Hutcherson R W, Jenkins H A, Canalis R F, Handler S D, Eichel B S
Arch Otolaryngol. 1979 May;105(5):267-70. doi: 10.1001/archotol.1979.00790170037010.
We discuss our experience in the diagnosis and management of seven cases of neurogenic sarcomas of the head and neck. These uncommon tumors, which affect all ages, arise most frequently from the brachial plexus, sympathetic chain, and the cranial nerves or their branches. An enlarging mass is the most common initially appearing symptom. Diagnosis may be difficult and rests heavily on gross evidence of a relationship between the tumor and its nerve of origin. Histologically, the lesions are composed of spindle cells with varying degrees of pleomorphism in a pattern that is frequently undistinguishable from fibrosarcomas. Ultrastructural studies may be helpful in establishing the neurogenic origin of these tumors. Neurogenic sarcoma of the head and neck has an extremely poor prognosis. Surgery remains the cornerstone of treatment, although radiotherapy is important for palliation.
我们讨论了七例头颈部神经源性肉瘤的诊断和治疗经验。这些罕见肿瘤可发生于各年龄段，最常起源于臂丛神经、交感神经链、颅神经及其分支。肿块增大是最常见的首发症状。诊断可能困难，很大程度上依赖于肿瘤与其起源神经关系的大体证据。组织学上，病变由具有不同程度多形性的梭形细胞组成，其形态常与纤维肉瘤难以区分。超微结构研究可能有助于确定这些肿瘤的神经源性起源。头颈部神经源性肉瘤预后极差。手术仍是治疗的基石，尽管放疗对缓解症状很重要。
