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102例小肠胃肠间质瘤的MDCT与临床病理特征:单机构经验

MDCT and clinicopathological features of small bowel gastrointestinal stromal tumours in 102 patients: a single institute experience.

作者信息

Baheti A D, Shinagare A B, O'Neill A C, Krajewski K M, Hornick J L, George S, Ramaiya N H, Tirumani S H

机构信息

1 Department of Radiology, Brigham and Women's Hospital, Harvard Medical School, Boston, MA, USA.

2 Department of Imaging, Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA, USA.

出版信息

Br J Radiol. 2015 Sep;88(1053):20150085. doi: 10.1259/bjr.20150085. Epub 2015 Jun 25.

Abstract

OBJECTIVE

Small bowel (SB) is the second most common site of gastrointestinal stromal tumours (GISTs). We evaluated clinical presentation, pathology, imaging features and metastatic pattern of SB GIST.

METHODS

Imaging and clinicopathological data of 102 patients with jejunal/ileal GIST treated at Dana-Farber Cancer Institute and Brigham and Women's Hospital (Boston, MA) between 2002 and 2013 were evaluated. Imaging of treatment-naive primary tumour (41 patients) and follow-up imaging in all patients was reviewed.

RESULTS

90/102 patients were symptomatic at presentation, abdominal pain and lower gastrointestinal blood loss being the most common symptoms. On pathology, 21 GISTs were low risk, 17 were intermediate and 64 were high risk. The mean tumour size was 8.5 cm. On baseline CT (n = 41), tumours were predominantly well circumscribed, exophytic and smooth/mildly lobulated in contour. Of 41 tumours, 16 (39%) were homogeneous, whereas 25 (61%) were heterogeneous. Of the 41 tumours, cystic/necrotic areas (Hounsfield units < 20) were seen in 16 (39%) and calcifications in 9 (22%). CT demonstrated complications in 13/41 (32%) patients in the form of tumour-bowel fistula (TBF) (7/41), bowel obstruction (4/41) and intraperitoneal rupture (2/41). Amongst 102 total patients, metastases developed in 51 (50%) patients (27 at presentation), predominantly involving peritoneum (40/102) and liver (32/102). 7/8 (87%) patients having intraperitoneal rupture at presentation developed metastases. Metastases elsewhere were always associated with hepatic/peritoneal metastases. At last follow-up, 28 patients were deceased (median survival, 65 months).

CONCLUSION

SB GISTs were predominantly large, well-circumscribed, exophytic tumours with or without cystic/necrotic areas. Complications such as TBF, bowel obstruction and intraperitoneal perforation were visualized at presentation, with patients with perforation demonstrating a high risk of metastatic disease. Exophytic eccentric bowel wall involvement and lack of associated adenopathy are useful indicators to help differentiate GISTs from other SB neoplasms.

ADVANCES IN KNOWLEDGE

SB GISTs are predominantly large, well-circumscribed, exophytic tumours, and may present with complications. They often are symptomatic at presentation, are high risk on pathology and metastasize to the peritoneum more commonly than the liver.

摘要

目的

小肠是胃肠道间质瘤(GIST)的第二大常见发病部位。我们评估了小肠GIST的临床表现、病理、影像学特征及转移模式。

方法

对2002年至2013年间在达纳-法伯癌症研究所和布莱根妇女医院(马萨诸塞州波士顿)接受治疗的102例空肠/回肠GIST患者的影像学和临床病理数据进行评估。回顾了未经治疗的原发性肿瘤(41例患者)的影像学表现以及所有患者的随访影像学资料。

结果

102例患者中90例在初诊时有症状,最常见的症状是腹痛和下消化道出血。病理检查显示,21例GIST为低风险,17例为中风险,64例为高风险。肿瘤平均大小为8.5厘米。在基线CT检查(n = 41)中,肿瘤大多边界清晰,呈外生性生长,轮廓光滑/轻度分叶。41例肿瘤中,16例(39%)密度均匀,25例(61%)密度不均匀。41例肿瘤中,16例(39%)可见囊性/坏死区域(亨氏单位<20),9例(22%)可见钙化。CT显示41例患者中有13例(32%)出现并发症,表现为肿瘤-肠瘘(TBF)(7/41)、肠梗阻(4/41)和腹腔内破裂(2/41)。102例患者中,51例(50%)发生转移(27例初诊时即有转移),主要累及腹膜(40/102)和肝脏(32/102)。初诊时发生腹腔内破裂的8例患者中有7例(87%)发生转移。其他部位的转移总是与肝/腹膜转移相关。在最后一次随访时,28例患者死亡(中位生存期65个月)。

结论

小肠GIST大多为边界清晰、呈外生性生长的大肿瘤,可有或无囊性/坏死区域。初诊时可见TBF、肠梗阻和腹腔内穿孔等并发症,发生穿孔的患者发生转移性疾病的风险较高。外生性偏心性肠壁受累且无相关淋巴结肿大是有助于将GIST与其他小肠肿瘤相鉴别的有用指标。

知识进展

小肠GIST大多为边界清晰、呈外生性生长的大肿瘤,可能伴有并发症。它们初诊时通常有症状,病理上为高风险,转移至腹膜比转移至肝脏更常见。

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