1/15. Ectopic ACTH syndrome: molecular bases and clinical heterogeneity.There are roughly two types of ectopic ACTH syndrome (EAS). one associated with overt malignancies and one with occult neoplasms. The prototype of the first condition is Cushing's syndrome sustained by small-cell lung cancer (SCLC), while bronchial carcinoid tumors are the most common occult sources of ACTH. patients with EAS and SCLC may have an atypical presentation with muscle wasting and weight loss that are more frequently observed than the classic cushingoid features. These patients have a poor prognosis because SCLC associated with the EAS is more resistant to chemotherapy and the severe hypercortisolism is responsible for a high rate of life-threatening complications during treatment. Conversely, the clinical and biochemical features of the EAS associated with carcinoid may overlap those seen in pituitary-dependent Cushing's syndrome. An extensive radiological and hormonal work-up is necessary to detect the extrapituitary source of ACTH. However, the differentiation between the pituitary, or eutopic, from the non-pituitary, or ectopic, source of ACTH secretion may be extremely difficult in some cases despite the wide diagnostic armamentarium available. molecular biology studies have demonstrated that the carcinoid cells achieve a process of corticotroph differentiation being able to express the proopiomelanocortin (POMC) gene and to process POMC correctly to release large amounts of intact ACTH. Conversely, SCLC processes POMC in an aberrant way releasing high concentrations of ACTH precursors and less intact ACTH in the circulation.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
2/15. Ectopic ACTH syndrome associated with ovarian steroid-cell tumor.Several cases of ovarian neoplasms resulting in Cushing's syndrome due to ectopic secretion of ACTH or ectopic secretion of F have been reported. Tumors producing ACTH include adenocarcinoma, androblastoma, Sertoli cell carcinoma, carcinoid tumor and teratoma. Cortisol secretion has been reported in ovarian steroid cell tumor (unclassified steroid cell tumor). We present a case of a 19-year-old woman with Cushing's syndrome in course of an ovarian steroid cell tumor with ectopic ACTH production. To our knowledge, it is the first reported case of ACTH secreting ovarian steroid cell tumor causing Cushing's syndrome.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
3/15. Small cell carcinoma with two paraendocrine syndromes.Simultaneous elevated levels of ectopic arginine vasopressin (AVP) and ectopic adrenocorticotropin (ACTH) were found in a patient with small cell carcinoma (SCC). The finding of one of these paraendocrine syndromes at the time of diagnosis is common; however, the simultaneous presence of both syndromes has been reported in the literature only on four occasions in the past 25 years. This is the only report in which elevated plasma levels of both hormones are documented in a patient who simultaneously fulfills the criteria for the syndrome associated with each ectopically produced peptide. In the English-language literature, this is the first case that demonstrates by immunohistochemical staining the presence of both of these hormones in the patient's neoplasm. In addition to the use of radiographs, the presence of paraendocrine disorders can provide a method of monitoring the patient's response to therapy. The levels of ACTH and AVP were assayed during this patient's course and correlated with disease refractory to therapy, resulting in poor survival.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
4/15. Medullary carcinoma of the thyroid as a cause of Cushing's syndrome: a case with ectopic adrenocorticotropin secretion characterized by double enzyme immunostaining.A case of a medullary carcinoma of the thyroid gland that secreted both calcitonin and adrenocorticotropin (ACTH) is reported. The patient was a 32-year-old man who was referred to the Clinical Center of the National Institutes of health with radiologic evidence of intrathoracic and hepatic masses accompanied by florid Cushing's syndrome. serum levels of calcitonin and ACTH were elevated. The thoracic and hepatic masses were resected. The histologic findings were typical of medullary carcinoma of the thyroid with extensive metastases to the liver. The neoplasm had a predominantly solid pattern, and the neoplastic cells were either round or spindled, many with cytologic atypia. Immunohistochemical analysis of fixed, paraffin-embedded sections demonstrated chromogranin, calcitonin, and ACTH in the neoplastic cells. The immunostaining for chromogranin was intense in all of the cells, whereas weaker staining for calcitonin and ACTH was present in scattered cells. Electron microscopy revealed sparse secretory granules in the majority of tumor cells; a minority of neoplastic cells contained numerous granules. We further characterized this neoplasm by performing dual immunohistochemical analysis. This technique clearly demonstrated the presence of ACTH and calcitonin within the same neoplastic cells. Thus, the medullary carcinoma of the thyroid in this patient was the source of ectopic ACTH secretion causing Cushing's syndrome. In addition, this report highlights the value of using double immunostaining to localize both the ACTH and calcitonin within the same cells.- - - - - - - - - - ranking = 2keywords = neoplasm (Clic here for more details about this article) |
5/15. Small cell carcinoma of the uterine cervix showing Cushing's syndrome caused by ectopic adrenocorticotropin hormone production.A uterine cervical cancer is reported in a woman who developed Cushing's syndrome. The tumor measured 1.3 x 0.7 cm, and was a pure small cell carcinoma, identical to that in the lung. The primary tumor cells showed argyrophilia with Grimelius staining and reacted positively to the anti-chromogranin antibody. Clinically, the neoplasm behaved in an aggressive manner in spite of adjuvant chemotherapy and radiotherapy, and the patient died of widespread metastasis. Cushing's syndrome was noted after the occurrence of liver metastasis with an elevation of the serum adrenocorticotropin hormone (ACTH) level. At autopsy, metastatic tumor cells from the liver reacted immunohistochemically positively not only to anti-ACTH but also to antichromogranin, anti-gastrin and anti-calcitonin antibodies. This is the first report of an immunohistochemical analysis of, and comparison of primary and metastatic sites in cervical carcinoma showing Cushing's syndrome.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
6/15. Oncocytic carcinoid of the kidney associated with periodic Cushing's syndrome.An oncocytic carcinoid of the kidney producing a periodic Cushing's syndrome in an adolescent is described. The tumor displayed gross, histologic, and ultrastructural features similar to renal oncocytoma, another unusual renal neoplasm. A review of renal carcinoids and possible associations between oncocytic change and periodic hormone production are discussed.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
7/15. Corticotroph cell pituitary adenoma within an ovarian teratoma. A new cause of Cushing's syndrome.A 24-year-old woman with severe Cushing's syndrome was found to have corticotroph cell pituitary adenoma arising within a benign cystic ovarian teratoma. The patient manifested sustained hypercortisolemia and lack of suppression of either adrenocorticotropin (ACTH) or cortisol production. There was no evidence of a pituitary mass or secretion of other hormones. After careful clinical evaluation, no other tumor masses were found. Resection of the ovarian tumor led to normalization of ACTH and cortisol levels. Densely granulated corticotroph tumor cells with prominent Type I microfilaments and intracytoplasmic ACTH immunoreactivity characterized the neoplasm as a pituitary corticotroph cell adenoma. This is, to our knowledge, the first case reported of a functioning pituitary adenoma arising within a benign cystic teratoma.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
8/15. Occult ectopic secretion of corticotropin.The clinical, biochemical, radiographic, and morphologic features of ectopic corticotropin (ACTH)-dependent Cushing's syndrome are often indistinguishable from those of Cushing's disease (pituitary-dependent Cushing's syndrome). We encountered ten patients whose ectopic ACTH-secreting neoplasms were not clinically apparent for two months to 12 years after the diagnosis of hypercortisolism or in whom the site remains unknown. Five of these patients underwent unnecessary pituitary microsurgery, and a sixth was referred for surgery. The occult ectopic ACTH syndrome occurs with equal frequency in men and women and hypokalemia is present in 60%, in contrast to the female predominance and rarity of hypokalemia in Cushing's disease. We emphasize the importance of selective venous sampling for ACTH to establish the correct diagnosis. Thirty-nine similar cases from the literature help characterize this syndrome further.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
9/15. Cushing's syndrome produced by a bronchial carcinoid tumor.A case of bronchial carcinoid tumor with ectopic corticotropin production is described. Cushing's syndrome refractory to therapy, including pituitary irradiation and hypophysectomy, had been present for 12 years before the neoplasm was discovered at autopsy. The tumor was a small nodule localized in the pulmonary parenchyma and an adjacent hilar lymph node. Corticotropin, serotonin, and neuron-specific enolase were localized in the neoplasm by immunoperoxidase staining.- - - - - - - - - - ranking = 2keywords = neoplasm (Clic here for more details about this article) |
10/15. Immunohistochemical evaluation of a complex endocrinopathy.A patient with clinical and biochemical evidence of ectopic ACTH production is described. autopsy revealed endocrine tumors of bronchus and pancreas with metastases to the liver, kidney, abdominal lymph nodes, and bone. Immunohistochemical methods established that the bronchial and pancreatic tumors were separate primary neoplasms each with its own hormone production; and allowed us to determine that the bronchial carcinoid was the source of ACTH and gave rise to the liver metastases, and that insulin was present in the cells of the pancreatic islet cell tumor. These methods also revealed a mild C-cell hyperplasia of the thyroid gland. We wish to stress the importance of the immunoperoxidase method in the evaluation of this and similar polyendocrine cases.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
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