| 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 |
| neurofibromin |
| 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) |
| URI: | http://hdl.handle.net/2115/17243 |
| 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)
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