Proteus syndrome with giant myelolipoma in the pelvis.

Abstract

Proteus syndrome is a very rare and complex disorder with malformations and overgrowth of multiple tissues. This disorder was designated Proteus syndrome by Wiedemann et al. to denote its variable clinical expression. Our patient presented with macrodactyly, cerebriform appearance and a huge abdominal mass. A biopsy under laparotomy was performed, and histopathological examination revealed myelolipoma. Tumor resection was performed. To our knowledge, no case of Proteus syndrome presenting a myelolipoma in the retroperitoneal cavity has been reported before.

• Authors: Shinya Matsumoto, Satoru Takahashi, Takahiro Goto, Kyoichi Tomita, Takumi Takeuchi, Tadaichi Kitamura
• Journal: International journal of urology : official journal of the Japanese Urological Association (Int J Urol) Vol. 12 Issue 11 Pg. 1005-6 (Nov 2005) ISSN: 0919-8172 [Print] Australia
• PMID: 16351661 (Publication Type: Case Reports, Journal Article)

Topics

• Humans
• Male
• Middle Aged
• Myelolipoma (diagnosis, surgery)
• Proteus Syndrome (diagnosis)
• Retroperitoneal Neoplasms (diagnosis, surgery)