title: Treatment of multifocal primary cutaneous B-cell lymphoma: a clinical follow-up study of 29 patients.
abstract: OBJECTIVE
  Although patients with primary cutaneous B-cell lymphoma (CBCL) and localized skin lesions are generally treated with radiotherapy and have an excellent prognosis, the clinical behavior and optimal treatment of CBCL presenting with multifocal skin lesions are less well defined. In this study, we evaluated the clinical behavior of and results of treatment for multifocal CBCL in 29 patients, and we formulated therapeutic guidelines.
  
  
  METHODS
  The study group included 16 patients with primary cutaneous follicular center-cell lymphoma (PCFCCL), eight with primary cutaneous immunocytoma (PCI), and five with primary cutaneous large B-cell lymphoma presenting on the legs (PCLBCL of the leg).
  
  
  RESULTS
  Only one of the 24 patients with multifocal PCFCCL or PCI developed extracutaneous disease, and no patient died from lymphoma (median follow-up, 54 months). In patients with PCFCCL, treatment with either multiagent chemotherapy (nine patients) or radiotherapy directed toward all skin lesions (five patients) proved equally effective in terms of complete remission, relapse, and survival. In contrast, all five patients with PCLBCL of the leg developed extracutaneous disease, and four of the five died from systemic lymphoma, 8 to 36 months (median, 21 months) after diagnosis.
  
  
  CONCLUSIONS
  The results of these preliminary studies suggest that patients with PCFCCL or PCI presenting with multifocal skin lesions have the same excellent prognosis that patients with localized PCFCCL or PCI have and that radiotherapy directed toward all skin lesions is as effective as multiagent chemotherapy. Patients with PCLBCL of the leg have a more unfavorable prognosis, particularly patients presenting with multifocal skin lesions. This last group should always be treated with multiagent chemotherapy.
authors.affiliation: Departments of Dermatology and Pathology, Free University Hospital, Amsterdam.
authors.forename: M W
authors.initials: MW
authors.lastname: Bekkenk
authors.forename: M H
authors.initials: MH
authors.lastname: Vermeer
authors.forename: M L
authors.initials: ML
authors.lastname: Geerts
authors.forename: E M
authors.initials: EM
authors.lastname: Noordijk
authors.forename: F
authors.initials: F
authors.lastname: Heule
authors.forename: P C
authors.initials: PC
authors.lastname: van Voorst Vader
authors.forename: W A
authors.initials: WA
authors.lastname: van Vloten
authors.forename: C J
authors.initials: CJ
authors.lastname: Meijer
authors.forename: R
authors.initials: R
authors.lastname: Willemze
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000465:Algorithms; D000900:Anti-Bacterial Agents / Q000627:therapeutic use; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D003131:Combined Modality Therapy; D003520:Cyclophosphamide / Q000008:administration & dosage; D004317:Doxorubicin / Q000008:administration & dosage; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D016393:Lymphoma, B-Cell / Q000188:drug therapy* / Q000473:pathology / Q000532:radiotherapy*; D016403:Lymphoma, Large B-Cell, Diffuse / Q000188:drug therapy* / Q000473:pathology / Q000532:radiotherapy*; D008297:Male; D008875:Middle Aged; D009378:Neoplasms, Multiple Primary / Q000188:drug therapy* / Q000473:pathology / Q000532:radiotherapy; D011241:Prednisone / Q000008:administration & dosage; D012878:Skin Neoplasms / Q000188:drug therapy* / Q000473:pathology / Q000532:radiotherapy*; D014750:Vincristine / Q000008:administration & dosage
chemical_list: D000900:Anti-Bacterial Agents; D014750:Vincristine; D004317:Doxorubicin; D003520:Cyclophosphamide; D011241:Prednisone