1/155. Sertoli cell tumors of ovary: light microscopic and ultrastructural study with histogenetic considerations. Two cases of pure Sertoli cell tumors of the ovary are presented. The tumors were grey to brown and generally solid, but with several cystic areas. They showed a highly differentiated, but variable, histologic patterns with solid cords of neoplastic cells, as well as tubular formations. Ultrastructurally, the basal part of the cells rested on a nonfibrillary basement membrane layer while the free border showed occasional cilia. Laterally, the cells showed tight junctions and desmosomes. Abundant rough endoplasmic reticulum and some cystically dilated smooth endoplasmic reticulum were evident. The theories regarding the histogenesis are discussed. Enough similarities, both by light and by electron microscopy, are presented between these tumors and the Sertoli cell to justify classifying these ovarian tumors as Sertoli cell in type. The development of cilia is considered to be a manifestation of focal metaplasia of the neoplastic Sertoli cell. ( info) |
2/155. A case of large cell calcifying sertoli cell tumor in a child with a history of nasal myxoid tumor in infancy. A case of an 8-year-old Japanese boy with a testicular large cell calcifying sertoli cell tumor (LCCSCT) is presented. This report appears to be the first Japanese case of LCCSCT. The patient presented with left testicular swelling and gynecomastia. His family history was not contributory; however, his past history was remarkable for a benign myxoid tumor in the nasal cavity, which was removed at the age of 2 months. After removal of the testicular tumor, the gynecomastia disappeared gradually and no recurrence or metastasis developed during a 15 month follow-up period. Although the tumor was initially interpreted as a leydig cell tumor, a review of the slides after the patient's past history of nasal myxoid tumor was revealed led us to the diagnosis of LCCSCT. An accurate diagnosis of LCCSCT is crucial because this tumor is occasionally associated with carney complex, which can comprise various pathological conditions, including cardiac myxoma, that may be life-threatening. myxoma of carney complex has been described to occur in the heart, skin, oral cavity and breast in a wide age range, but there have been no reports referring to nasal myxoid tumor associated with carney complex. ( info) |
3/155. carney complex: in a patient with multiple blue naevi and lentigines, suspect cardiac myxoma. carney complex is characterized by spotty pigmentation (blue naevi and lentigines), myxomas (cardiac, cutaneous, mammary), endocrine over-activity (Cushing's syndrome, acromegaly), testicular tumours, and schwannomas. We report a male with multiple blue naevi, lentigines, testicular large cell calcifying Sertoli-cell tumour and four cardiac myxomas. The myxomas caused two cerebrovascular accidents and a myocardial infarction. All patients with multiple blue naevi or lentigines should be investigated for the life-threatening association of cardiac myxomas. ( info) |
| To date, only I I cases of sclerosing Sertoli cell tumors have been reported in the literature, representing a distinctive subtype of sertoli cell tumor in humans. We present a 12th case with a review of the current urological and pathological literature. ( info) |
5/155. Malignant sertoli cell tumor of the ovary metastatic to the lung mimicking neuroendocrine carcinoma: report of a case. A Sertoli cell carcinoma of the ovary with lung metastases mimicking neuroendocrine carcinoma is presented. lung metastases frequently occur. Primary and secondary tumors may exhibit similar growth patterns and differentiating primary from secondary tumors may be troublesome. This process may be more difficult when metastases occur from a tumor in which metastases are uncommon and morphologically resemble only a small portion of the primary tumor. We report the case of a 52-year-old woman who underwent resection of a 4,550-g sertoli cell tumor of the ovary. Histologically, in addition to the characteristic tubular pattern of growth, 5% of the tumor consisted of poorly differentiated areas with tumor cells in sheets, a high mitotic rate, and areas of necrosis. Eleven months after this surgery she presented at a different institution with multiple pulmonary nodules. Microscopic examination of a subsequently resected lung nodule showed histologic findings similar to those of the poorly differentiated areas of the ovarian tumor and initial immunohistochemical studies showed positive staining for cytokeratin, neuron-specific enolase, and focal positivity for synaptophysin. Without knowledge of the ovarian tumor the lung lesion was interpreted as large-cell neuroendocrine carcinoma. On review of the clinical history and comparison with the previous surgical material, however, both tumors showed similar light microscopy and immunohistochemical reactivity, and a final diagnosis of metastatic sertoli cell tumor was made. Immunohistochemical staining for inhibin revealed weak positivity in the poorly differentiated areas of the ovarian tumor but not in the lung metastasis. This is one of the rare reports of ovarian sertoli cell tumor metastasizing to the lungs and it emphasizes the importance of complete clinical histories, ancillary studies, appropriate sampling, and review of archival material in such unusual cases. ( info) |
6/155. Malignant large cell calcifying sertoli cell tumor of the testis (LCCSCTT). Report of a case in an elderly man and review of the literature. Malignant Large Cell Calcifying sertoli cell tumor of the testis (LCCSCTT) is a rare histological variant of sex cordstromal tumors. It usually arises in young males, sometimes is associated with endocrine abnormalities and has a benign course. It is exceptional in elderly men and the outcome is rarely fatal. We report a case of LCCSCTT in a 73 year-old man with fatal outcome. The tumor involved the right testis and several areas of the tunica albuginea were grossly invaded. serum levels of HCG, LH and testosterone were normal. Lymphoangiography performed after orchiectomy showed an involvement of the iliac and preaortic lymph nodes. X-ray of the chest showed no lung metastases. A thorough study of the light microscopic, immunohistochemical and ultrastructural appearances was performed. immunohistochemistry revealed positivity to vimentin, S-100 and NSE. Our observations confirm the previous findings concerning malignant LCCSCTT and point out the histogenesis of the tumor from sertoli cells. ( info) |
7/155. Large cell calcifying sertoli cell tumor of the testis: a clinicopathological, immunohistochemical, and ultrastructural study of two cases. Large cell calcifying sertoli cell tumor of the testis (LCCSCT) is a rare tumor that is usually benign and multifocal. It may be associated with hereditary endocrine anomalies such as Carney's and Peutz-Jeghers syndromes. Malignant forms are exceptional. Two cases of LCCSCT, one malignant and one benign are described. Both were composed of cords and trabeculae of large polygonal cells embedded in a myxoid and fibrous stroma with areas of calcification. The malignant tumor showed nuclear atypia, necrosis, and abundant mitoses. The cells were positive for vimentin and S-100 protein, and the intercellular space for laminin and collagen type IV. By electron microscopy, nucleolonemas and multilayered basal lamina were seen. The benign tumor was positive for vimentin and S-100 protein, and ultrastructurally showed less basal lamina. ( info) |
8/155. sertoli cell tumor in androgen insensitivity syndrome--a case report. A 26-year-old individual with androgen insensitivity syndrome was operated on for a 3200-g sertoli cell tumor of the left gonad with retroperitoneal metastases. Six courses of bleomycin, etoposide, and cisplatin chemotherapy followed surgical treatment. Eighteen months after the initial surgery the patient is free of disease and in good health. The association of sertoli cell tumor with androgen insensitivity syndrome is discussed and the relevant literature is briefly reviewed. ( info) |
9/155. Androgen-producing ovarian tumors: a clinicopathological study of 3 cases. The clinical course and pathological findings of 3 rare cases of androgen-producing ovarian tumors are presented. The ages of the 3 patients (Cases 1, 2, and 3, respectively) were 43, 34, and 57 years, respectively. Their preoperative serum testosterone levels were 506, 491, and 231 ng/dl, respectively. The pathological diagnoses of Cases 1, 2, and 3 were a Sertoli-stromal cell tumor of intermediate differentiation, a stromal tumor containing leydig cells, and a stromal tumor with minor sex cord elements, respectively. Patient 1 experienced a recurrence, of a lesion at the vaginal stump 1 year and 2 months after the initial surgery. The clinical courses of Cases 2 and 3 have been non-contributory. ( info) |
| gynecomastia in boys with peutz-jeghers syndrome and Sertoli cell tumors of gonadal origin results from increased estrogen production due to increased aromatase activity within the testicular tumor. We present a prepubertal boy with peutz-jeghers syndrome, gynecomastia and bilateral neoplastic Sertoli cell proliferation in whom the only abnormal hormonal profile was increased concentration of inhibin B and Pro-alpha C in serum. ( info) |