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Eur J Pediatr Surg 1997; 7(5): 304-307
DOI: 10.1055/s-2008-1071179
Case report

© Georg Thieme Verlag KG Stuttgart · New York

Bilateral Adrenal Neuroblastoma

Y.  Zaizen1 , S.  Suita1 , K.  Yamanaka1 , M.  Nagoshi1 , T.  Tsuda2
  • 1Department of Pediatric Surgery, Faculty of Medicine, Kyushu University, Fukuoka 812-82, Japan
  • 2Department of Work System and Health, University of Occupational and Environmental Health, Kitakyushu 807, Japan
Further Information

Publication History

Publication Date:
25 March 2008 (online)

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Abstract

Neuroblastoma has been recognized as the most common solid tumor of infancy and childhood. The occurrence of bilateral adrenal neuroblastoma, however, is extremely rare and only a small number of cases have been previously reported. The authors herein report the clinical, histopathological and molecular biological features of two bilateral adrenal cases out of 125 neuroblastoma patients treated at Kyushu University Hospital over a 35-year period. The clinical and histopathological data of the two cases of bilateral adrenal neuroblastoma were reviewed. In Case 1, which had multiple liver metastases, a post-mortem examination revealed bilateral adrenal involvement. In Case 2, which had been detected by mass screening, CT showed masses in both adrenal glands. No big differences in size were recognized between the tumors in either of the cases. A histopathological examination revealed rosette fibrillary type neuroblastomas in both cases. The DNA of these tumor samples stored at -80°C was extracted and the number of copies of the N-myc gene was determined by a Southern blot analysis. Fourteen copies of the gene were detected in Case 1, whereas neither of the tumors in Case 2 showed any amplification. The clinical outcome, histopathological findings and N-myc gene analysis of two cases might support the variety of biological features of this rare group of neuroblastoma.

Zusammenfassung

Neuroblastome gehören zu den häufigen soliden Tumoren im Kindesalter. Allerdings ist das Auftreten eines bilateralen Neuroblastoms extrem selten. Die Autoren fanden unter 125 Neuroblastomen, die am Universitäts-Krankenhaus von Kyushu in einem Zeitraum von 35 Jahren operiert worden waren, nur 2 Falle eines bilateralen Auftretens eines Neuroblastoms. Diese beiden Fälle werden vorgestellt.

Im ersten Falle wurde das bilaterale Auftreten erst post mortem diagnostiziert, im Fall 2 im Rahmen eines CTs. Die Histopathologie bestätigte die Diagnose in beiden Fällen. Die DNA wurde aus beiden Tumoren extrahiert und die N-myc Gene wurden untersucht. 14 Kopien der Gene wurden im Fall 1, keine jedoch im Fall 2 gefunden. Diese Befunde stehen möglicherweise in Korrelation zum Verlauf der Erkrankung bei den beiden Kindern.

Key words

Neuroblastoma - Bilateral adrenal origin - N-myc

Schlüsselwörter

Neuroblastom, bilaterales - Simultanes Auftreten - N-myc - Gene

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