Unusual Paraneoplastic Syndrome Accompanies Neuroendocrine Tumours of the Pancreas

Abstract EN

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.