Familial gigantiform cementoma

Citation
Sj. Finical et al., Familial gigantiform cementoma, PLAS R SURG, 103(3), 1999, pp. 949-954
Citations number
16
Categorie Soggetti
Surgery,"Medical Research Diagnosis & Treatment
Journal title
PLASTIC AND RECONSTRUCTIVE SURGERY
ISSN journal
00321052 → ACNP
Volume
103
Issue
3
Year of publication
1999
Pages
949 - 954
Database
ISI
SICI code
0032-1052(199903)103:3<949:FGC>2.0.ZU;2-3
Abstract
Familial gigantiform cementoma is a rare autosomal dominant tumor that is b enign but can result in disfigurement of the facial skeleton. Two families with a total of five patients presented for treatment. Because of a lack of opportunity to obtain treatment early, three of the patients presented in adult life with massive tumors requiring extensive resection and complex re construction in multiple stages. The two female patients had chronic anemia caused by multifocal polypoid adenomas of the uterus and required hysterec tomy before treatment The last three patients had elevated alkaline phospha tase levels before turner resection, and these levels decreased after surge ry. With extensive resection of the tumors and reconstruction of both the s oft tissues and facial skeleton, goad functional and aesthetic results can be obtained. There has been no tumor recurrence with 3 years of follow-up.