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Erschienen in: Endocrine Pathology 2/2007

01.05.2007

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

verfasst von: György Illyés, Andrea Luczay, Gábor Benyó, Attila Kálmán, Katalin Borka, Katalin Köves, Károly Rácz, Tivadar Tulassay, Zsuzsa Schaff

Erschienen in: Endocrine Pathology | Ausgabe 2/2007

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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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Metadaten
Titel
Cushing’s Syndrome in a Child with Pancreatic Acinar Cell Carcinoma
verfasst von
György Illyés
Andrea Luczay
Gábor Benyó
Attila Kálmán
Katalin Borka
Katalin Köves
Károly Rácz
Tivadar Tulassay
Zsuzsa Schaff
Publikationsdatum
01.05.2007
Verlag
Humana Press Inc
Erschienen in
Endocrine Pathology / Ausgabe 2/2007
Print ISSN: 1046-3976
Elektronische ISSN: 1559-0097
DOI
https://doi.org/10.1007/s12022-007-0018-4

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