Isolated primary non-Hodgkin's lymphoma of the esophagus

Isolated primary non-Hodgkin's lymphoma of the esophagus

A 76-year-old man presented with fever, night sweats, dysphagia, and a 6.5 -kg weight loss. He was immunosuppressed, having been maintained on azathioprine for several years for large granular cell lymphocytic leukemia. He reported several months of progressive solid food dysphagia and odynophagia.

Physical examination findings were negative for lymphadenopathy or hepatomegaly. Complete blood cell count and routine serum chemistries were within normal limits.

Barium esophagram was performed.

A lesion was detected which was slightly lobulated, eccentric, 4 x 3 x 1.5 cm in the midesophagus. The lesion protruded into the esophageal lumen but allowed passage of liquid barium without obstruction. CT of the chest, abdomen, and pelvis was performed.

CT scan obtained at the level of the carina.

Staging radionuclide scan (whole body planar and single-photon emission computed tomography: SPECT imaging was performed with gallium-67 citrate.

Gallium 67 SPECT image shows single focus of uptake. Sagittal image taken slightly from right of midline. No other foci of abnormal uptake were present.

Follow-up

Endoscopy confirmed the esophageal mass. Pathologic evaluation of the endoscopic biopsy revealed large-cell non-Hodgkin's lymphoma. Subsequent bone marrow biopsy showed no evidence of lymphoma. With the Ann Arbor classification, the patient's lymphoma was staged IE-B (isolated extranodal location with B* symptoms).

Discussion

Non-Hodgkin's lymphoma accounts for 1-10% of all gastrointestinal tract malignancies. The gastrointestinal tract is the most common extranodal site (30-45%) of non-Hodgkin's lymphoma, accounting for 5-20% of all cases, with the stomach affected in approximately 50%, the small bowel or ileocecal valve in approximately 33%, and the large bowel in approximately 10%.

Isolated primary esophageal lymphoma is a distinctly rare event. Previously described cases show a diverse spectrum of radiographic appearances for primary esophageal lymphoma. These include polypoid, or ulcerated, mass or infiltrating stricture mimicking esophageal cancer; diffuse enlargement and tortuosity of submucosal folds, sometimes producing a varicoid appearance; discrete submucosal nodules and even a diffuse fine nodular pattern; achalasialike appearance; and tracheoesophageaal fistula formation. Given the varied and somewhat nonspecific radiographic appearances of esophageal lymphoma, we believe it is a difficult specific radiologic diagnoiss.

Esophageal involvement by lymphoma is more commonly caused by local extension from adjacent structures. The most frequent radiographic finding in esophageal lymphoma is an irregular narrowing of the distal esophagus, caused by local extenison of tumor from the gastric fundus. This appearance may be indistinguishable from gastric carcinoma. Extrinsic compression of the esophagus by mediastinal lymphadenopathy can also occur. This may result in smooth indentation of the esophagus with obtuse gently sloping borders. Further invasion of the esophageal wall by tumor will result in a more irregular or serrated contour abnormality and may ultimately cause diffuse esophageal narrowing.