Paraneoplastic neurological syndromes associated with ovarian tumors.

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

doi:10.1007/s00432-014-1745-9

Mikolaj Piotr Zaborowski, Slawomir Michalak + Show 2 more

Open Access

https://doi.org/10.1007/s00432-014-1745-9

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Abstract

IntroductionParaneoplastic 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.MethodsWe 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.ResultsPCD 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.ConclusionsThe 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.

Highlights

Paraneoplastic neurological syndromes (PNS) are neurologic deficits triggered by an underlying remote tumor
Anti-NMDAR encephalitis that affects women with ovarian teratoma responds well to tumor resection and subsequent immunotherapy which may lead to almost complete recovery
Ovarian tumors associated with anti-NMDAR encephalitis include mainly teratomas (Dalmau et al 2007, 2011)

Summary

Introduction

Paraneoplastic neurological syndromes (PNS) are neurologic deficits triggered by an underlying remote tumor. Anti-NMDAR antibodies detected in patient affected with encephalitis are highly suggestive of ovarian teratoma. Anti-NMDAR encephalitis that affects women with ovarian teratoma responds well to tumor resection and subsequent immunotherapy which may lead to almost complete recovery. 3. A non-classical syndrome with onconeural antibodies (well characterized or not) and cancer that develops within 5 years of the diagnosis of the neurological disorder

Results

Conclusion