Ekpe E E
Cardiothoracic Surgery Unit, Department of Surgery, University of Uyo Teaching Hospital, P.M.B. 1136 Uyo, Akwa Ibom, Nigeria.
West Afr J Med. 2014 Jan-Mar;33(1):77-9.
Although von Recklinghausen disease is relatively common at an incidence of 1 in 4000 live births, malignant transformation in a neurofibromatosis is quite rare estimated at about 5% therefore the importance of life-long follow up of all patients von Recklinghausen disease.
This is a case report of a 45-year old Nigerian-Ibibio male farmer who presented with six months history of rapid growth in one of the numerous skin nodules which had been present since his teenage age. The clinical diagnosis of malignant change in von Recklinghausen's disease was confirmed by histopathologic examination of an incisional biopsy specimen. Radiological investigations also showed the metastatic status of the disease. The patient could not afford the cytotoxic chemotherapeutic agents prescribed. He therefore left the hospital and failed to return when a free-treatment voucher was ready for treatment in the following two days. He is assumed dead at home.
Lifelong follow up for von Recklinghausen disease is emphasized to enable early detection of malignant change and also expansion of the national health insurance scheme to benefit the unemployed citizens.
尽管冯·雷克林豪森病相对常见，活产发病率为1/4000，但神经纤维瘤病的恶变相当罕见，估计约为5%，因此对所有冯·雷克林豪森病患者进行终身随访很重要。
这是一例45岁尼日利亚伊比比奥族男性农民的病例报告，他自青少年时期就出现了多个皮肤结节，其中一个结节在六个月内迅速生长。通过对切开活检标本的组织病理学检查，证实了冯·雷克林豪森病恶变的临床诊断。影像学检查也显示了该疾病的转移情况。患者无力承担所开的细胞毒性化疗药物费用。因此，他离开了医院，在接下来的两天里，当有免费治疗券准备好进行治疗时，他未能返回。推测他已在家中死亡。
强调对冯·雷克林豪森病进行终身随访，以便早期发现恶变，并扩大国家医疗保险计划，使失业公民受益。
