Hassan I, Celik I, Nies C, Zielke A, Gerdes B, Moll R, Ramaswamy A, Wagner H-J, Bartsch D K
Department of Surgery, Institute of Theoretical Surgery, Philipps University of Marburg, Marburg, Germany.
Pancreatology. 2005;5(2-3):289-94. doi: 10.1159/000085285. Epub 2005 Apr 22.
The solid-pseudopapillary tumor (SPT) is a very rare pancreatic neoplasm that predominantly affects young females. About 450 cases have been described in the world literature and approximately 20% of the reported patients were children. The occurrence of SPT with distant metastases in children is extremely rare with only two previously reported cases. We now report a 16-year-old Asian girl with a large SPT and synchronous multiple liver metastases who was successfully treated in a 2-step strategy, including initial pylorus-preserving partial duodenopancreatectomy, right hemicolectomy, resection and allografting of the portal vein and secondary resection of 12 liver metastases. The patient is disease free after a follow-up of 18 months after resection of the primary tumor, suggesting that an aggressive surgical treatment might also be justified for metastasized SPT.
实性假乳头状肿瘤（SPT）是一种非常罕见的胰腺肿瘤，主要影响年轻女性。世界文献中已描述了约450例病例，报告患者中约20%为儿童。儿童SPT发生远处转移极为罕见，此前仅有两例报告。我们现报告一名16岁亚洲女孩，患有巨大SPT并伴有同步多发肝转移，采用两步策略成功治疗，包括初始保留幽门的十二指肠胰腺部分切除术、右半结肠切除术、门静脉切除及同种异体移植，以及二期切除12处肝转移灶。原发肿瘤切除后随访18个月，患者无疾病复发，提示对于发生转移的SPT积极的手术治疗也可能是合理的。
