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
References
- ↑ 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
- ↑ 2.0 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.
- ↑ 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
- ↑ 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