Department of Oral Diagnosis, Semiology and Pathology Areas, Piracicaba Dental School, University of Campinas, Piracicaba, São Paulo, Brazil.
Department of Pathology, University of São Paulo Medical School, São Paulo, Brazil.
Oral Oncol. 2022 Dec;135:106243. doi: 10.1016/j.oraloncology.2022.106243. Epub 2022 Nov 13.
Low-grade fibromyxoid sarcoma (LGFMS) is a malignant neoplasm of soft tissue, which occurs mainly in extremities. In this study, we described a rare case of LGFMS arising in the labial mucosa, in a 7-year-old male patient, who presented a painless multilobulated growth with an evolution time of 12 months. An incisional biopsy was performed and microscopic examination revealed a proliferation of bland regular spindle cells in an alternating myxoid and hyalinized stroma. The tumor cells showed a strong cytoplasmic immunoreactivity for MUC4 and low cell proliferation index measured by Ki-67 antibody. The diagnosis of LGFMS was established and the patient was referred for surgical treatment at a pediatric oncology hospital.
低度恶性纤维黏液样肉瘤（LGFMS）是一种主要发生于四肢的软组织恶性肿瘤。本研究描述了 1 例罕见的发生于 7 岁男性患者唇部黏膜的 LGFMS，其表现为 12 个月无痛性多叶状生长。行切开活检，镜下观察到在黏液样和玻璃样变的基质中交替存在规则的梭形细胞增生。肿瘤细胞胞质强表达 MUC4，Ki-67 抗体标记的细胞增殖指数低。诊断为 LGFMS，患者转至儿科肿瘤医院行手术治疗。
