Prévot S, Bienvenu L, Vaillant J C, de Saint-Maur P P
Department of Pathology, Saint-Antoine Hospital, AP-HP, Paris, France.
Am J Surg Pathol. 1999 Apr;23(4):431-6. doi: 10.1097/00000478-199904000-00007.
We report five cases of schwannomas of the digestive tract. The patients were two men and three women, whose ages ranged from 56 to 74 years. Three cases arose in the stomach, one in the ascending colon, and one in the esophagus; the latter was a hitherto unreported location for this tumor. The schwannomas ranged from 2 to 11 cm in diameter. They were well circumscribed but not encapsulated, with interlacing bundles of spindle cells, nuclear atypia and no mitosis, interspersed with collagenous strands. Inflammatory cells were scattered throughout the tumors and a peripheral cuff of lymphoid aggregates was observed in all cases. Intracellular periodic acid-Schiff (PAS)-positive crystalloids were found in three cases; no skeinoid fibers were seen. A diffuse and intense positivity for vimentin and S-100 protein was detected in all five cases together with a variable and sometimes focal positivity for glial fibrillary acidic protein and neuron-specific enolase. None of the tumors showed expression of CD34 or the smooth muscle antigens tested. The four cases with a sufficient follow-up had a favorable outcome without any recurrence or metastasis. The morphologic and immunohistochemical features of digestive schwannomas were compared with those of other gastrointestinal stromal tumors. Schwannomas have many differences. Digestive schwannomas can be readily recognized on histologic and immunohistochemical examination. They are spindle cell tumors without epithelioid features, with a peripheral cuff of lymphoid tissue. Specific intracellular needle-shaped PAS-positive crystalloids are found in some cases, whereas skeinoid fibers are not. These tumors always express S-100 protein in a diffuse and strong manner, and they express glial fibrillary acidic protein but not express CD34. Digestive schwannomas usually are gastric tumors and have never been reported in the small bowel. They pursue a benign course and are far rarer than gastrointestinal autonomic nerve tumors.
我们报告了5例消化道神经鞘瘤。患者为2名男性和3名女性，年龄在56岁至74岁之间。3例发生于胃，1例发生于升结肠，1例发生于食管；后者是该肿瘤迄今未报道的发病部位。神经鞘瘤直径为2至11厘米。它们边界清楚但无包膜，有梭形细胞交织束，无核异型性和核分裂象，其间散在胶原纤维束。炎性细胞散在分布于肿瘤各处，所有病例均可见周边淋巴细胞聚集。3例发现细胞内过碘酸希夫（PAS）阳性结晶；未见束状纤维。5例均检测到波形蛋白和S-100蛋白弥漫性强阳性，胶质纤维酸性蛋白和神经元特异性烯醇化酶呈可变的、有时为局灶性阳性。所有肿瘤均未显示CD34或所检测的平滑肌抗原表达。4例有足够随访时间的患者预后良好，无复发或转移。将消化道神经鞘瘤的形态学和免疫组化特征与其他胃肠道间质瘤进行了比较。神经鞘瘤有许多不同之处。消化道神经鞘瘤在组织学和免疫组化检查中易于识别。它们是无上皮样特征的梭形细胞肿瘤，有周边淋巴组织。部分病例可发现特异性细胞内针状PAS阳性结晶，而无束状纤维。这些肿瘤总是弥漫性强表达S-100蛋白，并表达胶质纤维酸性蛋白但不表达CD34。消化道神经鞘瘤通常为胃肿瘤，从未在小肠报道过。它们病程良性，远比胃肠道自主神经肿瘤少见。
