Rogalski R P, Louis D S
Section of Orthopaedic Surgery, University of Michigan Hospitals, Ann Arbor 48109-0328.
J Hand Surg Am. 1991 Sep;16(5):873-6. doi: 10.1016/s0363-5023(10)80153-4.
A retrospective review of all malignant hand tumors seen at the University of Michigan from 1950 to 1987 demonstrated six biopsy-proven cases of neurofibrosarcoma involving the upper extremity. There were four male and two female patients; average age at presentation was 37 years and ages ranged from 15 to 63 years. All patients had a history of von Recklinghausen's disease. Three patients were seen initially with painful enlarging masses, one with a painful mass with a neurological deficit, and two with painless enlarging masses. Three patients had radical excisions requiring upper extremity or forequarter amputation, and three patients received limited or no surgical treatment. Five patients died of metastases an average of 3 years after diagnosis. One patient remains alive 18 years after upper extremity amputation despite an early local recurrence. Early diagnosis and radical surgical excision offer the best chance of long-term survival from this highly lethal cancer.
对1950年至1987年在密歇根大学所见的所有手部恶性肿瘤进行的回顾性研究显示，有6例经活检证实的累及上肢的神经纤维肉瘤病例。患者中有4名男性和2名女性；就诊时的平均年龄为37岁，年龄范围在15岁至63岁之间。所有患者均有冯·雷克林豪森病病史。3例患者最初表现为疼痛性肿块增大，1例伴有疼痛性肿块及神经功能缺损，2例为无痛性肿块增大。3例患者接受了根治性切除，需要进行上肢或上肢肩部截肢，3例患者接受了有限的手术治疗或未接受手术治疗。5例患者死于转移，平均在诊断后3年。1例患者在上肢截肢18年后仍然存活，尽管早期有局部复发。早期诊断和根治性手术切除为这种高度致命的癌症提供了最佳的长期生存机会。
