Adenoid cystic carcinoma of the esophagus. Complete response to combination chemotherapy

Abstract

A 55-year-old man had adenoid cystic carcinoma of the esophagus metastatic to the lungs and right supraclavicular fossa. He was treated with local radiation therapy to the esophagus and supraclavicular fossa, followed by combination chemotherapy with doxorubicin, mitomycin C, and 5-fluorouracil (5-FU). After a modest initial response, disease progression was noted in the pulmonary nodules. He was then treated with cisplatin, cyclophosphamide, vincristine, and doxorubicin. After two cycles of this regimen, there was complete regression of his pulmonary nodules, which was sustained for 5 months. A review of 44 literature cases of esophageal adenoid cystic carcinoma contrasted with adenoid cystic carcinoma of salivary gland origin indicated that the esophageal adenoid cystic carcinomas have a high tendency to metastasize (76% of cases) and a much poorer prognosis, with only 23% 1-year survival rate. It was concluded that esophageal adenoid carcinoma is clinopathologically distinct from the salivary gland variant, and that combination chemotherapy may be an effective treatment modality for this cancer.

Adenoid cystic carcinoma of the cardia: Report of a rare case and review of the Chinese literature

“…Since the first study, reported in 1954 by Gregg and Stamler (4), there have only been 17 cases reported in China and ~60 cases in other countries. Petursson (5) reviewed 45 cases of esophageal ACC. The mean age of patients with esophageal ACC was 65 years, and the male to female ratio was 3.4:1.…”

“…Meanwhile, the therapeutic effect of radiotherapy and chemotherapy for esophageal ACC remains controversial. The study by Petursson (5) reported that a combination of chemotherapy with cyclophosphamide, vincristine, adriamycin and cisplatin completely remitted ACC. The TNM staging of the patient in the present study was stage IIB.…”

Primary Esophageal Adenoid Cystic Carcinoma

“…The most common endoscopic findings include a fungating or polypoid mass rather than an ulcerative or infiltrative growth pattern. 5 Only three cases have been reported where they were presented as submucosal mass (Table 1). [8][9][10] One of the tumors was investigated by endoscopic ultrasonography.…”

“…3 The clinical behavior of esophageal ACC is not well known due to the small number of reported cases. [5][6][7] The average age of patients is 65 years, with a male to female ratio of 3.4 to 1. The most common symptom is progressive dysphagia.…”

“…The most common symptom is progressive dysphagia. 5,7 ACCs have most frequently been reported in the middle third of the esophagus (63%), less often in the lower third (30%) and rarely in the upper third (7%). The most common endoscopic findings include a fungating or polypoid mass rather than an ulcerative or infiltrative growth pattern.…”