Department of Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and PUMC, Beijing 100730, China;
Department of Surgery, University of Chicago, Chicago Il, USA.
Hepatobiliary Surg Nutr. 2013 Feb;2(1):51-60. doi: 10.3978/j.issn.2304-3881.2012.08.01.
To discuss the diagnosis and treatment of peripheral primitive neuroectodermal tumors of the pancreas based on our case and all the cases in the world.
The first case of peripheral primitive neuroectodermal tumors of the pancreas in Asia was preliminarily reported by our group in 2006. The patient underwent three operations for the primary tumor and recurrences over 41 months prior to the patient's death in November 2007. All 14 reported cases of pancreatic PNETs in the world were analyzed. The corresponding literatures on its diagnosis and treatment of were reviewed.
A 13 year-old female patient was diagnosed with pancreatic PNETs by the clinical, microscopic, immunohistochemical features, and cytogenetic analysis after the resection of the tumor located in the uncinate process of the pancreas at PUMC Hospital. During the follow-up course, radiotherapy and chemotherapy were given after the first operation. Two additional operations were performed 10 months and 25 months after the first one, respectively, because of tumor recurrence. The patient died 41 months after the initial diagnosis with the recurrence and metastasis that were not suitable for a further surgery. Primitive neuroectodermal tumors of the pancreas are extremely rare. A review of the world's literature on this tumor identified fourteen cases with a mean survival time of 12 months (ranging from 6 to 50 months). These patients often have no specific clinical symptoms, but most do present with abdominal pain and/or jaundice. The diagnosis is established by small round tumor cells seen on light microscopy, immunohistochemical features of positive P30/32(MIC2) with at least two positive neuronal markers., and cytogenetic analysis showing characteristic translocation of t[11;22][q24;q12]. Since pancreatic PNETs are highly aggressive, early diagnosis, immediate surgical resection and re-resection if possible, early radiotherapy and chemotherapy and close follow-up are required.
Peripheral primitive neuroectodermal tumors can arise in pancreas. The diagnosis and treatment should be made as early as possible, aggressive surgeries for the primary and recurrences may help to improve the prognosis.
通过分析我们的病例和全世界所有病例，探讨胰腺外周原始神经外胚层肿瘤的诊断和治疗。
2006 年，我们小组首次报道了亚洲首例胰腺外周原始神经外胚层肿瘤病例。该患者在死亡前 41 个月内共经历了 3 次针对原发肿瘤和复发肿瘤的手术。对全世界报道的 14 例胰腺 PNET 病例进行了分析，并回顾了相关的诊断和治疗文献。
在 PUMC 医院进行肿瘤位于胰钩突处的切除术后，根据患者的临床表现、显微镜下特征、免疫组织化学特征和细胞遗传学分析，该 13 岁女性患者被诊断为胰腺 PNET。首次手术后进行了放疗和化疗。由于肿瘤复发，分别在首次手术后 10 个月和 25 个月进行了另外 2 次手术。在初次诊断后 41 个月，患者因复发和转移而死亡，无法进一步手术。胰腺外周原始神经外胚层肿瘤极为罕见。对全世界有关该肿瘤的文献进行回顾，共确定了 14 例病例，平均生存时间为 12 个月（6 至 50 个月）。这些患者通常没有特异性的临床症状，但大多数都有腹痛和/或黄疸。通过光镜下观察到小圆细胞肿瘤、免疫组织化学特征显示至少两种阳性神经元标志物阳性的 P30/32（MIC2）以及细胞遗传学分析显示特征性 t[11;22][q24;q12]易位，可明确诊断。由于胰腺 PNET 侵袭性强，需要早期诊断、立即进行手术切除，如果可能的话进行再次切除、早期放疗和化疗以及密切随访。
胰腺可发生外周原始神经外胚层肿瘤。应尽早做出诊断和治疗，对原发肿瘤和复发肿瘤进行积极的手术可能有助于改善预后。
