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Hypokalemic rhabdomyolysis due to WDHA syndrome caused by VIP-producing composite pheochromocytoma in a case of neurofibromatosis type 1

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Title: Hypokalemic rhabdomyolysis due to WDHA syndrome caused by VIP-producing composite pheochromocytoma in a case of neurofibromatosis type 1
Authors: Onozawa, Masahiro Browse this author
Fukuhara, Takashi Browse this author
Minoguchi, Madoka Browse this author
Takahata, Mutsumi Browse this author
Yamamoto, Yasushi Browse this author
Miyake, Takayoshi Browse this author
Kanagawa, Koichi Browse this author
Kanda, Makoto Browse this author
Maekawa, Isao Browse this author
Keywords: composite pheochromocytoma
vasoactive intestinal polypeptide
hypokalemic rhabdomyolysis
watery diarrhea, hypokalemia and achlorhydria syndrome
Issue Date: Sep-2005
Publisher: Oxford University Press
Journal Title: Japanese Journal of Clinical Oncology
Volume: 35
Issue: 9
Start Page: 559
End Page: 563
Publisher DOI: 10.1093/jjco/hyi139
PMID: 16027147
Abstract: A 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.
Rights: © 2005 Foundation for Promotion of Cancer Research
Type: article (author version)
Appears in Collections:北海道大学病院 (Hokkaido University Hospital) > 雑誌発表論文等 (Peer-reviewed Journal Articles, etc)

Submitter: 小野澤 真弘

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