Should primary cutaneous Ki-1(CD30)-positive anaplastic large cell lymphoma in childhood be treated with multiple-agent chemotherapy?

Should primary cutaneous Ki-1(CD30)-positive anaplastic large cell lymphoma in childhood be treated with multiple-agent chemotherapy?

638 Letters J AM ACAD DERMATOL OCTOBER 2001 Should primary cutaneous Ki-1(CD30)positive anaplastic large cell lymphoma in childhood be treated with ...

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638 Letters

J AM ACAD DERMATOL OCTOBER 2001

Should primary cutaneous Ki-1(CD30)positive anaplastic large cell lymphoma in childhood be treated with multiple-agent chemotherapy? To the Editor: We read with interest the case report by Tomaszewski, Moad, and Lupton in the May 1999 issue of the Journal regarding primary cutaneous CD30-positive large T-cell lymphoma in children (J Am Acad Dermatol 1999;40:857-61). The authors describe 3 children with a CD30+ anaplastic large cell lymphoma (ALCL) limited to the skin. In all 3 patients intensive multiple-agent chemotherapy (vincristine, doxorubicin, and prednisone, in combination with either cytoxan or methotrexate and 6mercaptopurine) as well as intrathecal methotrexate resulted in complete remission. However, in all cases cutaneous relapses developed during follow-up: 8, 5, and 3 months after initial diagnosis. Therefore one wonders whether multiple-agent chemotherapy is appropriate for these young patients with a CD30+ ALCL confined to the skin. Primary cutaneous CD30+ ALCL in childhood is rare, and differentiation from lymphomatoid papulosis (LyP) may be extremely difficult. In a recent study of the Dutch Cutaneous Lymphoma Group on 219 patients with a primary or secondary CD30+ lymphoproliferative disorder only 1 of 79 patients (1%) with a primary cutaneous CD30+ ALCL was younger than 20 years, compared with 12 of 118 patients with LyP (10%).1 The difficulties in making a correct diagnosis are illustrated by 3 children in our study, who presented with large, rapidly growing, ulcerating lesions. Because of this very distressing clinical presentation, suggesting an aggressive lymphoma, mul-

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tiple-agent chemotherapy was first considered. However, because the histologic appearance was more suggestive of LyP and staging procedures did not show any evidence of extracutaneous disease, it was decided to wait for several weeks. After this period spontaneous resolution of the larger lesions occurred in all patients, and new smaller lesions characteristic of LyP developed in all 3 patients. Our group also contained a 12-year-old boy with a 2-year history of classic LyP with recurrent self-healing papulonecrotic lesions, in whom a large ulcerating tumor developed; the tumor was 5 cm in diameter and located on the back, similar to case 2 described by Tomaszewski et al. There was no lymphadenopathy and computed abdominal and thoracic tomography showed no abnormalities. It was therefore decided to postpone planned radiotherapy for several weeks. In the following weeks the tumor’s size gradually decreased and it eventually disappeared completely without any treatment, whereas small papules continued to develop. A similar favorable course was observed in 3 adult patients. Previous studies have indicated that progression of LyP to CD30+ ALCL with only skin lesions (ie, the development of a large [ulcerating] tumor) does not affect the favorable clinical course of LyP.2 Therefore we suggest that when larger skin tumors develop in LyP and staging is negative, spontaneous remission can be anticipated in 4 to 6 weeks. In case spontaneous resolution does not occur, such lesions can be excised or treated with radiotherapy. In both scenarios there is no good reason to treat such patients with multiple-agent chemotherapy. Primary cutaneous CD30+ ALCL has a high tendency to relapse in the skin. In our own study 32 of 79 patients (41%) had one or multiple skin relapses.1 Consistent with the observations of Tomaszewski et al, skin relapses were also observed in all 7 patients who had received multiple-agent chemotherapy because of the presence of multifocal skin lesions. Because multiple-agent chemotherapy seldom results in cures in this group and is unlikely to prevent the development of extracutaneous disease observed in a small minority of patients, we agree with other investigators that multiple-agent chemotherapy is only indicated in patients presenting with or developing extracutaneous disease, and not, or rarely, in patients with only skin lesions.3 Because available data suggest that primary cutaneous CD30+ ALCL in childhood is very similar to that in adults, and quite distinct from systemic CD30+ ALCL in children, we believe that these pediatric cases should be treated in the same way and that intensive chemotherapy is not warranted. Finally, it is important to note that none of the 79 primary cutaneous CD30+ ALCL and none of the 118

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patients with LyP in our study developed central nervous system involvement. Therefore we believe that intrathecal methotrexate in CD30+ ALCL with only skin lesions is not justified. Maarten H. Vermeer, MD Marcel W. Bekkenk, MD Rein Willemze, MD, PhD Department of Dermatology Leiden University Medical Center PO Box 9600 2300 RC Leiden, The Netherlands E-mail: [email protected] REFERENCES 1. Bekkenk MW, Geelen FAMJ, van Voorst Vader PC, Heule F, Geerts ML, van Vloten WA, et al. Primary and secondary cutaneous CD30+ lymphoproliferative disorders: a report from the Dutch Cutaneous Lymphoma Group on the long-term follow-up data of 219 patients and guidelines for diagnosis and treatment. Blood 2000;95:3653-61. 2. Beljaards RC, Willemze R. The prognosis of patients with lymphomatoid papulosis associated with malignant lymphomas. Br J Dermatol 1992;126:596-602. 3. Demierre MF, Goldberg LJ, Kadin ME, Koh HK. Is it lymphoma or lymphomatoid papulosis? J Am Acad Dermatol 1997;36:76572. 16/8/114571 doi:10.1067/mjd.2001.114571