Michael Jorgensen, MD, Kazi Ullah, MD, Lionel D'Souza, MD Stony Brook Medicine, Stony Brook, NY
Introduction: Mucosa associated lymphoid tissue lymphoma (MALToma) is a low-grade malignant B-cell lymphoma. Primary esophageal MALToma is extremely rare, with less than 20 reported cases in the literature, most of which are reports from Japan. Follow-up beyond two years is even more sparse. Treatment ranges from radiotherapy to surgical resection. Here, we report a case of primary esophageal MALToma successfully treated with endoscopic mucosal resection (EMR) without recurrence four years later.
Case Description/Methods: A 60-year-old female with history of acid reflux presented for endoscopic ultrasound (EUS) evaluation of incidental finding of small submucosal nodule during routine esophagogastroduodenoscopy (EGD). EUS showed a small 5 x2.5 mm submucosal nodule in the upper third of the esophagus; biopsies were taken with a cold jumbo forceps with removal of a large part of the lesion. Sonographically, the origin appeared to be within the muscularis mucosa. Pathology revealed atypical subepithelial lymphoid proliferation with stains suspicious for extra nodal marginal zone MALToma. Tumor board review further supported a diagnosis MALToma. PET scan revealed increased uptake in the cervical esophageal area, without additional site of involvement.
Repeat endoscopy a month later revealed a single 7 mm submucosal nodule consistent with known MALToma. Band ligator and snare mucosal resection with suction was performed. Pathology revealed atypical lymphoid proliferation of the subepithelial layer and lamina propria. Complete removal was accomplished with margins free of lymphoid infiltrate. The patient underwent surveillance EGD with EUS at the six month and four-year interval without evidence of disease recurrence. Biannual PET scan surveillance, now four years later, continues to show absence of disease.
Discussion: Primary esophageal MALT lymphoma is an exceedingly rare diagnosis, with no clear guidance for initial treatment or surveillance. This patient’s tumor was diagnosed incidentally, on screening EGD. The clinical course for MALT lymphoma remains unclear. Depending on nodule size, endoscopic resection is a viable alternative to surgical resection. This patient had successful resection of her lymphoma via EMR, without evidence of recurrence on biannual PET scan or repeat endoscopy with EUS four years later. Further clinical cases with associated follow up are needed to better understand this rare disease, potential treatment options and its subsequent clinical course.
Disclosures:
Michael Jorgensen indicated no relevant financial relationships.
Kazi Ullah indicated no relevant financial relationships.
Lionel D'Souza indicated no relevant financial relationships.
Michael Jorgensen, MD, Kazi Ullah, MD, Lionel D'Souza, MD. P2274 - Successful Endoscopic Mucosal Resection of Primary Esophageal MALT Lymphoma and Subsequent Surveillance, ACG 2024 Annual Scientific Meeting Abstracts. Philadelphia, PA: American College of Gastroenterology.