Primary cutaneous follicle centre lymphoma overview
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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 the most common type of primary cutaneous B-cell lymphoma. Primary cutaneous follicle centre lymphoma (PCFCL) can be defined as neoplastic proliferation of the follicle germinal center cells limited to the skin. This 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]Based on the growth pattern, primary cutaneous follicle centre lymphoma may be classified into follicular, diffuse, and mixed pattern.[2] 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.[3]There are no established causes for primary cutaneous follicle centre lymphoma. Primary cutaneous follicle centre lymphoma must be differentiated from other diseases such as eczema, psoriasis, and cutaneous T cell lymphoma. The incidence of primary cutaneous follicle centre lymphoma increases with age; the median age at diagnosis is 51 years. Males are more commonly affected with primary cutaneous follicle centre lymphoma than females.[1] There are no established risk factors for primary cutaneous follicle centre lymphoma. According to the the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine screening for primary cutaneous follicle centre lymphoma.[4]Prognosis is generally excellent, and the 5-year survival rate of patients with primary cutaneous follicle centre lymphoma is approximately 97%. The staging of primary cutaneous follicle centre lymphoma is based on the International Society for Cutaneous Lymphomas (ISCL) / EORTC proposal on TNM classification of cutaneous lymphoma other than mycosis fungoides / sezary syndrome.[5]The most common symptoms of primary cutaneous follicle centre lymphoma include fever, fatigue, weight loss, skin rash, night sweats, skin rash, chest pain, abdominal pain, bone pain, and painless swelling in the neck, axilla, groin, thorax, and abdomen.[1] Common physical examination findings of primary cutaneous follicle centre lymphoma include fever, rash, ulcer, firm erythematous or violaceous plaques, nodules, or tumors of varying sizes, chest tenderness, abdomen tenderness, bone tenderness, peripheral lymphadenopathy, and central lymphadenopathy.[1]Laboratory tests for primary cutaneous follicle centre lymphoma include complete blood count (CBC), blood chemistry studies, cytogenetic analysis, flow cytometry, immunohistochemistry, and immunophenotyping.[1] Lymph node biopsy is diagnostic of primary cutaneous follicle centre lymphoma. CT scan may be helpful in the diagnosis of primary cutaneous follicle centre lymphoma. [1]
Classification
Based on the growth pattern, primary cutaneous follicle centre lymphoma may be classified into follicular, diffuse, and mixed pattern.[2]
Pathophysiology
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.[3]
Causes
There are no established causes for primary cutaneous follicle centre lymphoma.
Differentiating Primary cutaneous follicle center lymphoma from other Diseases
Primary cutaneous follicle centre lymphoma must be differentiated from other diseases such as eczema, psoriasis, and cutaneous T cell lymphoma.
Epidemiology and Demographics
The incidence of primary cutaneous follicle centre lymphoma increases with age; the median age at diagnosis is 51 years. Males are more commonly affected with primary cutaneous follicle centre lymphoma than females.[1]
Risk Factors
There are no established risk factors for primary cutaneous follicle centre lymphoma.
Screening
According to the the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine screening for primary cutaneous follicle centre lymphoma.[4]
Prognosis
Prognosis is generally excellent, and the 5-year survival rate of patients with primary cutaneous follicle centre lymphoma is approximately 97%.
Diagnosis
Staging
The staging of primary cutaneous follicle centre lymphoma is based on the International Society for Cutaneous Lymphomas (ISCL) / EORTC proposal on TNM classification of cutaneous lymphoma other than mycosis fungoides / sezary syndrome.[5]
Symptoms
The most common symptoms of primary cutaneous follicle centre lymphoma include fever, fatigue, weight loss, skin rash, night sweats, skin rash, chest pain, abdominal pain, bone pain, and painless swelling in the neck, axilla, groin, thorax, and abdomen.[1]
Physical Examination
Common physical examination findings of primary cutaneous follicle centre lymphoma include fever, rash, ulcer, firm erythematous or violaceous plaques, nodules, or tumors of varying sizes, chest tenderness, abdomen tenderness, bone tenderness, peripheral lymphadenopathy, and central lymphadenopathy.[1]
Laboratory tests
Laboratory tests for primary cutaneous follicle centre lymphoma include complete blood count (CBC), blood chemistry studies, cytogenetic analysis, flow cytometry, immunohistochemistry, and immunophenotyping.[1]
Biopsy
Lymph node biopsy is diagnostic of primary cutaneous follicle centre lymphoma.
CT
CT scan may be helpful in the diagnosis of primary cutaneous follicle centre lymphoma. [1]
References
- ↑ 1.00 1.01 1.02 1.03 1.04 1.05 1.06 1.07 1.08 1.09 1.10 1.11 1.12 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 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
- ↑ 3.0 3.1 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.
- ↑ 4.0 4.1 Recommendations. U.S Preventive Services Task Force. http://www.uspreventiveservicestaskforce.org/BrowseRec/Search?s=Primary+cutaneous+follicle+centre+lymphoma. Accessed on March 01, 2016
- ↑ 5.0 5.1 Kim, Y. H.; Willemze, R.; Pimpinelli, N.; Whittaker, S.; Olsen, E. A.; Ranki, A.; Dummer, R.; Hoppe, R. T. (2007). "TNM classification system for primary cutaneous lymphomas other than mycosis fungoides and Sezary syndrome: a proposal of the International Society for Cutaneous Lymphomas (ISCL) and the Cutaneous Lymphoma Task Force of the European Organization of Research and Treatment of Cancer (EORTC)". Blood. 110 (2): 479–484. doi:10.1182/blood-2006-10-054601. ISSN 0006-4971.