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] Soroush Seifirad, M.D.[3]
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.Based on the growth pattern, primary cutaneous follicle centre lymphoma may be classified into follicular, diffuse, and mixed pattern. 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.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. 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.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.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. 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. MRI scan may be helpful in the diagnosis of primary cutaneous follicle centre lymphoma. PET scan may be helpful in the diagnosis of primary cutaneous follicle centre lymphoma. Other diagnostic studies for primary cutaneous follicle centre lymphoma include laparoscopy, laparotomy, bone marrow aspiration, and bone marrow biopsy. The predominant therapy for primary cutaneous follicle centre lymphoma is radiotherapy. Adjunctive chemotherapy may be required. Surgery is not the first-line treatment option for patients with primary cutaneous follicle centre lymphoma. Surgery is usually reserved for patients with localized disease.
Historical Perspective
Primary cutaneous follicle center lymphoma is a type of lymphoma. It was recognized as a distinct disease entity in the 2008 WHO classification. PCFCL had been previously conceived as a variant of follicular lymphoma (FL).
Classification
Based on the growth pattern, primary cutaneous follicle centre lymphoma may be classified into follicular, diffuse, and mixed pattern.
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. 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.
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.
Risk Factors
There are no established risk factors for primary cutaneous follicle centre lymphoma.
Screening
According to the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine screening for primary cutaneous follicle centre lymphoma.[2]
Natural History, Complications and Prognosis
Prognosis is generally excellent, and the 5-year survival rate of patients with primary cutaneous follicle centre lymphoma is approximately 97%.
Diagnosis
Diagnostic Study of Choice
Biopsy is the gold standard test for the diagnosis of primary cutaneous follicle centre lymphoma. Lymph node biopsy is diagnostic of primary cutaneous follicle centre lymphoma. 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
History and 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.
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.
Laboratory findings
Laboratory tests for primary cutaneous follicle centre lymphoma include complete blood count (CBC), blood chemistry studies, cytogenetic analysis, flow cytometry, immunohistochemistry, and immunophenotyping.
Electrocardiogram
There are no ECG findings associated with primary cutaneous follicle centre lymphoma.
X-ray
There are no x-ray findings associated with primary cutaneous follicle centre lymphoma.
Echocardiography and Ultrasound
There are no echocardiography/ultrasound findings associated with primary cutaneous follicle centre lymphoma.
CT scan
CT scan may be helpful in the diagnosis of primary cutaneous follicle centre lymphoma.
MRI
MRI scan may be helpful in the diagnosis of primary cutaneous follicle centre lymphoma.
Other Imaging Findings
PET scan may be helpful in the diagnosis of primary cutaneous follicle centre lymphoma.
Other Diagnostic Studies
Other diagnostic studies for primary cutaneous follicle centre lymphoma include laparoscopy, laparotomy, bone marrow aspiration, and bone marrow biopsy.
Treatment
Medical Therapy
The predominant therapy for primary cutaneous follicle centre lymphoma is radiotherapy. Adjunctive chemotherapy may be required.
INterventions
Surgery
Surgery is not the first-line treatment option for patients with primary cutaneous follicle centre lymphoma. Surgery is usually reserved for patients with localized disease.
Primary Prevention
Secondary Prevention
Cost-Effectiveness of Therapy
Future or Investigational Therapies
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
- ↑ Recommendations. U.S Preventive Services Task Force. http://www.uspreventiveservicestaskforce.org/BrowseRec/Search?s=Primary+cutaneous+follicle+centre+lymphoma. Accessed on March 01, 2016