Luteoma of pregnancy: case report.
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The clinical course of a 26-year-old primigravid woman with a virilizing luteoma of pregnancy is described. It was demonstrated by endocrine studies that the tumor secreted an excessive amount of testosterone. After removal of the luteoma in week 32 of pregnancy, the virilizing symptoms of the mother completely regressed. A normal male infant was delivered in week 39. From 100 luteomas of pregnancy described in the literature, 26 cases have been cited to elicit endocrine virilizing activity. An evaluative synopsis indicates that including the case described in this paper only 13 can be regarded as a clinical, morphologic, and functional entity. This entity should be differentiated from the inhomogeneous group of luteomas as the "androluteoma syndrome of pregnancy." It is characterized by the following major criteria: virilization, beginning with the second trimester of pregnancy; histologic criteria of a luteoma of pregnancy; production of testosterone, leading to excessive plasma levels (exceeding the concentration of androstenedione), and masculinization of female fetuses.
The binding of human luteinizing hormone and human follicle-stimulating hormone to ovarian tumor biopsy specimens from 29 patients was analyzed. The binding sites for human luteinizing hormone were demonstrated in one tumor of epithelial origin (mucinous cystadenoma) and in one of sex cord-stromal origin (theca cell tumor). The binding sites for human follicle-stimulating hormone were found in three tumors of epithelial origin (serous cystadenoma and mucinous cystadenoma) and in two of sex cord-stromal origin (theca cell tumor and theca-granulosa cell tumor). The surface-binding autoradiographic study revealed that the binding sites for gonadotropins were localized in the stromal tissue. The results suggest that gonadotropic hormones may play a role in the growth and differentiation of a certain type of human ovarian neoplasms.
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In a study of the frozen section biopsy of 120 ovarian neoplasms, correct results were obtained in 94.16%, incorrect in 4.16%, and inconclusive in 1.66% of the 120 cases. The "incorrect" results were all "false-negative." No inverse "false-positive" results were recorded, and the "incorrect" results occurred in cases of encapsulated neoplasms of a macroscopically benign appearance with small areas of malignant changes detected only by paraffin block technique. The authors conclude that frozen section biopsy of ovarian neoplasm is plausible so long as a pathologist is available who can be provided with the proper equipment during the surgery and that the technique is more indicated in patients up to the age of 40 because of the necessity of conservative management in this age group.
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The patient was born with ambiguous genitalia (stade III of Prader). The karyotype revealed a normal female genotype. A defect in 21-hydroxylase, at first suspected, was denied by the hormonal studies. Indeed, extremely high levels of pregnenolone, pregnenolone sulfate, progesterone were found in association with low plasma levels of delta 4-androstenedione, testosterone, dehydroepiandrosterone and its sulfate, while cortisol 17OH-progesterone and ACTH levels and plasma renin activity were normal. The hormonal pattern was thus consistent with 17,20-desmolase deficiency. The dynamic studies further supported this contention: all the progestagens rose further after ACTH stimulation and were suppressed by dexamethasone. Meanwhile, all androgens failed to rise after ACTH: the responses of cortisol were normal. The in utero virilization of the female fetus was not understood until an history of virilization was allegedly found in the mother (luteoma of pregnancy). This is the first case of 17-20 desmolase defect recognized in a female newborn. This child, born with ambiguous genitalia had presented the concurrence of two very rare conditions. The in utero virilization of maternal origin enabled us to make the diagnosis of the 17-20 desmolase defect, which otherwise would have been ignored in a XX subject in the neonatal period because it would obviously be unsymptomatic at this age.
An incidental pregnancy luteoma was identified microscopically in an ovary submitted for ruptured ectopic tubo-ovarian pregnancy. The lesion consisted of a localized, non-encapsulated proliferation of thecal cells in the wall of an atretic follicle and had all the pathologic features of the typical late pregnancy luteoma. This finding is presented to support the origin of the pregnancy luteoma from the theca interna of atretic follicles.
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Synchronous bilateral testis tumors of different histologic types are rare. All previous cases have demonstrated germ cell tumors on both sides. The simultaneous appearance of a germ cell tumor and a contralateral non-germ cell tumor has not been reported. We herein report a thirty-four-year-old man who presented with a mixed non-seminomatous germ cell tumor of the left testis and theca cell tumor of the right testis.
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Two cases of endometrial sarcoma that developed many years after exposure to unopposed exogenous and endogenous estrogens, with no previous pelvic irradiation, are described. The literature is reviewed and the possible effect of prolonged unopposed estrogen stimulation is suggested as an etiologic factor in such extremely rare conditions. These cases add additional support to the assumption that prolonged and excessive endogenous or exogenous unopposed estrogen stimulation of the uterus may increase the risk of endometrial sarcoma.