Primary Large B-cell Lymphoma of the Skin

Arch Dermatol. 2007 Sep;143(9):1144-50. Abstract quote

Objectives To describe clinicopathologic features and to identify prognostic factors in a large series of primary cutaneous diffuse large B-cell lymphoma, leg type (PCLBCL LT), as defined in the recent World Health Organization-European Organization for Research and Treatment of Cancer classification of cutaneous lymphomas.

DESIGN: Retrospective multicenter study from the French Study Group on Cutaneous Lymphomas.

SETTING: Nineteen departments of dermatology in 10 regions of France. Patients Sixty patients with a PCLBCL LT included in the registry of the French Study Group on Cutaneous Lymphomas.

MAIN OUTCOME MEASURES: Age, sex, outcome, therapy, B symptoms, cutaneous extent, number of lesions, location (leg vs nonleg), serum lactate dehydrogenase level, and MUM-1 and Bcl-2 expression were recorded. Disease-specific survival was used as the main end point. Prognostic factors were identified using a Cox proportional hazards model.

RESULTS: Primary cutaneous diffuse large B-cell lymphoma, leg type is characterized by a predilection for the leg (72%), a high proportion of Bcl-2 expression (85%), an advanced age at onset (mean age, 76 years), and frequent relapses and extracutaneous dissemination. The overall 5-year disease-specific survival rate was 41%. Location on the leg and multiple skin lesions were predictive of death in multivariate analysis. Although no variable related to therapy was significantly associated with survival, patients recently treated with combinations of anthracycline-containing chemotherapies and rituximab had a more favorable short-term outcome.

CONCLUSIONS: Primary cutaneous diffuse large B-cell lymphoma, leg type is a distinct entity with a poor prognosis, particularly in patients with multiple tumors on the legs. Despite the advanced age of many patients, the prognosis could be improved with combinations of anthracycline-containing chemotherapies and rituximab.

Pathogenesis
Gross Appearance and Clinical Variants
Histopathological Features and Variants
Special Stains/ Immunohistochemistry/ Electron Microscopy
Prognosis
Treatment
Commonly Used Terms
Internet Links

HISTOPATHOLOGY	CHARACTERIZATION
DIFFUSE CUTANEOUS LARGE B-CELL LYMPHOMA, LEG TYPE
Primary cutaneous diffuse large B-cell lymphoma, leg type: clinicopathologic features and prognostic analysis in 60 cases. Grange F, Beylot-Barry M, Courville P, Maubec E, Bagot M, Vergier B, Souteyrand P, Machet L, Dalac S, Esteve E, Templier I, Delaporte E, Avril MF, Robert C, Dalle S, Laroche L, Delaunay M, Joly P, Wechsler J, Petrella T. Department of Dermatology, Hôpital Robert Debré, Avenue du Général Koenig, Reims 51100, France.	Arch Dermatol. 2007 Sep;143(9):1144-50. Abstract quote Objectives To describe clinicopathologic features and to identify prognostic factors in a large series of primary cutaneous diffuse large B-cell lymphoma, leg type (PCLBCL LT), as defined in the recent World Health Organization-European Organization for Research and Treatment of Cancer classification of cutaneous lymphomas. DESIGN: Retrospective multicenter study from the French Study Group on Cutaneous Lymphomas. SETTING: Nineteen departments of dermatology in 10 regions of France. Patients Sixty patients with a PCLBCL LT included in the registry of the French Study Group on Cutaneous Lymphomas. MAIN OUTCOME MEASURES: Age, sex, outcome, therapy, B symptoms, cutaneous extent, number of lesions, location (leg vs nonleg), serum lactate dehydrogenase level, and MUM-1 and Bcl-2 expression were recorded. Disease-specific survival was used as the main end point. Prognostic factors were identified using a Cox proportional hazards model. RESULTS: Primary cutaneous diffuse large B-cell lymphoma, leg type is characterized by a predilection for the leg (72%), a high proportion of Bcl-2 expression (85%), an advanced age at onset (mean age, 76 years), and frequent relapses and extracutaneous dissemination. The overall 5-year disease-specific survival rate was 41%. Location on the leg and multiple skin lesions were predictive of death in multivariate analysis. Although no variable related to therapy was significantly associated with survival, patients recently treated with combinations of anthracycline-containing chemotherapies and rituximab had a more favorable short-term outcome. CONCLUSIONS: Primary cutaneous diffuse large B-cell lymphoma, leg type is a distinct entity with a poor prognosis, particularly in patients with multiple tumors on the legs. Despite the advanced age of many patients, the prognosis could be improved with combinations of anthracycline-containing chemotherapies and rituximab.
Large B-cell lymphoma of the leg: clinical and pathologic characteristics in a North American series. Brogan BL, Zic JA, Kinney MC, Hu JY, Hamilton KS, Greer JP. Departments of Medicine, Vanderbilt University Medical School, Nashville, Tennessee 37232-5227, USA.	J Am Acad Dermatol. 2003 Aug;49(2):223-8. Abstract quote BACKGROUND: Large B-cell lymphoma (LBCL) of the leg is an uncommon subset of primary cutaneous B-cell lymphoma that has been described in a series of European patients. OBJECTIVE: Our purpose was to evaluate the clinical manifestation, diagnostic histopathology, immunophenotype, clinical course, and response to treatment of LBCL of the leg. METHODS: We conducted a retrospective case series of 3 patients with primary LBCL of the leg. RESULTS: The 3 elderly patients presented with progressive erythematous nodules on bilateral or unilateral lower extremities. All 3 patients had pre-existing peripheral edema or peripheral vascular disease. Histopathologic examination of the nodules showed dense lymphocytic infiltrates composed predominantly of large dysplastic lymphocytes that marked as B cells (CD20(+)). In 2 cases, the neoplastic cells were BCL-2 positive. All patients responded to initial therapy with localized electron beam radiation and chemotherapy but had disease progression. One patient had a complete and durable second response to anti-CD20 monoclonal antibody (rituximab). CONCLUSIONS: The patients described have similar clinical and histopathologic features to those previously described. There may be an association between LBCL and pre-existing lower-extremity vascular disease. Treatment of LBCL is difficult, but 1 patient responded well to systemic anti-CD20 monoclonal antibody.
Primary cutaneous large B-cell lymphomas. Wechsler J, Bagot M. Department of Pathology, Henri Mondor Hospital, Creteil, France.	Semin Cutan Med Surg 2000 Jun;19(2):130-2 Abstract quote According to the European Organization for Research and Treatment of Cancer classification, primary cutaneous large B-cell lymphomas are subdivided into 2 groups: follicle center cell large B-cell lymphomas and large B-cell lymphomas of the leg. The first type predominantly affects middle-aged adults with an equal gender distribution and presents with lesions on the head and trunk. Histology shows proliferations of predominantly large cleaved cells. The prognosis is excellent, with a 5-year survival rate >90%. The second group predominantly affects elderly females. Histologically the lesions are composed of centroblasts and/or immunoblasts (large round cells). The prognosis is more unfavorable with a 5-year survival rate of 50%. Intravascular large B-cell lymphoma is still classified as a provisional entity.
Primary cutaneous diffuse large B-cell lymphoma: a clinicopathologic study of 15 cases. Hembury TA, Lee B, Gascoyne RD, Macpherson N, Yang B, House N, Medeiros LJ, Hsi ED. Department of Clinical Pathology, Cleveland Clinic Foundation, OH 44195, USA.	Am J Clin Pathol 2002 Apr;117(4):574-80 Abstract quote Primary cutaneous diffuse large B-cell lymphoma (DLBCL) is an uncommon lymphoma. Some authors have suggested that large B-cell lymphoma can be segregated based on anatomic site, with tumors of the lower extremity being unique. We report 15 cases of primary cutaneous DLBCL. Each case was analyzed immunohistochemically using antibodies specific for CD3, CD5, CD10, CD20, bcl-2, bcl-6, and p53. Polymerase chain reaction analysis for t(14;18)(q32;q21) also was performed. There were 13 men and 2 women (median age, 64 years). Thirteen tumors were composed predominantly of centroblasts, and 2 were immunoblastic. There was a median follow-up of 72 months. Of the 4 patients with primary cutaneous DLBCL of the lower extremity (thigh, knee, leg), 2 (50%) experienced a recurrence and 1 patient died of disease. In the non-lower extremity cases, 18% (2/11) recurred and no patients died of disease. We conclude that primary cutaneous DLBCL usually occurs in elderly patients with a male predominance. Recurrences are common, but death of disease is rare.
SUBCUTANEOUS FAT
Primary cutaneous B-cell lymphoma, leg type restricted to the subcutaneous fat arising in a patient with dermatomyositis. Levy A, Randall MB, Henson T. From the Division of Dermatology, Department of Medicine, University of Tennessee Health Sciences Center, Memphis, TN.	Am J Dermatopathol. 2008 Dec;30(6):578-81. Abstract quote Until now, cutaneous lymphoma limited to the subcutaneous fat has been described as being derived only from T cells. Subcutaneous panniculitis-like T-cell lymphoma has been reported as a rare, postthymic neoplasm with various associations, including dermatomyositis. Furthermore, primary cutaneous B-cell lymphoma of the leg is defined by a diffuse dermal infiltrate of neoplastic B cells with extension to both the papillary dermis and the subcutaneous fat. We report a case of a patient with dermatomyositis who developed a cutaneous lymphoma of B-cell origin restricted to the subcutis, the first of its kind reported in the literature. This malignancy spared the dermis and epidermis, and we suggest that this is a unique variant of primary cutaneous B-cell lymphoma of the leg.