Primary High-Grade Neuroendocrine Carcinoma of the Esophagus: A Clinicopathologic Study of 42 Cases
Qin Huang, Hongyan Wu, Qi Sun, Qing Ye. Nanjing Drum Tower Hospital, Nanjing, China; VA Boston Healthcare System and Harvard Medical School, West Roxbury
Background: High-grade neuroendocrine carcinoma of the esophagus (HNCE) is rare and poorly understood. We aimed to systematically investigate clinicopathologic features of this fatal cancer in Chinese patients treated at a high-volume medical center in Nanjing, China.
Design: We searched pathologic files for esophageal cancer resections at the Nanjing Drum Tower Hospital in China over the period from January 2004 through August 2010. Pathologic reports were reviewed, along with medical, radiologic, and endoscopic files. HNCE was defined and classified with the same criteria for pulmonary counterparts. All histologic slides of selected cases were evaluated (14 slides per case on average). A corresponding archival tumor tissue block was used for immunostains with conventional methods.
Results: In 1,105 consecutive resection cases of esophageal cancer over the 7-year period, 42 (3.8%) were qualified for the study with strong immunoreactivity to synaptophysin. Patient median age was 62 years (range 47-79). The M/F ratio was 3.7. The epicenter of tumors located in the upper, middle, and distal esophagus was in 2%, 57%, and 40%, respectively. Tumor gross appearances were exophytic (31%), fungating (17%), ulcerated (48%), or flat (5%). The mean tumor size was 3.5 cm (range: 0.2-8). By histology, the small, large, and mixed small-large cell types accounted for 64%, 10%, and 26%, respectively. The majority (86%) of tumors were poorly differentiated. Focal squamous, glandular, and signet-ring differentiation were noted in 19%, 14%, and 2%, respectively. Squamous cell carcinoma in situ was found in 53%. Synchronous tumors (12%) included 3 squamous cell carcinomas, 1 gastrointestinal stromal tumor, and 1 mesothelioma. Lymphovascular (50%)/perineural (33%) invasion and metastasis to regional (48%) and celiac (29%) lymph nodes were common. Neoplastic cells exhibited strong immunoreactivity to chromograinin (67%), synaptophysin (100%), CD56 (93%), TTF1 (71%), CK8/18 (90%), p63 (55%), CD117 (86%), and p16 (84%) protein antigens.
Conclusions: In Chinese patients, most HNCE tumors were the small-cell type, located in the middle esophagus, associated with squamous cell carcinoma in situ, and showed extensive lymphovascular, perineural, and nodal metastasis as well as a high expression rate for p63, TTF1, CD117, and p16 proteins, suggesting an aggresive behavior.
Wednesday, March 21, 2012 9:30 AM
Poster Session V # 64, Wednesday Morning