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[外周原始神经外胚层肿瘤——PNET]

[Peripheral primitive neuroectodermal tumor -- PNET].

作者信息

Koudelová J, Kunesová M, Koudela K, Matejka J, Novák P, Prausová J

机构信息

Radiodiagnostická klinika LF UK a FN, Plzen

出版信息

Acta Chir Orthop Traumatol Cech. 2006;73(1):39-44.

Abstract

The authors describe three cases of peripheral primitive neuroectodermal tumor. The tumor was found in soft tissues of the crus, shoulder girdle and perineum, and was also located paravertebrally and epidurally at the level of L1-L2 vertebrae. Radiological findings were not specific for this disease. The results of imaging methods (sonography, CT, MRI, DSA) were important for the assessment of tumor size, its boundary and invasion of the surrounding tissues, and for the evaluation of tumor response to therapy and detection of recurrent disease. The PNET diagnosis was based on immunohistochemical, biochemical and cytogenetic examinations. One patient died 5 months after the first clinical signs were manifested; the two patients surviving for 2 and 1 3/4 years after first sign manifestation, respectively, remained in the care of cancer specialists. Key words: skeletal Ewing's sarcoma, extra-skeletal Ewing's sarcoma, Ewing's sarcoma family of tumors, peripheral primitive neuroectodermal tumor.

摘要

作者描述了3例外周原始神经外胚层肿瘤。肿瘤见于小腿、肩胛带和会阴的软组织,也位于L1-L2椎体水平的椎旁和硬膜外。影像学表现对本病无特异性。成像方法(超声、CT、MRI、DSA)的结果对于评估肿瘤大小、边界及对周围组织的侵犯,以及评估肿瘤对治疗的反应和检测复发性疾病很重要。PNET诊断基于免疫组化、生化和细胞遗传学检查。1例患者在出现首发临床症状后5个月死亡;另外2例患者在出现首发症状后分别存活了2年和1又3/4年,仍由癌症专科医生照料。关键词:骨尤文肉瘤、骨外尤文肉瘤、尤文肉瘤肿瘤家族、外周原始神经外胚层肿瘤

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