Endocrine Abstracts

Introduction: Monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL), formerly known as type II enteropathy-associated T-cell lymphoma, is a rare and aggressive subtype of lymphoma of the gastrointestinal track typically noted in Asian or Hispanic populations. Adrenal involvement as part of MEITL is extremely rare. Herein we present a patient of Greek origin with MEITL and bilateral adrenal metastases.

Case Presentation: A 74-year-old man presented with a 2-week history of right upper quadrant abdominal pain, decreased appetite and fatigue, as well as weight loss (10 kgs in 8 months). Abdominal CT imaging revealed heterogenous bilateral adrenal lesions (maximum diameter 11 cm on the left and 10 cm on the right, respectively). On clinical examination, there were no signs of cortisol excess, no skin pigmentation, no palpable lymph nodes and the abdomen was soft. Hormonal investigations revealed primary hypocortisolism (basal cortisol: 61 nmol/l, ACTH: 189 pg/ml) and treatment with hydrocortisone was initiated; twenty-four-hour urine metanephrines were normal. Further investigations showed anemia and increased LDH (1365 IU/L, normal values: 134-279), β2-microglobulin (4.99 ng/L, normal values: 0.97-2.64) and inflammation markers (ESR, CRP and ferritin). Primary bilateral adrenal lymphomas were suspected and fine needle biopsy of the left adrenal lesion was performed. Staging CT scans showed no brain or lung metastases but gastric wall thickening and infiltration of the right kidney by the adrenal mass were noted on the abdominal scan. Gastroscopy revealed a mucosal lesion with a micronodular and ulcerated surface which was biopsied. Histology from both the adrenal and the gastric lesions was consistent with MEITL; Ki-67 was positive in approximately 80% of tumor cells. Bone marrow biopsy and aspiration did not show any signs of infiltration by the lymphoma. Treatment with high dose dexamethasone was initiated but the patient rapidly deteriorated. He developed sepsis with multi-organ failure and finally passed away before receiving combined chemotherapy.

Discussion: MEITL is a rare aggressive T-cell lymphoma arising from intestinal intraepithelial lymphocytes with a poor prognosis. Most often it involves the small bowel, particularly the jejunum and ileum, but it can also involve the colon or stomach, like our case. Involvement of the adrenal glands in patients with MEITL is extremely rare. To our knowledge, this is the second report of MEITL with bilateral adrenal metastases. Clinical experience, awareness and a multidisciplinary approach in such perplexing cases is required.