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An. bras. dermatol ; An. bras. dermatol;91(6): 748-753, Nov.-Dec. 2016. tab, graf
Article de Anglais | LILACS | ID: biblio-837985

RÉSUMÉ

Abstract BACKGROUND: Kaposiform hemangioendothelioma is a rare, intermediate, malignant tumor. The tumor's etiology remains unknown and there are no specific treatments. OBJECTIVE: In this study, we performed exome sequencing using DNA from a Kaposiform hemangioendothelioma patient, and found putative candidates for the responsible mutations. METHOD: The genomic DNA for exome sequencing was obtained from the tumor tissue and matched normal tissue from the same individual. Exome sequencing was performed on HiSeq2000 sequencer platform. RESULTS: Among oncogenes, germline missense single nucleotide variants were observed in the TP53 and APC genes in both the tumor and normal tissue. As tumor-specific somatic mutations, we identified 81 candidate genes, including 4 nonsense changes, 68 missense changes and 9 insertions/deletions. The mutations in ITGB2, IL-32 and DIDO1 were included in them. CONCLUSION: This is a pilot study, and future analysis with more patients is needed to clarify: the detailed pathogenesis of this tumor, the novel diagnostic methods by detecting specific mutations, and the new therapeutic strategies targeting the mutation.


Sujet(s)
Humains , Mâle , Enfant d'âge préscolaire , Mutation faux-sens , Syndrome de Kasabach-Merritt/génétique , Syndrome de Kasabach-Merritt/anatomopathologie , Exome , Hémangioendothéliome/génétique , Hémangioendothéliome/anatomopathologie , Valeurs de référence , Analyse de mutations d'ADN , Imagerie par résonance magnétique , Gènes p53/génétique , Gènes APC , Tissu sous-cutané/anatomopathologie , Études d'associations génétiques , Fréquence d'allèle
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