Paraneoplastic cerebellar degeneration associated with lymphoepithelial carcinoma of the tonsil
1 Department of Neurology, Goethe University Frankfurt, Theodor-Stern-Kai 7, 60590 Frankfurt, Germany
2 Institute of Neurooncology, Goethe University Frankfurt, Senckenberganlage 31, 60325 Frankfurtm, Germany
3 Institute of Pathology, Goethe University Frankfurt, Senckenberganlage 31, 60325 Frankfurtm, Germany
4 Department of Nuclear Medicine, Goethe University Frankfurt, Senckenberganlage 31, 60325 Frankfurtm, Germany
5 Department of Neurology and Stroke, Hertie Institute for Clinical Brain Research, Eberhard-Karls-University Tübingen, 72076 Tübingen, Germany
BMC Neurology 2013, 13:147 doi:10.1186/1471-2377-13-147Published: 17 October 2013
Paraneoplastic cerebellar degeneration (PCD) is a classical tumor-associated, immune-mediated disease typically associated with gynecological malignancies, small-cell lung-cancer or lymphoma.
Here we present the case of a 38-year old male with an over 12 months rapidly progressive cerebellar syndrome. Extensive diagnostic workup revealed selective hypermetabolism of the right tonsil in whole-body PET. Histological examination after tonsillectomy demonstrated a lymphoepithelial carcinoma of the tonsil and the tongue base strongly suggesting a paraneoplastic cause of the cerebellar syndrome. To the best of our knowledge this is the first case of an association of a lymphoepithelial carcinoma, a rare pharyngeal tumor, with PCD.
In cases of classical paraneoplastic syndromes an extensive search for neoplasms should be performed including whole-body PET to detect tumors early in the course of the disease.