婴儿型纤维肉瘤的临床病理分析

Infantile fibrosarcoma: A clinicopathological study of 12 cases

目的探讨婴儿型纤维肉瘤的临床病理学特征、诊断、鉴别诊断和预后。方法收集12例手术切除的婴儿型纤维肉瘤的临床资料进行肉眼观察、光镜和免疫组化检查,并进行随访。结果12例中,男性患儿5例,女性7例;平均年龄(4.7±4.4)个月,术前病程(18.7±30.5)个月。肿块以胸背部和头颈部好发,平均直径(5.8±3.2)cm,无包膜。镜下梭形细胞形态较一致,致密,排列成鲱鱼骨样,核分裂显著,胶原纤维较少或缺乏。瘤细胞免疫组化染色表达vim entin,SMA、MSA染色结果不明确。手术切除后未见局部复发及转移的病例。结论婴儿型纤维肉瘤大多发生于1岁之内,染色体的检查可帮助诊断,治疗以广泛局部手术切除为主,可辅以化疗。需与婴幼儿纤维瘤病、恶性纤维组织细胞瘤、横纹肌肉瘤、良性或恶性神经鞘瘤、平滑肌肉瘤、滑膜肉瘤、血管外皮瘤及成人型纤维肉瘤相鉴别。

Purpose To study the clinicopathological features, diagnosis, differential diagnosis and prognosis of infantile fibrosarcoma. Methods 12 cases of infantile fibrosarcoma were studied with gross, microscopic （including immunohistochemical stain） examinations, as well as collecting the clinical data and the follow-up. Results The average age of the twelve children （5 male and 7 female） was （4. 7 ±4. 4） months when the masses were observed, while the course of disease was （ 18.7 ± 30. 5 ） months before the masses were excised. The chest and back, head and neck were the most common sites. The median size was （5.8 ±3. 2） cm in diameter, and there was non-capsule. Microscopically, the packed spindle-shaped cells were relatively uniform in appearance and arranged in bundles or fascicles, showing a herringbone appearance. There were prominent mitotic activity, and the collagen was inconspicuous or absent. Immunohistochemically, the spindle cells of infantile fibrosarcoma were positive for vimentin and variably for muscle-specific and smooth muscle actin. There was no local recurrence or metastasis after excision in our series. Conclusions Most of infantile fibrosarcoma in children occurs within the first year of age. The chromosomal analysis may be useful for diagnosis. The initial treatment for infantile fibrosareoma is wide local excision, and may combine with chemotherapy for some cases. The differential diagnosis of the tumor includes infantile fibromatosis, malignant fibrohistocytic tumor, rhabdomyosarcoma, benign or malignant nerve sheath tumors, leiomyosarcoma, synovial sarcoma, hemangiopericytoma and adult fibrosarcoma.