腹壁是恶性周围神经鞘肿瘤（MPNST）极为罕见的发生部位。除了MPNST在罕见的部位发生外，我们的病例的特殊之处在于其与林奇综合征的关联，据我们所知，这是首次报道的这种情况。我们报道了一例39岁男性的病例报告，该患者有结肠癌的个人病史。遗传咨询揭示了MLH1基因中的杂合型生殖细胞突变，确认为林奇综合征。右侧结肠切除术后9年，患者发现腹壁上有一个无症状的肿块。CT影像显示右外斜肌腱膜内有一个3cm的肿块。患者成功接受了腹壁肿瘤的切除手术。病理检查发现为MPNST。无需额外治疗，该患者术后六个月无复发迹象。腹壁前面的MPNST极为罕见且具有挑战性。一些研究调查了软组织肉瘤患者中错配修复（MMR）缺陷的存在。我们的案例支持了肉瘤与林奇综合征之间的关联假设，这提出了在这些治疗选择有限的病例中，免疫检查点抑制剂治疗的疗效问题。在了解MPNST的生长模式和导致瘤变的综合征（如林奇综合征）的背景下，对MPNST的生长模式有深入的了解至关重要。这一知识对于准确预测患者预后并制定适当的监测和治疗计划至关重要。版权所有 © 2023，由Elsevier Ltd.出版。

The abdominal wall is an extremely rare location for malignant peripheral nerve sheath tumors (MPNSTs). Besides presenting a rare location of MPNST, the peculiarity of our case lies in its association with Lynch syndrome, which is to our knowledge the first reported case of its kind.We present a case report of a 39-year-old male with a personal history of colonic cancer. Genetic counseling revealed Lynch syndrome with a heterozygous germline mutation in MLH1. Nine years after the right hemicolectomy, the patient presented with an asymptomatic lump in the abdominal wall. CT imaging showed a 3 cm mass in the aponeurosis of the right external oblique muscle. The patient underwent successful resection of the parietal tumor. Pathological examination revealed an MPNST. No additional treatment was warranted, and the patient exhibited no signs of relapse during the six months following the surgery.MPNSTs of the anterior abdominal wall are extremely rare and challenging. Some studies have investigated the presence of mismatch repair (MMR) deficiency in patients with sarcomas. Our case consolidates the hypothesis of an association between sarcomas and Lynch syndrome, which raises the question of the efficacy of immune checkpoint inhibitor therapy in these cases where treatment options remain limited.It is essential to have a deep understanding of the growth patterns of MPNSTs in the context of syndromes that predispose individuals to tumors, like Lynch syndrome. This knowledge is crucial for accurately predicting patient outcomes and developing appropriate plans for monitoring and treatment.Copyright © 2023. Published by Elsevier Ltd.