Primary cutaneous diffuse large B‐cell lymphoma, NOS and leg type: Clinical, morphologic and prognostic differences

Felcht, Moritz; Klemke, Claus‐Detlev; Nicolay, Jan P.; Weiß, Christel; Assaf, Chalid; Wobser, Marion; Schlaak, Max; Hillen, Uwe; Moritz, Rose K. C.; Tantcheva‐Poór, Iliana; Nashan, Dorothée; Beyer, Marc; Dippel, Edgar; Müller, Cornelia; Sachse, Michael Max; Meiß, Frank; Géraud, Cyrill; Marx, Alexander; Goerdt, Sergij; Geissinger, Eva; Kempf, Werner

doi:10.1111/ddg.13773

Cited by 13 publications

(20 citation statements)

References 36 publications

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“…Our case of DLBCL differed in several aspects: neck location overlying cervical lymph nodes (as opposed to the characteristic lower‐extremity distribution of PC‐DLBCL‐LT), germinal center B‐cell subtype (CD10+, BCL6+, and MUM1−), and evidence of significant extracutaneous disease at the time of diagnosis (including bone marrow involvement). Important clues to the diagnosis of secondary cutaneous involvement by systemic lymphoma include a diagnostic category for which there is no primary cutaneous equivalent (eg, mantle cell lymphoma, GZL), identification of germinal center B‐cell subtype in a DLBCL (particularly, presence of CD10 in association with lack of MUM1), and staging results 12,13 . Two other aggressive B‐cell lymphomas with primary cutaneous presentation include EBV‐positive DLBCL and CD5‐positive DLBCLs.…”

Section: Discussionmentioning

confidence: 99%

Secondary cutaneous involvement by direct extension in high‐grade B‐cell lymphomas

Subtil

Gru

2020

J Cutan Pathol

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Cutaneous involvement by a systemic lymphoma via direct extension is a very rare phenomenon, historically described in patients who had advanced-stage disease with bulky lymphadenopathy. Since its original description, most cases of cutaneous lymphomatous spread via direct extension are attributable to Hodgkin disease. Here, we report the occurrence of direct cutaneous dissemination in the setting of nodal highgrade B-cell lymphomas, specifically diffuse large B-cell lymphoma (DLBCL) and B-cell lymphoma, unclassifiable, with features intermediate between DLBCL and classical Hodgkin lymphoma (also called gray-zone lymphoma). Both cases are adequately documented with clinical, histopathologic, and immunophenotypic data, as well as additional cytogenetic analysis.

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Section: Discussionmentioning

confidence: 99%

Secondary cutaneous involvement by direct extension in high‐grade B‐cell lymphomas

Subtil

Gru

2020

J Cutan Pathol

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“…Our patient belonged to the typical age group. Negative prognostic markers are female gender, Bcl-2 expression, and MYC rearrangements [2], [3], [4]. We did not perform an MYC rearrangement analysis.…”

Section: Discussionmentioning

confidence: 99%

“…Negative prognostic markers are Bcl-2, MYC rearrangements, and female gender, while proliferation rate (Ki67) is not of prognostic significance. Overall survival (OS) is poorer for PCLBCL-LT than PCFCL-LC or PCLBCL/NOS [2], [3], [4].…”

Section: Introductionmentioning

confidence: 99%

Large B - Cell Lymphoma of the Leg – Unfavourable Course with Rituximab/Bendamustin

Wollina¹,

Schmidt²,

Schönlebe³

et al. 2019

Open Access Maced J Med Sci

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BACKGROUND: Cutaneous B-cell lymphomas represent about 25% of all cutaneous lymphomas. Peripheral diffuse large B-cell lymphoma of the leg type is the most aggressive subtype seen mainly in elderly patients. Treatment is not standardised. CASE REPORT: An 87-year-old female patient was presented in May 2018 because of the development of painless subcutaneous nodules on the legs since late 2017. On examination, we observed up to 5 cm large erythematous nodules on the legs and a smaller plaque in the left submammary fold. The histology of a skin demonstrated tumour infiltrate that was separated from the overlying epidermis by a grenz zone. It consisted of densely packed, blastoid lymphocytic cells with numerous, and some atypical mitoses. The cells were positive for CD20, CD79A and CD5. Almost 100% of the cells were labelled with Ki67. The diagnosis of a diffuse large B-cell lymphoma (PCLBCL-LT) of the leg was confirmed. Histologic analysis of a bone marrow biopsy demonstrated a hypercellular bone marrow without malignant lymphatic infiltrates. Diagnostic ultrasound of cervical nodes and computerised tomography (CT) scans (native and with contrast medium) of head, neck and trunk excluded an extracutaneous manifestation of the PCLBCL-LT. Treatment with rituximab plus bendamustibe was initiated, but tumour progress was noted after the second course. Suggested palliative therapy with radiation and rituximab was refused. The patient died 7 months after diagnosis. CONCLUSIONS: Although some trials suggested a beneficial effect of immuno-chemotherapy, the prognosis of (PCLBCL-LT) remains poor. Standardised treatment is missing due to the relative rarity of this malignancy.

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“…Mit einer jährlichen Inzidenz von etwa 1 : 100 000 sind PCL relativ selten, stellen aber nach den primären gastrointestinalen Lymphomen die zweithäufigste Form der extranodalen NHL dar [2]. Das kutane T‐Zell‐Lymphom (CTCL) mit seinen verschiedenen Entitäten ist mit fast drei Vierteln aller PCL die größte Gruppe, während kutane B‐Zell‐Lymphome (CBCL) weniger häufig vorkommen [3–7].…”

Section: Einführungunclassified

Stellenwert diagnostischer Verfahren und Risiko von Zweittumoren bei primär kutanen Lymphomen

Scheu

Schnabl

Steiner

et al. 2021

J Deutsche Derma Gesell

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ZusammenfassungHintergrundPrimär kutane Lymphome (PCL) unterscheiden sich oft stark im klinischen Verhalten und in der Prognose von systemischen Lymphomen des gleichen histopathologischen Typs. Ziel der Studie war es, die Verteilung der PCL‐Subtypen, die Zeitspanne von der Krankheitsmanifestation bis zur Diagnosestellung, den Stellenwert diagnostischer Verfahren, das Auftreten von Zweittumoren und die verschiedenen Behandlungsmodalitäten im Rahmen des Krankheitsverlaufs zu untersuchen.Patienten und MethodikRetrospektive Analyse von 152 Patienten mit PCL, die von 2010–2012 an der Universitäts‐Hautklinik Tübingen behandelt wurden.Ergebnisse105 Patienten mit primär kutanem T‐Zell‐Lymphom (CTCL) (69,1 %) und 47 Patienten mit primär kutanem B‐Zell‐Lymphom (CBCL) (30,9 %) wurden eingeschlossen. Die Zeitspanne von der Krankheitsmanifestation bis zur Diagnose betrug durchschnittlich vier Jahre. Mycosis fungoides (MF) (47,4 %) wurde am häufigsten diagnostiziert. Die First‐Line‐Therapien umfassten hier entweder eine alleinige Phototherapie (PUVA, n = 48; UVB 311 nm, n = 7) oder Kombinationstherapien (PUVA mit systemischen Retinoiden, n = 18). Häufigste Second‐Line‐Therapie war Interferon (INF)‐α plus PUVA (n = 15). Der Behandlungsverlauf war insgesamt günstig (45,2 % Remission, 28,6 % stabile Erkrankung, 22,6 % Progress). Maligne Komorbiditäten wurden im Vergleich zu einer gesunden Vergleichsgruppe häufiger beobachtet.SchlussfolgerungenBis zur Diagnosestellung der PCL dauert es oft mehrere Jahre. Der Wert der Staging‐Verfahren ist gering. Die Behandlungsmodalitäten in früheren MF‐Stadien basieren hauptsächlich auf der Phototherapie.

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Primary cutaneous diffuse large B‐cell lymphoma, NOS and leg type: Clinical, morphologic and prognostic differences

Cited by 13 publications

References 36 publications

Secondary cutaneous involvement by direct extension in high‐grade B‐cell lymphomas

Secondary cutaneous involvement by direct extension in high‐grade B‐cell lymphomas

Large B - Cell Lymphoma of the Leg – Unfavourable Course with Rituximab/Bendamustin

Stellenwert diagnostischer Verfahren und Risiko von Zweittumoren bei primär kutanen Lymphomen

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