J Korean Pediatr Soc.  1994 Aug;37(8):1162-1168.

One Case of Maffucci's Syndrome with Testicular Teratoma

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 sysdrome 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.


MeSH Terms

Biopsy
Chondroma
Enchondromatosis
Hemangioma
Humans
Liver
Mesoderm
Ribs
Teratoma*
Tibia
Tomography, X-Ray Computed
Ultrasonography
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