Radiation Oncology/NHL/Cutaneous B-cell lymphoma

Primary Cutaneous B-Cell Lymphoma (PCBCL) For cutaneous T-cell lymphomas, see Mycosis fungoides Pathology B-cell lymphomas 20-25% of all cutaneous lymphomas Subtypes: primary cutaneous follicular center-cell lymphoma (PCFCCL), primary cutaneous immunocytoma (PCI), primary cutaneous large B-cell lymphoma (PCLBCL) of the leg WHO Classification Most common histology: diffuse large B-cell lymphoma EORTC Classification Note: in the EORTC, diffuse large B-cell lymphomas of non-leg site are classified as follicle center cell (i.e., considered indolent instead of aggressive) Epidemiology Overall (data from SEER, 15908651) Mean age 64 yrs. Male 54%. Incidence increasing Histology (WHO): DLBCL in 33%, NOS 24%, follicular 17%, MZL 8% Site: head or neck 50%, trunk 19%, arm 12%, leg 11%, disseminated 8% PCFCCL Localized to skin; rarely disseminate to extracutaneous sites Usually involve head or trunk 5-yr OS > 95% PCI Usually involve arms and legs Excellent prognosis PCLBCLs of the leg Affects elderly patients More often involves extracutaneous sites Poor prognosis: 5-yr OS 58% SEER; 2008 (1973-2004) 18349400 -- "Analysis of primary CD30+ cutaneous lymphoproliferative disease and survival from the Surveillance, Epidemiology, and End Results database." (Yu JB, J Clin Oncol. 2008 Mar 20;26(9):1483-8.) SEER review. 268 cases; 58% males, 87% white. 157 (59%) primary localized PCLPD Outcome: 3-year OS 81% vs. population-matched OS 87%; 5-year DSS 92%. H&N worse outcome Conclusion: Rare disease, with excellent OS; more common in white men, H&N sites worse survival Prognostic Index Yale; 2005 - 15908651 — "The cutaneous B-cell lymphoma prognostic index: a novel prognostic index derived from a population-based registry." (Smith BD, J Clin Oncol. 2005 May 20;23(15):3390-5.) From SEER registry (1973-2001). 1343 cases. Histology according to WHO classification. Developed prognostic index based on histology and site. CBCL-PI Group Histology Site 5-yr OS IA Indolent (follicular, marginal zone, small lymphocytic, lymphoplasmacytic) Any 81% IB Diffuse large B-cell Favorable (head/neck, arm) 72% II DLBCL Unfavorable 48% Immunoblastic diffuse large B-cell Favorable III Immunoblastic diffuse large B-cell Unfavorable 27% Outcomes Yale (1990-2002) Retrospective, 34 pts. Treated with RT as primary treatment; Median dose 40 Gy (range 20-48 Gy). No systemic disease. Comparison of EORTC and WHO pathologic classification systems, which differ in characterizing these lesions, and therefore recommendations for treatment may differ (based on characterization as indolent or aggressive lymphomas) 2004 14966086 -- "Primary cutaneous B-cell lymphoma treated with radiotherapy: a comparison of the European Organization for Research and Treatment of Cancer and the WHO classification systems." (Smith BD, J Clin Oncol. 2004 Feb 15;22(4):634-9.) -- Median f/u 3.4 yrs 5-yr OS 96%, RFS 55%, LR-free survival 81%, extracutaneous relapse-free survival 79%. OS and RFS was 67% and 33% for leg-type DLBCL and 100% and 62-73% for other types 62% (8/13) of initial relapses confined to skin (localregional or distant), 24% (8/34) of all pts. Distant relapse (+/- skin) in 38% (5/13) of all relapses or 15% (5/34) of all pts. Pathologic: 68% of lesions classified as follicle center cell (i.e. indolent) by EORTC criteria are classified as DLBCL (i.e. aggressive) by WHO system, but the biologic behavior is indolent. Increased risk of LR with dose < 36 Gy: 5-yr LR-free survival 50% (< 36 Gy) vs 90% (>= 36 Gy). Conclusion: 40 Gy dose is recommended. Worse outcome for leg type; however, for the other lesions, DLBCL histology appears more indolent than in nodal lymphomas. PCLBCL: European multicenter (1979-98) - 11504742, 2001 — "Prognostic factors in primary cutaneous large B-cell lymphomas: a European multicenter study." Grange F et al. J Clin Oncol. 2001 Aug 15;19(16):3602-10. Retrospective. 48 pts with PCLBCL of leg; 97 with PCFCCL. On multivariate analysis, worse outcomes for location on leg, multiple lesions, and round cell morphology. Treatment Solitary lesion: Most often presents as a solitary lesion (~85%). Radiotherapy is preferred treatment for solitary lesions. Dose 40 Gy. Multifocal disease: Netherlands (1985-97) - 10561311, 1999 — "Treatment of multifocal primary cutaneous B-cell lymphoma: a clinical follow-up study of 29 patients." Bekkenk MW et al. J Clin Oncol. 1999 Aug;17(8):2471-8. Retrospective. Included 29 pts who had involvement of >= 2 nonadjacent skin areas that did not fit into one radiation field without extracutaneous disease (14% of patients seen at this center). Pts treated with either chemotherapy (CHOP in most) or RT. 16 pts had PCFCCL, 8 PCI, 5 PCLBCL of leg. Excellent results in PCFCCL or PCI for either chemo or RT. All pts with PCLBCL relapsed with extracutaneous disease.