Esophageal Lymphoma Workup

Updated: Sep 03, 2019
  • Author: Victor Velocci, MD; Chief Editor: BS Anand, MD  more...
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Workup

Approach Considerations

No specific laboratory blood tests are required for the diagnosis of esophageal lymphoma. In primary esophageal lymphoma, the WBC count is, by definition, within the reference range.

Anemia and thrombocytopenia are common in AIDS patients who present with lymphoma. The CD4 count is usually low. In a series of 22 AIDS patients with non-Hodgkin lymphoma, 2 of whom had esophageal involvement, the mean CD4 cell count was 80.8 ± 119.6/µL.

Staging

Initial staging studies include a complete blood count (CBC), a bone marrow biopsy, and computed tomography (CT) scans of the chest, abdomen, and pelvis. Classification of esophageal lymphoma as a primary lesion can only be made once the criteria of Dawson et al are fulfilled. [5] (See Overview.)

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Radiography and CT Scanning

Radiography

The radiographic appearance of esophageal lymphoma varies and is somewhat nonspecific; therefore, esophageal lymphoma is a difficult diagnosis to confirm with radiographic studies.

Barium swallow

Esophageal lymphoma has no pathognomonic appearance. Barium swallow studies of the esophagus may reveal thickened folds with nodular, polypoid, ulcerated, or stenotic features. A radiographic picture consistent with pseudoachalasia may also be present. However, these features cannot help to differentiate esophageal lymphoma from other benign or malignant esophageal diseases.

CT scanning

CT scanning is not diagnostic in esophageal lymphoma; instead, it is used in staging of the disease and in evaluating response to therapy. [10]

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Esophagogastroduodenoscopy

Esophagogastroduodenoscopy (EGD) is the only way to directly visualize and biopsy esophageal lymphoma. [11] The morphologic appearance of the tumor may be of little help, especially in patients with acquired immunodeficiency syndrome (AIDS), in whom candidal superinfection is common. An exception to this occurs in cases of mantle cell lymphoma with esophageal involvement in which multiple white submucosal nodules or plaques have been described. [12, 13, 14]  In these settings, endoscopic findings vary from other types of esophageal lymphoma, wherein the tumor may appear as an ulcerated, polypoid, or submucosal mass.

Routine endoscopic biopsies may not be useful in making a diagnosis because of the submucosal nature of the lesion. In one study, all patients required repeat endoscopy and biopsy to confirm the diagnosis. Endoscopic biopsies may have a false-negative rate of greater than 30%. Endoscopic mucosal resection of the esophageal lesion has a greater diagnostic yield.

In one series, 27 patients with lymphomatous involvement of the esophagus were identified. Of these, 3 had primary esophageal lymphoma. EGD confirmed the diagnosis in 81% of patients, although 19% required surgical exploration to establish the diagnosis.

If suspicion for esophageal lymphoma is high, consider other diagnostic modalities (eg, computed tomography scans, surgical biopsy).

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Endoscopic Ultrasonography

In one case report of primary esophageal lymphoma, the endosonographic features were reported as diffuse, homogenous, hypoechogenic esophageal wall thickening. Esophageal lymphomas may demonstrate anechoic areas.

Endoscopic ultrasonographically guided fine-needle aspiration (EUS-FNA) of suspicious submucosal lesions may help to obtain tissue and to establish a diagnosis, such as mucosa-associated lymphoid tissue lymphoma (MALT). [15] In patients who receive chemotherapy or radiation for primary esophageal lymphoma, EUS may be helpful in evaluating the response to treatment.

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Histologic Findings

Most reported cases of primary esophageal lymphoma are diffuse large cell lymphomas of the B-cell immunotype. In general, surface markers of the tumor cells reveal positive immunofluorescent staining results for immunoglobulin G (IgG) and kappa light chain.

Mucosa-associated lymphoid tissue (MALT) lymphoma has been found in the esophagus and is being increasingly reported, although still very rare. Unlike MALT lymphoma of the stomach, MALT lymphoma of the esophagus does not appear to be associated with Helicobacter pylori. [16, 17]

Other histologic variants include anaplastic large cell lymphoma, with 3 cases reported in the literature, and mantle cell lymphoma. [18, 12]

Gupta et al summarized the histologic findings of 17 patients with primary esophageal lymphoma. All but 1 had non-Hodgkin lymphoma, with large cell lymphoma being the most common histologic subtype.

If the tumor type is difficult to determine on hematoxylin and eosin staining, using monoclonal antibodies to the leukocyte common antigen may help to differentiate hematopoietic from nonhematopoietic malignancies. This may be useful for patients in whom it is difficult to distinguish a poorly differentiated carcinoma or sarcoma from a lymphoma.

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