Primary cutaneous follicular center lymphoma C82.6

Author: Prof. Dr. med. Peter Altmeyer

Co-Autor: Jeton Luzha

All authors of this article

Last updated on: 29.10.2020

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Synonym(s)

Brill-Symmers disease; Brill-Symmers M.; Centroblastic-centrocytic lymphoma; Crosti-Lymphoma; Cutaneous follicle center lymphoma; Cutaneous germinal center lymphoma; Follicular B-cell lymphoma of the skin; Follicular center lymphoma; follicular lymphoma; Follicular lymphoma; Germ-centered lymphoma of the skin; Germination center lymphoma; Large follicular lymphadenopathy; Large follicular lymphoblastoma; Lymphadenopathy large follicular; Lymphoblastoma large follicular; Lymphoma centroblastic-centrocytic; Lymphoma follicular; Malignant lymphoma with small cleaved and large non cleaved follicle center cells; M. Brill-Symmers; Primary cutaneous follicular B-cell lymphoma; rosaceous follicular lymphoma

Definition
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Most common primary cutaneous B-cell lymphoma (indolent, low malignant character), originating from neoplastic follicular centres. The lymphoma generally consists of a mixture of centrocytes (small and medium-sized cells with a notched nucleus) and centroblasts (large cells with large bright, non-notched nuclei and prominent corpuscles), which may have a follicular, mixed follicular/diffuse or diffuse growth pattern.

Occurrence/Epidemiology
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In absolute terms, primary cutaneous follicular B-cell lymphomas (PCBCL) are rare, although they form by far the largest group (40-70% of this group) among primary cutaneous B-cell lymphomas (PCBCL).

The incidence of the total number of primary cutaneous and non-cutaneous follicular B-cell lymphomas is reported to be 4/100,000 per year.

The proportion of primary cutaneous B-cell lymphomas in larger B-cell lymphoma collectives is about 1% (Kim et al. 2015).

Etiopathogenesis
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The pathogenesis is unknown. In diffuse large-cell follicular lymphomas, activating MYD88 mutations are found in 20-60%. Furthermore, mutations of the NF-κB (Nuclear Factor Kappa B) signalling pathway are detectable, which lead to its upregulation and hyperactivity. NF-κB is of great importance for the regulation of the immune response, cell proliferation and cell death. The activation of NF-κB is considered critical for the development of inflammation. Furthermore, this signaling agent is associated with the pathogenesis of numerous carcinoma diseases.

Manifestation
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Middle-aged adults (40-60 years). m:w=1:1

Localization
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Trunk, capillitium, face (> 90%), rarely on the extremities (DD: cutaneous marginal zone lymphoma).

Clinical features
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Mostly solitary, more rarely multiple, diffusely arranged or grouped, 3.0-5.0 cm in diameter, red or brown-red, symptomless plaques or nodules with a smooth surface. No tendency to ulceration.

A special variant is the so-called crosti-lymphoma with large plaques on the back.

Histology
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Dense nodular or diffuse infiltration of the dermis and subcutis. Clear orientation on the adnexal structures with subepidermally free zone (free border zone). No epidermotropy. A clear follicular pattern is observed, especially when localized on the head. Cytomorphologically there are smaller cells with clearly notched nuclei and numerous granules (characteristics of centrocytes) as well as larger cells with large round nuclei and one or more prominent nucleoli (characteristics of centroblasts). The infiltrate is supplemented by immunoblasts (large nuclei with central nucleolus), small lymphocytes, histiocytes, rarely eosinophilic granulocytes and plasma cells. Especially in early lesions there are numerous reactive T cells.

With the progression of the tumor, follicular structures are less frequently expressed, the number of reactive T cells is relatively reduced. Monomorphic populations of large germinal centre cells predominate.

Immunohistology: Expression of B-cell associated antigens: CD19, CD20, CD79a. In follicular structures: expression of CD10; also BCL6, CD21 (dendritic reticulum cells).

PCR: in about 70% of cases, detection of the clonal tumor cell population with monoclonal rearrangement of the heavy chain immunoglobulin genes.

The typical translocation t(14;18) (q32;q21) of nodal germinal center tumors is found only in a minority of primary cutaneous follicular lymphomas. Chromosomal translocation leads to an overexpression of Bcl-2 an anti-apoptotic oncogene.

Differential diagnosis
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Therapy
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Basically, follicular lymphomas are considered lymphomas with an excellent prognosis.

  • In patients with "low burden follicular lymphoma", i.e. only few HV and no other clinical symptoms, a "wait and see" strategy can be pursued in coordination with the patient. Chemotherapy at this stage is not recommended.
    • Alternative: In a large study with 384 patients (RESORT) it could be shown that the early application of Rituximab was superior to the "wait and see" group (time to treatment failure).
    • Alternative: Patients with solitary or few (< 10) lesions can be treated either surgically or (locally) radiotherapeutically.
  • Recurrences are not always a sign of tumor progression. In this respect they can also be treated locally.
  • For patients with advanced follicular lymphomas, standard chemotherapy according to the CHOP scheme or R-CHOP scheme ( rituximab + CHOP) is recommended. R-CHOP can significantly increase the time to therapy failure (TTF) according to studies.
  • Alternatively: CVP (cyclophosphamide, vincristine, prednisone) or R-CVP (rituximab + CVP). The combination R-CVP is also superior here, the time to progression (TTP) is extended by 20 months according to studies.
  • In patients with multiple lesions, good results were achieved with systemically or intralesionally applied anti-CD20-Ak(rituximab).

Progression/forecast
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Negative bone marrow infiltration results in an excellent prognosis with a 5-year survival rate of 95%. With detectable bone marrow infiltration the 5-year survival rate drops to 63%. Remarkably, the prognosis worsens significantly with localization on the leg (5-year survival rate of only 40% - mustard 2007-). The reason for this is unclear.

Literature
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  1. Cerroni L, Kerl H (2003) Cutaneous B-cell lymphomas. In: Kerl H, Garb C, Cerroni L, Wolff H histopathology of the skin. Springer, Berlin, S. 902-905
  2. Heinzerling LM et al (2000) Reduction of tumor burden and stabilisation of disease by systemic therapy with anti-CD20-antibody (rituximab) in patients with primary cutaneous B-cell-lymphoma: initial results in dermatologic patients. Br J Dermatol 144: 1239-243
  3. Kazakov DV et al (2002) Primary subcutaneous follicular centre cell lymphoma with involvement of the galea: a case report and short review of the literature. Br J Dermatol 146: 663-666
  4. Kim MJ et al (2015) Clinical features and treatment outcomes of primary cutaneous B-cell lymphoma: a single-center analysis in South Korea. Int J Hematol. 101: 273-278
  5. Nicolay JP (2016) B-cell lymphomas of the skin - pathogenesis, diagnosis and therapy. J Dtsch Dermatol Ges 14:1207-1225.
  6. Prince HM et al (2003) Primary cutaneous B-cell lymphomas. Clin Exp Dermatol 28: 8-12
  7. Senff NJ et al (2007) Reclassification of 300 primary cutaneous B-cell lymphomas according to the newWHO-EORTC
    classification for cutaneous lymphomas: comparison with previousclassifications
    and identification of prognostic markers. J Clin Oncol 25:1581-1587.
  8. Wilcox RA (2015) Cutaneous B-cell lymphomas: 2015 update on diagnosis, risk-stratification, and management. On J Hematol 90:73-76
  9. Willemze R et al (2005) WHO-EORTC classification for cutaneous lymphomas. Blood 105: 3768-3785

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Last updated on: 29.10.2020