Primary cutaneous diffuse large B-cell lymphoma, leg-type
first presentation report as Marjolin ulcer on chronic venous ulcer
Abstract
Primary Cutaneous Diffuse Large B-cell Lymphoma, Leg-type (CBCL-LT) accounts for 4% of the primary cutaneous lymphomas (PCL). It is characterized by being aggressive, with a five-year survival rate of around 55%. Marjolin’s ulcer is a malignant neoplasms arising in an area of chronic lesions, being squamous cell carcinoma the most frequent one.
The study presents the case of a 74 year old male patient who suffered from bilateral chronic venous insufficiency, carrier of a chronic ulcer with 15 years of evolution in his right leg. Since three years ago he presents two exophytic lesions on the ulcer’s bottom, with accelerated progression in the last months, Marjolin’s ulcer being the possible diagnosis.
Histopathology showed an diffuse proliferation of atypical lymphoid cells of blastic type lymphoid cells. Immunohistochemistry evidenced CD20+, CD10-, MUM1+, Bcl-2+, Bcl-6-, Ki67 90%. Studies were compatible with a diagnosis of primary cutaneous diffuse large B-cell Lymphoma, Leg-type. Imagenology assessment failed to evidence deep adenopahties or visceromegally. Bone marrow biopsy could not be completed due to difficulties resulting from the patient’s obesity and pain intolerance. Local radiation therapy was used with a total dose of 54Gy, evolution being excellent and tumor lesions disappeared. Chemotherapy associated to rituximab is the first line therapy for primary cutaneous diffuse large B-cell Lymphoma, leg-type, with or without radiation therapy.
If the patient’s condition is not compatible with aggressive treatment, then rituximab monotherapy or radiation therapy are considered. This study corresponds to the second case of primary cutaneous lymphoma reported in the global literature, that debuts as Marjolin´s ulcer on a chronic venous lesion, and the first one of the primary cutaneous diffuse large B-cell lymphoma, leg- type sub-type.
References
(2) Groves FD, Linet MS, Travis LB, Devesa SS. Cancer surveillance series: non-Hodgkin’s lymphoma incidence by histologic subtype in the United States from 1978 through 1995. J Natl Cancer Inst 2000; 92(15):1240-51.
(3) Suárez AL, Pulitzer M, Horwitz S, Moskowitz A, Querfeld C, Myskowski PL. Primary cutaneous B-cell lymphomas: part I. Clinical features, diagnosis, and classification. J Am Acad Dermatol 2013; 69(3):329.e1-13.
(4) Dores GM, Anderson WF, Devesa SS. Cutaneous lymphomas reported to the National Cancer Institute’s surveillance, epidemiology, and end results program: applying the new WHO-European Organisation for Research and Treatment of Cancer classification system. J Clin Oncol 2005; 23(28): 7246-8.
(5) Bradford PT, Devesa SS, Anderson WF, Toro JR. Cutaneous lymphoma incidence patterns in the United States: a population-based study of 3884 cases. Blood 2009; 113(21): 5064-73.
(6) Vermeer MH, Geelen FA, van Haselen CW, van Voorst Vader PC, Geerts ML, Van Vloten WA, et al. Primary cutaneous large B-cell lymphomas of the legs: a distinct type of cutaneous B-cell lymphoma with an intermediate prognosis. Dutch Cutaneous Lymphoma Working Group. Arch Dermatol 1996; 132(11):1304-8.
(7) Suárez AL, Querfeld C, Horwitz S, Pulitzer M, Moskowitz A, Myskowski PL. Primary cutaneous B-cell lymphomas: part II. Therapy and future directions. J Am Acad Dermatol. 2013; 69(3):343.e1-11.
(8) Senff NJ, Noordijk EM, Kim YH, Bagot M, Berti E, Cerroni L, et al. European Organization for Research and Treatment of Cancer and International Society for Cutaneous Lymphoma consensus recommendations for the management of cutaneous B-cell lymphomas. Blood 2008; 112(5):1600-9.
(9) Hahn SB, Kim DJ, Jeon CH. Clinical study of Marjolin’s ulcer. Yonsei Med J 1990; 31(3):234-41.
(10) Smith J, Mello LF, Nogueira Neto NC, Meohas W, Pinto LW, Campos VA, et al. Malignancy in chronic ulcers and scars of the leg (Marjolin’s ulcer): a study of 21 patients. Skeletal Radiol 2001; 30(6):331-7.
(11) Kowal-Vern A, Criswell BK. Burn scar neoplasms: a literature review and statistical analysis. Burns 2005; 31(4): 403-13.
(12) Trent JT, Kirsner RS. Wounds and malignancy. Adv Skin Wound Care 2003; 16(1):31-4.
(13) Cendras J, Sparsa A, Bedane C, Delage M, Touati M, Bonnetblanc JM. Primary cutaneous large B-cell lymphoma in chronic venous leg ulcer. Ann Dermatol Venereol 2007; 134(4 Pt 1):357-61.
(14) Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, et al, eds. WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues. 4 ed. Lyon: IARC Press, 2008.
(15) Grange F, Beylot-Barry M, Courville P, Maubec E, Bagot M, Vergier B, et al. Primary cutaneous diffuse large B-cell lymphoma, leg type: clinicopathologic features and prognostic analysis in 60 cases. Arch Dermatol 2007; 143(9): 1144-50.
(16) Kodama K, Massone C, Chott A, Metze D, Kerl H, Cerroni L. Primary cutaneous large B-cell lymphomas: clinicopathologic features, classification, and prognostic factors in a large series of patients. Blood 2005; 106(7):2491-7.
(17) Grange F, Bekkenk MW, Wechsler J, Meijer CJ, Cerroni L, Bernengo M, et al. Prognostic factors in primary cutaneous large B-cell lymphomas: a European multicenter study. J Clin Oncol. 2001; 19(16):3602-10.
(18) Thomas V, Dobson R, Mennel R. Primary cutaneous large B-cell lymphoma, leg type. Proc (Bayl Univ Med Cent) 2011; 24(4):350-3.
(19) Geelen FA, Vermeer MH, Meijer CJ, Van der Putte SC, Kerkhof E, Kluin PM, et al. Bcl-2 expression in primary cutaneous large B-cell lymphoma is site-related. J Clin Oncol 1998; 16(6):2080-5.
(20) Hoefnagel JJ, Dijkman R, Basso K, Jansen PM, Hallermann C, Willemze R, et al. Distinct types of primary cutaneous large B-cell lymphoma identified by gene expression profiling. Blood 2005; 105(9):3671-8.
(21) Hoefnagel JJ, Vermeer MH, Janssen PM, Fleuren GJ, Meijer CJ, Willemze R. Bcl-2, Bcl-6 and CD10 expression in cutaneous B-cell lymphoma: further support for a follicle centre cell origin and differential diagnostic significance. Br J Dermatol 2003; 149(6):1183-91.
(22) Paulli M, Viglio A, Vivenza D, Capello D, Rossi D, Riboni R, et al. Primary cutaneous large B-cell lymphoma of the leg: histogenetic analysis of a controversial clinicopathologic entity. Hum Pathol 2002; 33(9):937-43.
(23) Demirkesen C, Tuzuner N, Esen T, Lebe B, Ozkal S. The expression of IgM is helpful in the differentiation of primary cutaneous diffuse large B cell lymphoma and follicle center lymphoma. Leuk Res 2011; 35(9):1269-72.
(24) Koens L, Vermeer MH, Willemze R, Jansen PM. IgM expression on paraffin sections distinguishes primary cutaneous large B-cell lymphoma, leg type from primary cutaneous follicle center lymphoma. Am J Surg Pathol 2010; 34(7): 1043-8.
(25) Hallermann C, Kaune KM, Gesk S, Martin-Subero JI, Gunawan B, Griesinger F, et al. Molecular cytogenetic analysis of chromosomal breakpoints in the IGH, MYC, BCL6, and MALT1 gene loci in primary cutaneous B-cell lymphomas. J Invest Dermatol 2004; 123(1):213-9.
(26) Dijkman R, Tensen CP, Jordanova ES, Knijnenburg J, Hoefnagel JJ, Mulder AA, et al. Array-based comparative genomic hybridization analysis reveals recurrent chromosomal alterations and prognostic parameters in primary cutaneous large B-cell lymphoma. J Clin Oncol 2006; 24(2): 296-305.
(27) Grange F, Petrella T, Beylot-Barry M, Joly P, D’Incan M, Delaunay M, et al. Bcl-2 protein expression is the strongest independent prognostic factor of survival in primary cutaneous large B-cell lymphomas. Blood 2004; 103(10):3662-8.
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