Author/Authors :
mao, yilei chinese academy of medical sciences and pumc - peking union medical college hospital - department of liver surgery, Beijing, China , sang, xinting chinese academy of medical sciences and pumc - peking union medical college hospital - department of liver surgery, Beijing, China , liang, naixin chinese academy of medical sciences and pumc - peking union medical college hospital - department of surgery, Beijing, China , yang, huayu chinese academy of medical sciences and pumc - peking union medical college (pumc) hospital - department of liver surgery, Beijing, China , lu, xin chinese academy of medical sciences and pumc - peking union medical college hospital - department of surgery, Beijing, China , yang, zhiying chinese academy of medical sciences and pumc - peking union medical college hospital - department of surgery, Beijing, China , du, shunda chinese academy of medical sciences and pumc - peking union medical college hospital - department of liver surgery, Beijing, China , xu, yiyao chinese academy of medical sciences and pumc - peking union medical college (pumc) hospital - department of liver surgery, Beijing, China , zhao, haitao chinese academy of medical sciences and pumc - peking union medical college (pumc) hospital - department of liver surgery, Beijing, China , zhong, shouxian chinese academy of medical sciences and pumc - peking union medical college hospital - department of surgery, Beijing, China , huang, jiefu chinese academy of medical sciences and pumc - peking union medical college hospital - department of surgery, Beijing, China , millis, j. michael university of chicago - department of surgery, Chicago, USA
Abstract :
Objective: To discuss the diagnosis and treatment of peripheral primitive neuroectodermal tumors of thepancreas based on our case and all the cases in the world.Methods: The first case of peripheral primitive neuroectodermal tumors of the pancreas in Asia waspreliminarily reported by our group in 2006. The patient underwent three operations for the primary tumorand recurrences over 41 months prior to the patient’s death in November 2007. All 14 reported cases ofpancreatic PNETs in the world were analyzed. The corresponding literatures on its diagnosis and treatmentof were reviewed.Results: 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 theuncinate process of the pancreas at PUMC Hospital. During the follow-up course, radiotherapy andchemotherapy were given after the first operation. Two additional operations were performed 10 months and25 months after the first one, respectively, because of tumor recurrance. The patient died 41 months afterthe 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 tumoridentified fourteen cases with a mean survival time of 12 months (ranging from 6 to 50 months). Thesepatients 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, immunohistochemicalfeatures of positive P30/32MIC2 with at least two positive neuronal markers., and cytogenetic analysis showingcharacteristic translocation of t[11;22][q24;q12]. Since pancreatic PNETs are highly aggressive, earlydiagnosis, immediate surgical resection and re-resection if possible, early radiotherapy and chemotherapyand close follow-up are required.Conclusions: Peripheral primitive neuroectodermal tumors can arise in pancreas. The diagnosis andtreatment should be made as early as possible, aggressive surgeries for the primary and recurrences may helpto improve the prognosis.
