Abstract
We report a rare case of ectopic ACTH syndrome associated with undifferentiated carcinoma of the ascending colon. A 62-year-old woman developed hypokalaemia and metabolic alkalosis associated with markedly elevated serum cortisol and plasma ACTH levels. High-dose dexamethasone (8 mg/day) did not suppress increased urinary 17-hydroxycorticosteroid and 17-ketosteroid excretion. Barium enema and abdominal computerised tomography showed a Borrmann II type tumour in the ascending colon, multiple metastatic nodules in the liver and bilateral enlargement of the adrenal glands. Histological examination of the resected primary colon cancer and metastatic liver tumour showed undifferentiated carcinoma with areas of distinct neuroendocrine, exocrine, and squamous differentiation. ACTH production by the tumour was confirmed by radioimmunoassay and immunohistochemistry. This is a unique case report of carcinoma of the colon with distinct multidirectional differentiation causing ectopic ACTH syndrome.
Similar content being viewed by others
References
Imura H, Matsukura S, Yamamoto H, Hirata Y, Nakai Y, Endo J, Tanaka A, Nakamura M (1975) Studies on ectopic ACTH-producing tumors. II. Clinical and biochemical features of 30 cases. Cancer 35:1430–1437
Miura K, Demura H, Sato E, Sasano N, Shimizu N (1970) A case of ACTH secreting cancer of the colon. J Clin Endocrinol Metab 31:591–595
Balsam A, Berstein B, Goldman J, Sachs BA, Rifkin H (1972) Ectopic adrenocorticotropin syndrome associated with carcinoma of the colon. Gastroenterology 62:636–641
Suda T, Demura H, Demura R, Wakabayashi I, Nomura K, Odagiri E, Shizume K (1977) Corticotropin-releasing factor-like activity in ACTH producing tumors. J Clin Endocrinol Metab 44:440–446
Coates PJ, Doniach I, Howlett TA, Rees LH, Besser GM (1986) Immunocytochemical study of 18 tumors causing ectopic Cushing's syndrome. J Clin Pathol 39:955–960
Remick SC, Hafez GR, Carbone PP (1987) Extrapulmonary small cell carcinoma. A review of the literature with emphasis on therapy and outcome. Medicine 66:457–471
Sterling RK (1993) Ectopic ACTH syndrome associated with anorectal carcinoma. Report of a case and review of the literature. Dig Dis Sci 38:955–959
Shimatsu A, Kato Y, Tanaka I, Nakai Y, Fukunaga M, Imura H (1983) Plasma calcitonin and ACTH responses to lysine vasopressin, calcium and pentagastrin in a patient with medullary thyroid carcinoma associated with Cushing's syndrome. Clin Endocrinol 18:119–125
Clery AP, Dockerty MB, Waugh JM (1961) Small cell carcinoma of the colon and rectum. Arch Surg 83:164–172
Gould VE, Chejfec G (1978) Neuroendocrine carcinoma of the colon. Am J Surg Pathol 2:31–38
Staren ED, Gould VE, Warren WH, Wool NL, Bines S, Baker J, Bonomi P, Roseman DL, Economou SG (1988) Neuroendocrine carcinomas of the colon and rectum. A clinicopathologic evaluation. Surgery 104:1080–1089
Defold CR, Menefee JK, Nicholson WE, Orth DN (1988) Proopiomelanocortin gene is expressed in many normal human tissues and tumors not associated with ectopic adrenocorticotropin syndrome. Mol Endocrinol 2:862–870
Damjanov I, Amenta PS, Bosman FT (1983) Undifferentiated carcinoma of the colon containing exocrine, neuroendocrine and squamous cells. Virchows Arch [A] 401:57–66
Petrelli M, Tetangco E, Reid JD (1981) Carcinoma of the colon with undifferentiated, carcinoid, and squamous cell features. Am J Clin Pathol 75:581–584
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Onishi, R., Sano, T., Nakamura, Y. et al. Ectopic adrenocorticotropin syndrome associated with undifferentiated carcinoma of the colon showing multidirectional neuroendocrine, exocrine, and squamous differentiation. Vichows Archiv A Pathol Anat 427, 537–541 (1996). https://doi.org/10.1007/BF00199515
Received:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF00199515