title: [Angiomatoid fibrous histiocytoma in children: 6 cases].
abstract: BACKGROUND
  Angiomatoid fibrous histiocytoma (AFH) is a soft-tissue tumour of uncertain differentiation most often arising in the extremities of children and young adults. AFH is a little-known neoplasm and its rarity may result in it being misdiagnosed as either a reactive lesion or a benign or higher-grade tumour. We report 6 cases of AFH in children and we review the clinicopathological and molecular features of this neoplasm published in the literature.
  
  
  METHODS
  The children (aged 4 to 16 years) presented a single nodule involving the forearm (4/6), the trunk or the buttock, and all 5 nodules appeared spontaneously. Microscopic examination revealed well-circumscribed nodular lesions comprising a fibrous pseudo-capsule, haemorrhagic non-endothelial-lined pseudocystic spaces, and sheets of spindle and ovoid cells with dense surrounding lymphoplasmacytic infiltrate. Tumours were positive for desmin, CD68, CD99 and smooth-muscle actin markers. A fusion gene (EWSR1-ATF1) was found in the 3 cases in which molecular investigation was performed.
  
  
  CONCLUSIONS
  In our series, a diagnosis of AFH had in no event been evoked after clinical examination and radiological investigation. The diagnosis was based in all cases on recognition of characteristic features during histological examination and it was confirmed in 3 cases by the recognition of fusion genes. Complete excision with wide margins allowed complete cure in all cases, supporting a good prognosis of AFH, although long-term follow-up is still mandatory to rule out relapse or metastases, which although rare, are responsible for fatal cases. To avoid unnecessary surgery in patients with AFH, an ultrasound core-needle biopsy should be performed as a first step in order to provide precise diagnosis enabling complete excision to be performed, with the margins being decided in multidisciplinary meetings involving teams specialised in soft-tissue tumours.
authors.affiliation: Service de dermatologie, hôpital Avicenne, AP-HP, 125, rue de Stalingrad, 93000 Bobigny, France.
authors.forename: G
authors.initials: G
authors.lastname: Bohelay
authors.affiliation: Departments of Dermatology, Allergology and Venereology, Institute of Clinical Medicine, University of Helsinki, Skin and Allergies Hospital, Helsinki University Central Hospital, Meilahdentie 2, PO Box 160, 00029 HUS, Finlande.
authors.forename: N
authors.initials: N
authors.lastname: Kluger
authors.affiliation: Service d'anatomie pathologique, hôpital Saint-Louis, AP-HP, 1, avenue Claude-Vellefaux, 75010 Paris, France.
authors.forename: M
authors.initials: M
authors.lastname: Battistella
authors.affiliation: Service d'anatomie pathologique, université Paris-Descartes Paris V, hôpital Necker-Enfants-Malades, AP-HP, 149, rue de Sèvres, 75743 Paris cedex 15, France.
authors.forename: A
authors.initials: A
authors.lastname: Biaggi-Frassati
authors.affiliation: Laboratoire d'anatomie pathologique, hôpital Cochin, AP-HP, 27, rue du Faubourg-Saint-Jacques, 75014 Paris, France.
authors.forename: F
authors.initials: F
authors.lastname: Plantier
authors.affiliation: Cabinet de dermatologie, 34, rue du Général-Gallieni, 78510 Triel-sur-Seine, France.
authors.forename: A
authors.initials: A
authors.lastname: Harraudeau
authors.affiliation: Service de dermatologie, université René-Descartes Paris V, hôpital Cochin, AP-HP, 27, rue du Faubourg-Saint-Jacques, 75014 Paris, France.
authors.forename: M-F
authors.initials: MF
authors.lastname: Avril
authors.affiliation: Institut de recherche sur le cancer et le vieillissement de Nice (IRCAN), laboratoire de génétique des tumeurs solides, faculté de médecine, CHU de Nice, 28, avenue de Valombrose, 06107 Nice cedex 02, France.
authors.forename: F
authors.initials: F
authors.lastname: Pedeutour
authors.affiliation: Service d'anatomie pathologique, université Paris-Descartes Paris V, hôpital Necker-Enfants-Malades, AP-HP, 149, rue de Sèvres, 75743 Paris cedex 15, France. Electronic address: sylvie.fraitag@nck.aphp.fr.
authors.forename: S
authors.initials: S
authors.lastname: Fraitag
mesh_terms: D000071443:12E7 Antigen; D000199:Actins / Q000032:analysis; D000293:Adolescent; D015703:Antigens, CD / Q000032:analysis; D015214:Antigens, Differentiation, Myelomonocytic / Q000032:analysis; D014408:Biomarkers, Tumor / Q000032:analysis* / Q000235:genetics; D001707:Biopsy, Needle / Q000379:methods; D015815:Cell Adhesion Molecules / Q000032:analysis; D002648:Child; D002675:Child, Preschool; D003893:Desmin / Q000032:analysis; D005260:Female; D051677:Histiocytoma, Malignant Fibrous / Q000737:chemistry / Q000175:diagnosis* / Q000235:genetics / Q000473:pathology / Q000601:surgery; D006801:Humans; D008297:Male; D015514:Oncogene Proteins, Fusion / Q000032:analysis* / Q000235:genetics; D011379:Prognosis; D012074:Remission Induction; D012878:Skin Neoplasms / Q000737:chemistry / Q000175:diagnosis* / Q000235:genetics / Q000473:pathology / Q000601:surgery; D018084:Ultrasonography, Interventional; D000097382:CD68 Molecule
chemical_list: D000071443:12E7 Antigen; D000199:Actins; D015703:Antigens, CD; D015214:Antigens, Differentiation, Myelomonocytic; D014408:Biomarkers, Tumor; D015815:Cell Adhesion Molecules; D003893:Desmin; D015514:Oncogene Proteins, Fusion; C067980:CD68 antigen, human; D000097382:CD68 Molecule; C061828:CD99 protein, human; C560860:EWSR1-ATF1 fusion protein, human
keywords: ATF1; Angiomatoid fibrous histiocytoma; Children; EWSR1; Enfant; Histiocytome fibreux angiomatoïde; Sarcoma; Sarcome