Case 405 -- Esophageal perforation with an unusual etiology

Contributed by Amy Davis, MD and Fiona Craig, MD
Published on line in October, 2004


The patient is a female in her mid 80's who underwent emergent esophagectomy after an attempt at routine esophageal dilatation for stricture resulted in perforation. The patient had no significant medical history; specifically of a hematolymphoid malignancy.


The specimen included the esophagus and proximal portion of stomach, 22cm in total length. Examination of the mucosal surface of the esophagus revealed no obvious perforation and no mucosal lesions (image 1). A submucosal defect with thinning of the muscularis propria was noted. There appeared to be a probe-patent separation between layers of the esophageal wall. Multiple lymph nodes were identified and submitted for histologic evaluation.

Based on the clinical history and gross examination, there was no indication of a lymphoid process, so no fresh tissue was sent for flow cytometry, molecular, or cytogenetic studies. After initial slide review, the case was transferred to the hematopathology service.

H & E:

Low power histologic evaluation revealed a dense submucosal lymphocytic infiltrate extending into the muscular wall of the esophagus (images 2 & 3). Focal necrosis of the wall was present. At high power, the infiltrate had a monocytoid appearance with small lymphocytes containing abundant pale cytoplasm (image 4). A few larger cells were also present. Several reactive appearing follicles with germinal centers were noted. The small portion of gastric fundic mucosa showed reactive epithelial changes with mild chronic inflammation.

Sections from the regional lymph nodes demonstrated disruption of the architecture by an infiltrate with a predominance of small lymphocytes with abundant pale cytoplasm similar to that observed in the esophagus. The infiltrate had a vaguely nodular growth pattern (image 5). A few germinal centers were recognized (image 6).


Multiple immunohistochemical stains were performed on both the esophagus and a regional lymph node. In the esophagus, CD20 demonstrated numerous positive cells in the lymphoid infiltrate (image 7). BCL-6 was positive in germinal centers and negative in the neoplastic cells (image 8). Ki-67 proliferation index highlighted many positive cells in follicles and fewer positive cells outside of follicles (image 9). Cyclin D1 for mantle cell lymphoma and the helicobacter pylori immunohistochemical stains were negative.

In the lymph node, CD20 demonstrated many positive cells extending from the cortex to the medullary cord region (image 10). As in the esophagus, BCL-6 was positive in germinal centers and negative in the infiltrate (image 11). CD10 was positive in the germinal centers (image 12), and kappa was positive focally in excess of lambda (image 13). BCL-2 was negative in germinal centers, positive in T-cells, and variable in the infiltrate. Cyclin D1 was negative. CD3 highlighted a few residual T-cell nodules and scattered positive cells but was negative in the neoplastic infiltrate. CD5 and CD43 were similar in distribution to CD3.


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