title: Paediatric primary cutaneous marginal zone B-cell lymphoma: does it differ from its adult counterpart?
abstract: BACKGROUND
  Primary cutaneous marginal zone B-cell lymphoma (PCMZL) has rarely been reported in patients younger than 20 years.
  
  
  OBJECTIVE
  To report our experience with PCMZL in the paediatric/adolescent age group.
  
  
  METHODS
  Medical records of patients diagnosed with PCMZL before age 20 years and managed at two cutaneous lymphoma clinics in the U.S.A. and Israel from 1992 to 2015 were reviewed.
  
  
  RESULTS
  The study group included 11 patients (six girls; median age 16 years, range 6-19·5); 10 had generalized/multifocal (T3) and one had regional/localized (T2) disease. Lesions were located on the limbs in all patients and the trunk in six; two had facial lesions. Staging in all but one was based on whole-body computed tomography or positron emission tomography. Initial management in most patients included nonradiation modalities: one patient with localized disease received intralesional steroids; six patients with multifocal disease received the following: topical/intralesional steroids (n = 3); excision (n = 2); 'watch and wait' (n = 1). No extracutaneous progression was noted during a median follow-up of 5·5 years (mean 7·5, range 0·5-14). At present, five patients are in complete remission.
  
  
  CONCLUSIONS
  Based on our data (largest series in the literature with the longest follow-up), the clinicopathological presentation and course of PCMZL in the paediatric/adolescent age group are similar to those in adults. Given the indolent course and the long life expectancy of these young patients, the cumulative risk of imaging studies and the age-related potential toxicity of treatment, especially radiation, should be taken into consideration.
authors: Department of Dermatology, Davidoff Center, Rabin Medical Center - Beilinson Hospital, Petach Tikva, Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.
authors: I
authors: I
authors: Amitay-Laish
authors: Department of Dermatology, Stanford Cancer Center, Stanford, CA, U.S.A.
authors: M
authors: M
authors: Tavallaee
authors: Department of Pathology, Stanford Cancer Center, Stanford, CA, U.S.A.
authors: J
authors: J
authors: Kim
authors: Department of Radiation Oncology, Stanford Cancer Center, Stanford, CA, U.S.A.
authors: R T
authors: RT
authors: Hoppe
authors: Department of Radiation Oncology, Stanford Cancer Center, Stanford, CA, U.S.A.
authors: L
authors: L
authors: Million
authors: Department of Pathology, Davidoff Center, Rabin Medical Center - Beilinson Hospital, Petach Tikva, Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.
authors: M
authors: M
authors: Feinmesser
authors: Department of Radiotherapy Unit, Davidoff Center, Rabin Medical Center - Beilinson Hospital, Petach Tikva, Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.
authors: E
authors: E
authors: Fenig
authors: Department of Dermatology, Stanford Cancer Center, Stanford, CA, U.S.A.
authors: M E L
authors: MEL
authors: Wolfe
authors: Department of Dermatology, Davidoff Center, Rabin Medical Center - Beilinson Hospital, Petach Tikva, Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.
authors: E
authors: E
authors: Hodak
authors: Department of Dermatology, Stanford Cancer Center, Stanford, CA, U.S.A.
authors: Y H
authors: YH
authors: Kim
mesh_terms: D000279:Administration, Cutaneous; D000284:Administration, Oral; D000293:Adolescent; D000970:Antineoplastic Agents / Q000008:administration & dosage; D002648:Child; D005260:Female; D006801:Humans; D015552:Injections, Intralesional; D018442:Lymphoma, B-Cell, Marginal Zone / Q000473:pathology* / Q000628:therapy; D008297:Male; D009367:Neoplasm Staging; D049268:Positron-Emission Tomography; D012878:Skin Neoplasms / Q000473:pathology* / Q000628:therapy; D013256:Steroids / Q000008:administration & dosage; D014057:Tomography, X-Ray Computed; D057832:Watchful Waiting; D055815:Young Adult
chemical_list: D000970:Antineoplastic Agents; D013256:Steroids