梭形细胞横纹肌肉瘤
Spindle Cell Rhabdomyosarcoma
夏成青 赵明 刘正智
更新时间：2020-12-13 14:24:00
发病部位：睾丸旁，头颈部，膀胱、腹部和四肢软组织
诊断要点：
1. 好发于儿童和青少年，男性多见，最常见于睾丸旁，其次为头颈部，肿瘤呈结节状，界限清楚，无包膜；
2. 镜下见由长梭形细胞呈束状排列，也可呈席纹状、交织的束状或漩涡状排列，或呈波浪状排列；
3. 细胞质强嗜酸性，原纤维状，核呈长卵圆形，两端稍钝，多数病例可见散在的横纹肌母细胞；
4. 瘤细胞的异型性不明显，可见少数核分裂像；
5. 少数病例可见典型的原始胚胎性横纹肌肉瘤成分；
6. 间质可见数量不等的胶原纤维。
免疫组织化学染色：
分子标记：
鉴别诊断：
肌纤维母细胞肉瘤
纤维肉瘤
MPNST
肿瘤类型
Myogenin
Desmin
SMA
H-caldesmon
S100
GFAP
梭形细胞横纹肌肉瘤
++
+
+/-
-
-
-
平滑肌肉瘤
-
+
++
+
-
-/+
低级别肌纤维母细胞肉瘤
-
+/-
+
-
-
-
纤维肉瘤
-
-
+/-
-
-
-
MPNST
-
-
-
-
+/-
+/-
促纤维增生性恶性黑色素瘤
-
-
-
-
++
+/-
预后：
治疗：
参考文献：
Carroll S J, Nodit L. Spindle cell rhabdomyosarcoma: a brief diagnostic review and differential diagnosis[J]. Archives of Pathology & Laboratory Medicine, 2013, 137(8):1155-1158.
Nascimento A F, Fletcher C D. Spindle cell rhabdomyosarcoma in adults[J]. American Journal of Surgical Pathology, 2005, 29(8):1106-1113.
Agaram NP, LaQuaglia MP, Alaggio R, et al. MYOD1-mutant spindle cell and sclerosing rhabdomyosarcoma: an aggressive subtype irrespective of age. A reappraisal for molecular classification and risk stratification. Mod Pathol 2018.
Alaggio R, Zhang L, Sung YS, et al. A molecular study of pediatric spindle and sclerosing rhabdomyosarcoma:
identification of novel and recurrent VGLL2-related fusions in infantile cases. Am J Surg Pathol 2016;40(2):224–35.
World Health Organization (WHO) Classification of Tumours Editorial Board. Soft Tissue and Bone Tumours.
WHO Classification of tumours. Lyon International Agency for Research on Cancer. 5th edition, Vol. 3. 2020.
