TY  - JOUR
AU  - Stingu, Elena
AU  - Dobrowolski, Jerome-Maurice
AU  - Bombach, Paula
AU  - Nann, Dominik
AU  - Singer, Stephan
AU  - Horger, Marius
AU  - Lauer, Ulrich M
AU  - Zender, Lars
AU  - Hinterleitner, Clemens
AU  - Hinterleitner, Martina
TI  - Myasthenia gravis as initial presentation of a pancreatic neuroendocrine tumor: A case report.
JO  - Experimental and Therapeutic Medicine
VL  - 26
IS  - 5
SN  - 1792-0981
CY  - Athens
PB  - Spandidos Publ.
M1  - DKFZ-2023-02109
SP  - 523
PY  - 2023
AB  - Myasthenia gravis (MG) is a heterogeneous autoimmune disease, which is characterized by a postsynaptic neuromuscular transmission defect, with antibodies directly targeting the acetylcholine receptor (AChR) or other structural proteins of the neuromuscular junction. The majority of MG cases are associated with thymic pathologies, including thymoma, thyroiditis, autoimmune diseases or malignant hematologic neoplasia. The present study reported a rare case of AChR-positive and late-onset ocular MG, which rapidly progressed to a generalized myasthenic syndrome as an initial presentation of a pancreatic neuroendocrine neoplasia (pNEN). Following complete surgical resection of the pNEN, the myasthenic syndrome was improved and the anti-AChR antibody titers were reduced. It has been reported that MG is a paraneoplastic syndrome in thymic neoplasms and less common in hematologic malignancies. However, currently, only few cases of MG as initial presentation of a solid tumor, and more particular of a neuroendocrine neoplasm, have been reported in the literature. In conclusion, surveillance for extrathymic solid malignancies in newly diagnosed patients with MG could promote the early diagnosis of associated tumor diseases.
KW  - acetylcholine receptor (Other)
KW  - chromogranin A (Other)
KW  - myasthenia gravis (Other)
KW  - pancreatic neuroendocrine neoplasia (Other)
KW  - paraneoplastic syndrome (Other)
KW  - solid tumor (Other)
LB  - PUB:(DE-HGF)16
C6  - pmid:37854502
C2  - pmc:PMC10580239
DO  - DOI:10.3892/etm.2023.12222
UR  - https://inrepo02.dkfz.de/record/284810
ER  -