphologic evidence of metaplastic process has been described. In this study, we intended to identify the earliest morphologic changes of endometriosis within the ovary to examine if evidence of metaplasia exists. Included in this study were 110 ovarian endometriosis cases and 30 benign ovaries without endometriosis but with ovarian epithelial inclusions (OEIs). Among the 110 well-established ovarian endometriosis cases, 34 cases showed areas of initial endometriosis (IE), which is defined as lesions showing direct transitions from normal-looking ovarian tissue to areas of minimal formation of endometriosis and/or to areas of full-blown endometriosis. We further divided IE into two types: type I IE was present on the ovarian surface, which was associated with ovarian surface epithelia; type II was located within the ovarian cortex, which was associated with OEIs. Sections containing IE, OEIs, and well-formed endometriosis were subject to CD10 and aromatase immunostaining. In IE lesions, the number of CD10-positive cells were significantly higher than the number of that in OEIs, but lower than that of well-formed endometriosis areas (p < 0.05). Aromatase expression was detected in both epithelial and stromal components of the IE lesions, indicating that estrogen local production may be involved in this initial process of endometriosis. Microvessel density was higher in IE lesions than in areas of OEI (p < 0.05). Based on the morphologic characteristics of IE, we believe that IE represent a spectrum of the earliest morphologic changes of endometriosis identifiable by routine microscopy. The morphologic transitions from ovarian surface epithelium or OEI to IE lesions provide direct metaplastic evidence for the pathogenesis of ovarian endometriosis. This metaplastic process may not only involve the ovarian epithelial cells, but also stromal components. Local production of estrogen, probably in high-levels, may be related to the initial process of endometriosis, although detailed mechanisms remain to be clarified.     

Uterus-like ovarian mass presenting near menarche.

An eleven year-old girl presented with right lower quadrant abdominal pain near menarche that coincided with the onset of her most recent menstrual period. A smooth-lined cystic structure with a thick wall was discovered in the right adnexa at surgery that was adherent to a multicystic ovary and a dilated fallopian tube. Histologic examination determined the lesion to be a uterus-like ovarian mass. The patient had a history of a resected right pelvic kidney at four months of age. This case is believed to represent a congenital malformation of the urogenital system rather than smooth muscle metaplasia of ovarian stromal cells.     

Bilateral Brenner tumors of the ovary

Two cases have been presented in which there were Brenner tumors in each ovary.This makes an approximate total of 29 instances of bilateral Brenner tumors that have been reported.In our first case the bilateral Brenner tumors were similar in appearance and of ovarian stromal origin.In the second case there was apparently a mucinous cystadenoma and 2 separate Brenner tumors in the left ovary and 3 Brenner tumors in the right ovary. The word “apparently” is used because serial blocks of the ovaries were not obtained. In the left ovary there was an area in a Brenner tumor where a cystic cavity was lined in part by mucinous and in part by Brenner epithelium.     

Coexistence of brenner tumor and struma ovarii: case report

BACKGROUND: There has been controversy regarding the histogenesis of Brenner tumors. It is generally accepted that Brenner tumors are derived directly from ovarian surface epithelium, which undergoes metaplasia to form the typical urothelial-like components, whereas some investigators assume that Brenner tumors arise from immature germ cells. CASE: We describe a well-documented case of the coexistence of struma ovarii regarded as a form of teratoma and Brenner tumor in the same ovary. Immunohistologically, not only columnar cells of thyroid follicles, but also transitional cells of Brenner nests were positive for thyroglobulin. CONCLUSIONS: In the present case, Brenner tumors and thyroid elements coexisted and were positive for thyroglobulin. While there is strong evidence that pure Brenner tumors originate mostly from the ovarian surface, at least Brenner tumors associated with teratomatous elements may have a germ cell origin.     

Ovarian mucinous cystadenocarcinoma with virilization

BACKGROUND: Ovarian neoplasms, other than sex cord--stromal tumors, are rare causes of hyperandrogenism. Only two cases of primary mucinous carcinomas associated with virilization have been reported. CASE: A 50-year-old female was referred to our clinic with a large pelvic mass. On examination she had significant facial hirsutism, clitoromegaly, and male pattern pubic hair growth. Serum levels of testosterone and dihydroepiandrosterone sulfate were elevated. A 30-cm, multilocular, solid and cystic, left ovarian mass was resected. Histology revealed moderately to poorly differentiated mucinous cystadenocarcinoma. The ovarian stroma contained florid proliferation of luteinized cells. The right ovary showed cortical stromal hyperplasia. Abnormal hormone values normalized 10 days postoperatively. CONCLUSION: We report a rare case of mucinous cystadenocarcinoma with virilization, review the literature, and discuss the mechanisms of hormone production by these tumors.     

Ovarian hemangioma associated with tamoxifen therapy: a case report

Vascular tumors of the ovary are very rare. We report a case of ovarian hemangioma in a patient treated with tamoxifen for breast ductal carcinoma. CD31 and CD34 immunoreactivity confirmed the vascular origin of the tumor. It is interesting to note that estrogen and progesterone receptors were negative in endothelial cells of the hemangioma, but were positive in stromal ovarian cells. Tamoxifen is a synthetic, non-steroidal, anti-estrogenic drug widely used as adjuvant therapy for pre- and post-menopausal, early and metastatic, breast cancer patients with positive estrogen receptor proteins. The mechanism of action of tamoxifen in stimulating the development and/or growth of ovarian hemangioma is unknown. We may speculate that its prolonged, estrogenic effect on the ovary may be one of the stimulating factor.     

Malignant mixed müllerian tumor of the fallopian tube coincident with a primary serous carcinoma of the ovary. Case report

Malignant mixed müllerian tumors are very rare neoplasms of the fallopian tube, and treatment is not well defined. A case of malignant mixed müllerian tumor of the tube concomitant with a primary serous carcinoma of the ovary is reported. It was unclear if there were two distinct neoplasms in the same patient, or if it was a single tumor with a sarcomatous fallopian conversion of the serous component, as described for some recurrent ovarian carcinomas. Chemotherapy for ovarian carcinoma with intraperitoneal metastasis was performed, with about a three-year interval-free period of disease, as could be expected for ovarian carcinomas at the same stage. Such coexistence of these two tumors does not afford adequate staging of the malignancy. Therapy for the very rare cases similar to the one reported here needs to be improved.     

Signet-ring stromal tumor of the ovary: clinicopathologic analysis and comparison with Krukenberg tumor.

Signet-ring stromal tumor is a rare ovarian neoplasm that can mimic Krukenberg tumor because of the presence of signet-ring cells in both tumors. The clinicopathologic features of three signet-ring stromal tumors, one of which has been previously reported, were analyzed and compared with 10 Krukenberg tumors. Patients with signet-ring stromal tumor ranged in age from 34 to 41 years (mean: 36.7 years). All signet-ring stromal tumors were unilateral and stage IA, whereas 60% and 40% of Krukenberg tumors were bilateral or associated with extraovarian tumor, respectively. The signet-ring stromal tumors were devoid of epithelial differentiation (glands, nests, cords), whereas all of the Krukenberg tumors contained these epithelial structures at least focally. In contrast to signet-ring stromal tumors, the signet-ring cells of Krukenberg tumors were positive for periodic acid-Schiff with diastase and cytokeratins but negative for vimentin. The patients with signet-ring stromal tumors were alive without disease at follow-up interval of 1 month to 17.4 years (mean: 7.4 years). In summary, signet-ring stromal tumor is a rare, benign, ovarian tumor that may be mistaken for Krukenberg tumor. Although the combination of operative and histopathologic findings allow their distinction, histochemical and immunohistochemical stains may also be useful.     

Differential expression of interleukins IL-13 and IL-15 in normal ovarian tissue and ovarian carcinomas

OBJECTIVE: To determine the temporal and spatial expression of interleukins (IL)-13 and IL-15 in ovarian carcinoma compared to normal ovarian tissue. METHODS: Quantitative RT-PCR, ELISA and immunohistochemistry. RESULTS: Q-RT-PCR, ELISA and immunohistochemical analysis indicates that IL-13 and IL-15 mRNA and protein are expressed in normal ovary at various phases of the menstrual cycle with immunoreactive proteins detected in granulosa/theca and luteal cells and to a lesser extent in stromal cells and surface epithelial cells. Compared to normal ovary, ovarian carcinoma expresses elevated levels of IL-13 and IL-15 mRNA, with higher IL-13 expression in primary vs. metastatic tumors. IL-13 and IL-15 protein expression was also higher in the tumor tissues compared to ascites. In normal ovary, ovarian tumors and ascites, the ratio of IL-13/IL-15 favored IL-13. Immunoreactive IL-13 and IL-15 proteins were localized primarily in the tumor cells and infiltrated inflammatory cells with increased intensity with disease stage. CONCLUSION: Normal ovary and ovarian tumors express IL-13 and IL-15 and pattern of their expression in carcinomas suggests that these cytokines may function in various ovarian cellular activities including inflammatory/immune responses that are integrated cellular events taking place in normal ovary and ovarian tumors.