Paraneoplastic neurological syndromes associated with ovarian tumors

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.

Paraneoplastic Cholestasis Associated With Ovarian Dysgerminoma

“…[2][3][4] Similar paraneoplastic manifestations were noted in three patients with dysgerminomas. [6][7][8] Individual cases of paraneoplastic neurologic syndromes have also been reported in patients with teratomas [16][17][18] as well as hypercalcemia mediated by parathyroid-related protein 19 and a case of Cushing syndrome resulting from ectopic adrenocorticotropic hormone secretion caused by primary ovarian mature teratoma with carcinoid components.…”

“…These conditions include anti-N-methyl-d-aspartate receptor encephalitis, subacute cerebellar degeneration, peripheral neuropathy, Lambert-Eaton syndrome, cerebral ataxia, opsoclonus-myoclonus, and limbic encephalitis. 3,4 Most paraneoplastic syndromes have been reported with serous ovarian carcinomas. We report an unusual case of paraneoplastic cholestasis associated with an uncommon ovarian neoplasm, dysgerminoma.…”

Paraneoplastic cerebellar degeneration secondary to ovarian carcinosarcoma: a cerebellar conundrum

“…It is believed that PCD is triggered by an abnormal immune system response to an underlying malignant tumour, due to the sharing of common antigens with nervous tissue 3. Postmortem studies on patients with PCD have shown a significant loss of Purkinje cells and inflammatory infiltrates in the early stages of the disease 2.…”

Chronic Neuropsychological Sequelae in a Patient with Nontumorous Anti-NMDA-Receptor Encephalitis

“…The lack of receptors causes neuropsychiatric symptoms, attributable to ovarian teratomas and autoimmune processes in approximately half of the cases [2–4, 6, 8]. Recognizing the symptom complex is crucial to diagnosis [7].…”