Fujiwara Yushi, Kitagami Hidehiko, Kikkawa Tomohiro, Sakashita Keita, Kusumi Takaya, Nishida Yasunori
Department of Gastroenterological Surgery, Keiyukai Sapporo Hospital, Minami1-1, Hondori 9-Chome, Shiroishi-Ku, Sapporo, 003-0026, Japan.
Surg Case Rep. 2024 Mar 12;10(1):58. doi: 10.1186/s40792-024-01858-1.
Congenital esophageal stenosis (CES) is a rare condition. We encountered a case of esophageal cancer that developed in an adult with persistent CES. Although many studies have investigated the therapeutic outcomes and performed surveillance for symptoms after treatment for CES, few have performed long-term surveillance or reported on the development of esophageal cancer. We report this case because it is extremely rare and has important implications.
A 45-year-old woman with worsening dysphagia was transferred to our hospital. The patient was diagnosed with CES at 5 years of age and underwent surgery at another hospital. The patient underwent esophageal dilatation for stenosis at 36 years of age. Esophagoscopy performed at our hospital revealed a circumferential ulcerated lesion and stenosis 15-29 cm from the incisors. Histological examination of the biopsy specimen revealed squamous cell carcinoma. Computed tomography (CT) revealed abnormal circumferential wall thickening in parts of the cervical and almost the entire thoracic esophagus. F-fluorodeoxyglucose-positron emission tomography-CT revealed increased uptake in the cervical and upper esophagus. No uptake was observed in the muscular layers of the middle or lower esophagus. Based on these findings, the patient was diagnosed with clinical stage IVB cervical and upper esophageal cancer (T3N1M1 [supraclavicular lymph nodes]). The patient underwent a total esophagectomy after neoadjuvant chemotherapy. The esophagus was markedly thickened and tightly adhered to the adjacent organs. Severe fibrosis was observed around the trachea. Marked thickening of the muscular layer was observed throughout the esophagus; histopathological examination revealed that this thickening was due to increased smooth muscle mass. No cartilage, bronchial epithelium, or glands were observed. The carcinoma extended from the cervical to the middle esophagus, oral to the stenotic region. Finally, we diagnosed the patient with esophageal cancer developing on CES of the fibromuscular thickening type.
Chronic mechanical and chemical irritations are believed to cause cancer of the upper esophagus oral to a persistent CES, suggesting the need for long-term surveillance that focuses on residual stenosis and cancer development in patients with CES.
先天性食管狭窄（CES）是一种罕见疾病。我们遇到一例在患有持续性CES的成年人中发生的食管癌病例。尽管许多研究已经调查了CES治疗后的疗效并对症状进行了监测，但很少有研究进行长期监测或报告食管癌的发生情况。我们报告该病例是因为其极为罕见且具有重要意义。
一名吞咽困难加重的45岁女性被转诊至我院。该患者5岁时被诊断为CES，并在另一家医院接受了手术。患者36岁时因狭窄接受了食管扩张术。我院进行的食管镜检查显示距门齿15 - 29 cm处有一环形溃疡病变及狭窄。活检标本的组织学检查显示为鳞状细胞癌。计算机断层扫描（CT）显示颈部部分及几乎整个胸段食管壁周向异常增厚。氟脱氧葡萄糖正电子发射断层扫描 - CT显示颈部及上段食管摄取增加。食管中下段肌层未见摄取。基于这些发现，患者被诊断为临床IVB期颈部及上段食管癌（T3N1M1[锁骨上淋巴结转移]）。患者在新辅助化疗后接受了全食管切除术。食管明显增厚并与相邻器官紧密粘连。气管周围观察到严重纤维化。整个食管均观察到肌层明显增厚；组织病理学检查显示这种增厚是由于平滑肌质量增加所致。未见软骨、支气管上皮或腺体。癌肿从颈部延伸至中段食管，从口侧至狭窄区域。最后，我们诊断该患者为纤维肌增厚型CES基础上发生的食管癌。
慢性机械性和化学性刺激被认为会导致持续性CES上方上段食管发生癌变，这表明需要对CES患者进行长期监测，重点关注残余狭窄和癌症发生情况。
