Adrenal ganglioneuroma in a patient presenting with severe hypertension and diarrhea

C. A. Koch, F. M. Brouwers, K. Rosenblatt, K. D. Burman, M. M. Davis, Alexander Vortmeyer, K. Pacak

Research output: Contribution to journalArticle

35 Citations (Scopus)

Abstract

Ganglioneuromas (GNs) are neural crest cell-derived tumors and rarely occur in the adrenal gland. There are presently no markers that can reliably distinguish benign and malignant neuroendocrine tumors. Here we describe a 63-year-old woman who developed sudden chest pain and hypertension combined with increased stool frequency. An incidental adrenal mass 5 cm in size with a bright signal on T2-weighted magnetic resonance imaging was discovered. Biochemical evaluation and 131I-metaiodobenzylguanidine (MIBG) scintigraphy were negative. Histopathological examination revealed a mature adrenal GN. Neuroblastoma, the immature form of a GN, is known for deletions on chromosomal locus 1p36, and adrenal tumors frequently show allele loss on 17p. To further elucidate the histo- and pathogenesis of adrenal GN, we performed loss of heterozygosity studies on chromosomal loci 1p34-1 and 17p13 (the p53 gene locus) after careful microdissection of tumor and normal tissue. We did not detect allelic losses at these loci with the informative polymorphic markers used, suggesting that these loci are not involved in tumorigenesis. In addition, immunohistochemical investigation of the GN was positive for vasoactive intestinal peptide, a hormone commonly expressed in ganglion cells. We suggest that in our patient with an adrenal GN, the combination of biochemical, scintigraphic, molecular, immunohistochemical, and histopathological findings are all consistent with the benign morphology of this tumor.

Original languageEnglish (US)
Pages (from-to)99-107
Number of pages9
JournalEndocrine-Related Cancer
Volume10
Issue number1
DOIs
StatePublished - Mar 1 2003
Externally publishedYes

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Ganglioneuroma
Diarrhea
Hypertension
Loss of Heterozygosity
Microdissection
Glandular and Epithelial Neoplasms
Neuroendocrine Tumors
Peptide Hormones
Neural Crest
Vasoactive Intestinal Peptide
p53 Genes
Adrenal Glands
Chest Pain
Neuroblastoma
Ganglia
Radionuclide Imaging
Neoplasms
Carcinogenesis
Alleles
Magnetic Resonance Imaging

ASJC Scopus subject areas

  • Endocrinology, Diabetes and Metabolism
  • Oncology
  • Endocrinology
  • Cancer Research

Cite this

Koch, C. A., Brouwers, F. M., Rosenblatt, K., Burman, K. D., Davis, M. M., Vortmeyer, A., & Pacak, K. (2003). Adrenal ganglioneuroma in a patient presenting with severe hypertension and diarrhea. Endocrine-Related Cancer, 10(1), 99-107. https://doi.org/10.1677/erc.0.0100099

Adrenal ganglioneuroma in a patient presenting with severe hypertension and diarrhea. / Koch, C. A.; Brouwers, F. M.; Rosenblatt, K.; Burman, K. D.; Davis, M. M.; Vortmeyer, Alexander; Pacak, K.

In: Endocrine-Related Cancer, Vol. 10, No. 1, 01.03.2003, p. 99-107.

Research output: Contribution to journalArticle

Koch, CA, Brouwers, FM, Rosenblatt, K, Burman, KD, Davis, MM, Vortmeyer, A & Pacak, K 2003, 'Adrenal ganglioneuroma in a patient presenting with severe hypertension and diarrhea', Endocrine-Related Cancer, vol. 10, no. 1, pp. 99-107. https://doi.org/10.1677/erc.0.0100099
Koch, C. A. ; Brouwers, F. M. ; Rosenblatt, K. ; Burman, K. D. ; Davis, M. M. ; Vortmeyer, Alexander ; Pacak, K. / Adrenal ganglioneuroma in a patient presenting with severe hypertension and diarrhea. In: Endocrine-Related Cancer. 2003 ; Vol. 10, No. 1. pp. 99-107.
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