[Ectopic hormone formation in multiple endocrine type IIa neoplasia (author's transl)]

A patient with high-grade osteoporosis had elevated levels of ACTH, cortisol and carcinoembryonic antigen (CEA). Computed tomography demonstrated enlarged adrenal glands. Despite intensive search no ACTH-producing tumour was found. A central Cushing's syndrome was thus possible, and a radiologically normal sella turcica made a microadenoma of the hypophysis a possibility. However, a one-time hypertensive crisis with elevated catecholamines raised the suspicion of an additional phaeochromocytoma and thus provided the first pointer to a multiple endocrine neoplasia. Bilateral phaeochromocytomas, discovered at adrenalectomy, as well as raised serum-calcitonin levels, strongly suggested coexistence of a C-cell carcinoma, which previously had not been demonstrated either by thyroid scanning or fine-needle biopsy. A total thyroidectomy revealed a multifocal and metastasizing C-cell carcinoma. On extraction it contained a high concentration of calcitonin, typical for a C-cell carcinoma. An unusual finding was of ACTH in extracts of both the C-cell carcinoma and the phaeochromocytoma. It was immunologically identical with the synthetic ACTH used as a standard.