Dipartimento di Scienze Mediche e Chirurgiche, Fondazione Policlinico Universitario "A. Gemelli" IRCCS, Rome, Italy.
Eur Rev Med Pharmacol Sci. 2020 Oct;24(20):10703-10707. doi: 10.26355/eurrev_202010_23429.
Gastrointestinal schwannomas are rare benign mesenchymal tumors originating from Schwann cells, the nerve sheath belonging to the Auerbach's plexus or, less frequently, to Meisser's plexus. The esophagus is the least common site accounting for less than 2% of all esophageal tumors, and the upper to mid portion is usually involved. Esophageal schwannomas affect more frequently middle-aged Asian women. The most common symptom is dysphagia. Diagnosis requires histological and immunohistochemical studies and the standard of care is surgical resection.
We present the case of a 22-year-old Caucasian male who was admitted to our hospital for progressive dysphagia and acute chest pain. An EGDS showed an elongated bulging of the lower esophagus with signs of a subcentimetric mucosal erosion. A CT-scan showed a lower esophageal ectasia and a huge postero-lateral wall mass measuring 37x28x70 mm. An endoscopic ultrasonography showed a hypoechoic heterogeneous mass with multiple anechoic areas and a fine needle biopsy was performed. Histological examination showed tissue made up of spindle cells with mild eosinophilic cytoplasm and rare nuclear atypia, which were intensively and diffusely positive for the S100 protein on immunohistochemical studies thus allowing pre-operative diagnosis of "ancient" schwannoma. after a multidisciplinary discussion, the patient underwent a surgical resection. Since the tumor had a transmural extension, a subtotal esophagectomy was performed to achieve complete resection with negative margins.
This is the first case of a young Caucasian male patient with an "ancient" schwannoma of the lower esophagus, a benign but locally advanced lesion treated by subtotal esophagectomy.
胃肠道 schwannomas 是一种罕见的良性间叶性肿瘤，起源于 Schwann 细胞，神经鞘属于 Auerbach 丛，或较少见的 Meisser 丛。食管是最不常见的部位，占所有食管肿瘤的不到 2%，通常累及中上段。食管 schwannomas 更常影响中年亚裔女性。最常见的症状是吞咽困难。诊断需要组织学和免疫组织化学研究，标准治疗方法是手术切除。
我们报告了一例 22 岁白人男性，因进行性吞咽困难和急性胸痛入院。EGDS 显示下段食管呈细长隆起，伴有亚厘米大小的黏膜糜烂迹象。CT 扫描显示下段食管扩张和巨大的后外侧壁肿块，大小为 37x28x70mm。内镜超声检查显示低回声不均匀肿块，多个无回声区，细针活检。组织学检查显示组织由梭形细胞组成，细胞质轻度嗜酸性，核异型性罕见，免疫组织化学研究显示 S100 蛋白弥漫强阳性，因此术前诊断为“陈旧性” schwannoma。经多学科讨论后，患者接受了手术切除。由于肿瘤有壁内延伸，行次全食管切除术以实现完全切除和阴性切缘。
这是首例年轻白人男性下段食管“陈旧性” schwannoma 病例，为良性但局部进展的病变，行次全食管切除术治疗。
