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One Case of Maffucco's Syndrome with Testicular Teratoma

Journal of the Korean Pediatric Society 1994;37(8):1162-1168.
Published online August 15, 1994.
One Case of Maffucco's Syndrome with Testicular Teratoma
Seung Mo Park1, Duk Hi Kim1, Ho Seong Kim2
1Department of Pediatrics, Yonsei University, College of Medicine, Seoul, Korea
2Department of Pediatrics, College of Medicine, Ewha Womans University, Seoul, Korea
고환 종양을 동반한 Maffucci증후군 1례
박승모1, 김덕희1, 김호성2
1연세대학교 의과대학 소아과학교실
2이화대학교 의과대학 소아과학교실
Abstract
Maffucci's syndrome is a very rare, congenital and non-hereditary mesodermal dysplasia manifested by multiple enchondromas and soft tissue hemangiomas. Since Maffucci had reported this syndrome in 1881, there have been more than 100 cases reported, and also there has been reported that Maffuddi's syndrome has various interstitial tumor. However there hasn't been any report about Maffucci's syndrome with testicular teratoma. Here we report in this paper that the patient was diagnosed as Maffucci's syndrome of enchondroma and liver hamangioma and also had testicular teratoma. He was admitted for the evaluation of gynecomatia and diagnosed as enchondroma by bone biopsy of the right rib and tibia. Liver hemangioma was also found through abdominal ultrasonogram. CT scan and liver biopsy. And the testicular teratoma was confirmed through testicular biopsy. He is finally diagnosed as Maffucci's syndrome with testicular teratoma and literatures were reviewed.
Key Words: Maffucci's sydrome, Enchondroma, Hemangioma, Testicular teratoma


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