Improvement of Survival in Patients With Primary Cutaneous Diffuse Large B-Cell Lymphoma, Leg Type, in France

Grange, Florent; Joly, P.; Barbe, Coralie; Bagot, Martine; Dalle, S.; Ingen‐Housz‐Oro, Saskia; Maubec, Ève; D’Incan, M.; Ram‐Wolff, Caroline; Dalac, S.; Templier, I.; Estève, É.; Quéreux, G.; Machet, L.; Leduc, Marion; Dereure, O.; Laroche, Liliane; Saïag, Philippe; Vergier, Béatrice; Beylot‐Barry, M.

doi:10.1001/jamadermatol.2013.7452

Cited by 87 publications

(81 citation statements)

References 24 publications

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“…Unlike the indolent cutaneous MZL and follicle center lymphomas, primary cutaneous DLBCL, leg-type (PCDLBCL-LT) is aggressive, although rituximab has significantly improved survival outcomes [114, 115]. It classically, though not exclusively, presents as rapidly growing tumors on the legs of women over age 60 [1].…”

Section: Prognosis Of Extranodal Dlbcl Arising From Specific Anatomicmentioning

confidence: 99%

Extranodal Diffuse Large B Cell Lymphoma: Molecular Features, Prognosis, and Risk of Central Nervous System Recurrence

Ollila

Olszewski

2018

Curr. Treat. Options in Oncol.

115

107

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Diffuse large B cell lymphoma (DLBCL) arises from extranodal organs in about 30% of cases. Its prognosis and risk of recurrence in the central nervous system (CNS) vary according to the primary site of origin. Recent studies begin to clarify these differences using molecular classification. Testicular, breast, and uterine DLBCL (as well as possibly primary cutaneous DLBCL, leg-type) share a high prevalence of the non-germinal center B cell (non-GCB) phenotype and the MYD88/CD79B-mutated (MCD) genotype. These biologic features, which resemble primary CNS lymphoma, may underlie their stage-independent propensity for CNS involvement. Management of these lymphomas should involve CNS prophylaxis, preferably using systemic high-dose methotrexate to prevent intraparenchymal recurrence. Involvement of the kidneys, adrenal glands, ovary, bone marrow, lung, or pleura usually indicates disseminated disease, conferring worse prognosis. Involvement of these sites is often associated with high CNS-International Prognostic Index (IPI), concurrent MYC and BCL2 or BCL6 rearrangements, or intravascular lymphoma-risk factors warranting CNS prophylaxis. In contrast, craniofacial, thyroid, localized bone, or gastric lymphomas have a variable prevalence of the non-GCB phenotype and lack MYD88 mutations. Their outcomes with standard immunochemotherapy are excellent, and the risk of CNS recurrence is low. We recommend individualized consideration of CNS prophylaxis based on the CNS-IPI score and anatomical proximity in cases of epidural, orbital, or skull involvement. Rituximab-containing immunochemotherapy is a standard approach for all extranodal DLBCLs. Surgery is no longer required for any primary site, but routine consolidative radiation therapy is recommended for testicular lymphoma. Radiation therapy also appears to be associated with better progression-free survival in primary bone DLBCL. Future studies should better distinguish primary from secondary sites of extranodal involvement, and investigate the association of newly identified genotypes with the risk of CNS or systemic recurrence.

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Section: Prognosis Of Extranodal Dlbcl Arising From Specific Anatomicmentioning

confidence: 99%

Extranodal Diffuse Large B Cell Lymphoma: Molecular Features, Prognosis, and Risk of Central Nervous System Recurrence

Ollila

Olszewski

2018

Curr. Treat. Options in Oncol.

115

107

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“…Localisation on the leg was identified as an independent factor associated with a poor prognosis . In contrast, PCLBCLs‐LT have an aggressive course, requiring a combination of rituximab and polychemotherapy as first‐line treatment …”

Section: Introductionmentioning

confidence: 99%

“…3 In contrast, PCLBCLs-LT have an aggressive course, requiring a combination of rituximab and polychemotherapy as first-line treatment. 7 The information used to make the diagnosis includes morphology, phenotype, and, when available, some molecular features. According to the WHO classification, the tumour cytology and growth pattern may discriminate PCFCLs-LC (classified in the group of PCFCLs whatever the number of large cells) and PCLBCLs-LT. Immunolabelling for bcl-2, MUM1, FOXP1, CD10, bcl-6, CD21 (follicular dendritic cell meshwork) and Ki67 may help to differentiate PCFCLs from PCLBCLs-LT. 8 IgM expression also favours PCLBCL-LT. 9,10 Robson et al suggested that p63 expression is specific to PCLBCL-LT. 11 Our group showed that the MYD88 L265P mutation is a valuable criterion for PCLBCL-LT diagnosis, as it is absent in PCFCLs-LC.…”

Section: Introductionmentioning

confidence: 99%

Primary cutaneous large B‐cell lymphomas: relevance of the 2017 World Health Organization classification: clinicopathological and molecular analyses of 64 cases

et al. 2019

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Aims We applied the 2017 World Health Organization (WHO) classification criteria to categorise a series of 64 primary cutaneous large B‐cell lymphomas (PCLBCLs), containing a majority (≥80%) of large cells and a proliferative rate of ≥40%, raising the problem of the differential diagnosis between PCLBCL, leg type (PCLBCL‐LT) and primary cutaneous follicle centre lymphoma, large cell (PCFCL‐LC). The aims were to determine the reproducibility and prognostic relevance of the 2017 WHO criteria. Methods and results Morphology and phenotype identified 32 PCLBCLs‐LT and 25 PCFCLs‐LC; seven cases (11%) remained unclassified. Morphology was less reproducible than immunophenotype. Pertinent markers for the differential diagnosis were MUM1, FOXP1, CD10, and IgM. bcl‐2 and bcl‐6 were expressed by both PCFCLs‐LC and PCLBCLs‐LT at substantial levels. Neither Ki67 expression nor p63 expression was of diagnostic value. MYD88 was found to be mutated only in PCLBCLs‐LT (n = 22, 69%). According to Hans/Hans modified algorithms, 23 of 25 PCFCLs‐LC had germinal centre (GC) status, and the 32 PCLBCLs‐LT had non‐GC status. Overall survival was poorer for PCLBCLs‐LT than PCFCLs‐LC (P = 0.0002). Non‐GC cases had poorer overall survival than GC cases (P = 0.0007). In PCLBCLs‐LT, MYC expression was associated with cutaneous relapses (P = 0.014). When GC/non‐GC status was applied to unclassified cases, only a single case remained discordant. Conclusions Our results support the 2017 WHO classification criteria for PCLBCL diagnosis. The Hans modified algorithm using CD10 and MUM1 distinguished PCFCLs‐LC from PCLBCLs‐LT with optimal diagnostic value without requiring bcl‐6 immunolabelling (poorly reproducible). Rare unclassified cases may constitute a provisionally heterogeneous subgroup for which GC/non‐GC status (relevant for prognosis) may guide therapeutic decisions.

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“…Grange ve ark ise primer kutanöz diffüz geniş B-hücreli lenfomalı hastalarda rituksimab tedavisiyle hayatta kalım oranlarını değerlendirdikleri 115 hastalı bir çalışma planlamışlardır. Rituksimab ve polikemoterapiyi birlikte alan hastalarda 3 ve 5 yıllık hayatta kalım oranlarını %80 ve %74 oranında saptarken, daha az yoğun tedavi alanlarda %48-%38 oranında hayatta kalım tespit edilmiştir (24). Valencak ve ark 16 kutanöz B hücreli lenfoma hastasında rituksimabı monoterapi olarak kullandığında 14 hastada tam remisyon gözlemiştir (48).…”

Section: A-primer Kutanöz B Hücreli Lenfoma Ve Diğer Lenfomalarunclassified

Dermatolojide Rituksimab Kullanımı

2014

Dermatoz

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Improvement of Survival in Patients With Primary Cutaneous Diffuse Large B-Cell Lymphoma, Leg Type, in France

Cited by 87 publications

References 24 publications

Extranodal Diffuse Large B Cell Lymphoma: Molecular Features, Prognosis, and Risk of Central Nervous System Recurrence

Extranodal Diffuse Large B Cell Lymphoma: Molecular Features, Prognosis, and Risk of Central Nervous System Recurrence

Primary cutaneous large B‐cell lymphomas: relevance of the 2017 World Health Organization classification: clinicopathological and molecular analyses of 64 cases

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