Simsek Cem, Uner Meral, Ozkara Feride, Akman Orkun, Akyol Aytekin, Kav Taylan, Sokmensuer Cenk, Gedikoglu Gokhan
Department of Gastroenterology, Hacettepe University, Ankara 06230, Turkey.
Department of Pathology, Hacettepe University, Ankara 06230, Turkey.
World J Clin Cases. 2021 Apr 6;9(10):2218-2227. doi: 10.12998/wjcc.v9.i10.2218.
Neurogenic tumors are rare but represent an important consideration in the differential diagnosis of abdominal mesenchymal tumors. Reports on their incidence, pathological features and clinical characteristics are scarce.
To advance the overall knowledge on the histologic, immunohistochemical, clinical and radiologic characteristics of neurogenic tumors through this case series.
An established database of a nationwide tertiary referral center, covering a 15-year period (2005 and 2020), was retrospectively re-evaluated. Diagnoses of neurogenic tumor cases were confirmed by two experts following review of the macroscopic, histological and immunohistochemical records along with findings from analysis of archived tissue sections for each included patient. Tissue microarrays were constructed for cases lacking necessary immunohistochemical studies. Clinical data and follow-up information were collected from the hospital records and the patients themselves, when available.
The study included 19 cases of intraabdominal neurogenic tumors, representing 12 women and 7 men, between 18 and 86 years of age (median: 51 years). Final confirmed diagnoses were 12 schwannomas, 2 diffuse submucosal neuro-fibromatoses, 2 ganglioneuromas, 2 malignant peripheral sheath nerve tumors, and 1 mucosal Schwann cell hamartoma. Sizes of the tumors were variable, with a median diameter of 4 cm; the two largest (> 10 cm) were schwannomas. The majority of cases were asymptomatic at presentation, but the most frequent symptom was abdominal pain. Gastrointestinal tract lesions were detected with endoscopy and extra-luminal lesions were detected with cross-sectional imaging. All cases were S100-positive and CD117-negative; most cases were negative for desmin, epithelial membrane antigen, smooth muscle actin and CD34. In all but 5 cases, the Ki67 proliferation index was ≤ 1%.
Re-evaluation of 19 cases of abdominal neurogenic tumors demonstrated con-siderable variability in clinicopathologic characteristics depending on location, dimension and histological features.
神经源性肿瘤较为罕见，但在腹部间叶组织肿瘤的鉴别诊断中是一个重要的考虑因素。关于其发病率、病理特征和临床特点的报道较少。
通过该病例系列研究，增进对神经源性肿瘤的组织学、免疫组化、临床及影像学特征的全面认识。
对一家全国性三级转诊中心建立的数据库进行回顾性重新评估，该数据库涵盖15年期间（2005年至2020年）。两名专家在审查了宏观、组织学和免疫组化记录以及对每个纳入患者存档组织切片的分析结果后，确认了神经源性肿瘤病例的诊断。对于缺乏必要免疫组化研究的病例，构建了组织微阵列。临床数据和随访信息从医院记录以及患者本人（如有）处收集。
该研究纳入了19例腹腔内神经源性肿瘤病例，其中女性12例，男性7例，年龄在18至86岁之间（中位数：51岁）。最终确诊为12例施万细胞瘤、2例弥漫性黏膜下神经纤维瘤病、2例神经节神经瘤、2例恶性外周神经鞘膜瘤和1例黏膜施万细胞错构瘤。肿瘤大小不一，中位直径为4 cm；最大的两个（>10 cm）为施万细胞瘤。大多数病例在就诊时无症状，但最常见的症状是腹痛。胃肠道病变通过内镜检查发现，腔外病变通过断层成像检测。所有病例S100均呈阳性，CD117呈阴性；大多数病例结蛋白、上皮膜抗原、平滑肌肌动蛋白和CD34均为阴性。除5例病例外，所有病例的Ki67增殖指数均≤1%。
对19例腹部神经源性肿瘤的重新评估表明，根据肿瘤的位置、大小和组织学特征，其临床病理特征存在相当大的差异。
