DR [Dermatology Reports 2015; 7:5888] [page 1] Treatment of primary cutaneous anaplastic large cell lymphoma with superficial X-rays Malene E. Jepsen,1 Robert Gniadecki1,2 1Department of Dermatology, Bispebjerg Hospital; 2Faculty of Health Sciences, Copenhagen University, Denmark Abstract The optimal radiation schedule for primary cutaneous anaplastic lymphoma (PCALCL) has not been investigated. We report here satisfac- tory outcomes of low-dose (16-20 Gy, 3-5 frac- tions), superficial X-ray radiation (40-50 kV) in a series of 10 patients with PCALCL. Only 1 patient developed a local relapse during the median observation time of 25 months; com- plete remission was recorded in the other patients. This observation indicates that superficial, low dose X-ray therapy may provide a cost-effective alternative to the traditional 35-45 Gy schedules. Introduction Primary cutaneous anaplastic large cell lym- phoma (PCALCL) is a rare indolent cancer with a favorable prognosis and the 5-year spe- cific disease survival of 90%.1-5 The patients often present with solitary or localized nodules or tumors, sometimes with ulceration.3 Treatment in most cases comprises surgery or radiotherapy, sometimes in combination.1,5-8 However, due to rarity of this disease the evi- dence for the efficacy of these modalities is very low. Here we report the excellent outcome of PCALCL treatment with superficial radio- therapy in a small cohort of 11 patients. Materials and Methods Patients with the diagnosis PCALCL were identified from the clinical lymphoma registry in our institution between September 2007 and October 2014. Among 277 patients with cutaneous T-cell lymphomas, 36 patients had a CD30+ lymphoproliferative disorder, and of these 13 were registered as having PCALCL. We have excluded one patient had sponta- neous remission before treatment, one patient died of other causes before the outcome of the radiation therapy was registered and one patient who received electron beam radiation therapy (40 Gy) to multiple tumors in the scalp. The remaining 11 patients (Table 1) were treated with superficial radiotherapy using Gulmay D3100 X-ray unit (Gulmay Ltd., Surrey, UK). Results The male:female ratio was 2.67:1 (8 men, 3 women) which is compatible with the 2-3:1 ratio reported in the literature.3 The median age at diagnosis was 62 years. The median-fol- low-up time after radiation therapy was 26 months. Five patients had an associated malig- nancy: lymphomatoid papulosis (LyP) or myco- sis fungoides (MF). The patients were treated with the dose 16- 20 Gy given in 3-5 daily fractions (40-50 kV). All patients were evaluated after 3 months when complete response was observed in 8/10 patients and partial response (PR) in 3/10 patients (in patient 1 one of the tumors had a CR and one tumor had a PR). On a long-term observation one patient (Patient 9) had a local relapse and Patient 1 progressed and devel- oped new lesions (but not within the irradiat- ed site). No adverse effects were registered except for local hyperpigmentation and mild scarring within the irradiated site. Discussion and Conclusions Our data document satisfactory effect of superficial X-ray treatment of PCALCL tumors, using the doses 16-20 Gy and photon energy 40-50 kV. Theoretically, 50% of the radiation of this energy is absorbed within the most super- ficial 10 mm of the skin,9 which is less that the estimated thickness of some tumors. Dermatology Reports 2015; volume 7:5888 Correspondence: Robert Gniadecki, Department of Dermatology, Bispebjerg Hospital, Bispebjerg bakke 23, DK-2400 Copenhagen, Denmark. E-mail: r.gniadecki@gmail.com Key words: radiotherapy, cutaneous lymphoma. Contributions: the authors contributed equally. Conflict of interest: the authors declare no poten- tial conflict of interest. Received for publication: 25 February 2014. Accepted for publication: 25 February 2014. This work is licensed under a Creative Commons Attribution NonCommercial 3.0 License (CC BY- NC 3.0). ©Copyright M.E. Jepsen and R. Gniadecki, 2015 Licensee PAGEPress, Italy Dermatology Reports 2015; 7:5888 doi:10.4081/dr.2015.5888 Table 1. Patients’ characteristics, treatments and outcomes. Patient Sex Age at Clinical Size of Location Secondary Radiation Response at 3 Follow-up, Relapse Follow up diagnosis lesion(s) lesions, cm lymphoma dose months months 1 M 46 2 tumors 3 Left thigh and crus - 50 kV 4 Gyx5 CR + PR (crus) 56 Local PD 2 F 35 1 tumor 2.5 Left crus Lyp 50 kV 6 Gyx3 CR 85 New CR 3 M 78 1 tumor 1.5 Right forearm Lyp 50 kV 4 Gyx5 CR 13 None CR 4 M 70 Multiple tumors 35×20 Upper back - 50 kV4 Gyx5 CR 41 None CR 5 M 72 1 tumor 2×0.5 Right flank - 40 kV 4 Gyx4 CR 5 None CR 6 F 65 1 plaque 2×3 Behind right ear - 40kV 4 Gyx5 CR 14 None CR 7 F 48 3 tumors 7×5, 5×5.5, 1.5 Left upper arm, MF 50 kV 4 Gyx5 PR 13 None CR right foot, left first finger 8 M 67 1 tumor 4×3×0.5 Left foot - 50kV 4 Gyx5 CR 11 New CR 9 M 68 1 plaque 10, 3.5 Right calf, left hand Lyp 50 kV 4Gyx5 PR 8 None PR 10 M 78 1 tumor 1.5 Right upper arm Lyp, MF 40kV 4 Gyx5 CR 6 None CR LyP, lymphomatoid papulosis; MF, mycosis fungoides; CR, complete response; PR, partial response; PD, progressive disease. No n c om me rci al us e o nly [page 2] [Dermatology Reports 2015; 7:5888] Nevertheless, the clinical outcome of the superficial therapy has been excellent with 90% long-term CR. This indicates the high radio-sensitivity of PCALCL and possibly involvement of secondary anti-tumor mecha- nism such as bystander effect.10 Studies reporting outcome of radiotherapy in PCALCL are scarce.2-5,7,10 The Yale Center records from 2008 is the only study involving radiation therapy exclusively using the dose of 34-44 Gy given in 2-Gy fractions.7 All eight reported patients achieved CR after 12 months median-follow-up. We propose here that the number of fractions and total radiation dose can be reduced to 16-20 Gy, without a signifi- cant loss of long-term efficacy. The modified, low-dose schedule can be delivered as superfi- cial radiotherapy which is more cost-effective and will probably reduce the risk of side- effects. References 1. Kadin ME. 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