Caldwell C B, Bains M S, Burt M
Department of Surgery, Memorial Sloan-Kettering Cancer Center, New York, NY 10021.
J Thorac Cardiovasc Surg. 1991 Jan;101(1):100-7.
Primary noncarcinomatous malignant neoplasms of the esophagus are uncommon and data concerning treatment and results are sparse. To evaluate the results of therapy in this group, we reviewed the records of 32 patients with primary esophageal malignant tumors of unusual histologic type. Thirteen patients (41%) had sarcoma, eight (25%) melanoma, and 11 (34%) had oat cell carcinoma. Dysphagia was present in 78% (25/32) of the patients for a median of 13 weeks before diagnosis. Location of the esophageal primary tumor was upper third in four patients (12%), middle third in 12 (38%), and lower third in 16 (50%). Treatment consisted of esophagectomy in 10 of 13 patients with sarcoma (77%), seven of eight with melanoma (88%), and three of 11 with oat cell carcinoma (27%). Patients not undergoing resection received chemotherapy or radiation therapy, or both. The 3- and 5-year survival rates were 46% and 23% for sarcoma (median 20 months), 13% and 0% for melanoma (median 5 months), and 0% and 0% for oat cell carcinoma (median 5 months), respectively. Distant disease was the initial form of recurrence in 73% (11/15) of patients undergoing curative therapy. Surgical resection appears indicated for localized primary esophageal sarcoma. Optimum treatment of primary esophageal melanoma is less clear, but surgical resection may be of benefit in selected patients. Esophageal oat cell carcinoma is a systemic disease necessitating systemic therapy with local therapy reserved for palliation of dysphagia.
原发性非癌性食管恶性肿瘤并不常见，关于其治疗及结果的数据也很稀少。为评估该组患者的治疗结果，我们回顾了32例组织学类型罕见的原发性食管恶性肿瘤患者的病历。13例患者（41%）患有肉瘤，8例（25%）患有黑色素瘤，11例（34%）患有燕麦细胞癌。78%（25/32）的患者在诊断前中位13周出现吞咽困难。食管原发性肿瘤的位置，上三分之一段有4例患者（12%），中三分之一段有12例（38%），下三分之一段有16例（50%）。治疗方法包括：13例肉瘤患者中有10例（77%）接受了食管切除术，8例黑色素瘤患者中有7例（88%）接受了食管切除术，11例燕麦细胞癌患者中有3例（27%）接受了食管切除术。未接受手术切除的患者接受了化疗或放疗，或两者皆用。肉瘤患者的3年和5年生存率分别为46%和23%（中位生存期20个月），黑色素瘤患者为13%和0%（中位生存期5个月），燕麦细胞癌患者为0%和0%（中位生存期5个月）。在接受根治性治疗的患者中，73%（11/15）远处转移是复发的初始形式。对于局限性原发性食管肉瘤，手术切除似乎是合适的。原发性食管黑色素瘤的最佳治疗方法尚不太明确，但手术切除可能对部分患者有益。食管燕麦细胞癌是一种全身性疾病，需要进行全身治疗，局部治疗仅用于缓解吞咽困难。
