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Both benign and malignant tumors of nonendocrine tissue can secrete a variety of hormones, principally peptide hormones, and many tumors produce more than one hormone (Table 76-1). At the clinical level, ectopic hormone production is important for two reasons.

First, endocrine syndromes that result may either be the presenting manifestations of the neoplasm or occur late in the course. The endocrine manifestations in some instances are of greater significance than the tumor itself, as in pts with benign or slowly growing malignancies that secrete corticotropin-releasing hormone and cause fulminant Cushing's syndrome. The frequency with which ectopic hormone production is recognized varies with the criteria used for diagnosis. The most common syndromes of clinical import are those of adrenocorticotropic hormone (ACTH) hypersecretion, hypercalcemia, and hypoglycemia. Indeed, ectopic ACTH secretion is responsible for 15-20% of pts with Cushing's syndrome, and ~50% of pts with persistent hypercalcemia have a malignancy rather than hyperparathyroidism. Because of the rapidity of development of hormone secretion in some rapidly growing tumors, diagnosis may require a high index of suspicion, and hormone levels may be elevated out of proportion to the manifestations.

Second, ectopic hormones serve as valuable peripheral markers for neoplasia. Because of the broad spectrum of ectopic hormone secretion, screening measurements of plasma hormone levels for diagnostic purposes are not cost effective. However, in pts with malignancies that are known to secrete hormones, serial measurements of circulating hormone levels can serve as markers for completeness of tumor excision and for effectiveness of radiation therapy or chemotherapy. Likewise, tumor recurrence may be heralded by reappearance of elevated plasma hormone levels before mass effects of the tumor are evident. However, some tumors at recurrence do not secrete hormones, so hormone measurements cannot be relied on as the sole evidence of tumor activity.

Treatment: Paraneoplastic Endocrine Syndromes

Therapy of ectopic hormone-secreting tumors should be directed when possible toward removal of the tumor. When the tumor cannot be removed or is incurable, specific therapy can be directed toward inhibiting hormone secretion (octreotide for ectopic acromegaly or mitotane to inhibit adrenal steroidogenesis in the ectopic ACTH syndrome) or blocking the action of the hormone at the tissue level (demeclocycline for inappropriate vasopressin secretion).

Outline

Section 6. Hematology and Oncology