This cutaneous B cell lymphoma (CBCL) occurs mainly in elderly females. Many of these tumors present as erythematous nodules or tumor on one or both lower legs. These tumors are more aggressive than large B cell lymphomas of the head and neck with a 5 year survival rate of 50%. If the lesions are localized, radiotherapy may be effective. However, if multifocal skin lesions or extracutaneous lesions are present, multiagent chemotherapy should be used. Death usually occurs with disseminated disease.
Under the microscope, the tumor cells are monotonous collections of centroblasts and immunoblasts. Immunohistochemistry reveals positive staining for CD19, CD20, CD22, and CD79alpha and strong staining for bcl-2 protein. Surface and cytoplasmic Ig is also present. Clonal rearrangement of the immunoglobulin genes are present.
Pathogenesis Gross Appearance and Clinical Variants Histopathological Features and Variants Special Stains/
Prognosis Treatment Commonly Used Terms Internet Links
PATHOGENESIS CHARACTERIZATION GENERAL
Primary cutaneous large B-cell lymphoma of the leg: histogenetic analysis of a controversial clinicopathologic entity.
Paulli M, Viglio A, Vivenza D, Capello D, Rossi D, Riboni R, Lucioni M, Incardona P, Boveri E, Bellosta M, Orlandi E, Borroni G, Lazzarino M, Berti E, Alessi E, Magrini U, Gaidano G.
Department of Pathology, IRCCS Policlinico S.Matteo/University of Pavia, Italy.
Hum Pathol 2002 Sep;33(9):937-43 Abstract quote
This study analyzes the pathologic and molecular features of 5 cases of primary cutaneous large B-cell lymphoma of the leg (PCLBCL-leg), recently included in the European Organization for Research and Treatment of Cancer (EORTC) classification of primary cutaneous lymphoma. PCLBCL-leg accounts for 5% to 10% of all primary cutaneous B-cell lymphoma (PCBCL), usually affects elderly patients and carries a worse prognosis than other forms of PCBCL. It has been proposed that the malignant cells of PCLBCL-leg originate from germinal center (GC)-related cells, but their effective normal counterpart is unclear, and the rationale behind the inclusion of this lymphoma as a separate entity is based on its prognosis rather than on its proved histogenesis.
All of our cases of PCLBCL-leg morphologically resembled diffuse large B-cell lymphoma (DLBCL), but to better define their histogenesis, we also analyzed various phenotypic and genotypic markers, including mutations of the Ig and of BCL-6 genes, as well as expression of the bcl-6, MUM1, and CD138/syndecan-1 proteins. Immunohistochemically, all of our cases stained for the L-26/CD20cy and CD79a antigens and expressed the bcl-2, bcl-6, and MUM-1 proteins but were negative for both the CD10/CALLA and CD138 antigens.
With respect to molecular analysis, the lymphoma population of all PCLBCL-leg carried hypermutation of Ig genes, and all but 1 case also harbored mutations of the BCL-6 gene. Our results indicate that PCLBCL-leg are similar both under the morphofunctional and molecular profiles to most DLBCL of other sites. Thus, caution seems justified before definitely considering PCLBCL of the leg as a distinct entity.
- Genetic Aberrations in Primary Cutaneous Large B-Cell Lymphoma: A Fluorescence In Situ Hybridization Study of 25 Cases.
Wiesner T, Streubel B, Huber D, Kerl H, Chott A, Cerroni L.
From the *Department of Dermatology, Medical University Graz, Graz, Austria; and the daggerDepartment of Clinical Pathology, Vienna General Hospital, Medical University Vienna, Vienna, Austria.
Am J Surg Pathol. 2005 May;29(5):666-673. Abstract quote
In contrast to nodal large B-cell lymphomas, recurrent chromosomal aberrations have been studied only in a small number of cases of primary cutaneous diffuse large B-cell lymphoma (PCDLBCL). We investigated 25 PCDLBCLs (classified according to the WHO-EORTC classification into PCDLBCL, leg-type, 8; and PCDLBCL, other, 17), using an interphase fluorescence in situ hybridization technique.
All cases were analyzed for chromosomal aberrations commonly observed in nodal large B-cell lymphomas, including structural aberrations of the genes BCL2, BCL6, and c-MYC, and numerical aberrations of the chromosomes/genes 3, 7, 8, 11, 12, 13, 17, 18q, RB1, and p53. We observed genetic aberrations in 19 (76%) of 25 patients. The most frequent numerical aberrations were gains of chromosome 12 (7 of 25, 28%), 7 (5 of 25, 20%), 3 (5 of 25, 20%), 18q (3 of 25, 12%), 11 (3 of 25, 12%), X (3 of 25, 12%), and losses of chromosome/gene 17/p53 (3 of 25, 12%). BCL2, c-MYC, and BCL6 were rearranged with the IGH gene in 4 (16%), 1 (4%), and none (0%) of 25 cases, respectively. Most aberrations were homogeneously distributed among cases of PCDLBCL, leg-type and of PCDLBCL, other, cases located on the leg or at other body sites, cases with round and cleaved cell morphology, and Bcl-2+ and Bcl-2- cases.
These results suggest that PCDLBCLs show similar chromosomal aberrations irrespective of classification, anatomic site, cell morphology, and Bcl-2 expression, and that many similarities between primary cutaneous and nodal diffuse large B-cell lymphomas can be observed.
Primary and Secondary Cutaneous Diffuse Large B-Cell Lymphomas: A Multiparameter Analysis of 25 Cases Including Fluorescence In Situ Hybridization for t(14;18) Translocation.
Kim BK, Surti U, Pandya AG, Swerdlow SH.
Am J Surg Pathol 2003 Mar;27(3):356-64 Abstract quote
Although primary cutaneous diffuse large B-cell lymphomas (DLBCLs) except for those of the leg are grouped together with primary cutaneous follicle center cell lymphoma in the European Organization for Research and Treatment of Cancer classification of primary cutaneous lymphomas, they typically lack the usual phenotypic profile of follicular lymphoma. Whether they are truly of follicular center cell origin, have a molecular pathogenesis similar to nodal follicular lymphoma, or have any biologic features that distinguish them from secondary DLBCL involving skin remains uncertain.
To address these issues, a retrospective multiparameter study of 25 patients including clinical, histologic, immunophenotypic, and cytogenetic analyses was performed. A classic CD10+, bcl-6+ follicular center cell profile was found in 10 (40%) cutaneous DLBCL (2 of 11 primary, 5 of 8 secondary, 3 of 6 unclassified) with bcl-2 expression seen only in the nonprimary cases. Of the remaining cases, 14 cases (56%) were CD10-, bcl-6+, bcl-2+/- (9 primary) and one case (4%) was CD10-, bcl-6-, bcl-2+ (0 primary). Fluorescence in situ hybridization analysis showed a t(14;18) in 0 of 9 primary and 3 of 5 secondary cases.
Primary cases were frequently found in the head/neck region, whereas secondary cases were more common on the trunk and extremities. Patients with primary disease were all alive, usually having received only local therapy, at a median follow-up of 19 months. Most secondary cases were treated with chemotherapy with only one untreated patient dead of disease at a median follow-up of 5 months.
Primary cutaneous DLBCLs therefore appear to be distinctive as they have fewer features of follicular lymphoma than do secondary cases. Nevertheless, some appear to be of follicular center cell origin, even though they probably have a different molecular pathogenesis than most nodal follicular lymphomas.
CLINICAL/GROSS CHARACTERIZATION DISSEMINATED-GARLAND
- Unusual clinicopathological presentation of primary cutaneous diffuse large B-cell lymphoma, leg type, with multiple nodules and widespread garland-like lesions.
Department of Dermatology and Venereology, Medical Military Academy, Saint-Petersburg, Russia.
- Am J Dermatopathol. 2009 Jun;31(4):370-4. Abstract quote
We present a case of primary cutaneous diffuse large B-cell lymphoma, leg type, with an unusual clinical picture. A 41-year-old man presented with a 2-year history of slowly progressive plaques, nodules, and garland-like patches on his chest, right upper arm, and back. Complete staging investigations revealed no extracutaneous involvement.
Histological examination of a nodule revealed a diffuse nonepidermotropic infiltrate mainly composed of large blast cells with features of immunoblasts and centroblasts and spindle cells seen at the periphery of the infiltrate. Histological examination of a garland-like lesion showed perivascular infiltrates composed predominantly of small lymphocytes admixed with only occasional large blasts. The blasts from the nodule and garland-like lesions and spindle cells identified in the nodule exhibited an identical phenotype: they stained positively for CD20, CD79a, and bcl-2 and tested negative for bcl-6, CD5, CD10, and TdT. CD35 revealed no networks of follicular dendritic cells.
Widespread garland-like lesions are not a typical feature of primary cutaneous diffuse large B-cell lymphoma.
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.
- Primary cutaneous B-cell lymphoma, leg type restricted to the subcutaneous fat arising in a patient with dermatomyositis.
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.
CHARACTERIZATION SPECIAL STAINS IMMUNOPEROXIDASE
Primary cutaneous B-cell lymphoma: a clinical, histological, phenotypic and genotypic study of 21 cases.
Gronbaek K, Moller PH, Nedergaard T, Thomsen K, Baadsgaard O, Hou-Jensen K, Zeuthen J, Guldberg P, Ralfkiaer E.
Departments of Pathology, Herlev University Hospital, 75 Herlev Ringvej, DK-2730 Herlev, Denmark.
Br J Dermatol 2000 May;142(5):913-23 Abstract quote
The clinical, histological, phenotypic and genotypic features of 21 primary cutaneous B-cell lymphomas (CBCLs) have been investigated. The patients were 13 men and eight women aged 34-91 years (median 67) at diagnosis. Eighteen patients had localized disease, and three had multiple skin lesions at diagnosis. Twelve patients developed cutaneous or extracutaneous recurrences, and five died from malignant lymphoma 7-84 months (median 36) after diagnosis.
Histological examination showed features of marginal zone/mucosa-associated lymphoid tissue (MALT)-type lymphoma in 12 cases. Three of these had transformed to diffuse large B-cell lymphoma (DLBCL) in relapse biopsies. The remaining cases were seven primary DLBCLs and two cases tentatively classified as follicle centre cell (FCC) lymphoma. The neoplastic B cells showed similar phenotypes and genotypes in most cases (CD20+, CD79+, CD5-, CD10-, cyclin D1-, bcl-2+, bcl-x-, bax-, t(14;18)-negative). p53 protein was expressed in five cases, and four harboured mis-sense or loss-of-function mutations in the p53 gene.
Deletion or promoter region hypermethylation of the p16INK4a gene was detected in two patients with DLBCL. The level of retinoblastoma protein expression and the proliferative fraction were significantly higher in DLBCL (> 50%) than in MALT- or FCC-type lymphomas (< 10%). Features associated with an unfavourable prognosis were the presence of multiple skin lesions at diagnosis, transformation from MALT-type lymphoma to DLBCL, and possibly p16INK4a aberrations.
It is concluded that most CBCLs are dissimilar from FCC lymphomas and seem to be more closely related to marginal zone/MALT-type lymphomas. It is also suggested that there are fundamental differences between DLBCL and other histological categories of CBCL, indicating that cutaneous DLBCL is a separate entity with an increased growth potential and genetic features similar to DLBCL originating in other anatomical sites.
bcl-2 protein expression in primary cutaneous large B-cell lymphoma is site-related.
Geelen FA, Vermeer MH, Meijer CJ, Van der Putte SC, Kerkhof E, Kluin PM, Willemze R.
Department of Dermatology, Free University Hospital, Amsterdam, The Netherlands.
J Clin Oncol 1998 Jun;16(6):2080-5 Abstract quote
PURPOSE: Primary cutaneous large B-cell lymphoma (PCLBCL) that presents on the leg has recently been recognized as a distinct disease entity. These lymphomas have a reduced disease-free survival and a worse prognosis as compared with the more common, morphologically similar PCLBCL that present on the head or trunk. Studies in noncutaneous diffuse large B-cell lymphomas suggest a relationship between the expression of bcl-2 protein and clinical behavior. In the present study, we investigated whether these two groups of PCLBCL differ in the expression of bcl-2 protein and the presence of t(4;18), known as one of the causes of bcl-2 overexpression.
PATIENTS AND METHODS: Paraffin sections from pretreatment biopsies of 14 PCLBCLs of the head or trunk and nine PCLBCLs of the legs were investigated for expression of bcl-2 protein using immunohistochemistry, and for the presence of the 14;18 translocation using polymerase chain reaction (PCR) amplification with primers against both the major breakpoint region (mbr) and the minor cluster region (mcr) of bcl-2. For reasons of comparison, nine secondary cutaneous large B-cell lymphomas (SCLBCLs) were also studied.
RESULTS: Expression of bcl-2 protein was found in all nine PCLBCLs of the leg and in all nine SCLBCLs, but not in any of the 14 PCLBCLs on the head and trunk. The t(14;18) was only detected in two of seven SCLBCLs, but not in the five PCLBCLs of the leg or the eight PCLBCLs on the head or trunk studied.
CONCLUSION: The striking differences in bcl-2 expression between PCLBCL of the head or trunk and PCLBCL on the leg suggest that bcl-2 expression is site-related and may contribute to the different clinical behavior between these two groups of lymphomas. In addition, they underscore that PCLBCL on the head and trunk and PCLBCL on the leg are distinct disease entities, as recently recognized in the European Organization for Research and Treatment of Cancer (EORTC) classification for primary cutaneous lymphomas.
- Expression of the bcl-6 and MUM1/IRF4 proteins correlate with overall and disease-specific survival in patients with primary cutaneous large B-cell lymphoma: a tissue microarray study.
Sundram U, Kim Y, Mraz-Gernhard S, Hoppe R, Natkunam Y, Kohler S.
Department of Pathology, Stanford University School of Medicine, Stanford, CA, USA.
J Cutan Pathol. 2005 Mar;32(3):227-34. Abstract quote
Background: Systemic B-cell lymphomas have been studied using microarrays, which has led to a better understanding of their molecular characteristics. Initial microarray studies of these lymphomas have implicated several genes as important predictors of outcome. In this study, we used a tissue microarray (TMA) to characterize primary cutaneous large B-cell lymphomas (PCLBCL).
Methods: We studied 14 patients for whom clinical follow up was available, including four patients whose lesions were limited to the leg on presentation. Immunohistochemical staining with CD20, CD44, CD21, CD5, CD10, bcl-2, bcl-6, Ki67, p53, and multiple myeloma 1 (MUM1) was examined.
Results: Our results identify two subgroups of lymphomas. The first group showed staining with bcl-6 and had an overall survival of 176 months (p = 0.003). The majority of this group was negative for MUM1. The second group lacked staining with bcl-6 and had an overall survival of 26 months, with a majority of these cases staining with MUM1. Three of four patients with PCLBCL of the leg showed no staining with bcl-6.
Conclusions: Our study demonstrates the utility of TMAs in the analysis of PCLBCL and that expression of bcl-6 and MUM1 correlates with survival.
PROGNOSIS AND TREATMENT CHARACTERIZATION PROGNOSIS
Primary cutaneous diffuse large B-cell lymphoma: prognostic significance of clinicopathological subtypes.
Goodlad JR, Krajewski AS, Batstone PJ, McKay P, White JM, Benton EC, Kavanagh GM, Lucraft HH; Scotland and Newcastle Lymphoma Group.
Department of Pathology, Raigmore Hospital, Inverness, Scotland, United Kingdom.
Am J Surg Pathol. 2003 Dec;27(12):1538-45. Abstract quote
Classification and subdivision of primary cutaneous diffuse large B-cell lymphoma (PCDLBCL) are a matter of ongoing debate. In this study we assessed the morphologic, immunophenotypic, and clinical features of 30 cases of PCDLBCL identified during a review of all primary cutaneous B-cell lymphomas in the Scotland and Newcastle Lymphoma Group database.
We also determined the number of cases harboring t(14;18) using a polymerase chain reaction and primers to the major breakpoint cluster region. The effect on prognosis of a variety of clinical and pathologic factors was assessed for the group of 30 PCDLBCL and the 5-year disease-specific survival (DSS) of this cohort compared with that of 195 cases of stage I diffuse large B-cell lymphoma arising primarily in lymph nodes, also identified from within the Scotland and Newcastle Lymphoma Group database.
Location on the leg was the only independent prognostic factor for determining outcome in PCDLBCL (67% 5-year DSS compared with 100% for the upper body; P = 0.0047). The presence of multiple lesions, involvement of more than one body site, and expression or not of CD10, bcl-2, bcl-6, and CD10 and bcl-6, had no effect on survival. Compared with cases arising above the waist, those on the leg were more often female, were of an older age, and had a significantly higher incidence of bcl-2 expression (P = 0.002) as well as the aforementioned poorer prognosis. They also showed more frequent co-expression of CD10 and bcl-6, supporting a follicle center cell origin for some, but this difference was not statistically significant.
Although there was no significant difference in the 5-year DSS between the group of PCDLBCL and the cases of stage I nodal diffuse large B-cell lymphoma (88% 5-year DSS vs. 78%; P = 0.06), the latter were generally treated with more aggressive therapy. Moreover, a significant difference in 5-year DSS was seen when the nodal DLBCLs were compared with PCDLBCLs arising above the waist (78% vs. 100% respectively; P = 0.0135). These results support the current EORTC approach of subdividing PCLBCL on the basis of site to produce prognostically relevant groupings.
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.
Vermeer MH, Geelen FA, van Haselen CW, van Voorst Vader PC, Geerts ML, van Vloten WA, Willemze R.
Department of Dermatology, Free University Hospital, Amsterdam, The Netherlands.
Arch Dermatol 1996 Nov;132(11):1304-8 Abstract quote
BACKGROUND AND DESIGN: Primary cutaneous follicular center cell lymphomas represent a distinct type of cutaneous B-cell lymphoma, clinically characterized by localized skin lesions on the head or trunk and an excellent prognosis. Histologically similar lymphomas may occur on the legs. The clinical behavior of this group is still undefined, and controversy exists whether these lymphomas should be classified as follicular center cell lymphoma or B-immunoblastic lymphoma. We reviewed the clinical, histologic, and follow-up data of 18 patients with primary cutaneous large B-cell lymphoma of the legs.
RESULTS: Primary cutaneous large B-cell lymphoma of the legs generally occurred in elderly patients (median age at diagnosis, 76 years), in particular women (male-female ratio, 7:2), and preferentially affected the lower legs (14 of 18 patients). Radiotherapy and/or systemic polychemotherapy resulted in complete remissions in 16 of 17 patients. Follow-up data demonstrated estimated 2- and 5-year survival rates of 77% and 58%, respectively. Histologic evaluation showed diffuse dermal infiltrates with variable proportions of centroblasts (large noncleaved cells), large centrocytes (large cleaved cells), and B immunoblasts. Seventeen of 18 patients were diagnosed as having primary cutaneous follicular center cell lymphoma; only 1 patient, whose histologic examination showed more than 30% immunoblasts, was diagnosed as having B-immunoblastic lymphoma.
CONCLUSIONS: Primary cutaneous large B-cell lymphoma of the legs is a distinct clinicopathologic entity that mainly affects elderly patients and has an intermediate prognosis. Although most cases have a follicular center cell origin, primary cutaneous large B-cell lymphoma is proposed as the most appropriate term for this type of cutaneous lymphoma.
Primary cutaneous B-cell lymphomas of the lower limbs: a study of integrin expression in 11 cases.
Lair G, Parant E, Tessier MH, Jumbou O, Dreno B.
Clinique Dermatologique, CHU de Nantes, France.
Acta Derm Venereol 2000 Sep-Oct;80(5):367-9 Abstract quote
Primary cutaneous B-cell lymphoma is a rare disease. Among the cutaneous B lymphomas, B-cell lymphomas of the lower limbs appear as a special subgroup with a prognosis that is possibly worse than that of primary cutaneous B-cell lymphomas located on the trunk, arms or head, with more frequent relapses. In addition, some recent studies indicate that the level of expression of integrins on tumour cells could be related to the clinical course of the disease.
This study reports on 14 cases of primary cutaneous B-cell lymphomas of the lower limbs and their clinical course. A study of integrin expression by tumour cells was performed in 11 of these cases. With a mean follow-up of 31 months, the study confirmed the worse prognosis of lymphomas with a predominance of centroblasts and immunoblasts (3 deaths) compared with lymphomas with a predominance of centrocytes, as well as their higher rate of recurrence (7/11).
A correlation was confirmed between the course of the disease and the level of expression of lymphocyte function-associated antigen-1, intercellular adhesion molecule-1 and very late antigen-4 by tumour cells.
Prognostic factors in primary cutaneous large B-cell lymphomas: a European multicenter study.
Grange F, Bekkenk MW, Wechsler J, Meijer CJ, Cerroni L, Bernengo M, Bosq J, Hedelin G, Fink Puches R, van Vloten WA, Joly P, Bagot M, Willemze R.
Department of Dermatology, Hopital Pasteur, Colmar, France.
J Clin Oncol 2001 Aug 15;19(16):3602-10 Abstract quote
PURPOSE: Most primary cutaneous B-cell lymphomas have an excellent prognosis. However, primary cutaneous large B-cell lymphomas (PCLBCLs) of the leg have been recognized as a distinct entity with a poorer prognosis in the European Organization for Research and Treatment of Cancer (EORTC) classification. This distinction on the basis of site has been debated. Our aim was to identify independent prognostic factors in a large European multicenter series of PCLBCL.
PATIENTS AND METHODS: The clinical and histologic data of 145 patients with PCLBCL were evaluated. According to the EORTC classification, 48 patients had a PCLBCL of the leg and 97 had a primary cutaneous follicle center-cell lymphoma (PCFCCL). Data from both groups were compared. Univariate and multivariate analyses of specific survival were performed using a Cox proportional hazards model.
RESULTS: Compared with PCFCCL, PCLBCL-leg were characterized by an older age of onset, a more recent history of skin lesions, a more frequent predominance of tumor cells with round nuclei and positive bcl-2 staining, and a poorer 5-year disease-specific survival rate (52% v 94%; P <.0001). Univariate survival analysis in the entire study group showed that older age, a more recent onset of skin lesions, the location on the leg, multiple skin lesions, and the round-cell morphology were significantly related to death. In multivariate analysis, the round-cell morphology (P <.0001), the location on the leg (P =.002), and multiple skin lesions (P =.01) remained independent prognostic factors. The round-cell morphology was an adverse prognostic factor both in PCLBCL-leg and in PCFCCL, whereas multiple skin lesions were associated with a poor prognosis only in patients with PCLBCL-leg.
CONCLUSION: With site, morphology, and number of tumors taken into account, guidelines for the management of PCLBCL are presented.
Secondary lymph node involvement from primary cutaneous large B-cell lymphoma of the leg: sentinel lymph nodectomy as a new strategy for staging circumscribed cutaneous lymphomas.
Starz H, Balda BR, Bachter D, Buchels H, Vogt H.
Department of Dermatology and Allergology, Zentralkinikum, Augsburg, Germany.
Cancer 1999 Jan 1;85(1):199-207 Abstract quote
BACKGROUND: Primary cutaneous large B-cell lymphoma of the leg (LBCLL) is a recently defined type of non-Hodgkin's lymphoma. It forms a separate category in the new classification of primary cutaneous lymphomas elaborated by the European Organization for Research and Treatment of Cancer. It is associated with a less favorable prognosis than the most frequently occurring types of primary cutaneous B-cell lymphoma.
METHODS: The authors present four patients with the typical clinicopathologic constellation of LBCLL. Three of them died during the years 1993-1996. The authors reviewed their courses. The fourth patient was staged by sentinel lymph nodectomy (SLNE), i.e., the selective surgical removal and histologic examination of the first draining lymph node associated with the cutaneous tumor.
RESULTS: The courses of the three previous patients were characterized by secondary involvement of regional lymph nodes followed by systemic dissemination of the lymphoma in a third step. Although the conventional staging of the fourth patient had been negative for any extracutaneous lymphoma manifestation, the SLNE revealed initial regional lymph node involvement, which had decisive implications for the choice of therapy.
CONCLUSIONS: SLNE may gain a prominent role in the staging of circumscribed cutaneous lymphomas, in addition to its already established position in melanoma management. Further positive effects of SLNE are 1) better distinction of primary cutaneous lymphomas with secondary lymph node involvement from primary lymph node lymphomas with skin manifestation, and 2) better insight into the biology of different primary cutaneous lymphoma types.
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Robbins Pathologic Basis of Disease. Seventh Edition. WB Saunders 2005.
DeMay RM. The Art and Science of Cytopathology. Volume 1 and 2. ASCP Press. 1996.
Weedon D. Weedon's Skin Pathology Second Edition. Churchill Livingstone. 2002
Fitzpatrick's Dermatology in General Medicine. 6th Edition. McGraw-Hill. 2003.
Weiss SW and Goldblum JR. Enzinger and Weiss's Soft Tissue Tumors. Fifth Edition. Mosby Elesevier 2008
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Last Updated June 15, 2009
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