1/5. 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.- - - - - - - - - - ranking = 1keywords = cell tumour (Clic here for more details about this article) |
2/5. Ovarian sertoli-leydig cell tumor with coexisting vaginal angiomyxoma: case report and review of the literature.An extremely rare case of a postmenopausal patient with an ovarian Sertoli-Leydig cell tumour and a coexistent vaginal angiomyxoma is reported. A 71-year-old patient was admitted complaining of abdominal distension. A thorough diagnostic evaluation revealed a large tumour of the right ovary, and an oval-shaped greyish-white polypoid vaginal lesion. Total hysterectomy with bilateral salpingooophorectomy and lymph node sampling was performed, followed by excision of the vaginal lesion. Histological examination showed a Sertoli-Leydig cell tumour of the right ovary, and a vaginal angiomyxoma. Twenty-six months after the operation the patient is well with no signs of recurrence. To the best of our knowledge, no case of coexistence of an ovarian Sertoli-Leydig cell tumour with a myxoma has been previously reported.- - - - - - - - - - ranking = 3keywords = cell tumour (Clic here for more details about this article) |
3/5. The complex of myxomas, spotty pigmentation and endocrine overactivity.We describe a 40-year-old male with acromegaly, multiple and recurrent cutaneous myxomas, spotty cutaneous pigmentation, a mammary myxoma, a large-cell calcifying Sertoli cell tumour of the testis, and an unusual calcifying pigmented neuroectodermal tumour. He presented a combination of clinical and histological features not previously documented within the complex of myxomas, spotty pigmentation and endocrine overactivity, and therefore the findings broaden the spectrum of the syndrome.- - - - - - - - - - ranking = 1keywords = cell tumour (Clic here for more details about this article) |
4/5. Primary adrenocortical nodular dysplasia with Cushing's syndrome and cardiac myxomas. A peculiar familial disease.In a family with 4 children, 2 had slowly developing Cushing's syndrome and were adrenalectomized between the ages of 18 and 28 years. As in other cases with familial Cushing's syndrome, primary adrenocortical nodular dysplasia, the so-called adrenocortical adenomatosis, was demonstrated. The brother, now 39 years old, is in good health. The sister, however, died at the age of 36 years. autopsy revealed a cardiac myxoma of the left atrium. A cardiac myxoma had been found incidentally at autopsy of these two patients' oldest brother, a boy who died at the age of 4 years. Both siblings with Cushing's syndrome presented additional Peutz-Jegher-like hyperpigmentation and myxomatous tumours of the skin. Identical observations of familial adrenocortical nodular dysplasia, Cushing's syndrome and cardiac myxomas have not been reported to date. However, the familial occurrence of cardiac myxomas and adrenocortical dysplasia in combination with bilateral large cell calcifying Sertoli cell tumours of the testis has been published recently.- - - - - - - - - - ranking = 1keywords = cell tumour (Clic here for more details about this article) |
5/5. Bilateral testicular large-cell calcifying sertoli cell tumor and recurrent cardiac myxoma in a patient with Carney's complex.Large-cell calcifying Sertoli cell tumors are exceedingly rare testicular tumors which can be distinguished from pure Sertoli cell tumors by distinctive microscopic features and multifocal and bilateral calcifications [1]. We describe the characteristic sonographic appearance of the tumor with pathologic correlation. This tumor also constitutes one of the conditions of Carney's complex [2], which includes cardiac and skin myxomas, myxoid mammary fibroadenomas, spotty skin pigmentation, primary pigmented nodular adrenocortical disease, pituitary adenoma and unusual testicular tumors, especially large-cell calcifying sertoli cell tumor. We report the case of a 10-year-old boy with four of the above conditions. Recognition of this complex is important as cardiac myxomas can lead to lethal complications. Because of a tendency for familial occurrence, family members should be screened carefully [3].- - - - - - - - - - ranking = 554.58426338896keywords = sertoli cell, sertoli (Clic here for more details about this article) |