CNS tumor
Metastatic tumors
Paraneoplastic syndromes

Author: Nat Pernick, M.D. (see Authors page)

Revised: 12 September 2017, last major update January 2006

Copyright: (c) 2002-2017, PathologyOutlines.com, Inc.

PubMed Search: "Paraneoplastic syndromes" CNS

Cite this page: Pernick, N. Paraneoplastic syndromes. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/cnstumorparaneoplastic.html. Accessed October 19th, 2017.
Definition / general
  • Nervous system dysfunction associated with cancer but without direct tumor invasion, infection or vascular complications of neural tissue
  • May precede clinical recognition of tumors
  • Occurs in < 1% of cancer patients; usually small cell carcinoma of lung, breast cancer or ovarian cancer
  • Often due to autoantibodies
  • May respond to treatment of the underlying tumors
Cerebellar degeneration
Definition / general:
  • Destruction of Purkinje cells (antibody mediated) with perivascular lymphocytes
  • Causes progressive bilateral leg and arm ataxia, dysarthria, variable vertigo and diplopia
  • Associated with breast, gynecologic, Hodgkin lymphoma and small cell carcinoma of lung
  • Often associated with anti-Yo antibody in serum or CSF
  • References: Brain 2003;126:1409
Lambert-Eaton myasthenic syndrome
Definition / general:
  • Destruction of neuromuscular junction (presynaptic terminals) by small cell lung cancer; due to IgG antibody
Limbic encephalitis
Definition / general:
  • Associated with subacute dementia - perivascular inflammatory cuffs, microglial nodules, neuronal loss, gliosis of temporal lobe and resembles infectious process
  • Also in brainstem; most common with small cell lung cancer
Myelopathies
Definition / general:
  • Necrotizing myelopathy due to leukemia, lymphoma and lung carcinoma
  • Myelitis due to anti-Hu antibody associated with small cell lung carcinoma
Opsoclonus / myoclonus (spontaneous chaotic eye movements)
Definition / general:
  • Affects brain stem but site unknown
  • Due to breast and small cell lung cancer; also neuroblastoma
  • May be associated with anti-Ri autoantibody
Peripheral neuropathy
Definition / general:
  • Most common paraneoplastic syndrome
  • May be associated with anti-Hu antibody in lung cancer patients
Primary lateral sclerosis
Definition / general:
  • Rarely caused by breast cancer
  • 50% develop lower motor neuron signs eventually
Retinal degeneration
Definition / general:
  • Destruction of photoreceptors
  • Due to small cell carcinoma and gynecologic tumors
Stiff man syndrome
Definition / general:
  • Destruction of spinal interneurons by amphiphysin antibody
  • Usually breast cancer, also lung cancer and thymoma
Subacute sensory neuropathy
Definition / general:

Micro images:

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Dorsal root ganglionitis

Subacute motor neuronopathy
Definition / general:

Case reports:

Differential diagnosis: