Korean J Gastroenterol.  2024 Apr;83(4):157-162. 10.4166/kjg.2024.021.

A Case of Esophageal MALT Lymphoma Mimicking a Subepithelial Tumor

• Lee HE
• Kim GH
• Kim MJ
• Kim KB
• Joo DC
• Jeon HK
• Lee MW
• Lee BE

• KMID: 2555649
• DOI: http://doi.org/10.4166/kjg.2024.021

Abstract

Mucosa-associated lymphoid tissue (MALT) lymphoma, also known as extranodal marginal zone lymphoma, is a low-grade B-cell lymphoma that can develop in the mucosal layer of various organs, including the gastrointestinal tract, salivary glands, lungs, and skin. The most common site is the gastrointestinal tract, particularly the stomach. On the other hand, primary esophageal lymphomas are extremely rare. MALT lymphomas can undergo histological transformation into more aggressive B-cell lymphomas, such as diffuse large B-cell lymphoma, resulting in a poor prognosis. This paper reports a rare case of primary esophageal MALT lymphoma mimicking a subepithelial tumor located in the lower esophagus that was treated successfully with radiotherapy. MALT lymphoma should be included in a differential diagnosis when subepithelial tumors are found in the esophagus, particularly if endoscopic ultrasonography reveals the tumor to be located in the deep mucosal and submucosal layers. Following the precise diagnosis, accurate staging and appropriate treatment are crucial. Regular follow-up is necessary to assess the possibility of recurrence or transformation to high-grade lymphoma.

Keyword

Endoscopic ultrasonography; Esophagus; Lymphoma; Subepithelial tumor

Figure

• 

  Fig. 1 Endoscopic and endosonographic findings of an esophageal subepithelial tumor. (A) A 2 cm-sized subepithelial tumor covered with normal mucosa was observed in the lower esophagus. (B) Endoscopic ultrasonography (EUS) shows a relatively homogenously hypoechoic lesion with latticework structures in the deep mucosal and submucosal layers. (C) On EUS elastography, its consistency is not hard. (D) Contrast-enhanced EUS shows increased enhancement only in the septal portion of the latticework structures. (E) Follow-up endoscopy one month after radiotherapy shows complete remission of the previous lesion.

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  Fig. 2 Histopathological findings. (A) Atypical lymphocytes infiltrate the deep mucosa and submucosa (H&E stain, x100). (B) Small lymphocytes characterized by central nuclei, irregular nuclear membranes, and abundant clear cytoplasm invade the epithelium of the esophagus (H&E stain, x400). (C–E) Tumor cells are positive for CD20 (C) but negative for CD5 (D) and CD10 (E) (immunohistochemical stain, x100). (F) Ki-67 index is approximately 20% (Ki-67 stain, x100).

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  Fig. 3 PET-CT findings. (A) Initial PET-CT shows a hypermetabolic lesion (maximum standardized uptake value=5.4, arrow) in the lower thoracic esophagus. (B) Follow-up PET-CT one month after radiotherapy shows no abnormal glucose metabolism, confirming complete remission. PET-CT, positron emission tomography-computed tomography.

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