Primary cutaneous follicle centre lymphoma pathophysiology

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sowminya Arikapudi, M.B,B.S. [2]

Overview

Primary cutaneous follicle centre lymphoma is a tumor of neoplastic follicle centre cells, including centrocytes and variable numbers of centroblasts, with a follicular and diffuse growth pattern that generally presents on the head or trunk.[1]Genes involved in the pathogenesis of primary cutaneous follicle centre lymphoma include C-REL and BCL-2 genes.[1] Primary cutaneous follicle centre lymphoma (PCFCL) may be associated with borrelia burgdorferi, hepatitis C, and human herpesvirus 8. On gross pathology,solitary or grouped erythematous papules, plaques and tumor lesions, mostly non-ulcerated are characteristic findings of primary cutaneous follicle centre lymphoma. On microscopic histopathological analysis, centroblasts (large noncleaved cells), centrocytes (small and large cleaved cells), and reactive T cells are characteristic findings of primary cutaneous follicle centre lymphoma.[2]

Pathophysiology

Primary cutaneous follicle centre lymphoma (PCFCL) is characterized by a proliferation of follicle center cells (centrocytes and centroblasts) with a follicular, follicular and diffuse, or diffuse growth pattern.[3]

Genetics

Genes involved in the pathogenesis of primary cutaneous follicle centre lymphoma include:[1]

  • Amplification of C-REL gene
  • BCL-2 rearrangements

Association

Primary cutaneous follicle centre lymphoma (PCFCL) may be associated with:[2]

Gross Pathology

On gross pathology, solitary or grouped erythematous papules, plaques and tumor lesions, mostly non-ulcerated are characteristic findings of primary cutaneous follicle centre lymphoma.[2]

Microscopic Pathology

On microscopic histopathological analysis, centroblasts (large noncleaved cells), centrocytes (small and large cleaved cells), and reactive T cells are characteristic findings of primary cutaneous follicle centre lymphoma.[2]

  • Histopathological appearance of primary cutaneous follicle centre lymphoma include:[4]
  • Epidemis
  • Superficially regular and preserved
  • Deep dermis
  • Abundant lymphocytic infiltrate with the formation of follicular germinal center and the surrounding ‘mantle zone’
  • The infiltrate was composed of atypical, medium-sized and focally large lymphatic cells


The epidermis is preserved, with diffuse and nodular infiltrates of lymphoid cells.[4]
The infiltrate is composed of atypical, medium-sized and focally large lymphatic cells.[4]
Immunohistochemical staining for CD20 demonstrates diffusely positive reaction in tumour cells (A: IHC, CD20), coexpressing BCL6 (B: IHC, BCL6).[4]
Histopatological fingings. Biopsy of the mass of the scalp showing a dense and diffuse dermal infiltrate with nodular growth pattern (A) consisted of large atypical lymphocytes (H&E) (B), which are positive for CD20 (C) and Bcl6 (D) and negative for Bcl2 (E) on immunohistochemical staining. Bone marrow biopsy showing a hypercellular bone marrow with proliferation of megakaryocytes with hyperlobulated nuclei, sometimes in loose clusters (H&E) (F), positive for CD61 on immunohistochemical staining (G).[4]

References

  1. ↑ 1.0 1.1 1.2 Primary cutaneous follicle centre lymphoma. Surveillance, Epidemiology, and End Results Program. http://seer.cancer.gov/seertools/hemelymph/51f6cf5ae3e27c3994bd549b/. Accessed on March 02, 2016
  2. ↑ 2.0 2.1 2.2 2.3 Ceovic R, Jovanovic I, Kostovic K, Rados J, Dotlic S, Radman I; et al. (2013). "Radiotherapy of primary cutaneous follicle center lymphoma: case report and review of literature". Radiat Oncol. 8: 147. doi:10.1186/1748-717X-8-147. PMC 3702489. PMID 23786884.
  3. ↑ Transformation of a Cutaneous Follicle Center Lymphoma to a Diffuse Large B-Cell Lymphoma—An Unusual Presentation. Hindawi. http://www.hindawi.com/journals/crim/2010/296523/. Accessed on March 02, 2016
  4. ↑ 4.0 4.1 4.2 4.3 4.4 Radiotherapy of primary cutaneous follicle center lymphoma: case report and review of literature. BioMed Central. https://ro-journal.biomedcentral.com/articles/10.1186/1748-717X-8-147. Accessed on March 02, 2016


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