Chen Chengxin, Cao Jiachen, Song Lingxie, Wang Wenjie, Guo Dandan, Shi Qi, Zhang Ying, Chen Yunzhao, Liu Chunxia, Li Feng
Department of Pathology and Key Laboratory for Xinjiang Endemic & Ethnic Diseases, The First Affiliated Hospital, Shihezi University School of Medicine, Shihezi, China.
Department of Pathology and Medical Research Center, Beijing Institute of Respiratory Medicine and Beijing Chao-Yang Hospital, Capital Medical University, Beijing, China.
Front Oncol. 2023 Oct 30;13:1258769. doi: 10.3389/fonc.2023.1258769. eCollection 2023.
Lipoblastic nerve sheath tumors of soft tissue are characterized as schwannoma tumors that exhibit adipose tissue and lipoblast-like cells with signet-ring morphology. They have been documented to arise in various anatomic locations, including the thigh, groin, shoulder, and retroperitoneum. However, to our knowledge, this tumor has not been previously reported as a lymph node primary. We present herein the first case of a benign primary lipoblastic nerve sheath tumor arising in an inguinal lymph node in a 69-year-old man. Microscopic examination revealed a multinodular tumor comprising fascicles of spindle cells, as well as adipocytic and lipoblast-like signet-ring cell component in the context of schwannoma. Despite the presence of some bizarre cells with nuclear atypia, no obvious mitotic activity or necrosis was observed. Immunohistochemical analysis showed strong and diffuse expression of S-100, SOX10, CD56, and NSE in the spindle cells as well as in the signet-ring lipoblast-like cells and the mature adipocytes. Sequencing analysis of the neoplasm identified six non-synonymous single nucleotide variant genes, specifically , , , , , and , as well as four nonsense mutation genes including , , , and . The patient remained alive and well with no evidence of recurrence over a period of ten-year follow-up.
软组织脂肪母细胞性神经鞘瘤的特征是表现出脂肪组织和具有印戒形态的脂肪母样细胞的神经鞘瘤。文献记载其可发生于各种解剖部位，包括大腿、腹股沟、肩部和腹膜后。然而，据我们所知，此前尚未有该肿瘤原发于淋巴结的报道。在此，我们报告首例发生于一名69岁男性腹股沟淋巴结的良性原发性脂肪母细胞性神经鞘瘤。显微镜检查显示为多结节性肿瘤，由梭形细胞束以及神经鞘瘤背景下的脂肪细胞和脂肪母样印戒细胞成分组成。尽管存在一些具有核异型性的奇异细胞，但未观察到明显的有丝分裂活性或坏死。免疫组化分析显示梭形细胞以及印戒样脂肪母样细胞和成熟脂肪细胞中S-100、SOX10、CD56和NSE呈强弥漫性表达。对该肿瘤的测序分析鉴定出六个非同义单核苷酸变异基因，具体为 、 、 、 、 、 和 ，以及四个无义突变基因，包括 、 、 和 。在为期十年的随访中，患者存活良好，无复发迹象。
