title: Primary cranial vault lymphoma with brain infiltration: case report and review of the literature.
abstract: Primary cranial vault lymphomas (PCVL) are extremely rare tumours. An extensive review of the literature shows that only 26 previous PCVL were reported while only four presented with cortex infiltration. We present a singular case of a 74-year-old woman with a voluminous painful mass lesion of the right temporal scalp. Preoperative neuroradiological assessment showed a lesion involving the scalp, the cranial vault and the subdural space; underneath, a cerebral oedema suggested brain infiltration. The absence of any neurological signs despite brain infiltration makes this case exceptional. The preoperative diagnostic hypothesis was infiltrating meningioma, and a surgical excision was scheduled. A radical removal of the lesion was obtained, but the histopathological result was unexpectedly large B-cell non-Hodgkin lymphoma. Further investigations failed to identify pathology elsewhere confirming the diagnosis of PCVL. Postoperative radio and chemotherapy were administered. The patient is free from signs of disease recurrence at a 2-year follow-up. Radiological features are debated with the aim to stress the possible differential diagnosis, and prognostic factors are discussed. Even if an accepted therapeutic algorithm is far from being defined for these extremely rare tumours, we suggest that radical surgical removal associated with radio and chemotherapy could be the best choice in these particular cases.
authors.affiliation: Hopital Cantonal Fribourg, Departement Neurochirurgie, Fribourg, CH, Switzerland. ciarpa@hotmail.com
authors.forename: Riccardo
authors.initials: R
authors.lastname: Ciarpaglini
authors.forename: Philippe
authors.initials: P
authors.lastname: Otten
mesh_terms: D000368:Aged; D001932:Brain Neoplasms / Q000473:pathology* / Q000628:therapy; D003131:Combined Modality Therapy; D005260:Female; D006801:Humans; D016403:Lymphoma, Large B-Cell, Diffuse / Q000473:pathology* / Q000628:therapy; D009361:Neoplasm Invasiveness / Q000473:pathology; D035583:Rare Diseases / Q000473:pathology* / Q000628:therapy; D012888:Skull Neoplasms / Q000473:pathology* / Q000628:therapy