Double-hit primary high-grade gastric B-cell lymphoma presenting with pancytopaenia and atraumatic back pain: A case report

Abstract

BACKGROUND

Primary gastric lymphoma is relatively rare, accounting for 5% of primary gastric neoplasms, with the two most common lymphoma subtypes being diffuse large B-cell lymphoma and mucosa-associated lymphoid tissue lymphoma. Many patients with gastrointestinal lymphoma have a delayed presentation involving vague symptomatology. On endoscopy, there are three main injury patterns of gastric lymphoma: Ulceration, diffuse infiltration with enlarged mucosal folds, and the development of a polypoid mass, usually affecting the fundus or antrum. “Double-” and “triple-hit” high-grade B-cell lymphoma are an emerging subtype of B-cell lymphoma characterised by cellular melocytomatosis oncogene and B-cell lymphoma (BCL) 2 and/or BCL6 rearrangement identified by cytogenetic testing or fluorescence in situ hybridisation testing. This is an uncommon variant of B-cell lymphoma, which is associated with an adverse prognosis compared to its standard diffuse large B-cell lymphoma counterparts; however, there is limited literature pertaining to its clinical significance in the context of primary gastric high-grade lymphoma.

CASE SUMMARY

We present a case of a 71-year-old male with disseminated primary gastric high-grade B-cell lymphoma with bony metastases. This was identified after the patient presented to the emergency department on three separate occasions with vague symptomatology, including chest, abdominal and back pain, and loose stools. Chest X-ray identified an atraumatic left rib fracture, and this was further explored with an outpatient computed tomography chest, which showed left fifth, seventh and eighth rib fractures, with ill-defined lucencies suspicious for underlying lesions. Computed tomography lumbar spine showed possible metastatic disease in the lumbar vertebrae. Positron emission tomography scan revealed non-specific mild to moderate heterogeneous uptake in the stomach, with disseminated, mixed, predominantly lytic bone metastases throughout the axial and appendicular skeletons. Gastroscopy subsequently identified multiple 10 mm to 20 mm semi-sessile polyps in the stomach, and biopsy confirmed high-grade “double-hit” B-cell lymphoma with melocytomatosis oncogene and BCL6 rearrangements. The patient was treated with rituximab, etoposide, prednisolone, vincristine, cyclophosphamide, doxorubicin and intrathecal methotrexate.

CONCLUSION

This case report highlights an uncommon presentation of high-grade primary gastric lymphoma and re-emphasises the aggressive nature of the disease.

Keywords: Gastroenterology; Primary gastric lymphoma; B-cell lymphoma; Gastrointestinal lymphoma; Case report

Core Tip: “Double-hit” primary gastric high-grade B-cell lymphoma is a rare variant of B-cell lymphoma that can present quite aggressively and is associated with significant morbidity and potential mortality. Endoscopically, these lesions can present as a polypoid mass, ulceration or prominent mucosal folds, and, in the absence of clinical suspicion, can be easily missed. Timely diagnosis via endoscopy and treatment is important to ensure positive outcomes; however, the literature is limited with regard to the prognosis of “double-hit” primary gastric high-grade B-cell lymphoma compared to its nodal and extra-nodal counterparts.