title: Primary lymphoma of the cranial vault: case report and a systematic review of the literature.
abstract: BACKGROUND
  Bone involvement is a common finding in many types of lymphoma. Cranial vault involvement is extremely rare, and the majority of patients are found at staging to have concurrent disease in lymph nodes. Thirty-eight cases of primary lymphoma of the cranial vault have been reported to date.
  
  
  METHODS
  This article presents a rare case of primary cranial vault lymphoma and conducts a systematic review of the current literature. A total of 36 articles comprising 38 cases were included for analysis. The relevant demographic, clinical, and imaging characteristics, as well as the treatment and outcomes of this unique disease presentation were studied.
  
  
  RESULTS
  The average patient age was 60 years. There was no significant difference in patient gender. The predominant patient complaint was a subcutaneous scalp mass. Of the patients, 11.7% were immunocompromised. CT scans showed signs of osteolysis in 74% of lesions and hyperostosis in 5%, and the cranial vault was observed as normal in 18% of cases. MRI was performed in 23 cases. There was a wide range of histological subtypes, with a slight predominance of diffuse large B-cell lymphoma. Treatment consisted of surgery alone, surgery followed by radiotherapy, and surgery followed by radiotherapy and chemotherapy. The follow-up periods ranged from 5 months to 6 years, with a median value of 7 months. Sixteen patients were followed up to 12 months; 13 of them were alive at 1 year from diagnosis.
  
  
  CONCLUSIONS
  Primary cranial vault lymphoma is an extremely rare finding. It should be considered in the differential diagnosis of scalp masses. Although the analysis of outcome of the reported cases is difficult because of the small number of occurrences of this entity and the variability of follow-up, a combination of surgery, radiotherapy, and chemotherapy seems to offer better outcomes.
authors.affiliation: Department of Neurosurgery, Mohammed V Military Teaching Hospital, University of Mohammed V - Suissi, Rabat, Morocco. abad20031@hotmail.com
authors.forename: Abad Cherif
authors.initials: AC
authors.lastname: El Asri
authors.forename: Ali
authors.initials: A
authors.lastname: Akhaddar
authors.forename: Hassan
authors.initials: H
authors.lastname: Baallal
authors.forename: Omar
authors.initials: O
authors.lastname: Boulahroud
authors.forename: Cherkaoui
authors.initials: C
authors.lastname: Mandour
authors.forename: Hafsa
authors.initials: H
authors.lastname: Chahdi
authors.forename: Mohammed
authors.initials: M
authors.lastname: Mikdame
authors.forename: Miloudi
authors.initials: M
authors.lastname: Gazzaz
authors.forename: Brahim
authors.initials: B
authors.lastname: El Mostarchid
authors.forename: Mohamed
authors.initials: M
authors.lastname: Boucetta
mesh_terms: D018951:Antigens, CD20 / Q000032:analysis; D014408:Biomarkers, Tumor / Q000032:analysis*; D006801:Humans; D018442:Lymphoma, B-Cell, Marginal Zone / Q000737:chemistry / Q000175:diagnosis* / Q000473:pathology*; D008279:Magnetic Resonance Imaging; D008297:Male; D008577:Meningeal Neoplasms / Q000737:chemistry / Q000175:diagnosis* / Q000473:pathology*; D008875:Middle Aged; D012888:Skull Neoplasms / Q000737:chemistry / Q000175:diagnosis* / Q000473:pathology*
chemical_list: D018951:Antigens, CD20; D014408:Biomarkers, Tumor