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Hyperadrenocorticism in a Patient with Esophageal Squamous-Cell Carcinoma
- Stephen Migueles, MD;
- Susan Thompson, MD; and
- Sean McGrade, MD
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TO THE EDITOR:
The ectopic corticotropin syndrome in association with nonpancreatic gastrointestinal tract cancer is rare. We report an unusual case of hyperadrenocorticism in a patient with metastatic esophageal squamous-cell carcinoma.
A 64-year-old man presented with a 3-month history of dysphagia and 11.35-kg weight loss. Physical examination was remarkable for a blood pressure of 160/98 mm Hg, cachexia, numerous ecchymoses, and globally diminished strength and reflexes. Abnormal laboratory values included a hematocrit of 0.34; a serum potassium level of 2.1 mEq/L, a chloride level of 86 mEq/L, a bicarbonate level of 42 mEq/L, and a glucose level of 264 mg/dL.
Esophogastroduodenoscopy revealed a friable lesion in the mid-esophagus. Computed tomography showed an esophageal mass, a low-attenuation hepatic lesion, and bilateral adrenal enlargement. The following findings were diagnostic for the ectopic corticotropin syndrome: elevated urinary free cortisol excretion (4508 µg/24 hours [normal, 20 to 90 µg/24 hours]); increased late-afternoon serum adrenocorticotropic hormone (ACTH) concentration (88 pg/mL [normal, 4 to 52 pg/mL]); low corticotropin-releasing hormone level (20 pg/mL [normal, 24 to 40 pg/mL]); and lack of suppressibility on 2-day, high-dose dexamethasone suppression testing. Pathologic examination of the esophageal tissue revealed poorly differentiated squamous-cell carcinoma. Positive results on immunohistochemical analysis for synaptophysin, keratin, and enolase indicated neuroendocrine differentiation. Staining for ACTH was negative.
Ketoconazole was administered to inhibit adrenal steroid biosynthesis. For widespread metastases, the patient received cisplatinum and 5-fluorouracil. The patient died shortly after leaving the hospital against medical advice. Autopsy was refused.
Neoplasms of the lung, thymus, and pancreas are the main producers of ectopic ACTH. In 1968, Lohrenz and Custer [1] described the only other patient with the ectopic corticotropin syndrome associated with esophageal squamous-cell carcinoma.
Negative staining for ACTH in tumor tissue does not exclude ACTH biosynthesis [2]. Some explanations include a predominance of ACTH precursors that are not immunoreactive to a particular anti-ACTH serum and excessive hormone secretion rates leading to a reduced storage of pro-opiomelanocortin-derived peptides [3].
Neuroendocrine esophageal cancer and no evidence of a pulmonary, thymic, or pancreatic tumor render the former the most probable source of the ectopic ACTH syndrome in our patient.
Stephen Migueles, MD
Susan Thompson, MD
Sean McGrade, MD
Georgetown University Medical Center; Washington, DC 20007
The Editors welcome submissions for possible publication in the Letters section. Authors of letters should:
•Include no more than 300 words of text, three authors, and five references
•Type with double-spacing
•Send three copies of the letter, an authors' form signed by all authors, and a cover letter describing any conflicts of interest related to the contents of the letter.
Letters commenting on an Annals article will be considered if they are received within 6 weeks of the time the article was published. Only some of the letters received can be published. Published letters are edited and may be shortened; tables and figures are included only selectively. Authors will be notified that the letter has been received. If the letter is selected for publication, the author will be notified about 3 weeks before the publication date. Unpublished letters cannot be returned.
Annals welcomes electronically submitted letters.
- Copyright ©2004 by the American College of Physicians
