Long-term prognosis of patients with pediatric pheochromocytoma

Birke Bausch, Ulrich Wellner, Dirk Bausch, Francesca Schiavi, Marta Barontini, Gabriela Sanso, Martin K. Walz, Mariola Peczkowska, Georges Weryha, Patrizia Dall'Igna, Giovanni Cecchetto, Gianni Bisogno, Lars C. Moeller, Detlef Bockenhauer, Attila Patocs, Karoly Rácz, Dmitry Zabolotnyi, Svetlana Yaremchuk, Iveta Dzivite-Krisane, Frederic CastinettiDavid Taieb, Angelica Malinoc, Ernst Von Dobschuetz, Jochen Roessler, Kurt W. Schmid, Giuseppe Opocher, Charis Eng, Hartmut P H Neumann

Research output: Contribution to journalArticlepeer-review

Abstract

A third of patients with paraganglial tumors, pheochromocytoma, and paraganglioma, carry germline mutations in one of the susceptibility genes, RET, VHL, NF1, SDHAF2, SDHA, SDHB, SDHC, SDHD, TMEM127, and MAX. Despite increasing importance, data for long-term prognosis are scarce in pediatric presentations. The European-American-Pheochromocytoma-Paraganglioma-Registry, with a total of 2001 patients with confirmed paraganglial tumors, was the platform for this study. Molecular genetic and phenotypic classification and assessment of gene-specific long-term outcome with second and/or malignant paraganglial tumors and life expectancy were performed in patients diagnosed at

Original languageEnglish
Pages (from-to)17-25
Number of pages9
JournalEndocrine-Related Cancer
Volume21
Issue number1
DOIs
Publication statusPublished - Feb 2014

Keywords

  • Germline mutations
  • Long-term follow-up
  • Pheochromocytoma
  • Relapse

ASJC Scopus subject areas

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

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