안면부 선천성 침윤성 지방종증(congenital infiltrating lipomatosis)의 치험례

Abstract

Some confusion in the literature regarding the embryology, pathogenesis, and classification of fatty tumors may have delayed recognition of the important group of infiltrating lipomatoses. Lipomatoses are defined as infiltrating and/or diffuse neoplastic or non-neoplastic proliferations of mature fat tissue that resemble and may be confused with well-differentiated liposarcoma. There had been 3 clinical entities under the broad heading of lipomatosis, namely; cervical lipomatosis, pelvic lipomatosis and diffuse lipomatosis. Lipomatosis involving only the soft tissue of the face was not mentioned. Recently, three cases of proliferating and infiltrating lesions of adipose tissue, considered to be congenital in nature, involving only the face have reported and termed as a congenital infiltrating lipomatosis of the face. We experienced a 3 month-old male with the congenital infiltrating lipomatosis of the face spite evidence of tumor persistence at 6 year follow-up. The lesion had the following characteristics 1) proliferation of mature adipose tissue without encapsulation ; 2) infiltration of adjacent muscle ; 3) presence of fibrous tissue ; 4) absence of lipoblasts ; 5) absence of signs of malignancy ; 6) hypertrophy of subjacent bone with macrodontia ; 7) congenital in origin ; and 8) tendency to recur after surgical excision.

저자들은 얼굴에 발생한 선천성 침윤성 지방종증을 조기에 진단하여 치료하였다. 선천성 침윤성 지방종증은 성숙한 지방세포로 구성된 피낭이 없는 종양으로서 주위 근육을 침윤하지만, 조직학적으로 악성화되거나 지방아세포가 관찰되지 않으며 섬유 성분이 포함되어 있었다. 진행성으로 재발되므로 지방흡인술은 부적절한 수술 방법이며, 두 차례에 걸친 광범위 국소 지방절제술 및 절골술로써 안면부 용모가 많이 호전되었지만 앞으로 여러 차례에 걸친 보존적 국소 지방절제술, 치아교정 및 비후된 중안면골에 대한 절골술이 필요할 것으로 생각된다.