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paraneoplastic syndrome

Syndrome resulting from distant effects of a neoplasm. Classification: 1) endocrine a) Cushing's syndrome* - mediators: - ACTH - corticotropin-releasing hormone (CRH) - associated neoplasms: - small cell carcinoma of the lung - carcinoid tumors b) SIADH* - mediators: - arginine vasopressin - atrial natriuretic peptide - associated neoplasms: - lung cancer, especially small cell carcinoma - head & neck cancer c) hypercalcemia* - mediators: - PTH-like peptide (PTHrP) - parathyroid hormone (PTH) - associated neoplasms: - lung cancer, especially squamous cell carcinoma - breast cancer - myeloma - renal cell carcinoma - bladder cancer - head & neck cancer d) carcinoid syndrome - mediators: - serotonin ? - substance P ? - associated neoplasms: - GI neuroendocrine tumors - sarcomas e) hypoglycemia - mediators: - insulin - insulin-like growth factors - associated neoplasms: GI neuroendocrine tumors f) acromegaly - mediators: - growth hormone-releasing hormone (GHRH) - growth hormone (GH) - associated neoplasms: - carcinoid - small cell lung cancer - pancreatic islet cell tumors g) gynecomastia - mediator: chorionic gonadotropin (hCG) - associated neoplasms: - testicular cancer - lung cancer - carcinoid tumors: lung, gastrointestinal tract 2) neurologic (see paraneoplastic neurologic syndrome) - sensorimotor peripheral neuropathy* 3) neuromuscular a) Lambert-Eaton syndrome - mediator: - antibody against voltage-sensitive Ca+2 channel - associated neoplasms: - small cell carcinoma of the lung - breast cancer - prostate cancer - gastric cancer - evolution over weeks to months - signs/symptoms - proximal muscle weakness - fatigability - dry mouth - pathology - disruption of active zone of presynaptic terminals b) myasthenia gravis - mediator: - antibody against acetylcholine receptor subunit - associated neoplasms: thymoma - evolution over weeks to months - signs/symptoms - weakness - fatigability - ptosis - diplopia - pathology - disruption of postsynaptic junctional membrane folds 4) muscular a) polymyositis - mediator: unknown - associated neoplasms: - lung cancer - breast cancer - ovarian cancer - lymphoma - evolution over months to years - signs/symptoms - proximal muscle weakness - myalgias - cardiomyopathy - pathology - elevated serum creatine kinase - lymphocytic infiltration of muscle interstitium - myofiber necrosis - phagocytosis b) necrotizing myopathy - mediator: unknown - associated neoplasms: - bronchial carcinoma - small cell lung cancer - evolution over days to weeks - signs/symptoms - rapidly progressive proximal muscle weakness - dysphagia may be present - dyspnea - pathology - severe myonecrosis with minimal inflammation or phagocytosis 5) skin a) dermatomyositis - mediator: unknown - associated neoplasms: - lung cancer - breast cancer b) acanthosis nigricans - mediator: TGF-alpha - associated neoplasms: - intra-abdominal cancer, generally gastric cancer 6) hematologic a) venous thrombosis* - mediator: activators of clotting cascade & platelets - associated neoplasms: - adenocarcinomas, especially pancreatic & gastric - lung cancer - breast cancer - genitourinary cancer - ovarian cancer - prostate cancer - lymphoma b) non-bacterial thrombotic endocarditis - mediator: activators of clotting cascade & platelets - associated neoplasms: - adenocarcinomas, especially pancreatic & gastric c) erythrocytosis - mediator: erythropoietin - associated neoplasms: - renal cancers - hepatocellular carcinoma - cerebellar hemangioblastoma d) granulocytosis - mediators: G-CSF, GM-CSF, IL-6 - associated neoplasms: - lung cancer - gastrointestinal cancer - ovarian cancer - genitourinary cancer e) thrombocytosis - mediator: IL-6 - associated neoplasms: - lung cancer - gastrointestinal cancer - breast cancer - ovarian cancer - lymphoma f) eosinophilia - mediator: IL-5 - associated neoplasms: - lymphoma - leukemia - lung cancer 7) idiopathic - cachexia-anorexia syndrome - mediators: IL-6, TNF-alpha, ADAMTS1 - associated neoplasms: - lung cancer - others - inflammatory polyarthritis or tendonitis - hypertrophic osteoarthropathy * most common paraneoplastic syndromes Etiology: - cause of paraneoplastic syndromes is generally autoimmune Clinical manifestations: - may present as unexplained subacute, progressive neurologic symptoms [3] Laboratory: - serum autoantibody

Interactions

disease interactions

Specific

carcinoid syndrome cutaneous papillomatosis ectopic ACTH syndrome erythema gyratum paraneoplastic cerebellar degeneration paraneoplastic dermatosis paraneoplastic neurologic syndrome paraneoplastic photoreceptor retinal degeneration; paraneoplastic retinopathy stiff-man syndrome tumor-induced osteomalacia (oncogenic osteomalacia) tylosis

General

autoimmune disease cancer complication syndrome

References

  1. Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 677-678
  2. Harrison's Principles of Internal Medicine, 14th ed. Fauci et al (eds), McGraw-Hill Inc. NY, 1998, pg 619, 621
  3. Medical Knowledge Self Assessment Program (MKSAP) 15, 16, 17. American College of Physicians, Philadelphia 2009, 2012, 2015
  4. Pelosof LC, Gerber DE. Paraneoplastic syndromes: an approach to diagnosis and treatment. Mayo Clin Proc. 2010 Sep;85(9):838-54. PMID: 20810794
  5. Titulaer MJ, Soffietti R, Dalmau J et al Screening for tumours in paraneoplastic syndromes: report of an EFNS task force. Eur J Neurol. 2011 Jan;18(1):19-e3 PMID: 20880069
  6. Vedeler CA, Antoine JC, Giometto B et al Management of paraneoplastic neurological syndromes: report of an EFNS Task Force. Eur J Neurol. 2006 Jul;13(7):682-90. PMID: 16834698
  7. Dimitriadis GK, Angelousi A, Weickert MO et al Paraneoplastic endocrine syndromes. Endocr Relat Cancer. 2017 Jun;24(6):R173-R190. PMID: 28341725 Review.
  8. NINDS Paraneoplastic Syndromes Information Page https://www.ninds.nih.gov/disorders/all-disorders/paraneoplastic-syndromes-information-page