[Differential diagnosis of thecoma from ovarian fibroma based on cellular dimensions; function activity of thecomas; 11 case reports].
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Histologic and immunohistochemical evidence for considering ovarian myxoma as a variant of the thecoma-fibroma group of ovarian stromal tumors.
Ovarian myxomas recently have been reported as new, distinct pathologic entities that show a myxoid, moderately cellular proliferation of spindle and stellate cells interspersed with areas of fibrosis, hemorrhage, and delicate vascular spaces. These histologic features are frequently seen in the thecoma-fibroma group of ovarian stromal tumors. For this reason, we propose that ovarian myxomas are part of the spectrum of differentiation in thecomas-fibromas of the ovary. To provide histologic and immunohistochemical evidence for this proposal, four ovarian myxomas were compared with 48 primary ovarian stromal tumors in the thecoma-fibroma group from 46 patients. The thecoma-fibroma group of stromal tumors included 23 thecomas, 23 fibromas, and two sclerosing stromal tumors. We found significant (> 25% of histologic appearance) myxoid change in six thecomas and one sclerosing stromal tumor. This myxoid change resembled the histologic appearance of an ovarian myxoma. Immunohistochemical studies on paraffin-embedded material showed vimentin immunostaining in all tumors. Smooth-muscle actin was present in all of the myxomas, in two of the two sclerosing stromal tumors, and in 20 (90%) of the 23 thecomas, but it was present in only 11 (48%) of the 23 fibromas. Desmin staining was not present in any of the four ovarian myxomas or in the two sclerosing stromal tumors, and only three (13%) of the 23 thecomas showed focal staining for desmin. Nine (39%) of the 23 fibromas expressed desmin. S100 protein was expressed in one fibroma and one thecoma, weakly. None of the ovarian myxomas or the thecoma-fibroma group of stromal tumors expressed cytokeratins as detected by three different monoclonal antibody cocktails, ie, cytokeratin AE1/AE3, cytokeratin CAM 5.2, or cytokeratin MAK-6. The ovarian thecoma-fibroma group of stromal tumors form a histologic spectrum of lesions in which clear-cut distinguishing points between various entities are difficult to define. The myxoid change, present in the thecoma-fibroma group of tumors, was indistinguishable histologically and immunohistochemically from ovarian myxoma. For this reason, we propose that ovarian myxomas may be at one end of the spectrum of differentiation in the thecoma-fibroma group of tumors, in which no remaining stromal tumor is detectable.
Actins↗
[Clinical analysis of 74 cases with ovarian thecoma].
OBJECTIVE: To study the clinical pathologic characteristics, treatment and prognostic factors of ovarian thecoma. METHODS: From 1958 approximately 1998, a total of 74 patients with benign and malignant thecoma were retrospectively analyzed. RESULTS: The reviewed diagnosis were 8 patients with malignant thecoma, 66 patients were benign thecoma. Nine of 66 patients with benign thecoma appeared cell proliferative activity. The mean age was 52 years. The most frequent symptoms were abdominal-pelvic masses (56.8%), metromenorrhagia and abnormal vaginal bleeding, other symptoms were abdominal pain, infertility followed. Twenty-two of 66 patients had ascites, only 1 patient with ascites and hydrothorax simultaneously. Thirty-nine patients with diseases which associate with excessive estrogenic stimulation, including 1 endometrial adenocarcinoma and 1 endometrial dysplasia and other diseases such as: myoma; endometrial hyperplasia and polyp. Fifteen patients with benign thecoma were evaluated blood serum CA(125), the CA(125) were elevated in 9 of 15 patients. In 2 of 9 patients with cell proliferative activity, the tumors invaded adjacent tissues or relapsed, after received operation and pelvic radiotherapy, they are alive 11 years and 27 years respectively. Among 8 patients with malignant thecoma, 4 patients without postoperation therapy or with non-standard chemotherapy died in 2 years postoperation, the other 4 patients received operation, postoperational radiotherapy or (and) chemotherapy, 1 of 4 patients died of irradiation intestinal fistule 4 years later; two patients are alive without disease more than 10 years, another one for 2 years. CONCLUSIONS: Theca cell tumor of ovary have good prognosis, but we should pay attention to thecomas with proliferative activity. The prognosis of malignant thecomas is poor, postoperative systemic chemotherapy or radiotherapy to the patients with malignant thecomas can improve their survival.
Adolescent↗
Ultrastructure and immunohistochemical localization of estradiol in three thecomas.
Three ovarian thecomas were studied by ultrastructural and immunohistochemical techniques. In each tumor, a small number of tumor cells stained for estradiol. Vacuolated as well as plump non-vacuolated tumor cells were positive, but spindle-shaped cells were negative. Ultrastructural examination showed two principle cell types. Type I cells were immature mesenchymal cells that differed from typical steroid-secreting cells because only a minority had conspicuous smooth endoplasmic reticulum, although mitochondria often had tubular cristae. Type II cells were distinguished by abundant intermediate (10-nm) microfilaments and round mitochondria with incomplete cristae and empty centers. Focal smooth muscle differentiation was present in each tumor. Adherens-type intercellular junctions, including desmosomes in one case, and degenerate cells with markedly vesiculated cytoplasmic membrane systems were also present. Lipid was not abundant, but it was more conspicuous in degenerate cells and in type II cells. Thecoma cells thus closely resemble both ovarian stromal cells and theca interna cells, which are known to be capable of steroidogenesis. The localization of estradiol in a minority of tumor cells in each thecoma suggests that thecoma cells, too, are capable of steroid synthesis and supports the popular concept that hyperestrogenism in patients with thecomas may be the result of estradiol secretion by these tumors.
Adolescent↗
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.
Adolescent↗
A testosterone-secreting, gonadotropin-responsive pure thecoma and polycystic ovarian disease.
UNLABELLED: A 31-yr-old hirsute female with oligoamenorrhea since menarche had markedly elevated peripheral plasma testosterone (T) concentrations of 250-255 ng/100 ml (normal 20-60 ng/100 ml), which lacked a diurnal rhythm, were not suppressed by dexamethasone, were decreased by ACTH, and were massively increased to 2,530 ng/100 ml by human chorionic gonadotropin (hCG). The binding capacity of T-binding globulin (TeBG) was 0.2 mug/100 ml (normal = 1.1-3.3 mug/100 ml). Plasma delta 4-androstenedione (A) was elevated at 374-681 ng/100 ml (normal = 90-135 ng/100 ml). Plasma estrone (E1) and estradiol (E2) were normal. The endometrium was proliferative. A T-secreting tumor was suspected because the plasma T levels were higher than those observed in polycystic ovarian disease. Exploratory surgery revealed bilateral polycystic ovaries and a pure thecoma in the right ovary which was not visible on surface examination. The thecoma did not contain granulosa cells. Plasma T in the right ovarian vein, draining the tumor, was 28,200 ng/100 ml and in the left ovarian vein was 2,600 ng/100 ml. Plasma A was elevated in both ovarian veins: 11,170 ng/100 ml on the left and 8,360 ng/100 ml on the right. The thecoma contained 1.35 mug/g of T and only 0.014 mug/g and 0.007 mug/g of E2 and E1, respectively. Plasma A and T after bilateral oophorectomy and removal of the thecoma were normal at 184 ng/100 ml and 40 ng/100 ml, respectively. CONCLUSIONS: 1) This pure thecoma produced primarily T rather than E1 OR E2 and was gonadotropin-responsive. 2) A very high plasma androgen level in a female is an important clue to the presence of a tumor. A T-secreting tumor should be ssupected when the peripheral plasma T is over 250 ng/100 ml and when plasma T increases to over 1,000 ng/100 ml following hCG stimulation. 3) Tumors cannot be classified as estrogenic or androgenic on the basis of the character of the endometrium.
Adult↗
A review of granulosa cell tumours and thecomas of the ovary.
This review is a retrospective study of granulosa cell tumours and thecomas encountered in one hospital between 1970 and 1995. There were 16 granulosa cell tumours and 17 thecomas. The size of the granulosa cell tumours varied from 3 to 30 cms in diameter and no correlation was found between size and evidence of invasion. There was also no correlation between either mitotic counts or histological pattern and evidence of invasion. Evidence of oestrogen production was found in 11 of the sixteen granulosa cell tumours (2 with endometrial carcinoma and 9 with endometrial hyperplasia) and in 9 out the seventeen thecomas (2 with endometrial carcinoma and 7 with endometrial hyperplasia). Thecomas are regarded as benign tumours but granulosa cell tumours are characterised by a long natural history with a significant capacity to recur years after an apparent clinical cure. It is therefore important that patients with these tumours are followed up indefinitely.
Adult↗
Calcified thecomas in young women. A report of four cases.
Four young women, all of whom were less than 30 years of age and had a variety of menstrual irregularities, had ovarian thecomas that were remarkable because of the presence of extensive calcification. The calcification was visible on x-ray examination of the abdomen in one case and was appreciated grossly in all four. Microscopic examination revealed that the calcification was the most striking microscopic feature in all the cases; in one case it was psammomatous. One tumor also exhibited extensive adipose metaplasia. Only two other extensively calcified thecomas have been reported in detail in the literature; one of them also occurred in a young woman. Extensively calcified thecomas appear to occur in younger women more often than thecomas that are not calcified or minimally calcified, and enter into the differential diagnosis in a patient with adnexal calcification on x-ray examination of the abdomen.
Adolescent↗
Ovarian thecoma associated in the first trimester of pregnancy.
Ovarian thecoma is a relatively rare tumor which occurs before and after menopause. It is extremely rare that pregnancy is complicated with thecoma. Diagnosis of ovarian tumors during pregnancy is highly problematic due to difficulties in obtaining clinical manifestations, and treatment of these tumors poses an even greater challenge. Our patient was found to have estrogen-producing thecoma accompanied by accumulation of ascites in an early phase of pregnancy. The patient underwent abdominal surgery to remove the tumor on the 13th week of gestation. This resulted in disappearance of the ascites and a favorable clinical course. Diagnosis and treatment of ovarian thecoma occurring during pregnancy are discussed with relevant references.
Adult↗
Ovarian low-grade stromal sarcoma with thecomatous features: a critical reappraisal of the so-called "malignant thecoma".
A case of low-grade ovarian stromal sarcoma in a postmenopausal woman is described. Although pelvic recurrences of the tumor followed 5 and 7 years after the original surgery, the patient has remained well and without evidence of tumor 3 years since the last operation. Histopathologic, electronmicroscopic, and hormonal studies are described. There was evidence of estrogenic stimulation by the theca elements of the tumor in this patient. Cases previously reported in the world literature as malignant thecoma were analyzed, and most of them were considered inadequately documented; indeed most of them were probably either sarcomatoid granulosa cell tumors, stromal sarcomas, or fibrosarcomas. If a thecoma ever becomes malignant, the tumor cells dedifferentiate so that they cannot be recognized any longer as theca cells; instead, they proliferate as a stromal sarcoma or fibrosarcoma. It is proposed, therefore, that the term "malignant thecoma" not be used. On the other hand, very rare malignant ovarian stromal tumors do exist, consisting of undifferentiated stromal cells, fibroblasts, and theca cells, which can show evidence of hormonal activity.
Diagnosis, Differential↗
Luteinized thecoma with sclerosing peritonitis presenting as an acute abdomen.
The seventh reported case of luteinized thecoma with sclerosing peritonitis is described. Unlike the other reported patients with this condition, the patient presented with a short history of secondary amenorrhea, a subsequent acute abdomen, and an absence of abdominal distention and ascites. The tumor had undergone extensive hemorrhagic necrosis secondary to infarction, and the patient's amenorrhea may have been secondary to the release of steroid hormones from the necrotic cells. The presence of extensive necrosis and a brisk mitotic rate raised the suspicion of a malignant thecoma, but the finding of subserosal fibrosis in the biopsied omental tissue led to the diagnosis of a benign luteinized thecoma with sclerosing peritonitis.
Abdomen, Acute↗
Ovarian malignant luteinized thecoma--an unusual tumor in an adolescent.
Malignant luteinized thecoma is an extremely rare ovarian tumor. The fifth reported case of this tumor presenting in a 13-year-old epileptic child on anticonvulsant therapy with a rapidly enlarging pelvic-abdominal mass is discussed. At laparotomy, bilateral ovarian involvement, massive ascites, and omental and bowel serosal metastases were present. The pathological diagnosis was difficult with initial diagnoses of ovarian edema, endodermal sinus tumor, and ovarian fibrosarcoma. The final diagnosis of malignant luteinized thecoma was made by recognizing the presence of lutein cells with foamy vacuolated cytoplasm and intracytoplasmic staining for oil red O. Electron microscopy also demonstrated vacuolated cells with multiple fat globules. After surgical debulking the patient was treated with combination chemotherapy with complete response. The similarities of this case to three previously reported bilateral thecomas presenting in children on anticonvulsant therapy is discussed.
Adolescent↗
Ovarian luteinized thecoma with sclerosing peritonitis in an adult woman treated with leuprolide and toremifene in complete remission at 5 years.
BACKGROUND: Luteinized thecoma of the ovary associated with sclerosing peritonitis is a rare pathologic condition without a standard strategy of treatment. CASE: We present the case of an ovarian luteinizing sclerosing thecoma in a 39-year-old woman. The patient underwent three laparotomic operations for subocclusive symptoms, revealing in both occasions the presence of sclerosing peritonitis, with large abdominal masses, including cysts containing clear fluid. Treatment with toremifene 20 mg/day and leuprolide resulted in a dramatic improvement of the performance status and complete remission of all the abdominal lesions. After 60 months follow-up, the patient is still disease-free. DISCUSSION: Antiestrogens plus LHRH agonists might be a noninvasive, effective and well-tolerated therapy for sclerosing peritonitis in patient operated for luteinized thecomas.
Adult↗
Tetrasomy 12 in ovarian tumors of thecoma-fibroma group: A fluorescence in situ hybridization analysis using paraffin sections.
Recent cytogenetical studies have indicated that trisomy 12 is a feature of ovarian tumors in the thecoma-fibroma group. Ten cases of these ovarian tumors were studied in total, including two thecomas, two fibrothecomas, four fibromas, one cellular fibroma and one fibrosarcoma, to clarify the relationship between polysomy 12 and proliferative activity in these tumors. Each formalin-fixed, paraffin-embedded tumor tissue was examined by fluorescence in situ hybridization to determine copy numbers of chromosome 12 and by immunohistochemical staining of Ki-67 for evaluation of tumor cell proliferation. Gains of trisomy 12 were found in seven of the 10 cases, and the percentage of cells with tetrasomy 12, but not that of cells with trisomy 12, was significantly and positively correlated with percentage of Ki-67-positive cells, but significantly and inversely correlated with patient age. These findings suggest that tetrasomy 12 is an age-related aberration of chromosome 12 in ovarian tumors of the thecoma-fibroma group, and that such tumors exhibit more active proliferation in younger patients.
Adult↗
Adenosarcoma of the uterine corpus associated with ovarian thecoma.
We describe a case of adenosarcoma of the uterine corpus associated with ovarian thecoma in a 67-year-old woman. The patient underwent surgery under a diagnosis of ovarian carcinoma. The 110 x 70 mm-sized ovarian tumor was diagnosed as thecoma. The polypoid tumor of the uterine corpus which measured 30 x 15 mm was diagnosed as adenosarcoma. Cells of both epithelial and stromal elements of the adenosarcoma expressed estrogen receptors (determined by immunohistochemistry). These findings support the view that estrogen stimulation, including that by a pre-existing ovarian thecoma, may play a role in the development of mesenchymal and mixed epithelial / mesenchymal uterine tumors, including adenosarcoma.
Adenosarcoma↗
Sclerosing stromal tumors, thecomas, and fibromas of the ovary: an immunohistochemical profile.
The immunohistochemistry of 11 sclerosing stromal tumors (SSTs), 11 fibromas, and 5 thecomas was studied to determine criteria for the assessment of 5 densely sclerotic, calcified ovarian tumors of uncertain diagnosis occurring in young women. The results indicate that the staining pattern for alpha glutathione S-transferase can be used to distinguish SSTs, fibromas, and thecomas. CD34, by highlighting the vascular pattern and density, can be used to distinguish between SSTs and other tumors in the thecoma-fibroma group. Alpha-inhibin and calretinin mirrored the alpha glutathione S-transferase staining. Vimentin, smooth muscle actin, and muscle specific actin were generally positive, but desmin was negative in all but one tumor. These results suggest that at least four of the five tumors of uncertain diagnosis were SSTs that had undergone end-stage sclerosis and calcification.
Actins↗
Lipid cell thecomas of the ovary.
An ovarian tumour of mixed thecomatous and lipid cell structure is described and the clinical and pathological features of this and eleven previously reported cases are reviewed. Functionally the tumours resemble lipid cell tumours in being mainly androgenic and are similar in size range and age incidence to the 'adrenal' type. Three tumours however contained hilus (Leydig) cells with Reinke crystalloids. The tumours furnish additional evidence of the stromal origin of lipid cell tumours and the term 'lipid cell thecoma' is proposed for them as indicating their main morphological affinities. It is suggested that fibromas, fibrothecomas, thecomas, lipid cell thecomas and lipid cell tumours form a series of related stromatogenous tumours of relatively simple structure with functional correlations as one moves from left to right.
Adult↗
Sclerosing peritonitis associated with luteinized thecoma of the ovary.
A unique case of bilateral luteinized thecomas of the ovary associated with sclerosing peritonitis is reported and the clinical and pathological features of this and previously reported cases are reviewed. The patient, 52 years of age, presented with abdominal distension and diarrhea. Pelvic imaging studies revealed bilateral ovarian tumors with ascites. Total abdominal hysterectomy and bilateral salpingo-oophorectomy with adhesiotomy of the small bowel were performed. Histologically, the ovarian tumor was composed of closely packed spindle to round-shaped cells, and within the spindle cell population, lutein-like cells were scattered singly or in clusters. Mitotic counts of spindle cells revealed 12 mitotic figures (MF) per 10 high-power fields (HPF) in one part of the left ovarian tumor, but other areas of the tumor showed less than 3 MF/10 HPF on average. The lesion from the resected small bowel showed prominent fibrosis, confined to the serosa with no evidence of metastasis from the ovarian tumor. The patient has undergone adhesiotomy with partial resection of the small bowel seven times since the first laparotomy because of the recurrent small bowel obstruction. The patient has survived with complications due to short bowel syndrome for 7 years after the initial surgery and so far no recurrence or metastasis of the ovarian tumor has been identified. The case reported here also supports the idea that luteinized thecoma of the ovary associated with sclerosing peritonitis may be a distinct clinicopathologic entity, in terms of the unique association and of the unique features of thecoma; that is, bilateral, hormonally inactive and apparently benign in spite of its highly mitotic activity. Additional attention should be paid to the patient's quality of life, which is often degraded by peritoneal fibrosis and small bowel obstruction.
Female↗