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Chapter 76: Paraneoplastic Endocrine Syndromes

INTRODUCTION

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.

TABLE 76-1PARANEOPLASTIC SYNDROMES CAUSED BY ECTOPIC HORMONE PRODUCTION
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TABLE 76-1PARANEOPLASTIC SYNDROMES CAUSED BY ECTOPIC HORMONE PRODUCTION
Paraneoplastic Syndrome Ectopic Hormone Typical Tumor Typesa
Common
Hypercalcemia of malignancy PTHrP Squamous cell (head and neck, lung, skin), breast, genitourinary, gastrointestinal
  1,25 dihydroxyvitamin D Lymphomas
  PTH (rare) Lung, ovary
  PGE2 (rare) Renal, lung
SIADH Vasopressin Lung (squamous, small cell), gastrointestinal, genitourinary, ovary
Cushing’s syndrome ACTH Lung (small cell, bronchial carcinoid, adenocarcinoma, squamous), thymus, pancreatic islet, medullary thyroid carcinoma
  CRH (rare) Pancreatic islet, carcinoid, lung, prostate
  Ectopic expression of GIP, LH/hCG, other G protein–coupled receptors (rare) Macronodular adrenal hyperplasia
Less common
Nonislet cell hypoglycemia IGF-II Mesenchymal tumors, sarcomas, adrenal, hepatic, gastrointestinal, kidney, prostate
  Insulin (rare) Cervix (small cell carcinoma)
Male feminization hCGb Testis (embryonal, seminomas), germinomas, choriocarcinoma, lung, hepatic, pancreatic islet
Diarrhea or intestinal hypermotility Calcitoninc Lung, colon, breast, medullary thyroid carcinoma
  VIP Pancreas, pheochromocytoma, esophagus
Rare
Oncogenic osteomalacia Phosphatonin (FGF23) Hemangiopericytomas, osteoblastomas, fibromas, sarcomas, giant cell tumors, prostate, lung
Acromegaly GHRH Pancreatic islet, bronchial and other carcinoids
  GH Lung, pancreatic islet
Hyperthyroidism TSH Hydatidiform mole, embryonal tumors, struma ovarii
Hypertension Renin Juxtaglomerular tumors, kidney, lung, pancreas, ovary

aOnly the most common tumor types are listed. For most ectopic hormone syndromes, an extensive list of tumors has been reported to produce one or more hormones.

bhCG is produced eutopically by trophoblastic tumors. Certain tumors produce disproportionate amounts of the hCG α or hCG β subunit. High levels of hCG rarely cause hyperthyroidism because of weak binding to the TSH receptor.

cCalcitonin is produced eutopically by medullary thyroid carcinoma and is used as a tumor marker.

Abbreviations: ACTH, adrenocorticotropic hormone; CRH, corticotropin-releasing hormone; FGF23, fibroblast growth factor 23; GH, growth hormone; GHRH, Growth hormone–releasing hormone; GIP, gastric inhibitory peptide; hCG, human chorionic gonadotropin; IGF, insulin-like growth factor; LH, luteinizing hormone; PGE2, prostaglandin E2; PTH, parathyroid hormone; PTHrP, parathyroid hormone–related protein; SIADH, syndrome of inappropriate antidiuretic hormone secretion; TSH, thyroid-stimulating hormone; VIP, vasoactive intestinal peptide.

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 ...

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