title: Angiomatoid fibrous histiocytoma: a series of seven cases including genetically confirmed aggressive cases and a literature review.
abstract: Angiomatoid fibrous histiocytoma (AFH) is a rare soft tissue tumor of intermediate biologic potential. Because of its rarity and nonspecific radiological and diverse pathological findings, AFH is often clinically misdiagnosed. However, few clinical reports have described this tumor. As reported herein, we analyzed the clinical and radiological features and clinical outcomes of AFH.
  
  
  
  We retrospectively reviewed the medical records of seven cases histopathologically diagnosed as AFH. We examined clinical features, MRI findings, histopathological diagnoses, treatments, and outcomes.
  
  
  
  These seven cases comprised five male and two female patients with ages ranging from 8 to 50 years old. The primary locations included upper extremities in 2, lower extremities in 4, and the inguinal region in one patient. Of the tumors, 4 occurred in subcutaneous tissues and 3 occurred in deep tissues. No cases were diagnosed as AFH from MRI and needle biopsy results. All cases were diagnosed histopathologically after excision. After treatment, 2 patients (29%) had tumor recurrence and metastasis, one of whom died from disease progression. These 2 aggressive cases involved both EWSR1 and CREB1 gene rearrangements as determined by FISH. The other patients were alive and well without recurrence or metastasis.
  
  
  
  AFH is a rare tumor that is difficult to diagnose. Therefore, it tends to be misdiagnosed and to be treated inadequately by referring physicians. Surgeons must therefore be mindful of the presence of AFH, learn about appropriate treatment necessary for this tumor, and conduct careful follow-up because AFH can engender poor outcomes.
authors.affiliation: Division of Muscloskeletal Oncology, National Cancer Center Hospital, 5-5-1 Tsukiji, Chuo-ku, Tokyo, 104-0045, Japan.|Department of Orthopaedic Surgery, Gunma University Graduate School of Medicine, 3-39-22 Showa-machi, Maebashi, Gunma, 371-8511, Japan.
authors.forename: Kenichi
authors.initials: K
authors.lastname: Saito
authors.affiliation: Division of Muscloskeletal Oncology, National Cancer Center Hospital, 5-5-1 Tsukiji, Chuo-ku, Tokyo, 104-0045, Japan. ekobayas@ncc.go.jp.
authors.forename: Eisuke
authors.initials: E
authors.lastname: Kobayashi
authors.affiliation: Division of Pathology and Clinical Laboratories, National Cancer Center Hospital, 5-5-1 Tsukiji, Chuo-ku, Tokyo, 104-0045, Japan.
authors.forename: Akihiko
authors.initials: A
authors.lastname: Yoshida
authors.affiliation: Division of Muscloskeletal Oncology, National Cancer Center Hospital, 5-5-1 Tsukiji, Chuo-ku, Tokyo, 104-0045, Japan.
authors.forename: Yoshihiro
authors.initials: Y
authors.lastname: Araki
authors.affiliation: Division of Muscloskeletal Oncology, National Cancer Center Hospital, 5-5-1 Tsukiji, Chuo-ku, Tokyo, 104-0045, Japan.
authors.forename: Daisuke
authors.initials: D
authors.lastname: Kubota
authors.affiliation: Division of Muscloskeletal Oncology, National Cancer Center Hospital, 5-5-1 Tsukiji, Chuo-ku, Tokyo, 104-0045, Japan.
authors.forename: Yoshikazu
authors.initials: Y
authors.lastname: Tanzawa
authors.affiliation: Division of Muscloskeletal Oncology, National Cancer Center Hospital, 5-5-1 Tsukiji, Chuo-ku, Tokyo, 104-0045, Japan.
authors.forename: Akira
authors.initials: A
authors.lastname: Kawai
authors.affiliation: Department of Orthopaedic Surgery, Gunma University Graduate School of Medicine, 3-39-22 Showa-machi, Maebashi, Gunma, 371-8511, Japan.
authors.forename: Takashi
authors.initials: T
authors.lastname: Yanagawa
authors.affiliation: Department of Orthopaedic Surgery, Gunma University Graduate School of Medicine, 3-39-22 Showa-machi, Maebashi, Gunma, 371-8511, Japan.
authors.forename: Kenji
authors.initials: K
authors.lastname: Takagishi
authors.affiliation: Division of Muscloskeletal Oncology, National Cancer Center Hospital, 5-5-1 Tsukiji, Chuo-ku, Tokyo, 104-0045, Japan.
authors.forename: Hirokazu
authors.initials: H
authors.lastname: Chuman
mesh_terms: D000328:Adult; D002648:Child; D017809:Fatal Outcome; D005260:Female; D005500:Follow-Up Studies; D051677:Histiocytoma, Malignant Fibrous / Q000000981:diagnostic imaging* / Q000235:genetics* / Q000628:therapy; D006801:Humans; D008297:Male; D008875:Middle Aged; D009361:Neoplasm Invasiveness / Q000000981:diagnostic imaging; D055815:Young Adult
keywords: Angiomatoid fibrous histiocytoma; Diagnosis; Prognosis