A 57-year-old man in whom systemic sclerosis and interstitial pneumonia had been diagnosed 17 and 3 years earlier, respectively, complained of dysphagia. Endoscopy showed an ulcerative tumor (type 3) in the lower thoracic esophagus, and it was diagnosed as squamous cell carcinoma. Pulmonary hypertension was confirmed by echocardiography with preoperative examination. The patient underwent thoracic esophagectomy with thoracic digestive reconstruction using a gastric tube via laparotomy and right thoracotomy. The pathological examination showed moderately differentiated squamous cell carcinoma (pT3 N1 M0 : pStage III). The postoperative course was uneventful. Six months later, intestinal pneumonia worsened gradually, and steroid was administered. Eight months after surgery, home oxygentherapy was indicated. Ten months after surgery a metastatic node was detected in the mediastinum, and radiotherapy was performed. The metastatic node shrank. Fifteen months after surgery, acute exacerbation of interstitial pneumonia led to respiratory failure. The patient died, although steroidpulse therapy and mechanical ventilation were performed. Esophageal squamous cell carcinoma associated with systemic sclerosis is a rare entity. Since there are complications such as interstitial pneumonia and pulmonary hypertension in systemic scleroderma, clinical practice includes evaluation of the tolerance and management of comorbidities, and in addition to the usual systemic management careful response was deemed necessary.
