Primary Cutaneous Follicle Centre Lymphoma

From Compendium of Cancer Genome Aberrations
Revision as of 13:53, 3 November 2023 by Bailey.Glen (talk | contribs)
Jump to navigation Jump to search


editPREVIOUS EDITION
This page from the 4th edition of Haematolymphoid Tumours is being updated. See 5th edition Table of Contents.

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

  • 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

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)

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%

Characteristic Chromosomal Aberrations / Patterns

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].

Genomic Gain/Loss/LOH

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

Gene Mutations (SNV/INDEL)

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%

Epigenomics (Methylation)

  • Not known in this specific subgroup.

Genes and Main Pathways Involved

BCL2-mediated apoptosis pathway and NF-κB pathway

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

Clinical Significance (Diagnosis, Prognosis and Therapeutic Implications)

  • Chromosomal abnormalities in PCFCL involving BCL2 or MALT1 do not correlate with a poor prognosis[2].

Familial Forms

  • Not known in this specific subgroup.

Other Information

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

Links

  • None

References

*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.


  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)
Retrieved from "https://ccga.io/index.php?title=HAEM4:Primary_Cutaneous_Follicle_Centre_Lymphoma&oldid=12691"