Jankulovski Nikola, Stankov O, Banev S, Petrovski D, Mickovski A, Mitevski A, Filipovski V, Popov Z
University Clinic of Digestive Surgery, Skopje, R. Macedonia.
Prilozi. 2008 Dec;29(2):361-69.
The occurrence of an isolated malignant peripheral nerve sheath tumor (MPNST) of the kidney capsule is extremely rare and its presence may only be expressed by an insidious onset of non-specific and misleading symptoms with a predominance of lower back pain. A computer tomography (CT) scan (as the imaging procedure of choice) will demonstrate the tumor location and its relation to the surrounding structures. Tumor excision in toto is considered the treatment of choice, but it can be hazardous, especially if the tumor is adhering to the surrounding structures. Severe bleeding complications due to the damage of venous structures have to be considered, and establishing hemostasis may pose considerable difficulties. Therefore surgery should be attempted with full precautions and the patient must receive preoperative counseling. If malignancy can safely be excluded, a laparoscopic excision should be considered as an alternative treatment as recurrence is unlikely. Definition of the originating nerve might not always be possible, and a minor degree of neurological impairment has therefore to be anticipated. A case of an isolated MPNST of the kidney capsule without neurofibromatosis is presented. The tumor was located in the fatty and fibrous capsule. It was surgically removed. The patient was further managed with radiotherapy and chemotherapy. An MPNST in such a location is very unusual.
肾包膜孤立性恶性外周神经鞘瘤（MPNST）的发生极为罕见，其存在可能仅表现为隐匿起病的非特异性且具有误导性的症状，以下背部疼痛为主。计算机断层扫描（CT）（作为首选的成像检查）将显示肿瘤的位置及其与周围结构的关系。肿瘤全切被认为是首选治疗方法，但可能具有危险性，尤其是当肿瘤与周围结构粘连时。必须考虑因静脉结构损伤导致的严重出血并发症，而实现止血可能会带来相当大的困难。因此，手术应在充分预防措施下进行，患者必须接受术前咨询。如果能够安全排除恶性肿瘤，可考虑腹腔镜切除作为替代治疗方法，因为复发的可能性不大。确定起源神经可能并非总是可行，因此必须预期会有轻微程度的神经功能损害。本文介绍了一例无神经纤维瘤病的肾包膜孤立性MPNST病例。肿瘤位于脂肪和纤维包膜内。通过手术将其切除。患者随后接受了放疗和化疗。这种位置的MPNST非常罕见。
