Annals of Oncology 15:1097-1108, 2004
© 2004 European Society for Medical Oncology
CD56+ hematological neoplasms presenting in the skin: a retrospective analysis of 23 new cases and 130 cases from the literature
Background: The aim of this study was to define prognostic parameters and guidelines for diagnosis and treatment for CD56+ hematological neoplasms with first presentation in the skin.Patients and methods: The study group included 153 cases (23 new and 130 from the literature). According to the World Health Organization classification, the group included 15 nasal and 38 nasal-type natural killer (NK)/T-cell lymphomas, 63 blastic NK-cell lymphomas, 14 cutaneous CD30+ lymphoproliferations, 10 cases of myeloid leukemia, six cases of subcutaneous panniculitis-like T-cell lymphoma (SCPLTCL) and seven peripheral T-cell lymphomas, unspecified.
Results: In general, these CD56+ hematological neoplasms had a poor prognosis, with only 27% of patients alive after a median follow-up of 12 months. The median survival was 13 months. Nasal and nasal-type NK/T-cell lymphomas and CD56+ SCPLTCL had the worst prognosis, with a median survival of 5, 6 and 5 months, respectively. Only nasal-type NK/T-cell lymphomas presenting with only skin lesions had a somewhat better prognosis (median survival 27 months). In blastic NK-cell lymphomas (median survival 14 months), age 
40 years, aggressive treatment with acute leukemia protocols and high TdT expression were associated with a more favorable prognosis. Striking similarities in histology, immunophenotype, clinical presentation and clinical behavior were found between blastic NK-cell lymphomas and CD56+ myeloid leukemias.
Conclusions: CD56+ hematological neoplasms presenting in the skin have a poor prognosis, except for primary cutaneous CD30+ lymphoproliferations. The striking similarities between blastic NK-cell lymphomas and CD56+ myeloid leukemias presenting in the skin provide a rationale to treat these patients with more aggressive regimens, rather than with CHOP(-like) regimens and radiotherapy, which have proven to be inadequate therapies for this neoplasm.
Departments of 1 Dermatology; 2 Pathology, Leiden University Medical Center, Leiden; 3 Department of Pathology, Vrije Universiteit Medical Center, Amsterdam, The Netherlands
*Correspondence to: Dr M. Bekkenk, Department of Dermatology, B1-Q-93, Leiden University Medical Center, PO Box 9600, 2300 RC Leiden, The Netherlands. Tel: +31-71-526-2421; Fax: +31-71-524-8106; Email: m.w.bekkenk{at}lumc.nl
Key words: CD56, classification, cutaneous NK/T-cell lymphoma, leukemia, prognosis, treatment
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