First report of a mucosa-associated lymphoid tissue (MALT) lymphoma of the esophagus diagnosed by endoscopic ultrasound-guided fine-needle aspiration (EUS-FNA)

Mucosa-associated lymphoid tissue (MALT) lymphoma of the esophagus is a rare tumor [1] [2] [3] with varying presentations [4]. Only a few reports have described MALT lymphoma of the esophagus spanning the entire circumference and length of the esophagus [3]. Many of these cases were diagnosed surgically. Endoscopic ultrasound-guided fine-needle aspiration (EUS-FNA) is an established method of obtaining submucosal tissue specimens [5]. We report here the first case of MALT lymphoma of the esophagus diagnosed by EUS-FNA.

A 60-year-old woman attended our institution due to dysphagia. Upper gastrointestinal endoscopy revealed a smooth surface with visible capillaries over the entire circumference of the esophagus and a white, soft, elastic submucosal tumor covered by normal mucosa ([Fig. 1]). An esophagogram showed a slightly elevated submucosal tumor extending over the entire esophageal length ([Fig. 2]), and enhanced computed tomography demonstrated thickening of the left bronchial and esophageal walls along with swelling of the pharyngeal lymph nodes. Endoscopic ultrasonography revealed a tumorous lesion presenting as a primarily hypoechoic mass arising in the third layer, with a hyperechoic region.

Fig. 1 Endoscopic findings in a 60-year-old woman with dysphagia: a large, rounded mass with normal overlying mucosa is seen extending longitudinally along the entire esophagus.

Fig. 2 Esophagogram showing a large submucosal tumor with luminal narrowing of the whole of the esophagus.

Because a diagnosis was not reached using specimens obtained with biopsy forceps, EUS-FNA was performed ([Fig. 3]). The presence of small- to medium-sized lymphocytic cells with mildly irregular nuclei, along with CD5(–), CD10(–), CD19(+), CD20(+), CD22 (+), and λ monoclonality on flow cytometry, led to a diagnosis of MALT lymphoma of the esophagus ([Fig. 4]). We described the tumor as a malignant, extramarginal zone B-cell lymphoma of the MALT type, clinical stage IV (Lugano International Conference classification). The patient was treated with a total of six courses of chemotherapy with rituximab, cyclophosphamide, vincristine, doxorubicin, and prednisolone (R-CHOP), and a complete response was achieved.

Fig. 3 Endoscopic ultrasound showing a hypoechoic thickening in the third layer. Endoscopic ultrasound-guided fine-needle aspiration was carried out using a 22-G needle (arrows).

Fig. 4 Small- to medium-sized lymphocytic cells with mildly irregular nuclei.

To the best of our knowledge, this is the first report of EUS-FNA diagnosis of MALT lymphoma of the esophagus.

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• References

• 1 Lewin KL, Ranchod M, Dorfman RF et al. Lymphomas of the gastrointestinal tract: a study of 117 cases presenting with gastrointestinal disease. Cancer 1978; 42: 693-707
• 2 Brady LW, Asbell SO. Malignant lymphoma of the gastrointestinal tract. Erskine Memorial Lecture, 1979. Radiology 1980; 137: 291-298
• 3 Kishi K, Maedu H, Nakamura Y et al. Radiotherapy for mucosa-associated lymphoid tissue (MALT) lymphoma of the esophagus: a case report with a diagnostic and therapeutic discussion. Int J Clin Oncol 2011; Jun 10 (Epub ahead of print)
• 4 Carnovale RL, Goldstein HM, Zornoza J et al. Radiologic manifestations of esophageal lymphoma. AJR Am J Roentgenol 1977; 128: 751-754
• 5 Yamao K, Sawaki A, Mizuno N et al. Endoscopic ultrasound-guided fine-needle aspiration biopsy (EUS-FNAB): Past, present, and future. J Gastroenterol 2005; 40: 1013-1023