Akeyson E W, McCutcheon I E, Pershouse M A, Steck P A, Fuller G N
Department of Neurosurgery, University of Texas M. D. Anderson Cancer Center, Houston, USA.
J Neurosurg. 1996 Jul;85(1):163-9. doi: 10.3171/jns.1996.85.1.0163.
The authors describe a malignant peripheral primitive neuroectodermal tumor (PNET) that originated in the median nerve in an elderly adult. After the diagnosis was made by biopsy, the patient underwent radical local resection with interpositional vein grafting of the brachial artery. The tumor had the typical appearance of a primitive neural tumor with small, round cells forming rosettes. It stained positively for both the Ewing's sarcoma/peripheral PNET antigen (HBA-71) and neuron-specific enolase, confirming its neural origin. Ultrastructural examination revealed dense core granules and suggested neural differentiation of the neoplasm. Cytogenetic analysis suggested a chromosome (11;22) translocation typical of peripheral PNET. Early reports consisted of tumors arising solely in peripheral nerves, but recent series have focused mainly on tumors arising in the soft tissues other than nerves. There are no other cases of true PNET of peripheral nerve in the modern literature that have been fully characterized by immunohistochemical, ultrastructural, and cytogenetic criteria. Although peripheral PNETs occur more commonly in children, this unusual neoplasm should be considered in the differential diagnosis of peripheral nerve neoplasms in adults. Early diagnosis is desirable because of its aggressive nature and poor outcome.
作者描述了一例起源于一名老年成人正中神经的恶性外周原始神经外胚层肿瘤（PNET）。活检确诊后，患者接受了根治性局部切除并进行了肱动脉间置静脉移植。肿瘤具有原始神经肿瘤的典型外观，由小圆形细胞形成菊形团。它对尤因肉瘤/外周PNET抗原（HBA - 71）和神经元特异性烯醇化酶均呈阳性染色，证实了其神经起源。超微结构检查发现致密核心颗粒，提示肿瘤有神经分化。细胞遗传学分析提示存在外周PNET典型的染色体（11；22）易位。早期报告的病例仅发生于外周神经，但近期系列研究主要关注发生于神经以外软组织的肿瘤。现代文献中尚无其他以外周神经真正PNET通过免疫组织化学、超微结构和细胞遗传学标准进行充分特征描述的病例。尽管外周PNET在儿童中更常见，但在成人外周神经肿瘤的鉴别诊断中应考虑这种不寻常的肿瘤。因其侵袭性本质和不良预后，早期诊断很有必要。
