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Japanese Journal of Clinical Oncology Advance Access originally published online on July 18, 2005
Japanese Journal of Clinical Oncology 2005 35(9):559-563; doi:10.1093/jjco/hyi139
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© 2005 Foundation for Promotion of Cancer Research

Case Report

Hypokalemic Rhabdomyolysis due to WDHA Syndrome Caused by VIP-producing Composite Pheochromocytoma: A Case in Neurofibromatosis Type 1

Masahiro Onozawa1, Takashi Fukuhara1, Madoka Minoguchi1, Mutsumi Takahata1, Yasushi Yamamoto1, Takayoshi Miyake1, Koichi Kanagawa2, Makoto Kanda3 and Isao Maekawa1

1 Department of Internal Medicine, 2 Department of Urology and 3 Department of Pathology, Asahikawa City Hospital, Asahikawa, Hokkaido, Japan

For reprints and all correspondence: Masahiro Onozawa, Department of Gastroenterology and Hematology, Hokkaido University Graduate School of Medicine, Kita 14, Nishi 5, Kita-ku, Sapporo 060-8638, Hokkaido, Japan. E-mail: masahiro.onozawa{at}nifty.ne.jp

Received April 16, 2005; accepted June 1, 2005

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.

Key Words: composite pheochromocytoma • vasoactive intestinal polypeptide • hypokalemic rhabdomyolysis • watery diarrhea, hypokalemia and achlorhydria syndrome • neurofibromin


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