title: Case of high-risk epithelioid hemangioendothelioma of the neck.
abstract: BACKGROUND
  Epithelioid hemangioendotheliomas (EHEs) of the head and neck region are uncommon malignant neoplasms that exhibit various biologic behaviors characteristic of both low- and high-grade malignancy. A subgroup of EHEs identified as "high-risk" EHEs because of their size and mitotic activity is associated with an unfavorable clinical course and poor prognosis.
  
  
  RESULTS
  We describe the treatment of the first and, in terms of size, largest case of high-risk EHE arising from the neck. Despite wide excision, recurrence occurred 9 months after surgery, as had been expected. However, the tumor was found to express both vascular endothelial growth factor (VEGF) and VEGF receptor 2, indicating the potential of anti-VEGF therapy in the treatment of such cases.
  
  
  CONCLUSIONS
  The finding that a high-risk EHE arising from the head and neck region is characterized by expression of VEGF and its receptor provides further support for the development of targeted molecular therapies.
authors.affiliation: Department of Otolaryngology/Head and Neck Surgery, Jichi Medical University School of Medicine, Shimotsuke, Japan.
authors.forename: Shingo
authors.initials: S
authors.lastname: Matsuzawa
authors.forename: Takeharu
authors.initials: T
authors.lastname: Kanazawa
authors.forename: Takehiko
authors.initials: T
authors.lastname: Yamaguchi
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Nishino
authors.forename: Kazumi
authors.initials: K
authors.lastname: Kawada
authors.forename: Keiichi
authors.initials: K
authors.lastname: Ichimura
mesh_terms: D000368:Aged; D000970:Antineoplastic Agents / Q000008:administration & dosage; D001707:Biopsy, Needle; D005500:Follow-Up Studies; D015972:Gene Expression Regulation, Neoplastic / Q000187:drug effects; D006258:Head and Neck Neoplasms / Q000175:diagnosis / Q000473:pathology* / Q000601:surgery; D018323:Hemangioendothelioma, Epithelioid / Q000175:diagnosis / Q000601:surgery*; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D058990:Molecular Targeted Therapy / Q000379:methods*; D009364:Neoplasm Recurrence, Local / Q000188:drug therapy* / Q000473:pathology / Q000601:surgery; D009367:Neoplasm Staging; D010166:Palliative Care / Q000379:methods; D018570:Risk Assessment; D042461:Vascular Endothelial Growth Factor A / Q000187:drug effects* / Q000235:genetics; D040301:Vascular Endothelial Growth Factor Receptor-2 / Q000187:drug effects* / Q000235:genetics
chemical_list: D000970:Antineoplastic Agents; D042461:Vascular Endothelial Growth Factor A; D040301:Vascular Endothelial Growth Factor Receptor-2