This is a Continuing Medical Education article, and can be found with corresponding questions on the Internet at http://www.efns.org/EFNSContinuing-Medical-Education-online.301.0.html. Certificates for correctly answering the questions will be issued by the EFNS.
Screening for tumours in paraneoplastic syndromes: report of an EFNS Task Force
Article first published online: 28 SEP 2010
© 2010 The Author(s). European Journal of Neurology © 2010 EFNS
European Journal of Neurology
Volume 18, Issue 1, pages 19–e3, January 2011
How to Cite
Titulaer, M. J., Soffietti, R., Dalmau, J., Gilhus, N. E., Giometto, B., Graus, F., Grisold, W., Honnorat, J., Sillevis Smitt, P. A. E., Tanasescu, R., Vedeler, C. A., Voltz, R. and Verschuuren, J. J. G. M. (2011), Screening for tumours in paraneoplastic syndromes: report of an EFNS Task Force. European Journal of Neurology, 18: 19–e3. doi: 10.1111/j.1468-1331.2010.03220.x
- Issue published online: 15 DEC 2010
- Article first published online: 28 SEP 2010
- Received 15 July 2010 Accepted 24 August 2010
Background: Paraneoplastic neurological syndromes (PNS) almost invariably predate detection of the malignancy. Screening for tumours is important in PNS as the tumour directly affects prognosis and treatment and should be performed as soon as possible.
Objectives: An overview of the screening of tumours related to classical PNS is given. Small cell lung cancer, thymoma, breast cancer, ovarian carcinoma and teratoma and testicular tumours are described in relation to paraneoplastic limbic encephalitis, subacute sensory neuronopathy, subacute autonomic neuropathy, paraneoplastic cerebellar degeneration, paraneoplastic opsoclonus-myoclonus, Lambert-Eaton myasthenic syndrome (LEMS), myasthenia gravis and paraneoplastic peripheral nerve hyperexcitability.
Methods: Many studies with class IV evidence were available; one study reached level III evidence. No evidence-based recommendations grade A–C were possible, but good practice points were agreed by consensus.
Recommendations: The nature of antibody, and to a lesser extent the clinical syndrome, determines the risk and type of an underlying malignancy. For screening of the thoracic region, a CT-thorax is recommended, which if negative is followed by fluorodeoxyglucose-positron emission tomography (FDG-PET). Breast cancer is screened for by mammography, followed by MRI. For the pelvic region, ultrasound (US) is the investigation of first choice followed by CT. Dermatomyositis patients should have CT-thorax/abdomen, US of the pelvic region and mammography in women, US of testes in men under 50 years and colonoscopy in men and women over 50. If primary screening is negative, repeat screening after 3–6 months and screen every 6 months up till 4 years. In LEMS, screening for 2 years is sufficient. In syndromes where only a subgroup of patients have a malignancy, tumour markers have additional value to predict a probable malignancy.