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Tumor-Like Presentation of Primary Angiitis of the Central Nervous System
Hubert de Boysson1, Grégoire Boulouis2, Nelly Dequatre2
1From 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.
Background And Purpose:
We aimed to describe the clinical and imaging features of patients with tumor-like presentation of primary angiitis of the central nervous system.
Methods:
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.
Results:
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.
Conclusions:
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.
