摘要
目的 探讨腹外型韧带样纤维瘤病(desmoid-type fibromatoses, DTF)的临床病理特征、诊断、鉴别诊断及治疗。方法 回顾性分析16例经手术切除诊断为腹外型DTF的临床病理资料并复习相关文献。结果 16例中男性8例,女性8例,年龄15~74岁,中位年龄49岁。发生于四肢7例、头颈部3例、肩胛带2例、髋部2例、胸背部1例、乳腺1例。镜检:肿瘤由增生的纤细梭形纤维母细胞/肌纤维母细胞及数量不等的胶原纤维组成,瘤细胞排列成束状、波浪状、交织状,细胞无明显异型性,核分裂象罕见。免疫组化:瘤细胞胞质表达Vimentin、SMA,细胞核表达β-catenin,部分病例灶性表达CD68、S-100,不表达CK-Pan、EMA、Desmin、CD34、Myogenin、STAT6、CD99,Ki-67增殖指数很低。手术切除后随访6~79个月,仅1例复发。结论 DTF是一种罕见的局部侵袭性软组织中间型肿瘤,腹外型发病部位较多,尤其好发于四肢、头颈部和肩胛带,临床多表现为肌肉组织内或与肌肉相邻的实性占位,影像学表现具有一定的特征性,但需与多种良恶性梭形细胞病变鉴别,最终诊断依赖病理学检查及基因检测,免疫组化细胞核表达β-catenin对诊断及鉴别诊断有重要价值,手术切除效果好。
Objective To explore the clinicopathological features, diagnosis, differential diagnosis and treatment of extra-abdominal desmoid-type fibromatosis(DTF). Methods The clinicopathological features of 16 patients with extra-abdominal DTF diagnosed by surgical resection were retrospectively analyzed and the relevant literatures were also reviewed. Results There were 8 males and 8 females among 16 cases, with a median age of 49 years(ranging from 15 to 74 years). 7 cases were found in extremities, 3 in head and neck, 2 in shoulder girth, 2 in hip, 1 in chest and back, and 1 in breast. Microscopically, the tumor was composed of a proliferation of slender fibroblasts/myofibroblasts and different number of collagen fibers, which arranged in bundles, waves, and interweaving. Cytological atypia was absent and pathological mitosis was very few. Immunohistochemically, the tumor cells were positive for vimentin and SMA, β-catenin in the nuclei, partly positive for CD68 and S100 in some cases, and negative for CK-Pan, EMA, Desmin, CD34, myogenin, STAT6, and CD99. The proliferating index of Ki-67 was very low.Patients were followed up from 6 to 79 months after DTF resection, only 1 case recurred. Conclusion DTF is a rare, locally aggressive intermediate tumor of soft tissue;extra-abdominal DTF can occur anywhere, especially in the extremities, head and neck and shoulder girdle. The clinical manifestations are mostly solid masses in muscle tissue or adjacent to muscle, with certain imaging characteristics, but it needs to be differentiated from a variety of benign and malignant spindle cell lesions. Moreover, the final diagnosis depends on pathological examination and genetic testing. β-catenin is positive in the nuclei, which plays a critical role for diagnosis and differential diagnosis of DTF. Generally, DTF could be cured by surgical resection with prolonger survival.
作者
杨玲
赵云飞
黄亚兰
刘智
何姣
YANG Ling;ZHAO Yun-fei;HUANG Ya-lan;LIU Zhi;HE Jiao(Department of Pathology,Suining Central Hospital of Sichuan Provincey Suining 629000,China)
出处
《诊断病理学杂志》
2022年第4期319-322,325,共5页
Chinese Journal of Diagnostic Pathology
