Acehan Türker, Tomas Kadir, Bedir Recep
Department of Surgical Oncology, Training and Research Hospital, Recep Tayyip Erdogan University, Rize, Turkey.
Department of General Surgery, Training and Research Hospital, Recep Tayyip Erdogan University, Rize, Turkey.
Indian J Surg Oncol. 2022 Jun;13(2):372-376. doi: 10.1007/s13193-021-01477-4. Epub 2021 Nov 8.
Malignant peripheral nerve sheath tumors (MPNST) are rare. Although they originate from Schwann cells or pluripotent neural crest cells, they constitute less than 10% of all soft tissue sarcomas and more than 60% develop on the basis of neurofibromatosis. It is difficult to diagnose MPNST. Although it mostly occurs in the head and neck region or upper extremities, only 1% of cases are located in the retroperitoneal region. The main treatment is surgery, but survival results are not satisfactory even after surgery with R0 resection. They are not sensitive to chemotherapy and radiotherapy and tend to recur locally. The mass detected by imaging in a 57-year-old male patient who admitted to hospital with the complaint of abdominal pain was excised with clear surgical margins. The tumor was located in the left upper quadrant of the abdomen and seemed to invade the pancreas and left kidney in the computed tomography images. The patient had no history of neurofibromatosis or radiation. In this study, it was aimed to present our case diagnosed with retroperitoneal MPNST and treated with multiorgan resection, which is a rare entity, and to increase the awareness of clinicians about the diagnosis, treatment and prognosis of this rare tumor.
恶性外周神经鞘瘤（MPNST）较为罕见。尽管它们起源于施万细胞或多能神经嵴细胞，但在所有软组织肉瘤中所占比例不到10%，且超过60%是在神经纤维瘤病的基础上发生的。MPNST的诊断较为困难。虽然它大多发生在头颈部区域或上肢，但仅有1%的病例位于腹膜后区域。主要治疗方法是手术，但即使进行了R0切除手术，生存结果也不尽人意。它们对化疗和放疗不敏感，且容易局部复发。一名因腹痛入院的57岁男性患者经影像学检查发现的肿块被完整切除，手术切缘清晰。肿瘤位于腹部左上象限，在计算机断层扫描图像中似乎侵犯了胰腺和左肾。该患者无神经纤维瘤病或放疗史。在本研究中，旨在介绍我们诊断为腹膜后MPNST并接受多器官切除治疗的病例，这是一种罕见的情况，以提高临床医生对这种罕见肿瘤的诊断、治疗和预后的认识。
