Objective: To report the first case of an ectopic adrenocorticotropic hormone (ACTH) syndrome caused by a sacrococcygeal chordoma.
Methods: We present a case report with clinical, laboratory, and histologic details.
Results: A 76-year-old man was admitted to the hospital because of urinary obstruction. Five years previously, a urethral mass had been detected, and transurethral prostatectomy had been performed. Annual computed tomographic (CT) scans showed no change in size of the mass. In 1995, skin hyperpigmentation, central obesity, and bilateral edema were noted. The patient was admitted to the hospital in July 1996. A CT scan of the abdomen revealed a large mass close to the sacrum and compressing the bladder and rectum. Cortisol measurements (AM and PM) were 309 and 271 ng/mL, respectively. The plasma ACTH concentration was extremely elevated (3,125 pg/mL). Although resection of the mass was attempted, complete resection was not possible because the tumor had infiltrated the sacrum. Plasma cortisol concentrations in samples obtained 7 and 8 days postoperatively were normal. Plasma ACTH was substantially decreased (180 pg/mL) but remained above normal. The histologic features of the tumor were compatible with a chordoma. Neoplastic cells stained positively for ACTH.
Conclusion: This report describes the first case of an ectopic ACTH syndrome caused by a sacrococcygeal chordoma. A slow progression of symptoms in an ectopic ACTH syndrome had been described only for carcinoid tumors. These data add a new entry to the list of neoplasms capable of causing this syndrome.