paraneoplastic syndrome
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Introduction
Syndrome resulting from distant effects of a neoplasm.
Classification
- endocrine
- Cushing's syndrome*
- mediators:
- associated neoplasms:
- SIADH*
- mediators:
- associated neoplasms:
- lung cancer, especially small cell carcinoma
- head & neck cancer
- hypercalcemia*
- mediators:
- PTH-like peptide (PTHrP)
- parathyroid hormone (PTH)
- associated neoplasms:
- mediators:
- carcinoid syndrome
- mediators:
- serotonin ?
- substance P ?
- associated neoplasms:
- mediators:
- hypoglycemia
- mediators:
- associated neoplasms: GI neuroendocrine tumors
- acromegaly
- mediators:
- associated neoplasms:
- carcinoid
- small cell lung cancer
- pancreatic islet cell tumors
- gynecomastia
- mediator: chorionic gonadotropin (hCG)
- associated neoplasms:
- Cushing's syndrome*
- neurologic (see paraneoplastic neurologic syndrome)
- neuromuscular
- Lambert-Eaton syndrome
- mediator:
- associated neoplasms:
- evolution over weeks to months
- signs/symptoms
- proximal muscle weakness
- fatigability
- dry mouth
- pathology
- disruption of active zone of presynaptic terminals
- myasthenia gravis
- mediator:
- antibody against acetylcholine receptor subunit
- associated neoplasms: thymoma
- evolution over weeks to months
- signs/symptoms
- pathology
- disruption of postsynaptic junctional membrane folds
- mediator:
- Lambert-Eaton syndrome
- muscular
- polymyositis
- mediator: unknown
- associated neoplasms:
- evolution over months to years
- signs/symptoms
- pathology
- elevated serum creatine kinase
- lymphocytic infiltration of muscle interstitium
- myofiber necrosis
- phagocytosis
- 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
- polymyositis
- skin
- dermatomyositis
- mediator: unknown
- associated neoplasms:
- acanthosis nigricans
- mediator: TGF-alpha
- associated neoplasms:
- intra-abdominal cancer, generally gastric cancer
- dermatomyositis
- hematologic
- venous thrombosis*
- non-bacterial thrombotic endocarditis
- mediator: activators of clotting cascade & platelets
- associated neoplasms:
- adenocarcinomas, especially pancreatic & gastric
- erythrocytosis
- mediator: erythropoietin
- associated neoplasms:
- granulocytosis
- thrombocytosis
- eosinophilia
- idiopathic
- cachexia-anorexia syndrome
- mediators: IL-6, TNF-alpha, ADAMTS1
- associated neoplasms:
- lung cancer
- others
- inflammatory polyarthritis or tendonitis
- hypertrophic osteoarthropathy
- cachexia-anorexia syndrome
* most common paraneoplastic syndromes
Etiology
- cause of paraneoplastic syndromes is generally autoimmune
Clinical manifestations
- may present as unexplained subacute, progressive neurologic symptoms[3]
Laboratory
More general terms
More specific terms
- 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
References
- ↑ Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 677-678
- ↑ Harrison's Principles of Internal Medicine, 14th ed. Fauci et al (eds), McGraw-Hill Inc. NY, 1998, pg 619, 621
- ↑ 3.0 3.1 Medical Knowledge Self Assessment Program (MKSAP) 15, 16, 17. American College of Physicians, Philadelphia 2009, 2012, 2015
- ↑ Pelosof LC, Gerber DE. Paraneoplastic syndromes: an approach to diagnosis and treatment. Mayo Clin Proc. 2010 Sep;85(9):838-54. PMID: https://www.ncbi.nlm.nih.gov/pubmed/20810794
- ↑ 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: https://www.ncbi.nlm.nih.gov/pubmed/20880069
- ↑ 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: https://www.ncbi.nlm.nih.gov/pubmed/16834698
- ↑ Dimitriadis GK, Angelousi A, Weickert MO et al Paraneoplastic endocrine syndromes. Endocr Relat Cancer. 2017 Jun;24(6):R173-R190. PMID: https://www.ncbi.nlm.nih.gov/pubmed/28341725 Review.
- ↑ NINDS Paraneoplastic Syndromes Information Page https://www.ninds.nih.gov/disorders/all-disorders/paraneoplastic-syndromes-information-page