title: Refractory Kaposiform Hemangioendothelioma Associated with the Chromosomal Translocation t(13;16)(q14;p13.3).
abstract: We report a 7-year-old male with a history of recurrent kaposiform hemangioendothelioma (KHE) in the sacral area and multiple thoracic vertebral lesions. Tumor karyotyping revealed the balanced translocation t(13;16)(q14;p13.3). He had mildly decreased platelet counts but never experienced any episodes of Kasabach-Merritt phenomenon. He was treated with vincristine for 1 year but improvement was minor. Significant clinical improvement was seen with sirolimus therapy. To the best of our knowledge, this is the first report to demonstrate that KHE is associated with a clonal karyotypic abnormality.
authors.affiliation: 1  Department of Pathology and Laboratory Medicine, Children's Hospital Los Angeles, Los Angeles, CA, USA.|2  Keck School of Medicine, University of Southern California Los Angeles, Los Angeles, CA, USA.
authors.forename: Shengmei
authors.initials: S
authors.lastname: Zhou
authors.affiliation: 1  Department of Pathology and Laboratory Medicine, Children's Hospital Los Angeles, Los Angeles, CA, USA.|2  Keck School of Medicine, University of Southern California Los Angeles, Los Angeles, CA, USA.
authors.forename: Larry
authors.initials: L
authors.lastname: Wang
authors.affiliation: 2  Keck School of Medicine, University of Southern California Los Angeles, Los Angeles, CA, USA.|3  Division of Plastic and Maxillofacial Surgery, Department of Surgery, Children's Hospital Los Angeles, Los Angeles, CA, USA.
authors.forename: Andre
authors.initials: A
authors.lastname: Panossian
authors.affiliation: 2  Keck School of Medicine, University of Southern California Los Angeles, Los Angeles, CA, USA.|3  Division of Plastic and Maxillofacial Surgery, Department of Surgery, Children's Hospital Los Angeles, Los Angeles, CA, USA.
authors.forename: Dean
authors.initials: D
authors.lastname: Anselmo
authors.affiliation: 1  Department of Pathology and Laboratory Medicine, Children's Hospital Los Angeles, Los Angeles, CA, USA.|2  Keck School of Medicine, University of Southern California Los Angeles, Los Angeles, CA, USA.
authors.forename: Samuel
authors.initials: S
authors.lastname: Wu
authors.affiliation: 4  Department of Pediatrics, Texas Children's Cancer Center, Baylor College of Medicine, Houston, TX, USA.
authors.forename: Rajkumar
authors.initials: R
authors.lastname: Venkatramani
mesh_terms: D000903:Antibiotics, Antineoplastic / Q000627:therapeutic use; D002648:Child; D002882:Chromosomes, Human, Pair 13 / Q000235:genetics*; D002885:Chromosomes, Human, Pair 16 / Q000235:genetics*; D006390:Hemangioendothelioma / Q000235:genetics* / Q000473:pathology; D006801:Humans; D008297:Male; D009364:Neoplasm Recurrence, Local / Q000188:drug therapy / Q000473:pathology; D012447:Sacrum; D020123:Sirolimus / Q000627:therapeutic use; D014178:Translocation, Genetic
chemical_list: D000903:Antibiotics, Antineoplastic; D020123:Sirolimus
keywords: chromosomal translocation; kaposiform hemangioendothelioma; sirolimus