| Kun yan,Yanyan Ge,Youhong Ding,Shiguang Yan,Xiangyang Li. Peripheral primitive neuroectodermal tumors: a rare case report. Oncol Transl Med, 2014, 13: 392-394. |
| 外周性原始神经外胚层肿瘤1例报道 |
| Peripheral primitive neuroectodermal tumors: a rare case report |
| Received:June 22, 2014 Revised:July 31, 2014 |
| DOI：10.1007/s10330-014-0006-3 |
| 中文关键词: 外周性原始神经外胚层肿瘤；免疫组化；术前化疗；术后化疗 |
| 英文关键词: peripheral primitive neuroectodermal tumors; immunohistochemistry; adjuvant chemoradiation |
| 基金项目: |
| Author Name | Affiliation | E-mail | | Kun yan | Cancer Center, The 82nd Hospital of the Chinese PLA, Huai`an | 1992394813@qq.com | | Yanyan Ge | Anesthesia department,The nd Hospital of the Chinese PLA,Huai`an | | | Youhong Ding | Cancer Center,The nd Hospital of the Chinese PLA,Huai`an | | | Shiguang Yan | Cancer Center,The nd Hospital of the Chinese PLA,Huai`an | | | Xiangyang Li* | Cancer Center,The nd Hospital of the Chinese PLA,Huai`an | 1992394813@qq.com |
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| 中文摘要: |
| 目的 探讨外周性原始神经外胚层肿瘤(peripheral primitive neuroectodermal tumors，pPNETs)的诊断及治疗。方法 回顾性分析我院收治的1例经病理证实的pPNETs患者的诊治过程，并复习文献。结果 本病例患者47岁，两枚肿物位于左胸壁近腋窝处及左锁骨上，均为软组织。患者的主要症状为迅速增大的肿物伴疼痛，两处肿物穿刺病理均提示为鳞状细胞癌。B超及CT检查提示，显示肿物局部低密度影，内可见血流信号，未压迫周围血管。术中完整切除肿物，术后病理学及免疫组化均提示诊断为pPNETs。结论 pPNETs是一种少见的小圆细胞恶性肿瘤。CT及MRI检查可评估肿瘤可切除性；B超可了解肿物的血供及与周围血管的位置关系。pPNETs的确诊依靠病理及免疫组化，尤其是Homer-Wright菊形团及神经内分泌标记物对肿瘤的确诊有重要意义。手术是直接有效的治疗手段，术前新辅助化疗可以减少pPNETs局部淋巴结转移。 |
| 英文摘要: |
| We aimed to explore the diagnosis and treatment of peripheral primitive neuroectodermal tumors (pPNETs). We retrospectively analyzed the diagnosis and treatment process of a patient who was diagnosed with pPNETs by pathology. This case was a man with soft masses arising from the left chest wall near the armpit and left supraclavicular of a 47-year-old man. The patient mainly presented with the masses which increasing gradually with obvious pain. Needle biopsy showed that they were both metastatic adenocarcinoma. Ultrasonography B revealed blood flow of these two low density placeholders can be seen in the signal, not oppression axillary and vein. Radical resection of the masses were performed. Histopathologic study and immunohistochemistry (IHC) confirmed the masses to be peripheral primitive neuroectodermal tumors. pPNETs is a rare malignant small round cell tumor. CT and MRI examination can estimate the resectability of the tumor; Ultrasound B can make sure its inside blood supply and the positional relationship between the mass and the surrounding vasculature. The diagnosis
of pPNETs is based primarily on histopathologic study and IHC, especially those with the characteristics of the Homer-Wright and neuroendocrine markers. Radical resection of the tumor is the most effective therapeutic method. The effect of adjuvant chemo-radiation is worth affirmation. Autologous stem cell rescue besides adjuvant chemotherapy has been associated with prolonged survival. |
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