Abstract
The case of a 71-yr-old Caucasian patient who suffered from an adrenocorticotropic hormone (ACTH)-producing tumor and developed a fulminantAspergillus fumigatus septicemia is reported. The patient presented initially with a recent onset of hyperglycemia and had excessive plasma levels of cortisol and ACTH. A computer-assisted tomography scan showed a tumor associated within the head of the pancreas. During his hospital course, he developed a rapid progressive fatalAspergillus fumigatus pneumonia and an upper gastrointestinal bleeding. At necropsy multiple abscesses in the brain, lungs, heart, kidneys, small bowel and mesentery were present as a result of Aspergillus septicemia. A neuroendocrine pancreatic tumor, 8 cm in diameter, was found in the head of the pancreas without any signs of malignancy. The tumor showed immunohistochemical and electron microscopic evidence of ACTH expression, but of no other pituitary or pancreatic hormones. This case demonstrates the fulminant progress of a septicemia based on an immunologically compromised patient because of ectopic Cushing’s syndrome. Early diagnosis of ectopic ACTH syndromes is essential for adequate therapy in order to prevent complications and fatal infections.
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References
Brown WH. A case of pluriglandular syndrome. Lancet ii:1022–1023, 1928.
Cushing H. The basophil adenomas of the pituitary body and their clinical manifestations (pituitary basophilism). Bull Johns Hopkins Hosp 50:137–195, 1932.
Jex RK, van Heerden JA, Carpenter PC, Grant CS. Ectopic ACTH syndrome. Diagnostic and therapeutic aspects. Am J Surg 149:276–282, 1985.
Amikura K, Alexander HR, Norton JA, Doppman JL, Jensen RT, Nieman L, et al. Role of surgery in management of adrenocorticotropic hormone-producing islet cell tumors of the pancreas. Surgery 118:1125–1130, 1995.
Larsson LI, Grimelius L, Hakanson R, Rehfeld JF, Stadil F, Holst J, et al. Mixed endocrine pancreatic tumors producing several peptide hormones. Am J Pathol 79:271–284, 1975.
Lyons DF, Eisen BR, Clark MR, Pysher TJ, Welsh JD, Kem DC. Concurrent Cushing’s and Zollinger-Ellison syndromes in a patient with islet cell carcinoma. Case report and review of the literature. Am J Med 76:729–733, 1984.
Coates PJ, Doniach I, Howlett TA, Rees LH, Besser GM. Immunocytochemical study of 18 tumours causing ectopic Cushing’s syndrome. J Clin Pathol 39:955–960, 1986.
Melmed S, Yamashita S, Kovacs K, Ong J, Rosenblatt S, Braunstein G. Cushing’s syndrome due to ectopic proopiomelanocortin gene expression by islet cell carcinoma of the pancreas. Cancer 59:772–778, 1987.
Clark ES, Carney JA. Pancreatic islet cell tumor associated with Cushing’s syndrome. Am J Surg Pathol 8:917–924, 1984.
Heitz PU, Klöppel G, Polak JM, Staub JJ. Ectopic hormone production by endocrine tumors: localization of hormones at the cellular level by immunocytochemistry. Cancer 48:2029–2037, 1981.
Asa SL, Kovacs K, Vale W, Petrusz P, Vecsei P. Immunohistologic localization of corticotrophin-releasing hormone in human tumors. Am J Clin Pathol 87:327–333, 1987.
Capella C, Heitz PU, Hofler H, Solica E, Klöppel G. Revised classification of neuroendocrine tumours of the lung, pancreas and gut. Virchows Arch 425:547–560, 1995.
Eriksson B, Arnberg H, Lindgren PG, Lorelius, LE, Magnusson A, Lundqvist G, et al. Neuroendocrine pancreatic tumours: clinical presentation, biochemical and histopathological findings in 84 patients. J Intern Med 228:103–113, 1990.
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An erratum to this article is available at http://dx.doi.org/10.1007/BF02739781.
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Grobholz, R., Back, W., Pörner, T. et al. FatalAspergillus fumigatus septicemia in a patient with an ectopic ACTH-producing neuroendocrine pancreatic tumor. Endocr Pathol 10, 181–185 (1999). https://doi.org/10.1007/BF02739830
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DOI: https://doi.org/10.1007/BF02739830