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Regional Proteus syndrome: Report of an autopsy case
Author(s) -
Horie Yasushi,
Fujita Hajime,
Mano Shohei,
Kuwajima Minoru,
Ogawa Katsuo
Publication year - 1995
Publication title -
pathology international
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.73
H-Index - 74
eISSN - 1440-1827
pISSN - 1320-5463
DOI - 10.1111/j.1440-1827.1995.tb03497.x
Subject(s) - proteus syndrome , medicine , autopsy , hemihypertrophy , pathology , hemangioma , lipomatosis , anatomy , scalp
An uncommon autopsy case involving Proteus syndrome with multiple hamartomatous lesions in a 52 year old woman is reported. At birth, hemihypertrophy was noted on the right side of the face. Leiomyoma of the urinary bladder was extirpated at 37 years of age. She died of sepsis due to a brain abscess. At autopsy, diffuse asymmetrical lipomatosis was noted on the right side of the face, scalp, lip, oral mucosa, tongue and on the left side of the cerebellar peduncle. Muitiple meningiomas, cavernous hemangiomas and osseous hypertrophy overlapped in the intracranial regions. The present case is considered as a regional type of Proteus syndrome displaying a somatic mosaicism.