Primary Mucosa-associated Lymphoid Tissue Lymphoma of the Esophagus, Manifesting as a Submucosal Tumor

Jae Gu Jung; Hyoun Woo Kang; Suk Jae Hahn; Jong Sun Choi; Eung Joong Kim

doi:10.4166/kjg.2013.62.2.117

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Jung, Kang, Hahn, Choi, and Kim: Primary Mucosa-associated Lymphoid Tissue Lymphoma of the Esophagus, Manifesting as a Submucosal Tumor

Case Report

Korean J Gastroenterol 2013;62(2):117-121.

Published online: 4 October 2013

DOI: https://doi.org/10.4166/kjg.2013.62.2.117

Primary Mucosa-associated Lymphoid Tissue Lymphoma of the Esophagus, Manifesting as a Submucosal Tumor

Jae Gu Jung, Hyoun Woo Kang, Suk Jae Hahn, Jong Sun Choi¹, Eung Joong Kim²

Department of Internal Medicine, Dongguk University Ilsan Hospital, Goyang, Korea

¹Department of Pathology, Dongguk University Ilsan Hospital, Goyang, Korea

²Department of Thoracic and Cardiovascular Surgery, Dongguk University Ilsan Hospital, Goyang, Korea

Correspondence to: Hyoun Woo Kang, Department of Internal Medicine, Dongguk University Ilsan Hospital, 27 Donggung-ro, Ilsandong-gu, Goyang 410-773, Korea. Tel: +82-31-961-7128, Fax: +82-31-961-7141, E-mail: gangmali@naver.com

Received 20 December 2012 Revised 14 January 2013 Accepted 25 January 2013

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

We report a case of primary mucosa-associated lymphoid tissue (MALT) lymphoma in the esophagus that manifested as a large submucosal tumor (SMT). Primary esophageal lymphoma is very rare, occurring in less than 1% of all patients with gastrointestinal lymphoma. Only a few cases of MALT lymphoma in the esophagus have been reported in the English literature. A 53-year-old man was referred to Dongguk University Ilsan Hospital (Goyang, Korea) in July 2012 for further evaluation and treatment of an esophageal SMT. Endoscopy showed a cylindrically elongated submucosal mass with normal overlying mucosa in the mid esophagus, 25–30 cm from the incisor teeth. He underwent surgery to confirm the diagnosis. Pathologic findings showed diffuse small atypical lymphoid cells which were stained with Bcl-2, CD20, but not with CD3, CD5, CD23, Bcl-6, or cyclin D1. These cells showed a positive monoclonal band for immunoglobulin heavy chain gene rearrangement. Based on the pathological, immunohistochemical, and molecular biological features, the esophageal mass was diagnosed as extranodal marginal zone B-cell lymphoma of the MALT type.

Keywords: Esophagus, Lymphoma, B-Lymphocytes, Marginal zone B-cell lymphoma

References

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Fig. 1.

Endoscopic findings. (A) Endoscopic observation shows a cylindrically elongated submucosal mass in the mid esophagus (25–30 cm from the incisor teeth). (B) Endoscopic ultrasonography shows an ovoid homogenous hypoechoic mass (arrow) originating from the third hyperechoic layer (submucosa).

Fig. 2.

Chest computed tomography findings. (A) There is a mediastinal solid mass arising from the esophagus wall (arrow). Its density is homogenous. (B) Sagittal reconstruction view shows a well-circumscribed longitudinal mass, following the path of the esophagus (arrow). There is a posterior displacement of the esophageal lumen by the mass.

Fig. 3.

Pathologic features. (A) Diffuse infiltration of atypical lymphoid cells occupying the marginal zone of the lymphoid follicle (H&E; left, ×12.5; right, ×100). (B) Positive immunoreactivity for the bcl-2 protein (immunohistochemistry, ×200). (C) Positive immunoreactivity for the CD20 protein (immunohistochemistry, ×200).

Table 1.

Clinical Findings of Reported Primary Esophageal MALT Lymphomas

Patient No.	Author	Age (yr)	Sex	Past medical history	Complaint	Helicobacter pylori infection	Tumor feature			Therapy
Patient No.	Author	Age (yr)	Sex	Past medical history	Complaint	Helicobacter pylori infection	Site	Size (cm)	Endoscopic finding	Primary	Adjuvant
1	Chung et al.⁹	65	Male		Dysphagia	No	U‐ M	10×3×3	SMT	Chemotherapy (CHOP×6)
2	Nishryama et al.¹⁸	63	Female		None	No	M	10	SMT	ND
3	Hayashi et al.¹⁰	62	Female		None	No	ND		SMT	Chemotherapy (R‐ CHOP×3)
4	Hosaka et al.¹¹	83	Female		Heartburn	No	U	1 and 1	SMT	EMR
5	Kishi et al.¹²	59	Male		Tarry stool	No	U‐ L	15×6.5×6	SMT	Radiotherapy (36 Gy)
6	Kitamoto et al.¹³	74	Male	MCTD	None	No	M		SMT	Radiotherapy (36 Gy)
7	Miyazaki et al.¹⁴	49	Male		None	No	L		SMT	Operation
8	Shim et al.¹⁵	61	Male	Tbc, syphilis, autoimmune thyroiditis	None	Yes	M‐ L	2×8	SMT	Operation	H. pylori eradication
9	Soweid and Zachary¹⁶	61	Male		None	Yes (treated)	U	1×2 and 1×2	SMT	H. pylori eradication	Chemotherapy (regimen unknown)
10	Yano et al.¹⁷	70	Female		None	No	M	0.6×0.8 and 2.0×0.8	SMT	EMR	Radiotherapy (30 Gy)
11	Ogura et al.¹⁹	60	Female		Dysphagia		U‐ L		SMT	Chemotherapy (R‐ CHOP×6)

MALT, mucosa‐ associated lymphoid tissue; MCTD, mixed connective tissue disease; Tbc, tuberculosis; U, upper; M, middle; L, lower; ND, no description in the original reports; SMT, submucosal tumor; CHOP, cyclophosphamide, doxorubicin, vincristine, and prednisolone; R‐ CHOP, rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisolone; EMR, endoscopic mucosal resection.

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