Primary cutaneous follicle centre lymphoma

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Haematolymphoid Tumours (5th ed.)

editHAEM5 Conversion Notes
This page was converted to the new template on 2023-12-07. The original page can be found at HAEM4:Primary Cutaneous Follicle Centre Lymphoma.

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Primary Author(s)*

Linlin Gao, MD, PhD and Shivani Golem, PhD, FACMG

Cancer Category / Type

Cancer Sub-Classification / Subtype

  • Primary Cutaneous Follicle Center Lymphoma

Definition / Description of Disease

Primary cutaneous follicle center lymphoma (PCFCL) is a tumor arising in skin composed of germinal center B cells, including centrocytes and centroblasts[1].

Synonyms / Terminology

  • Reticulohistiocytoma of the dorsum
  • Crosti lymphoma

Epidemiology / Prevalence

  • It accounts for about 50% of primary cutaneous B-cell lymphomas with an incidence of 0.1-0.2 per 100,000 persons per year
  • It mainly occurs in middle-aged adults
  • Male: female ratio is approximately 1.5:1[2]

Clinical Features

Put your text here and fill in the table (Instruction: Can include references in the table)

Signs and Symptoms EXAMPLE Asymptomatic (incidental finding on complete blood counts)

EXAMPLE B-symptoms (weight loss, fever, night sweats)

EXAMPLE Fatigue

EXAMPLE Lymphadenopathy (uncommon)

Laboratory Findings EXAMPLE Cytopenias

EXAMPLE Lymphocytosis (low level)


editv4:Clinical Features
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  • Usually solitary, firm, and erythematous to violaceous plaques, nodules or tumors of variable size
  • Multifocal in 15% of patients
  • Lesions on the trunk may be surrounded by erythematous papules
  • The skin surface is usually smooth and rarely ulcerated[2]

Sites of Involvement

  • Head
  • Trunk

Morphologic Features

  • Perivascular, periadnexal, or diffuse infiltrates with sparing of the epidermis
  • The growth patterns include follicular, follicular and diffuse, and diffuse patterns[3]
  • The tumor is composed of centrocytes and variable numbers of centroblasts[1]
  • In a tumor with follicular growth pattern, follicles are poorly defined and composed of monotonous follicle center cells with no polarization
    • A follicular dendritic cell meshwork is present
    • Tingible body macrophages are usually absent
    • Mantle zones are attenuated or absent
    • Proliferation rate is low
  • In a tumor with diffuse growth pattern, tumor cells are mainly large centrocytes, some of which are multilobated or spindle-shaped[2]
    • Variable numbers of large centroblasts
    • Follicular dendritic cell meshwork may be lost
    • Proliferation rate is generally high

Immunophenotype

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Finding Marker
Positive (universal) EXAMPLE CD1
Positive (subset) EXAMPLE CD2
Negative (universal) EXAMPLE CD3
Negative (subset) EXAMPLE CD4


editv4:Immunophenotype
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Finding Marker
Positive (universal) CD20, CD79a, BCL6
Positive (tumor with a follicular growth pattern) CD10
Negative (universal) CD5, CD43
Negative (most cases) BCL2, MUM1, FOXP1

Chromosomal Rearrangements (Gene Fusions)

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Chromosomal Rearrangement Genes in Fusion (5’ or 3’ Segments) Pathogenic Derivative Prevalence Diagnostic Significance (Yes, No or Unknown) Prognostic Significance (Yes, No or Unknown) Therapeutic Significance (Yes, No or Unknown) Notes
EXAMPLE t(9;22)(q34;q11.2) EXAMPLE 3'ABL1 / 5'BCR EXAMPLE der(22) EXAMPLE 20% (COSMIC)

EXAMPLE 30% (add reference)

Yes No Yes EXAMPLE

The t(9;22) is diagnostic of CML in the appropriate morphology and clinical context (add reference). This fusion is responsive to targeted therapy such as Imatinib (Gleevec) (add reference).


editv4:Chromosomal Rearrangements (Gene Fusions)
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The t(14;18)(q32;q21), IGH/BCL2 translocation, the genetic hallmark of nodal follicular lymphoma, is rare in primary cutaneous follicle center lymphoma[4][5].

Chromosomal Rearrangement Genes in Fusion (5’ or 3’ Segments) Pathogenic Derivative Prevalence
t(14;18)(q32;q21) 5'BCL2/3'IGH der(14) 7%
t(14;18)(q32;q21) 5'MALT1/3'IGH der(14) 2%


editv4:Clinical Significance (Diagnosis, Prognosis and Therapeutic Implications).
Please incorporate this section into the relevant tables found in:
  • Chromosomal Rearrangements (Gene Fusions)
  • Individual Region Genomic Gain/Loss/LOH
  • Characteristic Chromosomal Patterns
  • Gene Mutations (SNV/INDEL)
  • Chromosomal abnormalities in PCFCL involving BCL2 or MALT1 do not correlate with a poor prognosis[2].

Individual Region Genomic Gain / Loss / LOH

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Chr # Gain / Loss / Amp / LOH Minimal Region Genomic Coordinates [Genome Build] Minimal Region Cytoband Diagnostic Significance (Yes, No or Unknown) Prognostic Significance (Yes, No or Unknown) Therapeutic Significance (Yes, No or Unknown) Notes
EXAMPLE

7

EXAMPLE Loss EXAMPLE

chr7:1- 159,335,973 [hg38]

EXAMPLE

chr7

Yes Yes No EXAMPLE

Presence of monosomy 7 (or 7q deletion) is sufficient for a diagnosis of AML with MDS-related changes when there is ≥20% blasts and no prior therapy (add reference).  Monosomy 7/7q deletion is associated with a poor prognosis in AML (add reference).

EXAMPLE

8

EXAMPLE Gain EXAMPLE

chr8:1-145,138,636 [hg38]

EXAMPLE

chr8

No No No EXAMPLE

Common recurrent secondary finding for t(8;21) (add reference).

editv4:Genomic Gain/Loss/LOH
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Chromosome Number Gain/Loss/Amp/LOH Region
1 Loss chr 1p36
18 Amplification chr 18q21.33

Characteristic Chromosomal Patterns

Put your text here (EXAMPLE PATTERNS: hyperdiploid; gain of odd number chromosomes including typically chromosome 1, 3, 5, 7, 11, and 17; co-deletion of 1p and 19q; complex karyotypes without characteristic genetic findings; chromothripsis)

Chromosomal Pattern Diagnostic Significance (Yes, No or Unknown) Prognostic Significance (Yes, No or Unknown) Therapeutic Significance (Yes, No or Unknown) Notes
EXAMPLE

Co-deletion of 1p and 18q

Yes No No EXAMPLE:

See chromosomal rearrangements table as this pattern is due to an unbalanced derivative translocation associated with oligodendroglioma (add reference).

editv4:Characteristic Chromosomal Aberrations / Patterns
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In a study of the genetic abnormalities of primary cutaneous follicle center lymphoma, 1p36 deletion was reported to occur in 22% (5/21) and BCL2 gene break in 10% (2/20) of the cases. TNFRSF14 nonsense and missense mutations were detected in 4/17 (23.5%) cases with concomitant 1p36 deletion in 2 cases. In 43% (9/21) of the cases, high EZH2 protein expression with a BCL2 negative phenotype was detected[4]. In another study that investigated 57 patients with PCFCL, 1 case was found to have BCL2 chromosomal amplification, 4 cases had IGH/BCL2 translocation, and 1 case had IGH/MALT1 translocation[6]. In a case report of an aggressive PCFCL, c-MYC translocation and CDKN2A (9p21) deletion were detected[7].

Gene Mutations (SNV / INDEL)

Put your text here and fill in the table (Instructions: This table is not meant to be an exhaustive list; please include only genes/alterations that are recurrent and common as well either disease defining and/or clinically significant. Can include references in the table. For clinical significance, denote associations with FDA-approved therapy (not an extensive list of applicable drugs) and NCCN or other national guidelines if applicable; Can also refer to CGC workgroup tables as linked on the homepage if applicable as well as any high impact papers or reviews of gene mutations in this entity.)

Gene; Genetic Alteration Presumed Mechanism (Tumor Suppressor Gene [TSG] / Oncogene / Other) Prevalence (COSMIC / TCGA / Other) Concomitant Mutations Mutually Exclusive Mutations Diagnostic Significance (Yes, No or Unknown) Prognostic Significance (Yes, No or Unknown) Therapeutic Significance (Yes, No or Unknown) Notes
EXAMPLE: TP53; Variable LOF mutations

EXAMPLE:

EGFR; Exon 20 mutations

EXAMPLE: BRAF; Activating mutations

EXAMPLE: TSG EXAMPLE: 20% (COSMIC)

EXAMPLE: 30% (add Reference)

EXAMPLE: IDH1 R123H EXAMPLE: EGFR amplification EXAMPLE:  Excludes hairy cell leukemia (HCL) (add reference).


Note: A more extensive list of mutations can be found in cBioportal (https://www.cbioportal.org/), COSMIC (https://cancer.sanger.ac.uk/cosmic), ICGC (https://dcc.icgc.org/) and/or other databases. When applicable, gene-specific pages within the CCGA site directly link to pertinent external content.


editv4:Gene Mutations (SNV/INDEL)
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Gene Mutation Oncogene/Tumor Suppressor/Other Presumed Mechanism (LOF/GOF/Other; Driver/Passenger) Prevalence (COSMIC/TCGA/Other)
TNFRSF14 W12Ter Tumor suppressor LOF 17.6%
TNFRSF14 C53G Tumor suppressor LOF 6%

Epigenomic Alterations

  • Not known in this specific subgroup.

Genes and Main Pathways Involved

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Gene; Genetic Alteration Pathway Pathophysiologic Outcome
EXAMPLE: BRAF and MAP2K1; Activating mutations EXAMPLE: MAPK signaling EXAMPLE: Increased cell growth and proliferation
EXAMPLE: CDKN2A; Inactivating mutations EXAMPLE: Cell cycle regulation EXAMPLE: Unregulated cell division
EXAMPLE:  KMT2C and ARID1A; Inactivating mutations EXAMPLE:  Histone modification, chromatin remodeling EXAMPLE:  Abnormal gene expression program
editv4:Genes and Main Pathways Involved
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BCL2-mediated apoptosis pathway and NF-κB pathway

Genetic Diagnostic Testing Methods

  • Fluorescence in situ hybridization with BCL2 break apart and 1p36/1q25 dual color probes
  • Polymerase chain reaction for IGH rearrangements
  • Chromosomal microarray or karyotype analysis for 1p36 deletion
  • DNA sequencing for TNFRSF14 mutations

Familial Forms

  • Not known in this specific subgroup.

Additional Information

Prognosis: Excellent prognosis with a 5-year survival rate >90%[8].

Links

  • None

References

(use the "Cite" icon at the top of the page) (Instructions: Add each reference into the text above by clicking on where you want to insert the reference, selecting the “Cite” icon at the top of the page, and using the “Automatic” tab option to search such as by PMID to select the reference to insert. The reference list in this section will be automatically generated and sorted. If a PMID is not available, such as for a book, please use the “Cite” icon, select “Manual” and then “Basic Form”, and include the entire reference.)

  1. ↑ 1.0 1.1 Gulia, Andrea; et al. (2011-11). "Clinicopathologic features of early lesions of primary cutaneous follicle center lymphoma, diffuse type: Implications for early diagnosis and treatment". Journal of the American Academy of Dermatology. 65 (5): 991–1000.e7. doi:10.1016/j.jaad.2010.06.059. ISSN 0190-9622. Check date values in: |date= (help)
  2. ↑ 2.0 2.1 2.2 2.3 World health organization classification of tumours of haematopoietic and lymphoid tissues, revised 4th edition, Swerdlow SH, Campo E, Harris NL, et al. (Eds), IARC, Lyon 2017.
  3. ↑ Senff, Nancy J.; et al. (2007-04-20). "Reclassification of 300 primary cutaneous B-Cell lymphomas according to the new WHO-EORTC classification for cutaneous lymphomas: comparison with previous classifications and identification of prognostic markers". Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology. 25 (12): 1581–1587. doi:10.1200/JCO.2006.09.6396. ISSN 1527-7755. PMID 17353548.
  4. ↑ 4.0 4.1 Gángó, Ambrus; et al. (2018-10). "Concomitant 1p36 deletion and TNFRSF14 mutations in primary cutaneous follicle center lymphoma frequently expressing high levels of EZH2 protein". Virchows Archiv: An International Journal of Pathology. 473 (4): 453–462. doi:10.1007/s00428-018-2384-3. ISSN 1432-2307. PMID 29858685. Check date values in: |date= (help)
  5. ↑ Goodlad, John R.; et al. (2003-12). "Primary cutaneous diffuse large B-cell lymphoma: prognostic significance of clinicopathological subtypes". The American Journal of Surgical Pathology. 27 (12): 1538–1545. doi:10.1097/00000478-200312000-00006. ISSN 0147-5185. PMID 14657713. Check date values in: |date= (help)
  6. ↑ Abdul-Wahab, Alya; et al. (2014-06). "Chromosomal anomalies in primary cutaneous follicle center cell lymphoma do not portend a poor prognosis". Journal of the American Academy of Dermatology. 70 (6): 1010–1020. doi:10.1016/j.jaad.2014.01.862. ISSN 0190-9622. Check date values in: |date= (help)
  7. ↑ Tsang, Hamilton C.; et al. (2017-03). "An Aggressive Primary Cutaneous Follicle Center Lymphoma With c-MYC Translocation and CDKN2A (9p21) Deletion: A Case Report and Review of the Literature". The American Journal of Dermatopathology. 39 (3): e44–e49. doi:10.1097/DAD.0000000000000738. ISSN 1533-0311. PMID 27759694. Check date values in: |date= (help)
  8. ↑ Lucioni, Marco; et al. (2016-10). "Primary cutaneous B-cell lymphoma other than marginal zone: clinicopathologic analysis of 161 cases: Comparison with current classification and definition of prognostic markers". Cancer Medicine. 5 (10): 2740–2755. doi:10.1002/cam4.865. ISSN 2045-7634. PMC 5083727. PMID 27665744. Check date values in: |date= (help)

Notes

*Primary authors will typically be those that initially create and complete the content of a page.  If a subsequent user modifies the content and feels the effort put forth is of high enough significance to warrant listing in the authorship section, please contact the CCGA coordinators (contact information provided on the homepage).  Additional global feedback or concerns are also welcome. *Citation of this Page: “Primary cutaneous follicle centre lymphoma”. Compendium of Cancer Genome Aberrations (CCGA), Cancer Genomics Consortium (CGC), updated 12/13/2023, https://ccga.io/index.php/HAEM5:Primary_cutaneous_follicle_centre_lymphoma.

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