Primary cutaneous follicle center lymphoma is a type of

lymphoma Lymphoma is a group of blood and lymph tumors that develop from lymphocytes (a type of white blood cell). In current usage the name usually refers to just the cancerous versions rather than all such tumours. Signs and symptoms may include enlar ...

. It was recognized as a distinct disease entity in the 2008 WHO classification. PCFCL had been previously conceived as a variant of

follicular lymphoma Follicular lymphoma (FL) is a cancer that involves certain types of white blood cells known as lymphocytes. The cancer originates from the uncontrolled division of specific types of B-cells known as centrocytes and centroblasts. These cells normal ...

(FL).

Cause

Unlike FL, PCFCL is not typically associated with t(14;18) translocation although presence of that translocation does not exclude PCFCL. It is usually not associated with overexpressed

Bcl-2 Bcl-2 (B-cell lymphoma 2), encoded in humans by the ''BCL2'' gene, is the founding member of the Bcl-2 family of regulator proteins that regulate cell death (apoptosis), by either inhibiting (anti-apoptotic) or inducing (pro-apoptotic) apoptosis. ...

. PCFCL represents about 55% to 60% of primary cutaneous B-cell lymphomas (PCBCL);

primary cutaneous marginal zone lymphoma Primary cutaneous marginal zone lymphomas represent a heterogeneous group of diseases characterized by solitary or multiple dermis, dermal or subcutaneous nodules. Lymphomas included in this group are: :* Primary cutaneous immunocytoma :* Margina ...

and

diffuse large B-cell cell lymphoma, leg type Diffusion is the net movement of anything (for example, atoms, ions, molecules, energy) generally from a region of higher concentration to a region of lower concentration. Diffusion is driven by a gradient in Gibbs free energy or chemical p ...

are the other primary cutaneous B-cell lymphomas. The cause of PCFCL is unknown.

Treatment

Surgical removal and/or

radiotherapy Radiation therapy or radiotherapy, often abbreviated RT, RTx, or XRT, is a therapy using ionizing radiation, generally provided as part of cancer treatment to control or kill malignant cells and normally delivered by a linear accelerator. Radia ...

is given for localized disease. Radiation using multiple radiation fields is given if the disease has wider extent with grouped lesions. For the less common situation of more extensive disease (still confined to skin),

rituximab Rituximab, sold under the brand name Rituxan among others, is a monoclonal antibody medication used to treat certain autoimmune diseases and types of cancer. It is used for non-Hodgkin lymphoma, chronic lymphocytic leukemia (in non-geriatric p ...

without chemotherapy is used. Intralesional

interferon alpha The type-I interferons (IFN) are cytokines which play essential roles in inflammation, immunoregulation, tumor cells recognition, and T cell, T-cell responses. In the human genome, a cluster of thirteen functional IFN genes is located at the 9p2 ...

(IFN-α) and intralesional rituximab have been used. Approximately one-third of PCFCL relapse, usually in the skin; treatment is similar to initial management and overall survival remains excellent.

Prognosis

Spread from the skin is unusual, and the

prognosis Prognosis (Greek: πρόγνωσις "fore-knowing, foreseeing") is a medical term for predicting the likely or expected development of a disease, including whether the signs and symptoms will improve or worsen (and how quickly) or remain stabl ...

is excellent with a 5-year survival of over 97%. The International Extranodal Lymphoma Study Group identified elevated LDH, more than two skin lesions, and nodular lesions as three prognostic factors, that are used to assess a cutaneous lymphoma international prognostic index (CLIPI), which is

prognostic Prognosis (Greek: πρόγνωσις "fore-knowing, foreseeing") is a medical term for predicting the likely or expected development of a disease, including whether the signs and symptoms will improve or worsen (and how quickly) or remain stable ...

of disease-free status.

References

{{reflist, 2, refs= {{Cite book , last=Armitage , first=JO, author2=Mauch PM , author3=Harris NL , title=Non-Hodgkin Lymphomas, publisher=Lippincott Williams & Wilkins, year=2010 , chapter=Chapter 24, edition=2nd , isbn= 9780781791168, display-authors=etal {{Cite book , vauthors=Jaffe ES, Harris NL, Vardiman JW, Campo E, Arber, DA, title=Hematopathology , publisher=Elsevier Saunders, year=2011 , edition=1st , isbn= 9780721600406 {{cite journal , vauthors=Sokol L, Naghashpour M, Glass LF , title=Primary cutaneous B-cell lymphomas: recent advances in diagnosis and management , journal=Cancer Control , volume=19 , issue=3 , pages=236–44 , year=2012 , pmid=22710899 , doi= 10.1177/107327481201900308 {{cite journal , vauthors=Suárez AL, Pulitzer M, Horwitz S, etal , title=Primary cutaneous B-cell lymphomas: part I. Clinical features, diagnosis, and classification , journal=J. Am. Acad. Dermatol. , volume=69 , issue=3 , pages=329.e1–13; quiz 341–2 , year=2013 , pmid=23957984 , doi=10.1016/j.jaad.2013.06.012 {{cite journal , vauthors=Suárez AL, Querfeld C, Horwitz S, etal , title=Primary cutaneous B-cell lymphomas: part II. Therapy and future directions , journal=J. Am. Acad. Dermatol. , volume=69 , issue=3 , pages=343.e1–11; quiz 355–6 , year=2013 , pmid=23957985 , doi=10.1016/j.jaad.2013.06.011 {{cite journal , vauthors=Senff NJ, Noordijk EM, Kim YH, etal , title=European Organization for Research and Treatment of Cancer and International Society for Cutaneous Lymphoma consensus recommendations for the management of cutaneous B-cell lymphomas , journal=Blood , volume=112 , issue=5 , pages=1600–9 , year=2008 , pmid=18567836 , doi=10.1182/blood-2008-04-152850 , doi-access=free {{cite book , first1=Steven H. , last1=Swerdlow , author2=International Agency for Research on Cancer , author3=World Health Organization , title=WHO classification of tumours of haematopoietic and lymphoid tissues , url=http://apps.who.int/bookorders/anglais/detart1.jsp?codlan=1&codcol=70&codcch=4002 , archive-url=https://web.archive.org/web/20131012173547/http://apps.who.int/bookorders/anglais/detart1.jsp?codlan=1&codcol=70&codcch=4002 , url-status=dead , archive-date=October 12, 2013 , year=2008 , publisher=International Agency for Research on Cancer , edition=4th , volume=2 , series=World Health Organization classification of tumours , isbn=9789283224310 {{cite journal , vauthors=Wilcox RA , title=Cutaneous B-cell lymphomas: 2015 update on diagnosis, risk-stratification, and management , journal=Am. J. Hematol. , volume=90 , issue=1 , pages=73–6 , year=2015 , pmid=25535037 , doi=10.1002/ajh.23863 , s2cid=10733762 , url=https://deepblue.lib.umich.edu/bitstream/2027.42/95353/1/23360_ftp.pdf, doi-access=free Lymphoma Lymphoid-related cutaneous conditions

Cause

Treatment

Prognosis

See also

References