胰腺间质瘤的诊治及预后

摘要

Objective To investigate the clinical characteristics, diagnosis, treatment and prognosis of pancreatic gastrointestinal stromal tumor (GIST). Methods We reported a case and reviewed the medical literature on pancreatic malignant GIST. We searched the Pubmed and main domestic database. The clinical data of the reported cases were studied, and their predictive factors for postoperative recurrence and metastasis were analyzed. Results Between January 1980 and July 2010, 16 cases of pancreatic GIST were reported. There were 7 males and 9 females, with a median age pf 56.5 (31-72)years. The clinical symptoms were nonspecific. The main presentation was upper abdominal pain or discomfort. A preoperative diagnosis was suspected on radiological examination. The tumor mainly appeared as a well-defined solid-cystic mass. Irregular enhancement appeared in the circumferential and solid portion of the tumor on enhanced CT scan sequences. The pancreatic and biliary ducts were rarely dilated. Endoscopic ultrasound guided fine needle aspiration cytology (EUS-FNA) was helpful in preoperative diagnosis. Of the 15 surgical patients, 14 underwent complete resection, while the remaining received cyst-jejunostomy. A correct diagnosis was made on histopathology and immunohisto-chemistry. On a mean follow up of 21 months (range, 1-60) in 14 patients, all patients were alive.Recurrence or metastasis occurred in 4 patients with tumors of high malignant potential. On univariate analysis, the only significant predictor for adverse outcome was mitoses≥10/50 HPF. Conclusions Pancreatic GIST is a rare tumor of relatively low malignant potential. It has a better prognosis than ductal adenocarcinoma. It is important to arrive at a correct diagnosis and treat the tumor with radical resection. Aggressive surgical resection is potentially curative. Imatinib is recommended in the treatment of patients with tumors with high malignant potential.%目的 探讨原发于胰腺的胃肠道外间质瘤的临床特点、诊断治疗和预后.方法 报道本院诊治的1例胰腺恶性间质瘤,并通过Pubmed和国内主要数据库检索,分析该病的临床资料,对其术后复发转移的危险因素进行预测.结果 1980年1月至2010年7月间,国内外共报道胰腺间质瘤16例,其中男7例,女9例,中位年龄56.5(31～72)岁.临床表现无特异性,主要为上腹疼痛不适.术前诊断依赖于影像检查,肿瘤多为囊实性,边界清晰,CT增强后肿瘤周边或实质内出现不规则强化,胰胆管扩张少见.内镜超声引导下细针穿刺吸取细胞学检查(EUS-FNA)有助于术前确诊.15例手术患者中,14例完整切除肿瘤,1例行囊肿空肠内引流.所有患者均经病理和免疫组化证实为胰腺间质瘤.14例患者的随访时间为1～60个月,平均21个月,所有患者均存活,4例出现复发转移.该病多为高度恶性潜质,单因素分析显示核分裂相≥10/50高倍视野(HPF)为术后复发转移的风险因素.结论 胰腺问质瘤是一种少见的恶性程度相对较低的肿瘤,预后较导管腺癌好.正确诊断和根治性切除可改善其预后.术后即使复发转移,也应积极手术切除,高危患者应服用伊马替尼治疗.