PEARLS Primary cutaneous lymphomas (PCL) are subdivided according to cell lineage. These include cutaneous B-cell lymphomas (CBCL) – 30% and cutaneous T-cell lymphomas (CTCL) – 70%. Overall, 1–1.5 new cases per 100,000/year. Annual age-adjusted incidence of CBCL was 4 per million; CTCL was 6 per million persons (Hoppe et al. 2004; Yahalom et al. 2004). Skin is the most common extranodal site of non-Hodgkin’s lymphoma (NHL), representing 2% of new cases of NHL. Affects older adults (55–60 years), 2:1 male predominance, blacks > whites. Hypothesized links with environmental factors or viral etiology not substantiated (de Jong et al. 2008) Presentation: skin lesions, but long natural history. Median time from skin lesion to diagnosis ∼5 years. Sézary cells: malignant T-cells. Sézary syndrome: erythroderma, lymphadenopathy, and Sézary cells in peripheral blood. Treatment should be tailored to specific subtype of PCL. Unlike other NHL’s, which use the Ann Arbor staging system, Mycosis fungoides/Sézary syndrome (MF/SS) uses a TNM staging system that correlates to prognosis (Kim et al. 2007; Horwitz et al. 2008). EORTC and WHO have classification schemes as well (Willemze et al. 2005; Golling et al. 2008).
KeywordsAJCC Cancer Stage Manual Regular Clinic Visit Patient Surface Primary Cutaneous Lymphoma Total Skin Electron Beam
- Olsen E, Vonderheid E, Pimpinelli N, et al. Revisions to the staging and classification of mycosis fungoides and Sézary syndrome: a proposal of the International Society for cutaneous lymphomas (ISCL) and the cutaneous lymphoma task force of the European Organization of Research and Treatment of Cancer (EORTC). Blood. 2007;110:1713-1722.PubMedCrossRefGoogle Scholar
- Senff NJ, Hoefnagel JJ, Neelis KJ, et al. for the Dutch Cutaneous Lymphoma Group. Results of radiotherapy in 153 primary cutaneous B-cell lymphomas classified according to the WHO-EORTC classification. Arch Dermatol. 2007;143(12):1520-1526.Google Scholar
- Golling P, Cozzio A, Dummer R, et al. Primary cutaneous B-cell lymphomas – clinicopathological, prognostic and therapeutic characterisation of 54 cases according to the WHO-EORTC classification and the ISCL/EORTC TNM classification system for primary cutaneous lymphomas other than mycosis fungoides and Sézary syndrome. Leuk Lymphoma. 2008;49(6):1094-1103.PubMedCrossRefGoogle Scholar
- Hoppe RT, Kim Y. Mycosis Fungiodes. In: Leibel SA, Phillips TL, editors. Textbook of radiation oncology. 2nd ed. Philadelphia: Saunders; 2004. pp. 1417-1431.Google Scholar
- Kim Y, Willemze R, Pimpinelli N, et al. TNM classification system for cutaneous lymphomas other than mycosis fungoides and sézary syndrome: a proposal of the International Society for Cutaneous Lymphomas (ISCL) and the Cutaneous Lymphoma Task Force of the European Organization of Research and Treatment of Cancer (EORTC). Blood. 2007;110:479-484.PubMedCrossRefGoogle Scholar
- National Comprehensive Cancer Network. Clinical Practice Guidelines in Oncology v1.2010: Multiple Myeloma. Available at http://www.nccn.org/professionals/physician_gls/PDF/nhl.pdf. Accessed on March 3, 2010.
- Yahalom J. Non-Hodgkin’s lymphoma. In: Leibel SA, Phillips TL, editors. Textbook of radiation oncology. 2nd ed. Philadelphia: Saunders; 2004. pp. 1393-1416.Google Scholar