Rebhandl W, Felberbauer F X, Puig S, Paya K, Hochschorner S, Barlan M, Horcher E
Division of Pediatric Surgery, Department of Surgery, General Hospital of Vienna, University of Vienna Medical School, Vienna, Austria.
J Surg Oncol. 2001 Apr;76(4):289-96. doi: 10.1002/jso.1048.
Solid-pseudopapillary tumor of the pancreas (SPT) is an exceptionally rare neoplasm in children. Its origin remains enigmatic. It is of low malignant potential and occurs most frequently in young females.
A cumulative review of the tumor's clinicopathological characteristics from the world's literature is presented. The clinical course, pathohistologic data and outcome of surgery of four Austrian children treated at the general hospital of Vienna are analyzed.
Between 1987 and 1999, four girls (age: 12--16 years) with SPT were diagnosed at our institution. All patients presented with an abdominal mass and uncharacteristic abdominal pain. Two tumors were located in the tail, one in the body and tail and one in the head of the pancreas (diameter: 7--15 cm). Surgical procedures included three distal pancreatectomies and one partial duodenopancreatectomy (Whipple procedure). One patient had two recurrences with metastases that could only be partially resected. Chemotherapy was initiated for this patient. In the follow-up period (range: 6 months to 12 years) all patients are alive with no evidence of recurrence.
SPT is a rare differential diagnosis of a pancreatic mass in children. It is mandatory to establish this diagnosis since complete surgical removal of the tumor even in case of metastases or local invasion offers an excellent prognosis.
胰腺实性假乳头状瘤（SPT）在儿童中是一种极为罕见的肿瘤。其起源仍然不明。它具有低恶性潜能，最常发生于年轻女性。
对来自世界文献的该肿瘤临床病理特征进行累积回顾。分析了在维也纳总医院接受治疗的4名奥地利儿童的临床病程、病理组织学数据及手术结果。
1987年至1999年间，我院诊断出4名患有SPT的女孩（年龄：12 - 16岁）。所有患者均表现为腹部肿块及非特异性腹痛。2个肿瘤位于胰尾，1个位于胰体尾，1个位于胰头（直径：7 - 15厘米）。手术方式包括3例胰体尾切除术和1例十二指肠胰头切除术（惠普尔手术）。1例患者出现2次复发并伴有转移，仅能部分切除。对该患者启动了化疗。在随访期（范围：6个月至12年），所有患者均存活，无复发迹象。
SPT是儿童胰腺肿块的一种罕见鉴别诊断。必须确立该诊断，因为即使在发生转移或局部侵犯的情况下，完整手术切除肿瘤仍可提供良好的预后。
