Gastric stromal tumors in Carney triad are different clinically, pathologically, and behaviorally from sporadic gastric gastrointestinal stromal tumors: findings in 104 cases.
Gastric stromal tumors in Carney triad are different clinically, pathologically, and behaviorally from sporadic gastric gastrointestinal stromal tumors: findings in 104 cases.
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DOI：
10.1097/pas.0b013e3181c20f4f
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发表时间：
2010-01
期刊：
影响因子：
--
通讯作者：
Carney JA
中科院分区：
文献类型：
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作者：
Zhang L;Smyrk TC;Young WF Jr;Stratakis CA;Carney JA
卡尼三联征最初于 1977 年描述，是 3 种肿瘤的联合：胃上皮样平滑肌肉瘤 [后更名为胃肠道间质瘤 (GIST)]、肾上腺外副神经节瘤和肺软骨瘤。这种疾病主要影响年轻女性，并且不是家族性的。我们研究了 104 例胃肿瘤综合征患者的临床和病理特征。大多数 (88%) 是年轻女性（平均年龄 22 岁），通常表现为胃出血。肿瘤通常位于胃窦（61%），为多灶性，尺寸范围为 0.2 至 18.0 厘米。大多数（86%）具有圆形和多边形（上皮样）细胞。 49 名患者 (47%) 发生转移：胃淋巴结 (29%)、肝脏 (25%) 和腹膜 (13%)。在所测试的肿瘤中检测到免疫阳性如下：KIT，100%； CD34，75%； PKCy，21%； PDGFRA，90%；和平滑肌肌动蛋白，6%。 14 名患者 (13%) 死于转移性 GIST，平均年龄为 45 岁（范围为 30 至 69 岁）。估计 10 年和 40 年生存率分别为 100% 和 73%。中位生存时间为 26.5 年（范围：16 至 60 年）。美国国立卫生研究院肿瘤风险分类与肿瘤行为之间没有相关性。与散发性胃GIST相比，卡尼三联征胃间质瘤表现出明显的特征：女性好发、患者年龄年轻、上皮样细胞占优势、多灶性、淋巴结转移频繁、肿瘤发生连续、行为不可预测。因此，卡尼三联征胃间质瘤在临床、病理和行为上与散发性胃间质瘤不同。
Carney triad, as originally described in 1977, was the association of 3 tumors: gastric epithelioid leiomyosarcoma [later renamed gastrointestinal stromal tumor (GIST)], extra-adrenal paraganglioma, and pulmonary chondroma. The disorder affected mostly young women and was not familial. We studied the clinical and pathologic features of the gastric neoplasm in 104 patients with the syndrome. Most (88%) were young women (mean age, 22 y), and the usual presentation was gastric bleeding. The tumors, commonly antral-based (61%), were multifocal, and ranged from 0.2 to 18.0 cm in dimension. Most (86%) featured round and polygonal (epithelioid) cells. Metastasis occurred in 49 patients (47%): to gastric lymph nodes (29%), liver (25%), and peritoneum (13%). Immunopositivity was detected in the tumors tested as follows: KIT, 100%; CD34, 75%; PKCy, 21%; PDGFRA, 90%; and smooth muscle actin, 6%. Fourteen patients (13%) died of metastatic GIST at a mean age of 45 years (range, 30 to 69 y). Estimated 10 and 40-year survivals were 100% and 73%, respectively. Median survival time was 26.5 years (range, 16 to 60 y). There was no correlation between the National Institutes of Health tumor risk classification and the tumor behavior. Compared with sporadic gastric GISTs, the gastric stromal tumor in Carney triad showed distinctive features: female predilection, young patient age, epithelioid cell predominance, multifocality, frequent lymph node metastasis, serial tumor occurrence, and unpredictable behavior. Thus, the Carney triad gastric stromal tumor is different clinically, pathologically, and behaviorally from sporadic gastric GIST.