A CASE OF COBB SYNDROME ASSOCIATED WITH RETRO-PERITONEAL GIANT LYMPHANGIOMA

Abstract

A 19-year-old woman was admitted to the hospital because of abdominal distension and an intra-abdominal cystic tumor. She had several hemangiomas in the right side of the body and a history of undergo-ing a resection of cervical spinal hemangioma at the age of 13 years old, when she was diagnosed as having Cobb syndrome. On admission, numerous angiomas covering from the right jaw to right thorasic part were seen, and a soft tumor extending to under the umbilicus was palpated. Abdominal ultrasonography, CT scan, and magnetic resonance imaging revealed a multilocular cystic tumor 14cm in diameter adjacent to the right kidney. It was diagnosed as retroperitoneal giant lymphangioma. At operation, a sharply demarcated, yellowish white cystic tumor was present which was successfully removed after communication with retroperitoneal lymphatic vessel under the left renal vein was confirmed. Histopathological diagnosis was lymphangioma. Cobb syndrome consists of a vascular skin nevus associated with an angioma in spinal cord and only about 40 cases were reported in the literature. This case had hemangiomas in other than the same derma-tomes as spinal hemangioma, and the abdominal lymphangioma might be considered as a finding of Cobb syn-drome.