NK/T cell lymphoma-induced ectopic ACTH syndrome: a case report

Primary adrenal natural killer (NK)/T cell lymphoma is extremely rare with rapidly aggressive clinical manifestation and poor prognosis. Here, we report a case of NK/T-cell lymphoma with bilateral adrenal involvement and secondary ectopic adrenocorticotropin syndrome (EAS).

This is a 56-year-old woman with main complaint of fatigue and slight weight loss. Bilateral adrenal mass, rapid progress of pancytopenia, and elevated cortisol and ACTH levels were discovered. Cushing's syndrome was diagnosed when serum cortisol was not suppressed after 1-mg dexamethasone suppression test and low-dose dexamethasone suppression test (LDDST). Morning cortisol and ACTH levels markedly increased after 1 week, whereas the pathology of bone marrow revealed NK/T cell lymphoma, which indicated the diagnosis of EAS. After two cycles of chemotherapy, the patient died 6 months after diagnosis.

NK/T cell lymphoma should be considered in the differential diagnosis of bilateral enlarged adrenal mass and could induce ectopic ACTH syndrome. For rapid progressing malignancy, the clinical features of Cushing's syndrome may be absent in patients with EAS.