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Cushing’s Syndrome in a Child with Pancreatic Acinar Cell Carcinoma

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Abstract

A case of pancreatic acinar cell tumor (ACC) is presented in a 10-year-old boy. The tumor manifested clinically with Cushing’s syndrome, high serum adrenocorticotropic hormone (ACTH) and cortisol concentrations. In addition, excessive serum levels of alpha-fetoprotein (AFP) were detected. Surgical resection was not possible due to retroperitoneal invasion. Biopsy of the mass showed a solid, poorly differentiated ACC of the pancreas. Periodic acid Schiff positive cytoplasmic granules, trypsinogen, keratins, alpha-1-antitrypsin, and AFP were identified in the tumor cells. Electron microscopy demonstrated zymogen granules as well as isolated dense core granules. Using immunochemiluminometric assay, a high quantity of ACTH was found in the fresh frozen tumor extract. ACTH, chromogranin A, and corticotropin-releasing factor were identified only in a few cells by immunohistochemistry. Combined radiochemotherapy was temporarily effective in reducing the tumor mass and serum AFP. Serum ACTH and cortisol levels dropped progressively and definitively to normal values after chemotherapy, and the Cushing’s syndrome subsided. Two years later, the patient died with metastatic disease. The presented case of ACC is interesting due to high serum AFP values and ectopic ACTH secretion resulting in Cushing’s syndrome.

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Acknowledgment

The authors wish to express their gratitude to Dr. Görcs TJ (Department of Human Morphology and Developmental Biology, Semmelweis University, Budapest) and Dr. Ildikó Miklós (Institute of Experimental Medicine of the Hungarian Academy of Sciences) for providing CRF antibodies and control tissues. Trypsinogen antibody was kindly given by Gábor Réz (Department of General Zoology, Eötvös University, Budapest). Many thanks to Ms. Ágnes Szík, Ms. Sándorné Tóth, and Ms Sklánitzné Somodai Erika for their devoted efforts to solve the technical problems arising in the laboratory work. The study was sponsored by the grants OTKA T 049559 and ETT-049/2006 by the Hungarian Ministry of Health.

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Authors and Affiliations

  1. First Department of Pediatrics, Semmelweis University, Budapest, Hungary

    Andrea Luczay, Gábor Benyó, Attila Kálmán & Tivadar Tulassay

  2. Department of Human Morphology and Developmental Biology, Semmelweis University, Budapest, Hungary

    Katalin Köves

  3. Second Department of Internal Medicine, Semmelweis University, Budapest, Hungary

    Károly Rácz

  4. Second Department of Pathology, Semmelweis University, Üllői út 93, 1091, Budapest, Hungary

    György Illyés, Katalin Borka & Zsuzsa Schaff

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  1. György Illyés
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  2. Andrea Luczay
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  3. Gábor Benyó
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  4. Attila Kálmán
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  5. Katalin Borka
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  6. Katalin Köves
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  9. Zsuzsa Schaff
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Correspondence to György Illyés.

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Andrea Luczay is working now at the Second Department of Pediatrics, Semmelweis University, Budapest, Hungary.

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Illyés, G., Luczay, A., Benyó, G. et al. Cushing’s Syndrome in a Child with Pancreatic Acinar Cell Carcinoma. Endocr Pathol 18, 95–102 (2007). https://doi.org/10.1007/s12022-007-0018-4

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  • DOI: https://doi.org/10.1007/s12022-007-0018-4

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