2024-03-28T19:49:43Zhttps://eprints.lib.hokudai.ac.jp/dspace-oai/requestoai:eprints.lib.hokudai.ac.jp:2115/172432022-11-17T02:08:08Zhdl_2115_20043hdl_2115_137Hypokalemic rhabdomyolysis due to WDHA syndrome caused by VIP-producing composite pheochromocytoma in a case of neurofibromatosis type 1Onozawa, MasahiroFukuhara, TakashiMinoguchi, MadokaTakahata, MutsumiYamamoto, YasushiMiyake, TakayoshiKanagawa, KoichiKanda, MakotoMaekawa, Isaocomposite pheochromocytomavasoactive intestinal polypeptidehypokalemic rhabdomyolysiswatery diarrhea, hypokalemia and achlorhydria syndromeneurofibromin493.29A 47-year-old woman with neurofibromatosis type 1 suffered from general muscle weakness and watery diarrhea. Laboratory findings showed elevated muscular enzymes, severe hypokalemia and excessive production of catecholamines and vasoactive intestinal polypeptide (VIP). A computed tomography scan showed a 10 cm left adrenal mass, in which [131I]-metaiodobenzylguanidine scintigraphy showed high uptake. After she underwent surgical removal of the tumor, all the symptoms and signs subsided. A histological study revealed that the mass consisted of pheochromocytoma and ganglioneuroma, respectively producing catecholamines and VIP. In immunohistochemical staining of neurofibromin, pheochromocytoma and ganglion cells showed positive staining, whereas the staining was negative for nerve bundles and Schwann cells. We concluded that the patient had hypokalemic rhabdomyolysis due to watery diarrhea, hypokalemia and achlorhydria (WDHA) syndrome, which was induced by a VIP-producing composite pheochromocytoma. Composite pheochromocytoma is a neuroendocrine tumor that is composed of pheochromocytoma and ganglioneuroma, both derived from the neural crest. Deficiency of neurofibromin in Schwann cells might have played an important role in the development and the growth of the composite pheochromocytoma in this patient.Oxford University PressJournal Articleapplication/pdfhttp://hdl.handle.net/2115/17243https://eprints.lib.hokudai.ac.jp/dspace/bitstream/2115/17243/1/JJCO35-9.pdf0368-28111465-3621Japanese Journal of Clinical Oncology3595595632005-09enginfo:pmid/16027147info:doi/10.1093/jjco/hyi139© 2005 Foundation for Promotion of Cancer Researchauthor