title: Outcome of primary cutaneous anaplastic large cell lymphoma: a 20-year British Columbia Cancer Agency experience.
abstract: Primary cutaneous anaplastic large cell lymphoma (PCALCL) is a rare CD30+lymphoproliferative disorder with excellent outcomes reported despite frequent cutaneous relapses. Limited information exists on the development of systemic lymphoma. The British Columbia Cancer Agency (BCCA) Lymphoid Cancer Database was searched to identify all adults diagnosed with PCALCL from 1993 to 2013. From 2005, the BCCA endorsed radiotherapy (RT) alone for limited stage with data failing to support chemotherapy. Forty-seven patients were identified with a male predominance (n = 31, 66%), median age of 64 years and predominantly limited stage (n = 40, 85%). Median follow-up was 8·4 years. The 5-year time to progression (TTP) was 58% (64% limited versus 29% advanced stage). The 5-year disease-specific survival (DSS) and overall survival was 86% and 75%, respectively. In an as-treated analysis, the 5-year DSS for limited stage patients was similar comparing RT to combined modality treatment or chemotherapy alone but the 5-year TTP favoured RT (82% vs. 33%, P = 0·004). The 5-year cumulative risk of developing systemic lymphoma was 14%. Our results confirm the favourable prognosis of PCALCL despite a high relapse rate. Limited stage patients treated with RT alone have excellent outcomes. There is a small risk of systemic lymphoma in PCALCL.
authors.affiliation: Department of Medical Oncology, Centre for Lymphoid Cancer, British Columbia Cancer Agency, Vancouver, BC, Canada.
authors.forename: Greg
authors.initials: G
authors.lastname: Hapgood
authors.affiliation: Department of Radiation Oncology, British Columbia Cancer Agency, Vancouver, BC, Canada.
authors.forename: Tom
authors.initials: T
authors.lastname: Pickles
authors.affiliation: Department of Medical Oncology, Centre for Lymphoid Cancer, British Columbia Cancer Agency, Vancouver, BC, Canada.
authors.forename: Laurie H
authors.initials: LH
authors.lastname: Sehn
authors.affiliation: Department of Medical Oncology, Centre for Lymphoid Cancer, British Columbia Cancer Agency, Vancouver, BC, Canada.
authors.forename: Diego
authors.initials: D
authors.lastname: Villa
authors.affiliation: Department of Medical Oncology, Centre for Lymphoid Cancer, British Columbia Cancer Agency, Vancouver, BC, Canada.
authors.forename: Richard
authors.initials: R
authors.lastname: Klasa
authors.affiliation: Department of Medical Oncology, Centre for Lymphoid Cancer, British Columbia Cancer Agency, Vancouver, BC, Canada.
authors.forename: David W
authors.initials: DW
authors.lastname: Scott
authors.affiliation: Department of Medical Oncology, Centre for Lymphoid Cancer, British Columbia Cancer Agency, Vancouver, BC, Canada.
authors.forename: Alina S
authors.initials: AS
authors.lastname: Gerrie
authors.affiliation: Department of Pathology, Centre for Lymphoid Cancer, British Columbia Cancer Agency, Vancouver, BC, Canada.
authors.forename: Randy D
authors.initials: RD
authors.lastname: Gascoyne
authors.affiliation: Department of Pathology, Centre for Lymphoid Cancer, British Columbia Cancer Agency, Vancouver, BC, Canada.
authors.forename: Graham W
authors.initials: GW
authors.lastname: Slack
authors.affiliation: Department of Radiation Oncology, British Columbia Cancer Agency, Vancouver, BC, Canada.
authors.forename: Christina
authors.initials: C
authors.lastname: Parsons
authors.affiliation: Department of Radiation Oncology, British Columbia Cancer Agency, Vancouver, BC, Canada.
authors.forename: James W
authors.initials: JW
authors.lastname: Morris
authors.affiliation: Department of Medical Oncology, Centre for Lymphoid Cancer, British Columbia Cancer Agency, Vancouver, BC, Canada.
authors.forename: Joseph M
authors.initials: JM
authors.lastname: Connors
authors.affiliation: Department of Medical Oncology, Centre for Lymphoid Cancer, British Columbia Cancer Agency, Vancouver, BC, Canada.
authors.forename: Kerry J
authors.initials: KJ
authors.lastname: Savage
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D001955:British Columbia; D003131:Combined Modality Therapy / Q000379:methods / Q000401:mortality; D016208:Databases, Factual; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D054446:Lymphoma, Primary Cutaneous Anaplastic Large Cell / Q000401:mortality / Q000628:therapy*; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D011878:Radiotherapy / Q000379:methods*; D012008:Recurrence; D015996:Survival Rate; D016896:Treatment Outcome
keywords: cutaneous lymphoid; lymphoid malignancies; malignant lymphomas