Department of General Surgery, Zhongshan Hospital, Fudan University, NO. 180 Fenglin Road, Shanghai, 200032, China.
Department of Pathology, Zhongshan Hospital, Fudan University, NO. 180 Fenglin Road, Shanghai, 200032, China.
J Cancer Res Clin Oncol. 2019 Jun;145(6):1559-1568. doi: 10.1007/s00432-019-02853-y. Epub 2019 Mar 28.
Gastrointestinal stromal tumors (GISTs) are typically solid neoplasms with small cystic change detected occasionally but in rare instances may present predominantly as cystic lesions. The histopathologic features and prognoses of cystic GISTs (cGISTs) are poorly understood.
We herein reviewed 20 cGISTs resected or consulted in our institution from January 1, 2003 to December 31, 2014.
Of the 20 patients included, the mean age was 61 years and the male-to-female ratio was 9:11. The original locations were the stomach (n = 10, 50%), the small intestine (n = 9, 45%) and the omentum (n = 1, 5%). Indistinct diagnosis or misdiagnosis was established in 15 cases based only on preoperative radiology. Grossly, the cystic component made up the bulk of masses and was filled by dark bloody fluid and necrotic debris in 18 cases. Microscopically, cyst wall was composed of neoplastic spindle (n = 14, 70%)/epithelioid cells (n = 6, 30%) and collagenous fiber, with necrotic debris and granulation tissue lining on the inner surface. cGISTs resembled their solid counterparts in terms of morphology and immunohistology but demonstrated fewer malignant parameters. c-kit or PDGFRα mutations were detected in eleven cases with the remaining being wild type for these two mutations. Although classified as intermediate or high (3 and 17, respectively) risk of recurrence according to modified National Institute of Health criterion, most patients with cGISTs experienced long-term recurrence-free survival without adjuvant imatinib.
Cystic GISTs is a relatively indolent subset of GISTs with favorable prognoses and adjuvant imatinib should be a prudent consideration.
胃肠道间质瘤（GIST）通常是实性肿瘤，偶尔会发现小囊变，但在极少数情况下，GIST 可能主要表现为囊性病变。囊性 GIST（cGIST）的组织病理学特征和预后尚不清楚。
我们回顾了 2003 年 1 月 1 日至 2014 年 12 月 31 日在我院切除或会诊的 20 例 cGIST。
20 例患者中，平均年龄为 61 岁，男女比例为 9:11。原发部位为胃（n=10，50%）、小肠（n=9，45%）和大网膜（n=1，5%）。仅基于术前影像学检查，15 例患者的诊断不明确或误诊。大体上，囊性成分构成肿块的大部分，18 例肿块充满暗红色血性液体和坏死碎片。镜下，囊壁由肿瘤梭形细胞（n=14，70%）/上皮样细胞（n=6，30%）和胶原纤维组成，内表面有坏死碎片和肉芽组织衬里。cGIST 在形态和免疫组织化学上与实性 GIST 相似，但恶性参数较少。11 例检测到 c-kit 或 PDGFRα 突变，其余 2 例突变类型为野生型。尽管根据改良 NIH 标准，cGIST 被分类为中等或高度（分别为 3 和 17）复发风险，但大多数 cGIST 患者无辅助伊马替尼治疗后长期无复发生存。
囊性 GIST 是 GIST 中相对惰性的亚群，预后良好，辅助伊马替尼治疗应慎重考虑。
