Paraneoplastic neurologic syndrome in ovarian carcinoma

Abstract

Objectives: Paraneoplastic neurologic syndrome (PNS) is a rare entity in ovarian cancer. The objective of this study was to assess the incidence, clinicopathologic features, and survival outcomes of patients with ovarian, primary peritoneal, or fallopian tube cancer (OC) diagnosed with PNS. Methods: We performed a retrospective review of all patients diagnosed with OC and PNS between 1995 and 2013. Characteristics of epithelial OC (EOC) with and without PNS diagnosed between 2003 and 2012 were also compared. Fisher's exact and Wilcoxon rank sum tests were used to compare categorical and continuous variables, respectively. Survival was estimated and compared using the product limit estimator of Kaplan–Meier and log-rank statistic. Results: Of the 4552 OC patients, 15 (0.3%) were diagnosed with PNS, 47% prior to OC diagnosis, 33% during primary treatment, and 20% at recurrence. Sixty-seven percent (n = 10) were confirmed with onconeural antibodies, primarily anti-Yo (9/10, 90%). Among patients with PNS, median age was 59 years (range, 21–74 years). Tumor histology was predominantly serous (67%), mixed epithelial (13%), or immature teratoma (7%). PNS presented with muscle weakness (53%), ataxia (47%), confusion (33%), fatigue (27%), nystagmus (20%), multifocal neuropathy (13%), altered mental status (13%), and paresthesias (13%). Median duration of PNS symptoms was 18 months (range, 2–68 months). Treatments for PNS, given alone or in combination, included intravenous immunoglobulin (67%), corticosteroids (53%), plasmapheresis (47%), and chemotherapy (20%). Overall, only 33% of patients responded completely to PNS therapy. Sixty percent required greater than one line of therapy. If PNS symptoms were not resolve after first-line therapy, complete resolution of symptoms was unlikely. In the analysis of 563 EOC, compared to patients without PNS (n = 553), patients with PNS (n = 10) had worse performance status (P= 0.005) and a trend toward higher median CA-125 levels at diagnosis (536 U/mL vs. 2580 U/mL, P = 0.08). There was no difference in OS between the two groups (34.9 vs. 59.5 months, P = 0.23). Conclusions: In our population, PNS was an uncommon complication but was more commonly seen in EOC. In general, complete response to PNS therapy was low, with a significant number of patients experiencing persistence of symptoms after treatment. Patients with PNS had no difference in OS compared to patients without PNS.