A Case of Maffucci's Syndrome Associated with Spindle-cell Hemangioendothelioma.
10.5021/ad.1998.10.3.203
- Author:
Kang Seok LEE
;
Byung Hwan CHUN
;
Seong Jun SEO
;
Chang Kwun HONG
;
Byung In RO
- Publication Type:Case Report
- Keywords:
Maffucci's syndrome;
Spindle-cell hemangioendothelioma;
Enchondroma
- MeSH:
Chondroma;
Female;
Fibroblasts;
Hand;
Hemangioendothelioma*;
Humans;
Juglans;
Peas;
Skin Pigmentation;
Upper Extremity
- From:Annals of Dermatology
1998;10(3):203-207
- CountryRepublic of Korea
- Language:English
-
Abstract:
A case of a 21-year old female with Maffucci's syndrome is presented where the patient developed multiple cutaneous spindle-cell hemangioendotheliomas. She had multiple pea to walnut sized, non-tender, normal skin colored or slight bluish nodules and bony mass-like lesions on the left upper extremity and hand. A histological examination of the lesions showed that they were composed of irregularly dilated, thin walled cavernous blood spaces containing phleboliths and collapsed vascular spaces separated by spindled fibroblastic cells. Radiologically, the bony lesions showed radiolucent densities with calcified spots within the second proximal phalanx and metacarpal bone of the left hand, which were consistent with enchondromas.
