Adrenal myelolipoma associated with Cushing's disease.
An 18-year-old man with a history of Cushing's disease was treated with a total right and a near total left adrenalectomy in 1956. Pathologic examination of the operative specimen revealed bilateral adrenal hyperplasia. After 13 years, recurrence of symptoms of cortisol excess necessitated cobalt irradiation to the pituitary, which was without clinical effect. After an initial response to the adrenolytic agent, o,p'-DDD, partial relapse occurred. At this time, the recognition of an abdominal mass prompted abdominal exploration revealing a huge adrenal myelolipoma containing adrenal cortical cells distributed diffusely throughout the tumor. Symptoms of adrenal insufficiency developed, and adrenal steroid secretion did not respond to exogenous adrenocorticotropic hormone postoperatively. The case illustrates that adrenal myelolipomas may become very large with continued stimulation by adrenocorticotropic hormone, may contain significant amounts of adrenal cortical tissue, and may be associated with clinical hypercortisolism.
D015065:17-Hydroxycorticosteroids / Q000652:urine; D000293:Adolescent; D000310:Adrenal Gland Neoplasms / Q000150:complications* / Q000473:pathology; D003480:Cushing Syndrome / Q000188:drug therapy / Q000209:etiology* / Q000652:urine; D006801:Humans; D008297:Male; D008939:Mitotane / Q000627:therapeutic use; D055728:Primary Myelofibrosis / Q000150:complications* / Q000473:pathology
D015065:17-Hydroxycorticosteroids; D008939:Mitotane