Mount Sinai St. Luke's and Mount Sinai Roosevelt New York, NY, United States
Angad Uberoi, MBBS1, Custon Tafadzwa T. Nyabanga, MD2, Syed Muhammad Hashim Abbas, MD3, Abdelsalam Sharabi, MD3, Michael S. Smith, MD, MBA4 1Mount Sinai St. Luke's and Mount Sinai Roosevelt, New York, NY; 2Mount Sinai Beth Israel, New York, NY; 3Icahn School of Medicine at Mount Sinai, New York, NY; 4Mount Sinai West & Mount Sinai Morningside Hospitals, New York, NY
Introduction: Burkitt Lymphoma (BL) is an aggressive form of non-Hodgkin Lymphoma (NHL) characterized by translocation of the c-myc gene on chromosome 8. There are three common variants of BL: endemic, immunodeficiency-associated and sporadic. The endemic variant is seen in African children and is associated with Epstein-Barr virus (EBV). Immunodeficiency-associated BL, is common among HIV and transplant patients. The sporadic variant, seen worldwide, accounts for 1-2% of adult lymphomas. Extra-nodal sporadic BL often involves the gastrointestinal (GI) tract. Here we describe a case of EBV-associated gastric BL which presented as linitis plastica in an otherwise immunocompetent adult.
Case Description/Methods: A 75-year-old white man was admitted with acute onset aphasia and dysarthria. His past medical history was only notable for hypertension. Acute stroke protocol was initiated, and included CT-angiography of the head and neck, which incidentally revealed hilar lymphadenopathy. Laboratory work-up was notable for acute kidney injury and elevation in serum calcium to 15.5 mg/dL. A dedicated non-contrast CT chest showed mediastinal and hilar lymphadenopathy and an upper abdomen with a diffusely thickened gastric wall (up to 3 cm). MRI brain did not reveal an acute stroke or intracranial mass. The patient’s aphasia and dysarthria gradually improved and calcium levels improved with medical treatment. An EGD was performed showing diffuse thickening of the gastric folds and overlying serpiginous superficial ulcerations (Figure 1a). Biopsies revealed aggressive B-cell NHL characterized by an infiltrative lymphoid process with prominent starry sky appearance (Figure 1b). FISH was positive for MYC-IGH [t(8;14)] fusion and immunohistochemical test was positive for EBER, consistent with a likely sporadic form of EBV-associated BL.
Discussion: Gastric BL is very rare, and in prior case reports it generally presents with predominantly GI symptoms and endoscopic findings of gastric mass or ulceration. Linitis plastica, a phenotype of infiltrative malignancy resulting in thickening and decreased distensibility of the stomach wall,
is commonly associated with diffuse type gastric adenocarcinoma. Our patient presented with predominantly neurological symptoms, no GI complaints, and linitis plastica on endoscopy. Though rare, endoscopists should consider Burkitt’s lymphoma on their differential diagnosis when they encounter diffuse thickening of the gastric wall.
Figure: Figure 1a: Endoscopic retroflexion view showing diffuse thickening of the gastric folds in the fundus and gastric body, along with some serpiginous superficial ulcerations.
Figure 1b: Gastric biopsy: Burkitt’s lymphoma with the typical “starry-sky” pattern: monomorphic proliferation of lymphocytes with round nuclei, clumped chromatin, basophilic cytoplasm and multiple small nucleoli; at low power magnification.
Angad Uberoi indicated no relevant financial relationships.
Custon Tafadzwa Nyabanga indicated no relevant financial relationships.
Syed Muhammad Hashim Abbas indicated no relevant financial relationships.
Abdelsalam Sharabi indicated no relevant financial relationships.
Michael Smith indicated no relevant financial relationships.
Angad Uberoi, MBBS1, Custon Tafadzwa T. Nyabanga, MD2, Syed Muhammad Hashim Abbas, MD3, Abdelsalam Sharabi, MD3, Michael S. Smith, MD, MBA4. P2052 - Sporadic Type Burkitt Lymphoma Presenting as Linitis Plastica, ACG 2021 Annual Scientific Meeting Abstracts. Las Vegas, Nevada: American College of Gastroenterology.