VandenHeuvel Katherine A, Carpentieri David F, Chen Jie, Fung Kar-Ming, Parham David M
1 Department of Pathology, University of Oklahoma Health Sciences Center, Oklahoma City, OK 73104, USA.
Pediatr Dev Pathol. 2014 May-Jun;17(3):226-30. doi: 10.2350/14-01-1433-CR.1. Epub 2014 Mar 20.
Soft-tissue tumors known as "triton" tumors are rare lesions containing neural tissue and skeletal muscle at varying levels of maturity and malignant potential. Benign triton tumors, also called "neuromuscular choristomas" or "neuromuscular hamartomas," consist of neural tissue containing mature skeletal muscle in intimate relationship with peripheral nerve. These tumors are rare in the head and neck in children. Ectomesenchymomas are similar tumors consisting of a malignant mesenchymal component, usually embryonal rhabdomyosarcoma, and a neuroectodermal component represented by mature ganglion cells or primitive neuroblastic/neuroectodermal foci (primitive ectomesenchymoma). Benign triton tumors have been regarded as benign, whereas ectomesenchymomas have been operationally considered to be variants of rhabdomyosarcoma. We present here a unique case that combines features of these 2 entities in a recurrent lesion on the tongue of a 35-month-old girl. This lesion raises questions about the "benign" nature of benign triton tumor and its possible relationship to ectomesenchymoma.
被称为“蝾螈”肿瘤的软组织肿瘤是罕见病变，包含不同成熟度和恶性潜能的神经组织和骨骼肌。良性蝾螈肿瘤，也称为“神经肌肉迷离瘤”或“神经肌肉错构瘤”，由与周围神经密切相关的含有成熟骨骼肌的神经组织组成。这些肿瘤在儿童头颈部很少见。外胚间叶瘤是类似的肿瘤，由恶性间叶成分（通常是胚胎性横纹肌肉瘤）和以成熟神经节细胞或原始神经母细胞/神经外胚层灶为代表的神经外胚层成分组成（原始外胚间叶瘤）。良性蝾螈肿瘤一直被认为是良性的，而外胚间叶瘤在临床上被认为是横纹肌肉瘤的变体。我们在此报告一例独特病例，该病例在一名35个月大女孩的舌部复发性病变中兼具这两种实体的特征。该病变引发了关于良性蝾螈肿瘤“良性”性质及其与外胚间叶瘤可能关系的疑问。
