title: Refractory kaposiform hemangioendothelioma that expressed vascular endothelial growth factor receptor (VEGFR)-2 and VEGFR-3: a case report.
abstract: This report describes the case of a 10-month-old boy who was diagnosed to have kaposiform hemangioendothelioma (KHE) with Kasabach-Merritt syndrome (KMS), which is a rare pediatric vascular tumor with a high mortality rate. Although both KHE with KMS were resistant to various therapies, such as oral prednisolone, sclerotherapy, and chemotherapy, repeated radiation therapy with methylprednisolone pulse therapy did reduce the volume of KHE and improved the symptoms of KMS. Unfortunately, a regrowth of KHE with KMS was observed 4 months after the cessation of treatment and the patient thereafter died from an intracranial hemorrhage and Pneumocystis carinii pneumonia, which is a complication related to repetitive radiation and steroid therapy. A histopathologic examination of autopsy specimens confirmed a diagnosis of KHE and immunohistologic staining was positive for vascular endothelial growth factor receptor (VEGFR)-2 and VEGFR-3. These findings may provide the rationale to further investigate the role of VEGFRs in the pathogenesis of KHE and also to elucidate its prognostic value, along with the application of inhibitors for VEGFRs for the treatment of refractory KHE.
authors.affiliation: Department of Pediatrics, Jichi Medical University School of Medicine, Simotsuke, Tochigi, Japan.
authors.forename: Mari
authors.initials: M
authors.lastname: Saito
authors.forename: Yuji
authors.initials: Y
authors.lastname: Gunji
authors.forename: Yoshifumi
authors.initials: Y
authors.lastname: Kashii
authors.forename: Jun
authors.initials: J
authors.lastname: Odaka
authors.forename: Tadahiko
authors.initials: T
authors.lastname: Yamauchi
authors.forename: Nobuyuki
authors.initials: N
authors.lastname: Kanai
authors.forename: Mariko Y
authors.initials: MY
authors.lastname: Momoi
mesh_terms: D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000627:therapeutic use; D000971:Antineoplastic Combined Chemotherapy Protocols; D001550:Benzamidines; D003520:Cyclophosphamide; D003609:Dactinomycin; D019008:Drug Resistance, Neoplasm; D006146:Guanidines / Q000627:therapeutic use; D006390:Hemangioendothelioma / Q000378:metabolism* / Q000503:physiopathology / Q000628:therapy; D006801:Humans; D007150:Immunohistochemistry; D007223:Infant; D016898:Interferon-alpha / Q000627:therapeutic use; D008279:Magnetic Resonance Imaging; D008297:Male; D009829:Oleic Acids / Q000627:therapeutic use; D017713:Platelet Transfusion; D011239:Prednisolone / Q000627:therapeutic use; D011379:Prognosis; D012597:Sclerosing Solutions / Q000627:therapeutic use; D015911:Sclerotherapy; D012983:Soft Tissue Neoplasms / Q000378:metabolism* / Q000503:physiopathology / Q000628:therapy; D013921:Thrombocytopenia / Q000209:etiology / Q000628:therapy; D040301:Vascular Endothelial Growth Factor Receptor-2 / Q000096:biosynthesis*; D040321:Vascular Endothelial Growth Factor Receptor-3 / Q000096:biosynthesis*; D014750:Vincristine
chemical_list: D000894:Anti-Inflammatory Agents, Non-Steroidal; D001550:Benzamidines; D006146:Guanidines; D016898:Interferon-alpha; D009829:Oleic Acids; D012597:Sclerosing Solutions; D003609:Dactinomycin; D014750:Vincristine; D003520:Cyclophosphamide; D011239:Prednisolone; D040301:Vascular Endothelial Growth Factor Receptor-2; D040321:Vascular Endothelial Growth Factor Receptor-3; C033343:ethanolamine oleate; C032855:nafamostat