Embryonal Rhabdomyosarcoma with Posttherapy Cytodifferentiation and Aggressive Clinical Course.

IF 0.7 Q4 PATHOLOGY Case Reports in Pathology Pub Date : 2021-11-08 eCollection Date: 2021-01-01 DOI:10.1155/2021/1800854
Maniraj Jeyaraju, Regina Ann Macatangay, Ashley Taylor-King Munchel, Teresa Anne York, Elizabeth A Montgomery, Michael E Kallen
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引用次数: 3

Abstract

Rhabdomyosarcoma is the most common soft tissue sarcoma in children and adolescents. Embryonal rhabdomyosarcoma (ERMS), its most common subtype, is a malignant soft tissue tumor with morphologic and immunophenotypic features of embryonic skeletal muscle. The histologic findings in ERMS typically include a range of differentiation in rhabdomyoblasts from primitive to terminally differentiated forms, and the latter become more prominent after chemotherapy-induced cytodifferentiation. Several reports have shown therapy-related cytodifferentiation to portend a good prognosis in ERMS. We discuss the case of a pediatric patient who presented with ERMS of the orbit. Although her tumor showed extensive posttreatment cytodifferentiation and several other good prognostic clinicopathologic factors, it pursued an aggressive course, resulting in early metastasis and death. This case represents an unusual course and may be instructive as to the clinicopathologic features impacting prognostication, and ultimately the biology, of this aggressive family of tumors.

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胚胎横纹肌肉瘤伴治疗后细胞分化及侵袭性临床病程。
横纹肌肉瘤是儿童和青少年中最常见的软组织肉瘤。胚胎横纹肌肉瘤(Embryonal rhabdomyosarcoma, ERMS)是其最常见亚型,是一种具有胚胎骨骼肌形态和免疫表型特征的恶性软组织肿瘤。ERMS的组织学表现通常包括横纹肌母细胞从原始分化到终末分化的一系列分化,后者在化疗诱导的细胞分化后变得更加突出。一些报道表明,治疗相关的细胞分化预示着ERMS的良好预后。我们讨论的情况下,儿科患者谁提出了眼眶ERMS。尽管她的肿瘤在治疗后表现出广泛的细胞分化和其他一些预后良好的临床病理因素,但它仍具有侵袭性,导致早期转移和死亡。这个病例代表了一个不寻常的过程,可能对影响预后的临床病理特征有指导意义,最终生物学上,这个侵袭性肿瘤家族。
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