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.