From the Chirurgica 1, Pancreatic and Endocrine Digestive Surgical Unit, Departments of Surgery, Oncology and Gastroenterology.
Medicine, University of Padua, Padua, Italy.
Pancreas. 2020 Aug;49(7):918-923. doi: 10.1097/MPA.0000000000001599.
Pancreatic neuroendocrine neoplasms (pNENs) and gastrointestinal stromal tumors (GISTs) represent rare neoplasms. Nonsyndromic cases of pNENs associated with a synchronous GIST were evaluated, and a review of the literature was performed.
We evaluated clinicopathologic features, postoperative outcome, and follow-up of patients operated on for nonsyndromic synchronous pNENs and GISTs in our unit (2003-2017).
Five (3.2%) of 156 patients with a pNEN had an associated GIST (3 male/2 female; average age, 67 years). They were diagnosed with a pNEN preoperatively and underwent pancreatic surgery. In 4 patients, GISTs were detected intraoperatively. Histology showed 3 G1 and 2 G2 pNENs. All GISTs were low risk (median size, 0.9 cm). Two patients were alive without disease 108 and 132 months after surgery. In the literature, 7 cases were described. They had low-risk GISTs, with a gastric location in 6 cases (median size, 2.85 cm).
Sporadic pNENs coexisting with a GIST have been demonstrated in 12 cases. This association is considered fortuitous, and its true incidence may be underestimated. Surgery should be performed on the GIST during the pancreatic surgery. The prognosis strictly depends on the pancreatic NENs.
胰腺神经内分泌肿瘤（pNENs）和胃肠道间质瘤（GISTs）均为罕见肿瘤。本研究评估了无综合征表现的 pNEN 合并同步 GIST 的病例，并对相关文献进行了复习。
回顾性分析了 2003 年至 2017 年在我院行手术治疗的无综合征表现的 pNEN 合并 GIST 患者的临床病理特征、术后转归和随访情况。
在 156 例 pNEN 患者中，有 5 例（3.2%）合并 GIST（3 例男性/2 例女性；平均年龄 67 岁）。术前诊断为 pNEN 并行胰腺手术，4 例术中发现 GIST。组织学显示 3 例 G1 和 2 例 G2 pNEN。所有 GIST 均为低危（中位直径 0.9cm）。2 例患者术后 108 和 132 个月时无瘤生存。文献中描述了 7 例病例，均为低危 GIST，其中 6 例位于胃（中位直径 2.85cm）。
在 12 例患者中观察到散发性 pNEN 合并 GIST。这种关联被认为是偶然的，其真实发生率可能被低估。在胰腺手术中应同期处理 GIST。预后严格取决于胰腺 NEN。
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