Ersek R A, Lele E, Surak G S, Denton D R, McCue K
University of Texas Health Science Center, San Antonio.
Ann Plast Surg. 1989 Nov;23(5):450-5. doi: 10.1097/00000637-198911000-00014.
Multiple hereditary lipomatosis has been reported very rarely in the literature. The largest number of lipomas previously reported in a single patient was 160. We report on a 45-year-old man who began to experience multiple lipomas at about age 26. The lipomas multiplied until they severely disfigured his entire torso, arms, and upper legs. Both sides of his body were affected to approximately equal degrees. A family history revealed that the patient's father, two brothers, and two nephews have all experienced multiple subcutaneous lipomas, although to a lesser degree. In treating the patient, what appeared to be very large lipomas were in fact found to be subcutaneous clusters of hundreds of small encapsulated lipomas. Lipomas were excised using suction lipectomy to treat different involved areas on eleven separate occasions. A total of 8,475 cc was removed over a two-year period. A modified, slightly less blunt cannula was developed to aspirate the clustered lipomas individually.
文献中关于多发性遗传性脂肪瘤的报道极为罕见。此前报道的单例患者身上最多的脂肪瘤数量为160个。我们报告了一名45岁男性,他大约在26岁时开始出现多发性脂肪瘤。脂肪瘤不断增多,直至严重损毁了他的整个躯干、手臂和大腿上部。他身体的两侧受到的影响程度大致相同。家族史显示,患者的父亲、两个兄弟和两个侄子都曾出现过多发性皮下脂肪瘤,不过程度较轻。在治疗该患者时,原本看似非常大的脂肪瘤实际上是数百个小的包膜性脂肪瘤的皮下簇集。采用抽脂术在11个不同场合切除了涉及不同部位的脂肪瘤。在两年时间里共吸出了8475立方厘米的组织。还研发了一种改良的、钝性略小的套管针,用于分别抽吸簇状脂肪瘤。