title: Tumor-Like Presentation of Primary Angiitis of the Central Nervous System.
abstract: We aimed to describe the clinical and imaging features of patients with tumor-like presentation of primary angiitis of the central nervous system.
  
  
  
  We retrospectively analyzed 10 patients enrolled in the French primary angiitis of the central nervous system cohort, who initially presented tumor-like brain lesions and compared them with other patients within the cohort.
  
  
  
  The 10 patients with tumor-like presentation in the cohort were younger and had more seizures at diagnosis than the other 75 patients (median of 37 [30-48] years versus 46 [18-79] years; P=0.008; 9 [90%] with seizures versus 22 [29%], P<0.001; respectively). All 10 patients had a biopsy (stereotactic procedure in 7 and open-wedge surgery in 3). Histological findings suggestive of vasculitis were observed in 9 patients in whom conventional cerebral angiography and magnetic resonance angiography were negative. In the remaining patient, vascular imaging demonstrated diffuse bilateral large- and medium-sized vessel involvement (biopsy did not reveal vasculitis). All patients with tumor-like presentation received glucocorticoids, combined with cyclophosphamide in 9 cases. With a median follow-up of 27 (12-130) months, 5 (50%) patients relapsed, but achieved remission again after treatment intensification.
  
  
  
  Patients with tumor-like presentation of primary angiitis of the central nervous system represent a subgroup characterized with mainly small-sized vessel disease that requires histological confirmation because vascular imaging is often normal. Although relapses are not uncommon, global outcomes are good under treatment with glucocorticoids and cyclophosphamide.
authors.affiliation: From the Departments of Internal Medicine (H.d.B., B.B., A.A.) and Department of Neurology, Caen University Hospital, University of Caen-Normandie, INSERM U919, France (E.T.); Department of Neuroradiology, Centre Hospitalier Sainte-Anne, University Paris-Descartes, INSERM UMR 894, France (G.B., O.N.); Department of Neurology, Lille University Hospital, France (N.D.); Department of Neurology, Angers University Hospital, France (S.G.); Department of Internal Medicine, Nantes University Hospital, France (A.N.); Department of Neurology, Montpellier University Hospital, INSERM UMR 894, France (C.A.); Department of Neurology, Grenoble Alpes University Hospital, France (O.D.); Department of Internal Medicine, Pitié-Salpêtrière Hospital, Paris, France (C.B.-Q.); Department of Vascular Neurology-Centre Hospitalier Saint Joseph, Université Paris-Descartes, INSERM UMR S 919, France (M.Z.); Department of Internal Medicine, Centre Hospitalier Cochin, Paris, France (L.G.); and the Vasculitis Clinic, Division of Rheumatology, Mount Sinai Hospital, Toronto (C.P.). deboysson-h@chu-caen.fr.
authors.forename: Hubert
authors.initials: H
authors.lastname: de Boysson
authors.affiliation: From the Departments of Internal Medicine (H.d.B., B.B., A.A.) and Department of Neurology, Caen University Hospital, University of Caen-Normandie, INSERM U919, France (E.T.); Department of Neuroradiology, Centre Hospitalier Sainte-Anne, University Paris-Descartes, INSERM UMR 894, France (G.B., O.N.); Department of Neurology, Lille University Hospital, France (N.D.); Department of Neurology, Angers University Hospital, France (S.G.); Department of Internal Medicine, Nantes University Hospital, France (A.N.); Department of Neurology, Montpellier University Hospital, INSERM UMR 894, France (C.A.); Department of Neurology, Grenoble Alpes University Hospital, France (O.D.); Department of Internal Medicine, Pitié-Salpêtrière Hospital, Paris, France (C.B.-Q.); Department of Vascular Neurology-Centre Hospitalier Saint Joseph, Université Paris-Descartes, INSERM UMR S 919, France (M.Z.); Department of Internal Medicine, Centre Hospitalier Cochin, Paris, France (L.G.); and the Vasculitis Clinic, Division of Rheumatology, Mount Sinai Hospital, Toronto (C.P.).
authors.forename: Grégoire
authors.initials: G
authors.lastname: Boulouis
authors.affiliation: From the Departments of Internal Medicine (H.d.B., B.B., A.A.) and Department of Neurology, Caen University Hospital, University of Caen-Normandie, INSERM U919, France (E.T.); Department of Neuroradiology, Centre Hospitalier Sainte-Anne, University Paris-Descartes, INSERM UMR 894, France (G.B., O.N.); Department of Neurology, Lille University Hospital, France (N.D.); Department of Neurology, Angers University Hospital, France (S.G.); Department of Internal Medicine, Nantes University Hospital, France (A.N.); Department of Neurology, Montpellier University Hospital, INSERM UMR 894, France (C.A.); Department of Neurology, Grenoble Alpes University Hospital, France (O.D.); Department of Internal Medicine, Pitié-Salpêtrière Hospital, Paris, France (C.B.-Q.); Department of Vascular Neurology-Centre Hospitalier Saint Joseph, Université Paris-Descartes, INSERM UMR S 919, France (M.Z.); Department of Internal Medicine, Centre Hospitalier Cochin, Paris, France (L.G.); and the Vasculitis Clinic, Division of Rheumatology, Mount Sinai Hospital, Toronto (C.P.).
authors.forename: Nelly
authors.initials: N
authors.lastname: Dequatre
authors.affiliation: From the Departments of Internal Medicine (H.d.B., B.B., A.A.) and Department of Neurology, Caen University Hospital, University of Caen-Normandie, INSERM U919, France (E.T.); Department of Neuroradiology, Centre Hospitalier Sainte-Anne, University Paris-Descartes, INSERM UMR 894, France (G.B., O.N.); Department of Neurology, Lille University Hospital, France (N.D.); Department of Neurology, Angers University Hospital, France (S.G.); Department of Internal Medicine, Nantes University Hospital, France (A.N.); Department of Neurology, Montpellier University Hospital, INSERM UMR 894, France (C.A.); Department of Neurology, Grenoble Alpes University Hospital, France (O.D.); Department of Internal Medicine, Pitié-Salpêtrière Hospital, Paris, France (C.B.-Q.); Department of Vascular Neurology-Centre Hospitalier Saint Joseph, Université Paris-Descartes, INSERM UMR S 919, France (M.Z.); Department of Internal Medicine, Centre Hospitalier Cochin, Paris, France (L.G.); and the Vasculitis Clinic, Division of Rheumatology, Mount Sinai Hospital, Toronto (C.P.).
authors.forename: Sophie
authors.initials: S
authors.lastname: Godard
authors.affiliation: From the Departments of Internal Medicine (H.d.B., B.B., A.A.) and Department of Neurology, Caen University Hospital, University of Caen-Normandie, INSERM U919, France (E.T.); Department of Neuroradiology, Centre Hospitalier Sainte-Anne, University Paris-Descartes, INSERM UMR 894, France (G.B., O.N.); Department of Neurology, Lille University Hospital, France (N.D.); Department of Neurology, Angers University Hospital, France (S.G.); Department of Internal Medicine, Nantes University Hospital, France (A.N.); Department of Neurology, Montpellier University Hospital, INSERM UMR 894, France (C.A.); Department of Neurology, Grenoble Alpes University Hospital, France (O.D.); Department of Internal Medicine, Pitié-Salpêtrière Hospital, Paris, France (C.B.-Q.); Department of Vascular Neurology-Centre Hospitalier Saint Joseph, Université Paris-Descartes, INSERM UMR S 919, France (M.Z.); Department of Internal Medicine, Centre Hospitalier Cochin, Paris, France (L.G.); and the Vasculitis Clinic, Division of Rheumatology, Mount Sinai Hospital, Toronto (C.P.).
authors.forename: Antoine
authors.initials: A
authors.lastname: Néel
authors.affiliation: From the Departments of Internal Medicine (H.d.B., B.B., A.A.) and Department of Neurology, Caen University Hospital, University of Caen-Normandie, INSERM U919, France (E.T.); Department of Neuroradiology, Centre Hospitalier Sainte-Anne, University Paris-Descartes, INSERM UMR 894, France (G.B., O.N.); Department of Neurology, Lille University Hospital, France (N.D.); Department of Neurology, Angers University Hospital, France (S.G.); Department of Internal Medicine, Nantes University Hospital, France (A.N.); Department of Neurology, Montpellier University Hospital, INSERM UMR 894, France (C.A.); Department of Neurology, Grenoble Alpes University Hospital, France (O.D.); Department of Internal Medicine, Pitié-Salpêtrière Hospital, Paris, France (C.B.-Q.); Department of Vascular Neurology-Centre Hospitalier Saint Joseph, Université Paris-Descartes, INSERM UMR S 919, France (M.Z.); Department of Internal Medicine, Centre Hospitalier Cochin, Paris, France (L.G.); and the Vasculitis Clinic, Division of Rheumatology, Mount Sinai Hospital, Toronto (C.P.).
authors.forename: Caroline
authors.initials: C
authors.lastname: Arquizan
authors.affiliation: From the Departments of Internal Medicine (H.d.B., B.B., A.A.) and Department of Neurology, Caen University Hospital, University of Caen-Normandie, INSERM U919, France (E.T.); Department of Neuroradiology, Centre Hospitalier Sainte-Anne, University Paris-Descartes, INSERM UMR 894, France (G.B., O.N.); Department of Neurology, Lille University Hospital, France (N.D.); Department of Neurology, Angers University Hospital, France (S.G.); Department of Internal Medicine, Nantes University Hospital, France (A.N.); Department of Neurology, Montpellier University Hospital, INSERM UMR 894, France (C.A.); Department of Neurology, Grenoble Alpes University Hospital, France (O.D.); Department of Internal Medicine, Pitié-Salpêtrière Hospital, Paris, France (C.B.-Q.); Department of Vascular Neurology-Centre Hospitalier Saint Joseph, Université Paris-Descartes, INSERM UMR S 919, France (M.Z.); Department of Internal Medicine, Centre Hospitalier Cochin, Paris, France (L.G.); and the Vasculitis Clinic, Division of Rheumatology, Mount Sinai Hospital, Toronto (C.P.).
authors.forename: Olivier
authors.initials: O
authors.lastname: Detante
authors.affiliation: From the Departments of Internal Medicine (H.d.B., B.B., A.A.) and Department of Neurology, Caen University Hospital, University of Caen-Normandie, INSERM U919, France (E.T.); Department of Neuroradiology, Centre Hospitalier Sainte-Anne, University Paris-Descartes, INSERM UMR 894, France (G.B., O.N.); Department of Neurology, Lille University Hospital, France (N.D.); Department of Neurology, Angers University Hospital, France (S.G.); Department of Internal Medicine, Nantes University Hospital, France (A.N.); Department of Neurology, Montpellier University Hospital, INSERM UMR 894, France (C.A.); Department of Neurology, Grenoble Alpes University Hospital, France (O.D.); Department of Internal Medicine, Pitié-Salpêtrière Hospital, Paris, France (C.B.-Q.); Department of Vascular Neurology-Centre Hospitalier Saint Joseph, Université Paris-Descartes, INSERM UMR S 919, France (M.Z.); Department of Internal Medicine, Centre Hospitalier Cochin, Paris, France (L.G.); and the Vasculitis Clinic, Division of Rheumatology, Mount Sinai Hospital, Toronto (C.P.).
authors.forename: Coralie
authors.initials: C
authors.lastname: Bloch-Queyrat
authors.affiliation: From the Departments of Internal Medicine (H.d.B., B.B., A.A.) and Department of Neurology, Caen University Hospital, University of Caen-Normandie, INSERM U919, France (E.T.); Department of Neuroradiology, Centre Hospitalier Sainte-Anne, University Paris-Descartes, INSERM UMR 894, France (G.B., O.N.); Department of Neurology, Lille University Hospital, France (N.D.); Department of Neurology, Angers University Hospital, France (S.G.); Department of Internal Medicine, Nantes University Hospital, France (A.N.); Department of Neurology, Montpellier University Hospital, INSERM UMR 894, France (C.A.); Department of Neurology, Grenoble Alpes University Hospital, France (O.D.); Department of Internal Medicine, Pitié-Salpêtrière Hospital, Paris, France (C.B.-Q.); Department of Vascular Neurology-Centre Hospitalier Saint Joseph, Université Paris-Descartes, INSERM UMR S 919, France (M.Z.); Department of Internal Medicine, Centre Hospitalier Cochin, Paris, France (L.G.); and the Vasculitis Clinic, Division of Rheumatology, Mount Sinai Hospital, Toronto (C.P.).
authors.forename: Mathieu
authors.initials: M
authors.lastname: Zuber
authors.affiliation: From the Departments of Internal Medicine (H.d.B., B.B., A.A.) and Department of Neurology, Caen University Hospital, University of Caen-Normandie, INSERM U919, France (E.T.); Department of Neuroradiology, Centre Hospitalier Sainte-Anne, University Paris-Descartes, INSERM UMR 894, France (G.B., O.N.); Department of Neurology, Lille University Hospital, France (N.D.); Department of Neurology, Angers University Hospital, France (S.G.); Department of Internal Medicine, Nantes University Hospital, France (A.N.); Department of Neurology, Montpellier University Hospital, INSERM UMR 894, France (C.A.); Department of Neurology, Grenoble Alpes University Hospital, France (O.D.); Department of Internal Medicine, Pitié-Salpêtrière Hospital, Paris, France (C.B.-Q.); Department of Vascular Neurology-Centre Hospitalier Saint Joseph, Université Paris-Descartes, INSERM UMR S 919, France (M.Z.); Department of Internal Medicine, Centre Hospitalier Cochin, Paris, France (L.G.); and the Vasculitis Clinic, Division of Rheumatology, Mount Sinai Hospital, Toronto (C.P.).
authors.forename: Emmanuel
authors.initials: E
authors.lastname: Touzé
authors.affiliation: From the Departments of Internal Medicine (H.d.B., B.B., A.A.) and Department of Neurology, Caen University Hospital, University of Caen-Normandie, INSERM U919, France (E.T.); Department of Neuroradiology, Centre Hospitalier Sainte-Anne, University Paris-Descartes, INSERM UMR 894, France (G.B., O.N.); Department of Neurology, Lille University Hospital, France (N.D.); Department of Neurology, Angers University Hospital, France (S.G.); Department of Internal Medicine, Nantes University Hospital, France (A.N.); Department of Neurology, Montpellier University Hospital, INSERM UMR 894, France (C.A.); Department of Neurology, Grenoble Alpes University Hospital, France (O.D.); Department of Internal Medicine, Pitié-Salpêtrière Hospital, Paris, France (C.B.-Q.); Department of Vascular Neurology-Centre Hospitalier Saint Joseph, Université Paris-Descartes, INSERM UMR S 919, France (M.Z.); Department of Internal Medicine, Centre Hospitalier Cochin, Paris, France (L.G.); and the Vasculitis Clinic, Division of Rheumatology, Mount Sinai Hospital, Toronto (C.P.).
authors.forename: Boris
authors.initials: B
authors.lastname: Bienvenu
authors.affiliation: From the Departments of Internal Medicine (H.d.B., B.B., A.A.) and Department of Neurology, Caen University Hospital, University of Caen-Normandie, INSERM U919, France (E.T.); Department of Neuroradiology, Centre Hospitalier Sainte-Anne, University Paris-Descartes, INSERM UMR 894, France (G.B., O.N.); Department of Neurology, Lille University Hospital, France (N.D.); Department of Neurology, Angers University Hospital, France (S.G.); Department of Internal Medicine, Nantes University Hospital, France (A.N.); Department of Neurology, Montpellier University Hospital, INSERM UMR 894, France (C.A.); Department of Neurology, Grenoble Alpes University Hospital, France (O.D.); Department of Internal Medicine, Pitié-Salpêtrière Hospital, Paris, France (C.B.-Q.); Department of Vascular Neurology-Centre Hospitalier Saint Joseph, Université Paris-Descartes, INSERM UMR S 919, France (M.Z.); Department of Internal Medicine, Centre Hospitalier Cochin, Paris, France (L.G.); and the Vasculitis Clinic, Division of Rheumatology, Mount Sinai Hospital, Toronto (C.P.).
authors.forename: Achille
authors.initials: A
authors.lastname: Aouba
authors.affiliation: From the Departments of Internal Medicine (H.d.B., B.B., A.A.) and Department of Neurology, Caen University Hospital, University of Caen-Normandie, INSERM U919, France (E.T.); Department of Neuroradiology, Centre Hospitalier Sainte-Anne, University Paris-Descartes, INSERM UMR 894, France (G.B., O.N.); Department of Neurology, Lille University Hospital, France (N.D.); Department of Neurology, Angers University Hospital, France (S.G.); Department of Internal Medicine, Nantes University Hospital, France (A.N.); Department of Neurology, Montpellier University Hospital, INSERM UMR 894, France (C.A.); Department of Neurology, Grenoble Alpes University Hospital, France (O.D.); Department of Internal Medicine, Pitié-Salpêtrière Hospital, Paris, France (C.B.-Q.); Department of Vascular Neurology-Centre Hospitalier Saint Joseph, Université Paris-Descartes, INSERM UMR S 919, France (M.Z.); Department of Internal Medicine, Centre Hospitalier Cochin, Paris, France (L.G.); and the Vasculitis Clinic, Division of Rheumatology, Mount Sinai Hospital, Toronto (C.P.).
authors.forename: Loïc
authors.initials: L
authors.lastname: Guillevin
authors.affiliation: From the Departments of Internal Medicine (H.d.B., B.B., A.A.) and Department of Neurology, Caen University Hospital, University of Caen-Normandie, INSERM U919, France (E.T.); Department of Neuroradiology, Centre Hospitalier Sainte-Anne, University Paris-Descartes, INSERM UMR 894, France (G.B., O.N.); Department of Neurology, Lille University Hospital, France (N.D.); Department of Neurology, Angers University Hospital, France (S.G.); Department of Internal Medicine, Nantes University Hospital, France (A.N.); Department of Neurology, Montpellier University Hospital, INSERM UMR 894, France (C.A.); Department of Neurology, Grenoble Alpes University Hospital, France (O.D.); Department of Internal Medicine, Pitié-Salpêtrière Hospital, Paris, France (C.B.-Q.); Department of Vascular Neurology-Centre Hospitalier Saint Joseph, Université Paris-Descartes, INSERM UMR S 919, France (M.Z.); Department of Internal Medicine, Centre Hospitalier Cochin, Paris, France (L.G.); and the Vasculitis Clinic, Division of Rheumatology, Mount Sinai Hospital, Toronto (C.P.).
authors.forename: Olivier
authors.initials: O
authors.lastname: Naggara
authors.affiliation: From the Departments of Internal Medicine (H.d.B., B.B., A.A.) and Department of Neurology, Caen University Hospital, University of Caen-Normandie, INSERM U919, France (E.T.); Department of Neuroradiology, Centre Hospitalier Sainte-Anne, University Paris-Descartes, INSERM UMR 894, France (G.B., O.N.); Department of Neurology, Lille University Hospital, France (N.D.); Department of Neurology, Angers University Hospital, France (S.G.); Department of Internal Medicine, Nantes University Hospital, France (A.N.); Department of Neurology, Montpellier University Hospital, INSERM UMR 894, France (C.A.); Department of Neurology, Grenoble Alpes University Hospital, France (O.D.); Department of Internal Medicine, Pitié-Salpêtrière Hospital, Paris, France (C.B.-Q.); Department of Vascular Neurology-Centre Hospitalier Saint Joseph, Université Paris-Descartes, INSERM UMR S 919, France (M.Z.); Department of Internal Medicine, Centre Hospitalier Cochin, Paris, France (L.G.); and the Vasculitis Clinic, Division of Rheumatology, Mount Sinai Hospital, Toronto (C.P.).
authors.forename: Christian
authors.initials: C
authors.lastname: Pagnoux
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D001921:Brain / Q000000981:diagnostic imaging / Q000473:pathology*; D001932:Brain Neoplasms / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000473:pathology; D002533:Cerebral Angiography; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D018810:Magnetic Resonance Angiography; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D020293:Vasculitis, Central Nervous System / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000473:pathology; D055815:Young Adult
keywords: biopsy; mass; primary angiitis of the central nervous system; tumor; tumor-like presentation