Adenoid cystic carcinoma of the esophagus: report of two cases and review of the Chinese literature
© Guo et al.; licensee BioMed Central Ltd. 2012
Received: 7 November 2012
Accepted: 4 December 2012
Published: 13 December 2012
Squamous cell carcinoma is the major pathology type of esophageal cancer in China, where adenocarcinoma is rare and adenoid cystic carcinoma (ACC) is more rare comparing to the western countries. We report the surgical and pathologic findings of two cases of primary ACC of the esophagus, and review of the Chinese literature of this tumor.
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KeywordsAdenoid cystic carcinoma Esophagus Surgery
Adenoid cystic carcinoma (ACC) is not uncommon in the salivary glands and respiratory tract; however, it occurs extremely rarely in the esophagus, where its behavior is biologically aggressive . It constitutes 0.1% of all esophageal malignancies, and only 60 cases have been reported so far in the literature . Herein, we present 2 cases of ACC of the esophagus and review the only 13 other cases of ACC of the esophagus reported in China, in order to clarify the clinicopathological features of ACC of the esophagus.
The resected tumor was a cauliflower-like mass, measuring 5.5 × 4.2 × 3 cm in size, and pathologically invaded adventitia with no metastases to lymph nodes (Figure 2D). The tumor cells were small and darkly stained with scanty cytoplasm and vesicular nuclei. A cribriform pattern was seen, but there was a tendency for the formation of solid or basaloid areas with amorphous eosinophilic material and comedo necrosis (Figure 2E). Immunohistochemically, tumor cells expressed CK, VIM and P63 protein. Scattered cells expressed S-100 protein. The surgical resection margins were clear and all biopsies of lymph nodes were free of metastases. The pathologic diagnosis was reported as primary ACC of the esophagus (pT3N0M0).
The patient was diagnosed with anastomotic leak on post-operative day 10, then we gave him jejunal feeding tube guided by esophagoscopy. Barium esophagogram indicated the anastomotic leak was cured one month later. Nine months postoperatively, the computed tomography of the abdomen demonstrated liver metastasis.
ACC of the esophagus was rare, since Gregg  first reported it in 1954, only 15 cases including our 2 cases were reported in China. There were studies showed that people who with hiatal hernia, obesity (visceral fat), smoking or alcohol abuse as well, are more likely to presence of gastroesophageal reflux disease . Then, infiltration of eosinophils into the esophagus could result to conditions such as eosinophilic esophagitis which may be the risk factor for development of ACC of the esophagus .
Clinical data on the 15 cases of ACC of the esophagus reported in China
42-70 (60.4 ±6.6)
Cervical: 1 Up-thoracic: 0 Mid-thoracic: 8 Low-thoracic: 6
Protruding: 10 Ulcerative: 5
Adenoid cystic carcinoma
Squamous cell carcinoma
1 (Misdiagnosed as upper esophageal sphincter achalasia)
1 (Preoperative brain metastasis)
Depth of invasion (Surgical cases)
Metastasis to lymph nodes (Surgical cases)
Biopsy of the tumor by preoperative endoscopy gave poor diagnostic results. In fact, only 1 of 14 such reported biopsies suggested ACC at that time. 10 biopsy specimens were misinterpreted as squamous cell carcinoma being the most frequent misdiagnosis, 2 cases were misdiagnosed as leiomyomata because of the normal esophageal mucosa overlying the tumor and 1 case was misdiagnosed as upper esophageal sphincter achalasia. Sweeney and Cooney reported  that one of the difficulties in diagnosing ACC of the esophagus from a biopsy specimen is related to the fact that small tissue samples may not display the characteristic architecture of the tumor. Esophageal adenoid cystic carcinoma diagnosed mainly rely on microscopic examination. Histologically, ACC of the esophagus show three different growth patterns similar to the salivary gland: cribriform, tubular and solid. The solid pattern appears to be associated with worse prognosis than the other two patterns . The most commonly depth of invasion was submucosa (7/14) in our data. This may clarify the fact that ACC of the esophagus arise from the deep submucosa glands of the esophagus.
Because of the rarity of ACC of the esophagus and paucity of data in the literature, few treatment guidelines are available for esophageal ACC. The first choice for treatment of ACC of the esophagus is radical excision. Chemotherapy is not usually chosen due to a poor response rate [8–10]. Postoperative radiotherapy may help improvement of progressive dysphagia [11–13]. Unfortunately, we can not give a complete follow-up data in Chinese literature. Foreign learners reported that the prognosis of ACC of the esophagus was poor, with organ metastasis occurring more frequently than lymph node metastasis . The 5-year survival rate is approximately 35%, but the long-term survival is poor. Eighty to 90% of patients die of this disease within 10–15 years .
In a word, ACC of the esophagus is extremely rare. Its diagnosis must be based upon histopathological characteristics. Surgery is the first choice for resectable lesions.
Written informed consent was obtained from the patient for publication of this Case Report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
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