Abstract
Antibodies to SOXB1 proteins in patients with paraneoplastic disorders are associated with small-cell lung cancer (SCLC), particularly in Lambert-Eaton myasthenic syndrome (LEMS). We aimed to establish if SOX2 antibodies could be used to identify SCLC and other tumours found in a range of paraneoplastic disorders and controls.SOX2 antibodies were detectable in 61% of patients with LEMS-SCLC, and in other paraneoplastic disorders, such as opsoclonus-myoclonus and paraneoplastic cerebellar degeneration, only when there was an underlying SCLC.SOX2 antibodies are specific (>90%) markers for SCLC, but are rarely found in patients with other tumours, whether neurological symptoms are present or not.
Highlights
Paraneoplastic neurological disorders (PNDs) are a diverse group of conditions in which distant tumour effects are thought to underlie the neurological presentation
There were only 6/126 (4.8%) Lambert-Eaton myasthenic syndrome (LEMS) patients whose Sry-like high-mobility group box protein 2 (SOX2) enzyme linked immunosorbent assay (ELISA) results from Nottingham differed from those performed in duplicate in Leiden: subsequent SOX2 western blotting performed at Nottingham in three of these patients, who were initially negative on SOX2 ELISA, were all positive, improving concordance between laboratories in all cases
Patients with small-cell lung cancer (SCLC) without neurological symptoms were similar in age, percentage female and SCLC disease extent at cancer diagnosis to those SCLC patients with neurological presentations whether they had SOX2 antibodies or not (Supplementary table 1). It was following the identification of a unique pattern of immunohistochemical staining of the Bergmann glia of the cerebellum in patients with paraneoplastic neurological disorders associated with SCLC that the putative antigen, SOX1, was discovered to be the antigen recognised by these AGNA-positive sera (Graus et al, 2005; Sabater et al, 2008)
Summary
Paraneoplastic neurological disorders (PNDs) are a diverse group of conditions in which distant tumour effects are thought to underlie the neurological presentation. These effects are generally thought to be due to an immune-mediated process with cell-mediated and humoral components. Patients with particular paraneoplastic neurological phenotypes often develop specific tumours (e.g. SCLC found in over 90% of paraneoplastic LEMS (Titulaer et al, 2011a) and ovarian teratoma in over 90% of patients with anti-NMDA-R encephalitis (Titulaer et al, 2013)), this is not always the case and some PND presentations can be associated with a number of different cancers (e.g. LE seen with tumours of the lung, testis, and breast, and with Hodgkin's lymphoma, teratoma, or thymoma) (Gozzard and Maddison, 2010)
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