Chung K C, Weiss S W, Kuzon W M
Section of Plastic and Reconstructive Surgery, University of Michigan Hospital, Ann Arbor, USA.
Br J Plast Surg. 1995 Jun;48(4):240-2. doi: 10.1016/0007-1226(95)90009-8.
A newborn male with multifocal congenital hemangiopericytomas complicated by Kasabach-Merritt syndrome was treated by excision of a large hemangiopericytoma in the right mandibular area. After excision of this lesion, the associated consumptive coagulopathy abated. Spontaneous regression of other subcutaneous tumours over the thighs and left flank was noted by age 6 months; this illustrates the benign course of most congenital hemangiopericytomas. Surgical excision of congenital hemangiopericytoma is indicated when there is a coexisting consumptive coagulopathy or for the definitive control of large lesions. For smaller lesions and in the absence of Kasabach-Merritt syndrome, the benign nature and predilection for spontaneous regression of congenital hemangiopericytomas render observation as the preferred strategy.
一名患有多灶性先天性血管外皮细胞瘤并伴有卡萨巴赫-梅里特综合征的男婴,接受了右下颌区一个大型血管外皮细胞瘤的切除术。切除该病变后,相关的消耗性凝血病得到缓解。到6个月大时,发现大腿和左腹的其他皮下肿瘤自发消退;这说明了大多数先天性血管外皮细胞瘤的良性病程。当存在并存的消耗性凝血病或为了彻底控制大型病变时,建议对先天性血管外皮细胞瘤进行手术切除。对于较小的病变且不存在卡萨巴赫-梅里特综合征的情况,先天性血管外皮细胞瘤的良性性质和自发消退倾向使得观察成为首选策略。