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Japanese Journal of Clinical Oncology Advance Access published online on July 18, 2005
Japanese Journal of Clinical Oncology, doi:10.1093/jjco/hyi139
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© 2005 Foundation for Promotion of Cancer Research
Received April 16, 2005
Accepted June 1, 2005

Case Report

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

Masahiro Onozawa 1*, Takashi Fukuhara 1, Madoka Minoguchi 1, Mutsumi Takahata 1, Yasushi Yamamoto 1, Takayoshi Miyake 1, Koichi Kanagawa 2, Makoto Kanda 3, and Isao Maekawa 1

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

* To whom correspondence should be addressed.
Masahiro Onozawa, E-mail: masahiro.onozawa{at}nifty.ne.jp


  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.

Keywords: composite pheochromocytoma; vasoactive intestinal polypeptide; hypokalemic rhabdomyolysis; watery diarrhea, hypokalemia and achlorhydria syndrome; neurofibromin.
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