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Review
. 2015 Jan;141(1):99-108.
doi: 10.1007/s00432-014-1745-9. Epub 2014 Jun 26.

Paraneoplastic neurological syndromes associated with ovarian tumors

Mikolaj Piotr Zaborowski  1 , Marek Spaczynski, Ewa Nowak-Markwitz, Slawomir Michalak
Affiliations
Review

Paraneoplastic neurological syndromes associated with ovarian tumors

Mikolaj Piotr Zaborowski et al. J Cancer Res Clin Oncol. 2015 Jan.

Abstract

Introduction: Paraneoplastic neurological syndromes (PNS) are neurologic deficits triggered by an underlying remote tumor. PNS can antedate clinical manifestation of ovarian malignancy and enable its diagnosis at an early stage. Interestingly, neoplasms associated with PNS are less advanced and metastasize less commonly than those without PNS. This suggests that PNS may be associated with a naturally occurring antitumor response.

Methods: We review the literature on the diagnosis, pathogenesis and management of PNS associated with ovarian tumors: paraneoplastic cerebellar degeneration (PCD) and anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis. An approach to the diagnostic workup of underlying tumors is discussed.

Results: PCD can precede the manifestation of ovarian carcinoma. Anti-NMDAR encephalitis in young women appears often as a result of ovarian teratoma. Since ovarian tumors and nervous tissue share common antigens (e.g., cdr2, NMDAR), autoimmune etiology is a probable mechanism of these neurologic disorders. The concept of cross-presentation, however, seems insufficient to explain entirely the emergence of PNS. Early resection of ovarian tumors is a significant part of PNS management and improves the outcome.

Conclusions: The diagnosis of PNS potentially associated with ovarian tumor indicates a need for a thorough diagnostic procedure in search of the neoplasm. In some patients, explorative laparoscopy/laparotomy can be considered.

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Conflict of interest statement

The authors have declared that no competing interests exist.

Figures

Fig. 1
Fig. 1
Ovarian cancer cell expresses cdr 2 antigen that triggers immune response against malignancy. The same cdr2 antigen is a intracellular protein in Purkinje cell in cerebellum. As a result, cytotoxic T lymphocytes (CTL) cross-react against nervous tissue. This mechanism represents the prevailing view on the pathogenesis of paraneoplastic cerebellar degeneration (PCD) related to ovarian cancer. The hallmarks of immune reaction, however, are not detected in all patients
Fig. 2
Fig. 2
NMDA receptor (NMDAR) is expressed on the surface of ovarian teratoma cells. As a result of immune reaction, anti-NNMAR antibodies are produced. The same receptor is present at the dendrites of neurons in many region of central nervous system. Anti-NMDAR antibodies enter nervous tissue through the vasculature and react against the target protein. Consequently, neural signaling mediated by NMDA receptor is considerably disturbed leading to both psychiatric and neurologic symptoms
See this image and copyright information in PMC

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