Supratentorial metastatic enteropathy-associated T-cell lymphoma: A case report and literature review

Abstract

Background:We are describing a rare case of supratentorial metastatic enteropathy-associated T-cell lymphoma (EATL). While these lesions are a rare complication of EATL, the implications are grave and they must be evaluated as a diagnostic possibility when a patient with known celiac disease presents with acute neurological deterioration. In addition, multidisciplinary care teams are recommended by the authors as critical to providing the most comprehensive patient care.Case Description:A 65-year-old female presented to the emergency room with uncontrolled abdominal pain, nausea, and vomiting. Initial abdominal computed tomography (CT) scan indicated a small bowel obstruction with a transition point at the jejunal area. Differential diagnosis included small bowel neoplasm, adhesions, or a reactive intestinal inflammatory process. Shortly after presentation, the patient's clinical condition worsened, requiring emergency small bowel resection. Histological analysis of the resected bowel segments demonstrated medium-sized infiltrating lymphocytes with characteristic pleomorphic nuclei and prominent nucleoli. Immunohistochemical stains revealed tumor cells positive for CD-3. Immunohistochemical analysis for Ki-67 showed a markedly increased proliferative index, with 90% of lymphocytes staining positive. Polymerase chain reaction analysis for T-cell receptor-gamma gene rearrangement was positive, demonstrating the presence of a clonal population of T-cells. The combined morphological and immunophenotypic features of this lesion were consistent with jejunal EATL.Five weeks post-diagnosis, she developed new onset neurological symptoms consisting of changes in her mental status and left facio-brachial weakness. Brain magnetic resonance imaging (MRI) demonstrated a single ill-defined, irregular, right fronto-parietal enhancing lesion surrounded by vasogenic edema. Surgical resection and histopathologic evaluation of the biopsied lesion confirmed the diagnosis of metastatic EATL involving the brain.Conclusion:Intracranial metastasis is a rare but grave complication of EATL and must be evaluated as a diagnostic possibility when a patient with known celiac disease presents with acute neurological deterioration. Although the prognosis of these patients is dismal, aggressive oncology management is mandatory.