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Unusual Paraneoplastic Syndrome Accompanies Neuroendocrine Tumours of the Pancreas
Author(s) -
Helga Bertani,
A. Messerotti,
Fabrizio Di Benedetto,
Raffaele Manta,
M. Greco,
Federica Casoni,
Luisa Losi,
Rita Conigliaro
Publication year - 2011
Publication title -
case reports in medicine
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.2
H-Index - 20
eISSN - 1687-9627
pISSN - 1687-9635
DOI - 10.1155/2011/309149
Subject(s) - medicine , enucleation , pancreas , myasthenia gravis , pathology , autoimmune pancreatitis , disease , surgery
Neuroendocrine tumours comprise a small percentage of pancreatic neoplasia (10%) (1). Diagnosis of neuroendocrine tumours is difficult, especially if the tumours are small and nonfunctional. CT scans, MRI, and nuclear scans are sufficiently sensitive assessment tools for tumours with diameters of at least 2 cm; otherwise, the sensitivity and specificity of these techniques is less than 50% (2). Myasthenia gravis (MG) is a heterogeneous neuromuscular junction disorder that is primarily caused when antibodies form against the acetylcholine receptors (Ab-AchR). MG can develop in conjunction with neoplasia, making MG a paraneoplastic disease. In those cases, MG is most commonly associated with thymomas and less frequently associated with extrathymic malignancies. The mechanism underlying this paraneoplastic syndrome has been hypothesized to involve an autoimmune response against the tumour cells (3). No published reports have linked malignant pancreatic diseases with MG. Here, we report the case of a young woman, negative for Ab-AchR, with a neuroendocrine tumour in the pancreatic head, who experienced a complete resolution of her MG-like syndrome after surgical enucleation of the tumour.

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