Surgical Management of the Ectopic ACTH Syndrome

Abstract

Most patients with extra-pituitary ACTH-secreting tumors die from carcinoma, but a few can benefit from operation. Of 96 patients with Cushing's syndrome, 11 probably had such tumors. There were three modes of presentation: (1) three had malignant tumors with visceral metastases initially. One (bronchial carcinoid) died without operation. Two with carcinoma (thyroid medullary and islet-cell) underwent adrenalectomy with remission, but died soon. (2) three had apparently benign tumors initially. One (appendicular carcinoid) underwent appendectomy and one (bronchial carcinoid with hilar node metastases) underwent lobectomy. Both had rapid remissions. The third (pheochromocytoma) died after resection of the tumor. (3) five patients had no obvious tumors and underwent adrenalectomy with remission. In one a benign bronchial carcinoid was removed later. Four others remain well, but without localizing signs of tumor. The main biochemical features in all were hypokalemic alkalosis and very high urinary excretion of free cortisol. Seven of the eight patients without visceral metastases are in remission from one to 15 years after operation.

Ectopic Adrenocorticotropic Hormone Syndrome

“…Some malignant tumors that have spread to local lymph nodes, especially bronchial carcinoids and medullary carcinomas of the thyroid, may be excised radically with a prospect of benefit, at least for some years (141). Some malignant tumors that have spread to local lymph nodes, especially bronchial carcinoids and medullary carcinomas of the thyroid, may be excised radically with a prospect of benefit, at least for some years (141).…”

Rare localization of an ACTH-producing tumour

“…В литературе описано не более 10 случаев обнаружения у больного АКТГ-эктопической опухоли указанной локализации и столько же работ с локализацией опухоли в ободоч-ной кишке [10][11][12][13][14][15].…”

“…В работе H. Dobnig и соавт. [12] уже че-рез 3 года после оперативного лечения синдрома Кушинга было достигнуто увеличение минеральной плотности костной ткани в поясничной области и шейке бедра (на 79% от дооперационного уровня).…”

Recovery from severe osteoporosis following cure from ectopic ACTH syndrome caused by an appendix carcinoid

“…Among carcinoids, it is most commonly the bronchial or thymic carcinoids, and only rarely carcinoids of other locations, that cause the ectopic ACTH syndrome. A literature search over the last 30 years revealed three reports in which the appendix was the proven source of ectopic ACTH secretion [15][16][17]. In two cases, the carcinoids of the appendix were also incidentally found during laparatomy [15,16], and in one, metastases of the liver caused the syndrome 9 years after removal of the primary tumour by appendectomy [17].…”

“…A literature search over the last 30 years revealed three reports in which the appendix was the proven source of ectopic ACTH secretion [15][16][17]. In two cases, the carcinoids of the appendix were also incidentally found during laparatomy [15,16], and in one, metastases of the liver caused the syndrome 9 years after removal of the primary tumour by appendectomy [17]. Until recently, it was thought that carcinoids of the midgut region do not cause the ectopic ACTH syndrome [18] and they are not included in the list of possible ectopic ACTH sources by major textbooks of endocrinology.…”