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Objective: Our purpose was to review patients with granulosa and theca cell tumors as filed in the Emil Novak Ovarian Tumor Registry. Study Design: Our study was a descriptive, retrospective study of 454 case records. Results: The reviewed diagnoses were for 97 patients with granulosa cell tumors, 116 with theca cell tumors, and 97 with granulosa-theca cell tumors. The remaining cases (n = 144) were reclassified as "nonspecific" gonadal stromal tumors (n = 61), luteomas of pregnancy (n = 7), and 76 "other" cases. These included poorly differentiated cancer, metastatic cancer, mixed mesodermal tumors, and sarcomas. The tumor-related mortality rate for the 310 patients with granulosa, theca, and granulosa-theca cell tumors was 7% (37.3% for granulosa cell tumors only). The surgical stage of disease was the most significant prognostic factor, with a mortality rate of at least 40%, given that the tumor had spread beyond the ovary. Conclusion: Because the differential diagnoses of particularly granulosa cell tumors included several conditions with an extremely poor prognosis, an accurate histologic diagnosis is crucial. (Am J Obstet Gynecol 1999;180:323-7.)

Four additional examples of theca-cell tumors of the ovary have been reported, three being removed surgically, one having been discovered incidental to autopsy. All the tumors were benign. One very large tumor occurred in a 17-year-old girl, producing no menstrual irregularities. One tumor associated with a fibroma of the opposite ovary showed infarction due to torsion of its pedicle. Convalescence and follow-up of the three cases have been uneventful. A complete summary of the literature has been presented.

The anatomic and histologic characteristics of theca cell tumors are described, together with their distinctive clinical and hormonal features. Descriptions are given of six cases illustrating variations from the more benign fibroma type to the highly cellular and malignant type. A correlation is made between the presence of hormonal changes and the presence of intracellular, doubly refractile fat containing cholesterol and cholesterol esters. It is suggested that some of the ovarian neoplasms previously diagnosed as fibromas or fibrosarcomas may on further investigation prove to be of theca cell origin.

Thirty ovarian tumors belonging to the granulosa and theca cell group of neoplasms are reported. They correspond in age incidence, clinical manifestations, and in gross and microscopic appearance with other reported cases. They differ in one major respect, that of malignancy. Only one tumor has proved to be malignant despite a follow-up record of four years or longer in 14 cases. By means of Laidlaw's silver stain granulosa-like cells were identified in all theca cell tumors and thecalike cells in a number of granulosa cell neoplasms. While our results are similar to those reported by Traut and Marchetti, we are not yet completely satisfied in regard to the recognition of these two types of cells by this method. Tumors of the theca cell group, however, had a later age incidence and presented sufficiently well-marked pathologic differences to warrant their continued recognition as a particular type of estrogen-secreting tumor. Two of the granulosa cell tumors were completely luteinized and one of these was of the unusual adenomatous type. The occurrence of 5 cases of carcinoma of the endometrium and 10 cases of uterine fibroids suggests a direct relationship between the ovarian neoplasm and the uterine tumors but does not warrant a definite conclusion in this regard.

The association of ovarian theca cell tumors with endometrial glandular hyperplasia and neoplasia is well established. A recognized association of these tumors with endometrial stromal neoplasia does not, however, appear to have emerged. The coexistence, on two occasions, of endometrial stromal neoplasia with theca cell tumors is herewith reported. Although no conclusion can be drawn from but two instances, the phenomenon is regarded as worthy of documentation in the event that further accumulation of similar evidence may permit of a definitive statement in the future.

A relationship of tumor cytology (anaplasia) and clinical course has not been established for the granulosa-theca cell tumor group. Granulosa-theca cell tumor cytology and clinical information from a single institution has been independently studied. A relationship of tumor cytology and clinical course does exist.

Theca-cell tumors are discussed relative to their histogenesis, clinical manifestations, pathology, and treatment. A small series of eleven cases from the Montreal General and Royal Victoria Hospitals is reported. These cases are representative of the problems involved in the investigation and management of theca-cell tumors generally. The ages of patients ranged from 21 to 69 years, with an average of 50 years. Seven of the patients were postmenopausal, only two of whom presented bleeding as a symptom. One of these showed hyperplasia, and one an adenocarcinoma of the endometrium. Three other patients demonstrated bleeding as a symptom, two at the menopausal age, and one earlier; all three of these had a hyperplasia of the endometrium. The one remaining patient exhibited amenorrhea as the principal symptom, and, after surgical removal of the thecoma, the menses returned to regularity. Three of the patients in the series had been previously radiated. Theca-cell tumors accounted for 1.8 per cent of all ovarian tumors during the ten years under review.

A case of malignant thecoma has been presented. Five previously reported cases have been reviewed and only three of these have criteria acceptable for malignant tumors. The incidence of malignancy in theca-cell tumors is approximately 3 per cent.

The clinical and pathologic data on 10 so-called theca cell tumors have been presented. These tumors occur most frequently following the menopause. They resemble granulosa cell tumors clinically, but pathologically possess certain distinguishing features which probably justify their consideration as an entity.

1. 1. One hundred and thirty-two cases of combined feminizing tumors of the ovary and carcinoma of the uterus were reviewed from the literature. 2. 2. Twenty-eight new cases of feminizing mesenchymoma of the ovary were reported. Eight cases of combined carcinoma and granulosa cell tumor were presented and evaluated. 3. 3. Evidence is offered that granulosa cell tumors, as well as thecomas, present a clinical and pathologic picture of hyperestrogenism. 4. 4. The relationship of estrogen to carcinogenesis in certain target or “receptor” tissues is discussed.

A case of a theca cell tumor removed at the sixteenth week of pregnancy which subsequently proceeded to term has been described. Only 7 cases of thecoma and 20 cases of granulosa cell tumor occurring with pregnancy have previously been reported. These cases have been reviewed.

Certain ovarian tumors exert hormonal effects, and of the feminizing type there are two, the granulosa cell tumors and the theca cell tumors. Theca cell tumors have only recently been recognized. Two such cases are reported. They produced hyperplasia of the myometrium and endometrium, and postclimacteric bleeding. In one case the bleeding was periodic, resembling the normal menstrual cycle. The tumors are composed of cells which have the histologic characteristics of theca interna cells. Apparently these tumors secreted theelin. This conclusion is supported by much experimental and deductive evidence from the literature that the theca cells secrete the estrogenic hormone. Unfortunately, the tumors were formalin fixed before implantation experiments could be made, and no blood or urine tests for hormone were made, but perhaps future investigators may be able to do this, for diagnosis as well as for investigation. In this connection we reiterate an admonition made in a previous communication, 37 namely, if in a woman past the menopause who is bleeding, nothing of an etiologic nature is found in the systemic or local examination, and curettage proves barren, one should perform a colpotomy and visualize the ovaries, in order that guilty ovarian tumors be not overlooked.

A malignant theca-cell tumor has been reported. The patient is living and well eight years after removal of only a portion of the neoplasm. Postoperative radiation therapy resulted in the disappearance of clinical evidence of the growth. Approximately 3 per cent of all theca-cell tumors are malignant. Criteria for the histologic diagnosis of malignancy in theca-cell tumors have been outlined and discussed.