Materiały źródłowe

• #1 A Descriptive Study of 103 Primary Cutaneous B-Cell Lymphomas: Clinical and Pathological Characteristics and Treatment from the Spanish Lymphoma Oncology Group (GOTEL)

  https://www.mdpi.com/2072-6694/16/5/1034

  Primary cutaneous B-cell lymphomas account for approximately 25% of all cutaneous lymphomas and 2% of all non-Hodgkin’s lymphomas. […] The exact incidence of this group of lymphomas is difficult to determine, but population-based data from the US Surveillance, Epidemiology, and End Results (SEER) program show an adjusted incidence of 0.31 cases per 100,000 person-years (0.4 for men and 0.23 for women), with an M/F ratio of 1.76. […] Approximately 2% of NHLs are estimated to be primary cutaneous B-cell lymphomas (PCBCLs). […] The most common histology was PCMZL (66%), followed by PCFCL (26%) and PCDLBCL-LT (8%). […] The low incidence of PCBCL has led to great variability in therapeutic management, often extrapolated from patients’ lymph node counterparts. […] The vast majority of patients (98%) were diagnosed at localized stages (I-II), which corresponds to most of the studies published with PCBCLs. […] Disease-specific survival (DSS) at 10 years was 97.2% in the whole cohort, which is also in agreement with the data known to date, reinforcing the idea that, globally, PCBCL has a very good prognosis.

• #2 Primary Cutaneous B-Cell Lymphomas

  https://atlasgeneticsoncology.org/haematological/1851/primary-cutaneous-b-cell-lymphomas?cookies_accepted=1

  Approximately 25% of all non-Hodgkin lymphoma (NHL) cases will present at an extranodal site without systemic involvement. The overall incidence of primary cutaneous lymphomas in Western countries is estimated to be 0.5 to 1 case per 100,000 people annually. The PCBCLs make up approximately 25% of all primary cutaneous lymphomas, the remainder predominantly T-cell lymphomas (Swerdlow 2013). The incidence varies geographically with lower rates in some countries (Abeldano 2018). PCMZL represents an indolent type of CBCL comprising approximately 7% of PCL and approximately 25% of CBCL. In the revised WHO classification of lymphoma, PCMZL is considered under extranodal marginal zone lymphoma of MALT type (Swerdlow 2016, Swerdlow 2017). PCMZL usually presents in the fifth decade of life, although the disease has been diagnosed in children (Kempf 2014 Aug). Men are diagnosed approximately twice as often as women. Most cases occur in non-Hispanic Whites (Kempf 2014 Mar). The PCFCL is the most common PCBCL, accounting for approximately 60% of such cases (Willemze 2005, Willemzie 2019). It is primarily a disease of middle aged to older patients (median age at onset is 51) and a male to female ratio of approximately 1.5:1 (Zinzani 2006, Bradford 2009, Swerdlow 2017). PCLBCL, leg type comprises 5% of all cutaneous lymphomas and 20% of all PCBCLs. The median age at presentation is in the mid to late seventies and is more common in females than in males with a male to female ratio of 1:3-4 (Swerdlow 2017). In contrast to the previous 2 entities, PCDLBCL-leg type is an aggressive disease and is associated with poor prognosis.

• #3 Primary cutaneous lymphoma | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/primary-cutaneous-lymphoma?embed_domain=hackmd.io%252525252F%2525252540yIPUAFeCSL2JsU8smR5nJQ%252525252Fbnjhjgjghjghjghfavicon.icoradiopaedia-icon-144.pngfavicon.icofavicon.ico&lang=gb

  They account for up to a fifth of all primary extranodal forms of non-Hodgkin lymphoma 1. […] Among the primary cutaneous lymphomas, B-cell types represent ~25% and T-cell types account for ~75% of the cases 2,4.

• #4 Cutaneous lymphoma incidence patterns in the United States: a population-based study of 3884 cases

  https://pmc.ncbi.nlm.nih.gov/articles/PMC2686177/

  In total, 3884 cases (IR 10.7/1000000 person-years) of CLs were diagnosed among residents of the 16 SEER registries during 2001-2005. CTCL was the most common CL subtype accounting for 2769 or 71% of cases (IR = 7.7/1000000 person-years). In contrast to CTCLs, CBCLs accounted for 1105 cases or 29% of all CLs (IR 3.1/1000000 person-years). The most common CBCL subtypes were (40%) primary cutaneous diffuse large-B cell lymphoma (pcDLBCL; IR 1.2/1000000 person-years) and cutaneous follicle center lymphoma (30%; IR 0.9/1000000 person-years), followed by cutaneous marginal zone B-cell lymphoma (25%; IR 0.8/1000000 person-years). […] Overall, males had a statistically significant higher IR of CLs than females (14.0 vs 8.2/1000000 person-years, respectively; male-female IR ratio [M/F IRR] = 1.72; P .001). The M/F IRRs ranged between 1.28 to 2.55 among the various T-cell and B-cell lymphoma subtypes and were significantly elevated except for Szary syndrome (M/F IRR = 2.11, P = .05, based on small numbers) and pcDLBCL-leg type (M/F IRR = 1.19, P = .44).

• #5

  https://link.springer.com/article/10.1007/s40257-022-00704-0

  Primary cutaneous lymphomas are a rare group of diseases, with an estimated incidence of 0.51 case per 100,000 people per year. Primary cutaneous B-cell lymphomas (pCBCLs) represent 25-30% of all primary cutaneous lymphomas. […] To date, treatment recommendations for cutaneous B-cell lymphomas have been largely based on small retrospective studies and institutional experience. […] The pharmacotherapeutic landscape has recently expanded to include drugs that may modify the underlying disease pathology. […] Primary cutaneous B-cell lymphomas have a broad spectrum of clinical presentations, which makes diagnostic and therapeutic strategies challenging. […] Incidence is estimated at 1 per 100,000 persons/year and increases with age. […] Overall prognosis is favorable, with a 5-year survival rate of 95-100%.

• #6 What Is the Treatment for Cutaneous B-Cell Lymphoma?

  https://www.icliniq.com/articles/cancer/cutaneous-b-cell-lymphoma

  Cutaneous B-cell lymphoma accounts for up to 25 percent to 29 percent of total primary cutaneous lymphomas, with an annual incidence of 0.5 to 1 new case per 100,000 population. […] Cutaneous B-cell lymphoma is more prevalent in non-Hispanic White men. However, the prevalence of cutaneous B-cell lymphoma increases with age.

• #7 A Descriptive Study of 103 Primary Cutaneous B-Cell Lymphomas: Clinical and Pathological Characteristics and Treatment from the Spanish Lymphoma Oncology Group (GOTEL)

  https://www.mdpi.com/2072-6694/16/5/1034

  Primary cutaneous B-cell lymphomas account for approximately 25% of all cutaneous lymphomas and 2% of all non-Hodgkin’s lymphomas. […] The exact incidence of this group of lymphomas is difficult to determine, but population-based data from the US Surveillance, Epidemiology, and End Results (SEER) program show an adjusted incidence of 0.31 cases per 100,000 person-years (0.4 for men and 0.23 for women), with an M/F ratio of 1.76. […] Approximately 2% of NHLs are estimated to be primary cutaneous B-cell lymphomas (PCBCLs). […] The most common histology was PCMZL (66%), followed by PCFCL (26%) and PCDLBCL-LT (8%). […] The low incidence of PCBCL has led to great variability in therapeutic management, often extrapolated from patients’ lymph node counterparts. […] The vast majority of patients (98%) were diagnosed at localized stages (I-II), which corresponds to most of the studies published with PCBCLs. […] Disease-specific survival (DSS) at 10 years was 97.2% in the whole cohort, which is also in agreement with the data known to date, reinforcing the idea that, globally, PCBCL has a very good prognosis.

• #8 Cutaneous B-Cell Lymphoma: Overview, Pathophysiology, Epidemiology

  https://emedicine.medscape.com/article/1099540-overview

  The frequency of cutaneous lymphomas is 0.3 case per 100,000 population per year, with 10% (in the United States) to 20% (in Europe) being cutaneous B-cell lymphomas (CBCLs), marginal zone lymphomas, or follicle center lymphomas (FCLs). One study reported a slightly lower rate of cutaneous B-cell lymphoma in Argentina compared with the United States and Europe. The 5-year overall survival rate for most cases of CBCL is greater than 90%, except in diffuse large B-cell lymphoma (DLBCL), for which the 5-year survival rate is 20-50%. […] No significant statistical data are available for CBCL with regard to any predisposition based on race. However, with regard to sex and age, DLBCL is predominantly seen in elderly women.

• #9 Cutaneous B-Cell Lymphoma: Overview, Pathophysiology, Epidemiology

  https://emedicine.medscape.com/article/1099540-overview

  The frequency of cutaneous lymphomas is 0.3 case per 100,000 population per year, with 10% (in the United States) to 20% (in Europe) being cutaneous B-cell lymphomas (CBCLs), marginal zone lymphomas, or follicle center lymphomas (FCLs). One study reported a slightly lower rate of cutaneous B-cell lymphoma in Argentina compared with the United States and Europe. The 5-year overall survival rate for most cases of CBCL is greater than 90%, except in diffuse large B-cell lymphoma (DLBCL), for which the 5-year survival rate is 20-50%. […] No significant statistical data are available for CBCL with regard to any predisposition based on race. However, with regard to sex and age, DLBCL is predominantly seen in elderly women.

• #10 Cutaneous lymphoma incidence patterns in the United States: a population-based study of 3884 cases

  https://pmc.ncbi.nlm.nih.gov/articles/PMC2686177/

  There have been no prior large population-based studies focusing on cutaneous lymphomas (CL) in the United States. Using the Surveillance, Epidemiology and End Results (SEER) program data, we analyzed age-adjusted CL incidence rates (IRs) and survival rates by sex and race/ethnicity. There were 3884 CLs diagnosed during 2001-2005. Cutaneous T-cell lymphomas (CTCLs) accounted for 71% (age-adjusted incidence rate [IR] = 7.7/1000000 person-years), whereas cutaneous B-cell lymphomas (CBCLs) accounted for 29% (IR = 3.1/1000000 person-years). Males had a statistically significant higher IR of CL than females (14.0 vs 8.2/1000000 person-years, respectively; male-female IR ratio [M/F IRR] = 1.72; P .001). CL IRs were highest among blacks and non-Hispanic whites (both 11.5/1000000 person-years), followed by Hispanic whites (7.9) and Asian/Pacific Islanders (7.1). The CTCL IR was highest among blacks (10.0/1000000 person-years), whereas the CBCL IR was highest among non-Hispanic whites (3.5). Over the past 25 years, the CL IR increased from 5.0/1000000 person-years during 1980-1982 to 14.3 during 2001-2003. During 2004-2005, the CL IR was 12.7. This recent apparent change could be incomplete case ascertainment or potential leveling off of IRs. CLs rates vary markedly by race and sex, supporting the notion that they represent distinct disease entities.

• #11 Radiation Oncology/NHL/Cutaneous B-cell lymphoma – Wikibooks, open books for an open world

  https://en.wikibooks.org/wiki/Radiation_Oncology/NHL/Cutaneous_B-cell_lymphoma

  Overall (data from SEER, PMID 15908651) […] Mean age 64 yrs. Male 54%. […] Incidence increasing […] Histology (WHO): DLBCL in 33%, NOS 24%, follicular 17%, MZL 8% […] Site: head or neck 50%, trunk 19%, arm 12%, leg 11%, disseminated 8% […] […] […] SEER review. 268 cases; 58% males, 87% white. 157 (59%) primary localized PCLPD […] Outcome: 3-year OS 81% vs. population-matched OS 87%; 5-year DSS 92%. HN worse outcome […] Conclusion: Rare disease, with excellent OS; more common in white men, HN sites worse survival

• #12 Primary Cutaneous B-Cell Lymphomas

  https://atlasgeneticsoncology.org/haematological/1851/primary-cutaneous-b-cell-lymphomas?cookies_accepted=1

  Approximately 25% of all non-Hodgkin lymphoma (NHL) cases will present at an extranodal site without systemic involvement. The overall incidence of primary cutaneous lymphomas in Western countries is estimated to be 0.5 to 1 case per 100,000 people annually. The PCBCLs make up approximately 25% of all primary cutaneous lymphomas, the remainder predominantly T-cell lymphomas (Swerdlow 2013). The incidence varies geographically with lower rates in some countries (Abeldano 2018). PCMZL represents an indolent type of CBCL comprising approximately 7% of PCL and approximately 25% of CBCL. In the revised WHO classification of lymphoma, PCMZL is considered under extranodal marginal zone lymphoma of MALT type (Swerdlow 2016, Swerdlow 2017). PCMZL usually presents in the fifth decade of life, although the disease has been diagnosed in children (Kempf 2014 Aug). Men are diagnosed approximately twice as often as women. Most cases occur in non-Hispanic Whites (Kempf 2014 Mar). The PCFCL is the most common PCBCL, accounting for approximately 60% of such cases (Willemze 2005, Willemzie 2019). It is primarily a disease of middle aged to older patients (median age at onset is 51) and a male to female ratio of approximately 1.5:1 (Zinzani 2006, Bradford 2009, Swerdlow 2017). PCLBCL, leg type comprises 5% of all cutaneous lymphomas and 20% of all PCBCLs. The median age at presentation is in the mid to late seventies and is more common in females than in males with a male to female ratio of 1:3-4 (Swerdlow 2017). In contrast to the previous 2 entities, PCDLBCL-leg type is an aggressive disease and is associated with poor prognosis.

• #13 Clinical Features, Epidemiology, Management and Prognosis of Patients With Primary Cutaneous Marginal Zone Lymphoma: An Observational Study | Actas Dermo-Sifiliográficas

  https://www.actasdermo.org/es-clinical-features-epidemiology-management-prognosis-articulo-S0001731023006919

  Primary cutaneous marginal zone lymphoma (PCMZL) is a low-grade B cell primary skin lymphoma, usually found in male adults around 50-55 years old. This disease can present as single or multiple erythematous macules, plaques, or tumors. The most common locations are the trunk and upper limbs. […] Treatment of this disease is based on only a limited number of short case series, and well-designed clinical trials are lacking; therefore, strong evidence is scarce. Currently, solitary lesions are treated via surgery or local radiotherapy, whereas multiple lesions are treated with radiotherapy, intravenous administration of rituximab, or watchful follow-up. The prognosis of this type of lymphoma is excellent, with a five-year survival rate of 95-100%. Nevertheless, PCMZL skin relapses are common (44-50%).

• #14 Primary Cutaneous B-Cell Lymphomas

  https://atlasgeneticsoncology.org/haematological/1851/primary-cutaneous-b-cell-lymphomas?cookies_accepted=1

  Approximately 25% of all non-Hodgkin lymphoma (NHL) cases will present at an extranodal site without systemic involvement. The overall incidence of primary cutaneous lymphomas in Western countries is estimated to be 0.5 to 1 case per 100,000 people annually. The PCBCLs make up approximately 25% of all primary cutaneous lymphomas, the remainder predominantly T-cell lymphomas (Swerdlow 2013). The incidence varies geographically with lower rates in some countries (Abeldano 2018). PCMZL represents an indolent type of CBCL comprising approximately 7% of PCL and approximately 25% of CBCL. In the revised WHO classification of lymphoma, PCMZL is considered under extranodal marginal zone lymphoma of MALT type (Swerdlow 2016, Swerdlow 2017). PCMZL usually presents in the fifth decade of life, although the disease has been diagnosed in children (Kempf 2014 Aug). Men are diagnosed approximately twice as often as women. Most cases occur in non-Hispanic Whites (Kempf 2014 Mar). The PCFCL is the most common PCBCL, accounting for approximately 60% of such cases (Willemze 2005, Willemzie 2019). It is primarily a disease of middle aged to older patients (median age at onset is 51) and a male to female ratio of approximately 1.5:1 (Zinzani 2006, Bradford 2009, Swerdlow 2017). PCLBCL, leg type comprises 5% of all cutaneous lymphomas and 20% of all PCBCLs. The median age at presentation is in the mid to late seventies and is more common in females than in males with a male to female ratio of 1:3-4 (Swerdlow 2017). In contrast to the previous 2 entities, PCDLBCL-leg type is an aggressive disease and is associated with poor prognosis.

• #15 Primary Cutaneous B-Cell Lymphomas

  https://atlasgeneticsoncology.org/haematological/1851/primary-cutaneous-b-cell-lymphomas?cookies_accepted=1

  Approximately 25% of all non-Hodgkin lymphoma (NHL) cases will present at an extranodal site without systemic involvement. The overall incidence of primary cutaneous lymphomas in Western countries is estimated to be 0.5 to 1 case per 100,000 people annually. The PCBCLs make up approximately 25% of all primary cutaneous lymphomas, the remainder predominantly T-cell lymphomas (Swerdlow 2013). The incidence varies geographically with lower rates in some countries (Abeldano 2018). PCMZL represents an indolent type of CBCL comprising approximately 7% of PCL and approximately 25% of CBCL. In the revised WHO classification of lymphoma, PCMZL is considered under extranodal marginal zone lymphoma of MALT type (Swerdlow 2016, Swerdlow 2017). PCMZL usually presents in the fifth decade of life, although the disease has been diagnosed in children (Kempf 2014 Aug). Men are diagnosed approximately twice as often as women. Most cases occur in non-Hispanic Whites (Kempf 2014 Mar). The PCFCL is the most common PCBCL, accounting for approximately 60% of such cases (Willemze 2005, Willemzie 2019). It is primarily a disease of middle aged to older patients (median age at onset is 51) and a male to female ratio of approximately 1.5:1 (Zinzani 2006, Bradford 2009, Swerdlow 2017). PCLBCL, leg type comprises 5% of all cutaneous lymphomas and 20% of all PCBCLs. The median age at presentation is in the mid to late seventies and is more common in females than in males with a male to female ratio of 1:3-4 (Swerdlow 2017). In contrast to the previous 2 entities, PCDLBCL-leg type is an aggressive disease and is associated with poor prognosis.

• #16 Primary Cutaneous B-Cell Lymphomas in Children and Adolescents: A SEER Population-Based Study – PubMed

  https://pubmed.ncbi.nlm.nih.gov/34417159/

  Although primary cutaneous B-cell lymphomas (PCBCL) comprise 25% of all cutaneous lymphomas, their incidence in the pediatric population is unknown, and the information on pediatric PCBCL has mostly been gathered from individual case reports or series from single centers. […] This was a population-based, retrospective cohort study of patients in 18 cancer registries in the United States diagnosed between 2000 to 2016 through the Surveillance, Epidemiology, and End Results (SEER) program. Age-adjusted incidence rates were calculated for PCBCL in pediatric (20 years) and adult (20 years) populations. […] A total of 48 pediatric and 5128 adult PCBCL cases were included. […] The age-adjusted pediatric PCBCL incidence rate (per 1,000,000 person-years) was 0.12 (95% CI 0.09-0.16). The incidence in the adult population was approximately 40-fold higher than the one observed in the pediatric group (IRR 41.4, 95% CI 31.2-56.2). […] Pediatric PCBCL is a very rare disease affecting mostly adolescents of both sexes. The major histologic subtype is marginal zone lymphoma, and the prognosis is favorable.

• #17 Cutaneous lymphoma incidence patterns in the United States: a population-based study of 3884 cases

  https://pmc.ncbi.nlm.nih.gov/articles/PMC2686177/

  In total, 3884 cases (IR 10.7/1000000 person-years) of CLs were diagnosed among residents of the 16 SEER registries during 2001-2005. CTCL was the most common CL subtype accounting for 2769 or 71% of cases (IR = 7.7/1000000 person-years). In contrast to CTCLs, CBCLs accounted for 1105 cases or 29% of all CLs (IR 3.1/1000000 person-years). The most common CBCL subtypes were (40%) primary cutaneous diffuse large-B cell lymphoma (pcDLBCL; IR 1.2/1000000 person-years) and cutaneous follicle center lymphoma (30%; IR 0.9/1000000 person-years), followed by cutaneous marginal zone B-cell lymphoma (25%; IR 0.8/1000000 person-years). […] Overall, males had a statistically significant higher IR of CLs than females (14.0 vs 8.2/1000000 person-years, respectively; male-female IR ratio [M/F IRR] = 1.72; P .001). The M/F IRRs ranged between 1.28 to 2.55 among the various T-cell and B-cell lymphoma subtypes and were significantly elevated except for Szary syndrome (M/F IRR = 2.11, P = .05, based on small numbers) and pcDLBCL-leg type (M/F IRR = 1.19, P = .44).

• #18 A Descriptive Study of 103 Primary Cutaneous B-Cell Lymphomas: Clinical and Pathological Characteristics and Treatment from the Spanish Lymphoma Oncology Group (GOTEL)

  https://www.mdpi.com/2072-6694/16/5/1034

  Primary cutaneous B-cell lymphomas account for approximately 25% of all cutaneous lymphomas and 2% of all non-Hodgkin’s lymphomas. […] The exact incidence of this group of lymphomas is difficult to determine, but population-based data from the US Surveillance, Epidemiology, and End Results (SEER) program show an adjusted incidence of 0.31 cases per 100,000 person-years (0.4 for men and 0.23 for women), with an M/F ratio of 1.76. […] Approximately 2% of NHLs are estimated to be primary cutaneous B-cell lymphomas (PCBCLs). […] The most common histology was PCMZL (66%), followed by PCFCL (26%) and PCDLBCL-LT (8%). […] The low incidence of PCBCL has led to great variability in therapeutic management, often extrapolated from patients’ lymph node counterparts. […] The vast majority of patients (98%) were diagnosed at localized stages (I-II), which corresponds to most of the studies published with PCBCLs. […] Disease-specific survival (DSS) at 10 years was 97.2% in the whole cohort, which is also in agreement with the data known to date, reinforcing the idea that, globally, PCBCL has a very good prognosis.

• #19 Primary Cutaneous B-Cell Lymphomas

  https://atlasgeneticsoncology.org/haematological/1851/primary-cutaneous-b-cell-lymphomas?cookies_accepted=1

  Approximately 25% of all non-Hodgkin lymphoma (NHL) cases will present at an extranodal site without systemic involvement. The overall incidence of primary cutaneous lymphomas in Western countries is estimated to be 0.5 to 1 case per 100,000 people annually. The PCBCLs make up approximately 25% of all primary cutaneous lymphomas, the remainder predominantly T-cell lymphomas (Swerdlow 2013). The incidence varies geographically with lower rates in some countries (Abeldano 2018). PCMZL represents an indolent type of CBCL comprising approximately 7% of PCL and approximately 25% of CBCL. In the revised WHO classification of lymphoma, PCMZL is considered under extranodal marginal zone lymphoma of MALT type (Swerdlow 2016, Swerdlow 2017). PCMZL usually presents in the fifth decade of life, although the disease has been diagnosed in children (Kempf 2014 Aug). Men are diagnosed approximately twice as often as women. Most cases occur in non-Hispanic Whites (Kempf 2014 Mar). The PCFCL is the most common PCBCL, accounting for approximately 60% of such cases (Willemze 2005, Willemzie 2019). It is primarily a disease of middle aged to older patients (median age at onset is 51) and a male to female ratio of approximately 1.5:1 (Zinzani 2006, Bradford 2009, Swerdlow 2017). PCLBCL, leg type comprises 5% of all cutaneous lymphomas and 20% of all PCBCLs. The median age at presentation is in the mid to late seventies and is more common in females than in males with a male to female ratio of 1:3-4 (Swerdlow 2017). In contrast to the previous 2 entities, PCDLBCL-leg type is an aggressive disease and is associated with poor prognosis.

• #20 Primary Cutaneous B-Cell Lymphomas

  https://atlasgeneticsoncology.org/haematological/1851/primary-cutaneous-b-cell-lymphomas?cookies_accepted=1

  Approximately 25% of all non-Hodgkin lymphoma (NHL) cases will present at an extranodal site without systemic involvement. The overall incidence of primary cutaneous lymphomas in Western countries is estimated to be 0.5 to 1 case per 100,000 people annually. The PCBCLs make up approximately 25% of all primary cutaneous lymphomas, the remainder predominantly T-cell lymphomas (Swerdlow 2013). The incidence varies geographically with lower rates in some countries (Abeldano 2018). PCMZL represents an indolent type of CBCL comprising approximately 7% of PCL and approximately 25% of CBCL. In the revised WHO classification of lymphoma, PCMZL is considered under extranodal marginal zone lymphoma of MALT type (Swerdlow 2016, Swerdlow 2017). PCMZL usually presents in the fifth decade of life, although the disease has been diagnosed in children (Kempf 2014 Aug). Men are diagnosed approximately twice as often as women. Most cases occur in non-Hispanic Whites (Kempf 2014 Mar). The PCFCL is the most common PCBCL, accounting for approximately 60% of such cases (Willemze 2005, Willemzie 2019). It is primarily a disease of middle aged to older patients (median age at onset is 51) and a male to female ratio of approximately 1.5:1 (Zinzani 2006, Bradford 2009, Swerdlow 2017). PCLBCL, leg type comprises 5% of all cutaneous lymphomas and 20% of all PCBCLs. The median age at presentation is in the mid to late seventies and is more common in females than in males with a male to female ratio of 1:3-4 (Swerdlow 2017). In contrast to the previous 2 entities, PCDLBCL-leg type is an aggressive disease and is associated with poor prognosis.

• #21 Cutaneous B-Cell Lymphoma: Overview, Pathophysiology, Epidemiology

  https://emedicine.medscape.com/article/1099540-overview

  The frequency of cutaneous lymphomas is 0.3 case per 100,000 population per year, with 10% (in the United States) to 20% (in Europe) being cutaneous B-cell lymphomas (CBCLs), marginal zone lymphomas, or follicle center lymphomas (FCLs). One study reported a slightly lower rate of cutaneous B-cell lymphoma in Argentina compared with the United States and Europe. The 5-year overall survival rate for most cases of CBCL is greater than 90%, except in diffuse large B-cell lymphoma (DLBCL), for which the 5-year survival rate is 20-50%. […] No significant statistical data are available for CBCL with regard to any predisposition based on race. However, with regard to sex and age, DLBCL is predominantly seen in elderly women.

• #22 Cutaneous lymphoma incidence patterns in the United States: a population-based study of 3884 cases

  https://pmc.ncbi.nlm.nih.gov/articles/PMC2686177/

  Blacks and non-Hispanic whites had the highest IR for CL overall (11.5/1000000 person-years), followed by Hispanic whites (7.9) and Asian/Pacific Islanders (7.1). The highest CTCL IR was among blacks (IR = 10.0/1000000 person-years) followed by non-Hispanic whites (8.1), and Asian/Pacific Islanders and Hispanic whites (both 5.1). In contrast to CTCLs, non-Hispanic whites had the highest IR for CBCLs (3.5/1000000 person-years), followed by Hispanic whites (2.8), Asian/Pacific Islanders (1.9), and blacks, who had the lowest IR (1.5). […] We found that the IR of CL in metropolitan counties (11.5/1000000 person-years) was higher than nonmetropolitan counties (8.9/1000000 person-years, P .001). The IR of CTCL was also higher in metropolitan counties (8.3/1000000 person-years) than nonmetropolitan counties (6.1/1000000 person-years; P .001). However, IRs of CBCL were similar in metropolitan (3.2/1000000 person-years) and nonmetropolitan counties (2.8/1000000 person-years; P = .222).

• #23 Cutaneous lymphoma incidence patterns in the United States: a population-based study of 3884 cases

  https://pmc.ncbi.nlm.nih.gov/articles/PMC2686177/

  Blacks and non-Hispanic whites had the highest IR for CL overall (11.5/1000000 person-years), followed by Hispanic whites (7.9) and Asian/Pacific Islanders (7.1). The highest CTCL IR was among blacks (IR = 10.0/1000000 person-years) followed by non-Hispanic whites (8.1), and Asian/Pacific Islanders and Hispanic whites (both 5.1). In contrast to CTCLs, non-Hispanic whites had the highest IR for CBCLs (3.5/1000000 person-years), followed by Hispanic whites (2.8), Asian/Pacific Islanders (1.9), and blacks, who had the lowest IR (1.5). […] We found that the IR of CL in metropolitan counties (11.5/1000000 person-years) was higher than nonmetropolitan counties (8.9/1000000 person-years, P .001). The IR of CTCL was also higher in metropolitan counties (8.3/1000000 person-years) than nonmetropolitan counties (6.1/1000000 person-years; P .001). However, IRs of CBCL were similar in metropolitan (3.2/1000000 person-years) and nonmetropolitan counties (2.8/1000000 person-years; P = .222).

• #24 What Is the Treatment for Cutaneous B-Cell Lymphoma?

  https://www.icliniq.com/articles/cancer/cutaneous-b-cell-lymphoma

  Cutaneous B-cell lymphoma accounts for up to 25 percent to 29 percent of total primary cutaneous lymphomas, with an annual incidence of 0.5 to 1 new case per 100,000 population. […] Cutaneous B-cell lymphoma is more prevalent in non-Hispanic White men. However, the prevalence of cutaneous B-cell lymphoma increases with age.

• #25 Primary Cutaneous B-Cell Lymphomas

  https://atlasgeneticsoncology.org/haematological/1851/primary-cutaneous-b-cell-lymphomas?cookies_accepted=1

  Approximately 25% of all non-Hodgkin lymphoma (NHL) cases will present at an extranodal site without systemic involvement. The overall incidence of primary cutaneous lymphomas in Western countries is estimated to be 0.5 to 1 case per 100,000 people annually. The PCBCLs make up approximately 25% of all primary cutaneous lymphomas, the remainder predominantly T-cell lymphomas (Swerdlow 2013). The incidence varies geographically with lower rates in some countries (Abeldano 2018). PCMZL represents an indolent type of CBCL comprising approximately 7% of PCL and approximately 25% of CBCL. In the revised WHO classification of lymphoma, PCMZL is considered under extranodal marginal zone lymphoma of MALT type (Swerdlow 2016, Swerdlow 2017). PCMZL usually presents in the fifth decade of life, although the disease has been diagnosed in children (Kempf 2014 Aug). Men are diagnosed approximately twice as often as women. Most cases occur in non-Hispanic Whites (Kempf 2014 Mar). The PCFCL is the most common PCBCL, accounting for approximately 60% of such cases (Willemze 2005, Willemzie 2019). It is primarily a disease of middle aged to older patients (median age at onset is 51) and a male to female ratio of approximately 1.5:1 (Zinzani 2006, Bradford 2009, Swerdlow 2017). PCLBCL, leg type comprises 5% of all cutaneous lymphomas and 20% of all PCBCLs. The median age at presentation is in the mid to late seventies and is more common in females than in males with a male to female ratio of 1:3-4 (Swerdlow 2017). In contrast to the previous 2 entities, PCDLBCL-leg type is an aggressive disease and is associated with poor prognosis.

• #26 Prevalence, clinical features, and survival outcome trends of 627 patients with primary cutaneous lymphoma over 29 years: a retrospective review from single tertiary center in Korea | Scientific Reports

  https://www.nature.com/articles/s41598-024-71210-y

  The relative frequency of primary cutaneous lymphoma (PCL) subtypes shows wide variation across different geographical regions. This retrospective study was conducted in a tertiary referral center located in Korea to describe the relative frequency, demographics, survival outcomes, and temporal trend in PCL. A total of 627 PCL cases diagnosed between January 1994 and December 2022 were included. The majority of PCL cases (87.2%) were of T-/NK-cell lineage (CTCL), while the remaining cases (12.8%) were B-cell lineage lymphomas (CBCL). The prevalence of mycosis fungoides (MF) in CTCL increased significantly over time, while other CTCL subtypes, including primary cutaneous extranodal NK/T-cell lymphoma and subcutaneous panniculitis-like T-cell lymphoma (SPTCL), decreased in frequency. Notably, the prevalence of CD4-positive small/medium T-cell lymphoproliferative disorder showed a substantial increase over time. Primary cutaneous marginal zone lymphoma was consistently the commonest CBCL subtype. Survival analysis demonstrated that CTCL had a more favorable 5-year overall survival (OS) than CBCL. OS rate of MF, SPTCL, and primary cutaneous peripheral T-cell lymphoma, NOS improved significantly over time. This study provides comprehensive insights into the dynamic change in the relative frequency and overall survival of PCL subtypes over time.

• #27 Cutaneous lymphoma incidence patterns in the United States: a population-based study of 3884 cases

  https://pmc.ncbi.nlm.nih.gov/articles/PMC2686177/

  In total, 3884 cases (IR 10.7/1000000 person-years) of CLs were diagnosed among residents of the 16 SEER registries during 2001-2005. CTCL was the most common CL subtype accounting for 2769 or 71% of cases (IR = 7.7/1000000 person-years). In contrast to CTCLs, CBCLs accounted for 1105 cases or 29% of all CLs (IR 3.1/1000000 person-years). The most common CBCL subtypes were (40%) primary cutaneous diffuse large-B cell lymphoma (pcDLBCL; IR 1.2/1000000 person-years) and cutaneous follicle center lymphoma (30%; IR 0.9/1000000 person-years), followed by cutaneous marginal zone B-cell lymphoma (25%; IR 0.8/1000000 person-years). […] Overall, males had a statistically significant higher IR of CLs than females (14.0 vs 8.2/1000000 person-years, respectively; male-female IR ratio [M/F IRR] = 1.72; P .001). The M/F IRRs ranged between 1.28 to 2.55 among the various T-cell and B-cell lymphoma subtypes and were significantly elevated except for Szary syndrome (M/F IRR = 2.11, P = .05, based on small numbers) and pcDLBCL-leg type (M/F IRR = 1.19, P = .44).

• #28 A Descriptive Study of 103 Primary Cutaneous B-Cell Lymphomas: Clinical and Pathological Characteristics and Treatment from the Spanish Lymphoma Oncology Group (GOTEL)

  https://www.mdpi.com/2072-6694/16/5/1034

  Primary cutaneous B-cell lymphomas account for approximately 25% of all cutaneous lymphomas and 2% of all non-Hodgkin’s lymphomas. […] The exact incidence of this group of lymphomas is difficult to determine, but population-based data from the US Surveillance, Epidemiology, and End Results (SEER) program show an adjusted incidence of 0.31 cases per 100,000 person-years (0.4 for men and 0.23 for women), with an M/F ratio of 1.76. […] Approximately 2% of NHLs are estimated to be primary cutaneous B-cell lymphomas (PCBCLs). […] The most common histology was PCMZL (66%), followed by PCFCL (26%) and PCDLBCL-LT (8%). […] The low incidence of PCBCL has led to great variability in therapeutic management, often extrapolated from patients’ lymph node counterparts. […] The vast majority of patients (98%) were diagnosed at localized stages (I-II), which corresponds to most of the studies published with PCBCLs. […] Disease-specific survival (DSS) at 10 years was 97.2% in the whole cohort, which is also in agreement with the data known to date, reinforcing the idea that, globally, PCBCL has a very good prognosis.

• #29 Primary Cutaneous B-Cell Lymphomas

  https://atlasgeneticsoncology.org/haematological/1851/primary-cutaneous-b-cell-lymphomas?cookies_accepted=1

  Approximately 25% of all non-Hodgkin lymphoma (NHL) cases will present at an extranodal site without systemic involvement. The overall incidence of primary cutaneous lymphomas in Western countries is estimated to be 0.5 to 1 case per 100,000 people annually. The PCBCLs make up approximately 25% of all primary cutaneous lymphomas, the remainder predominantly T-cell lymphomas (Swerdlow 2013). The incidence varies geographically with lower rates in some countries (Abeldano 2018). PCMZL represents an indolent type of CBCL comprising approximately 7% of PCL and approximately 25% of CBCL. In the revised WHO classification of lymphoma, PCMZL is considered under extranodal marginal zone lymphoma of MALT type (Swerdlow 2016, Swerdlow 2017). PCMZL usually presents in the fifth decade of life, although the disease has been diagnosed in children (Kempf 2014 Aug). Men are diagnosed approximately twice as often as women. Most cases occur in non-Hispanic Whites (Kempf 2014 Mar). The PCFCL is the most common PCBCL, accounting for approximately 60% of such cases (Willemze 2005, Willemzie 2019). It is primarily a disease of middle aged to older patients (median age at onset is 51) and a male to female ratio of approximately 1.5:1 (Zinzani 2006, Bradford 2009, Swerdlow 2017). PCLBCL, leg type comprises 5% of all cutaneous lymphomas and 20% of all PCBCLs. The median age at presentation is in the mid to late seventies and is more common in females than in males with a male to female ratio of 1:3-4 (Swerdlow 2017). In contrast to the previous 2 entities, PCDLBCL-leg type is an aggressive disease and is associated with poor prognosis.

• #30 Primary Cutaneous B-Cell Lymphomas

  https://atlasgeneticsoncology.org/haematological/1851/primary-cutaneous-b-cell-lymphomas

  Primary cutaneous B-cell lymphomas (PCBCL) are a heterogeneous group of mature B-cells neoplasms that present in the skin without evidence of nodal or systemic involvement. […] The overall incidence of primary cutaneous lymphomas in Western countries is estimated to be 0.5 to 1 case per 100,000 people annually. The PCBCLs make up approximately 25% of all primary cutaneous lymphomas, the remainder predominantly T-cell lymphomas. […] The incidence varies geographically with lower rates in some countries. […] PCMZL usually presents in the fifth decade of life, although the disease has been diagnosed in children. […] Men are diagnosed approximately twice as often as women. […] Most cases occur in non-Hispanic Whites. […] The PCFCL is the most common PCBCL, accounting for approximately 60% of such cases.

• #31 Primary Cutaneous B-Cell Lymphomas

  https://atlasgeneticsoncology.org/haematological/1851/primary-cutaneous-b-cell-lymphomas

  It is primarily a disease of middle aged to older patients (median age at onset is 51) and a male to female ratio of approximately 1.5:1. […] PCLBCL, leg type comprises 5% of all cutaneous lymphomas and 20% of all PCBCLs. […] The median age at presentation is in the mid to late seventies and is more common in females than in males with a male to female ratio of 1:3-4. […] In contrast to the previous 2 entities, PCDLBCL-leg type is an aggressive disease and is associated with poor prognosis. […] Subclassification and the extent of cutaneous involvement were identified as the most relevant prognostic factors in CBCL. […] PCFCL and PCMZL are indolent types of cutaneous lymphoma with excellent prognosis. […] The 5 year survival rate is 90-95%. […] However, like their nodal counterparts, they have tendency to recur in up to 40% of cases but dissemination to extracutaneous sites is rare. […] Both PCFCL and PCMZL can transform to DLBCL with a more aggressive behavior. […] The PCDLBCL, LT on the other hand is an aggressive disease with a 5 year survival of 20-50%.

• #32 Primary Cutaneous B-Cell Lymphomas in Children and Adolescents: A SEER Population-Based Study – PubMed

  https://pubmed.ncbi.nlm.nih.gov/34417159/

  Although primary cutaneous B-cell lymphomas (PCBCL) comprise 25% of all cutaneous lymphomas, their incidence in the pediatric population is unknown, and the information on pediatric PCBCL has mostly been gathered from individual case reports or series from single centers. […] This was a population-based, retrospective cohort study of patients in 18 cancer registries in the United States diagnosed between 2000 to 2016 through the Surveillance, Epidemiology, and End Results (SEER) program. Age-adjusted incidence rates were calculated for PCBCL in pediatric (20 years) and adult (20 years) populations. […] A total of 48 pediatric and 5128 adult PCBCL cases were included. […] The age-adjusted pediatric PCBCL incidence rate (per 1,000,000 person-years) was 0.12 (95% CI 0.09-0.16). The incidence in the adult population was approximately 40-fold higher than the one observed in the pediatric group (IRR 41.4, 95% CI 31.2-56.2). […] Pediatric PCBCL is a very rare disease affecting mostly adolescents of both sexes. The major histologic subtype is marginal zone lymphoma, and the prognosis is favorable.

• #33 Cutaneous B-cell lymphoma – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/cutaneous-b-cell-lymphoma/symptoms-causes/syc-20577439

  Cutaneous B-cell lymphoma is a rare type of cancer that begins in the white blood cells. This cancer attacks the skin. Cutaneous B-cell lymphoma affects one type of germ-fighting white blood cells called B cells. These cells also are called B lymphocytes. […] Cutaneous B-cell lymphoma is a type of non-Hodgkin lymphoma. Another type of non-Hodgkin lymphoma that affects the skin is called cutaneous T-cell lymphoma. Cutaneous T-cell lymphoma is more common than cutaneous B-cell lymphoma. […] The cause of cutaneous B-cell lymphoma often isn’t known. This cancer causes a growth of cells in the skin. It starts in germ-fighting white blood cells called lymphocytes. Cutaneous B-cell lymphoma affects specific lymphocytes called B lymphocytes. […] Risk factors for cutaneous B-cell lymphoma include: Weakened immune system. If the body’s germ-fighting immune system is weakened by medicine or illness, there might be a higher risk of cutaneous B-cell lymphoma. […] There is no way to prevent cutaneous B-cell lymphoma.

• #34 Cutaneous lymphoma incidence patterns in the United States: a population-based study of 3884 cases

  https://pmc.ncbi.nlm.nih.gov/articles/PMC2686177/

  Overall 5-year relative survival rates for patients with CTCL and CBCL were high (85% and 87%, respectively). CTCL survival rates ranged from 91% for patients with MF to 40% for patients with Szary syndrome. CBCL survival rates ranged from 93% to 96% for patients with cutaneous follicle center lymphoma or cutaneous marginal B-cell lymphoma. Survival rates were much lower among patients with pcDLBCL-leg (46%) than pcDLBCL-other (81%). Five-year relative survival rates were similar among males and females with CTCL (84% and 88%, respectively) and CBCL (both 86%). Overall CTCL and CBCL survival rates were highest among whites (both 87%) and lowest in blacks (82% and 83%, respectively). […] In conclusion, our result of primary cutaneous follicular center lymphoma and pcDLCL should be interpreted with caution until a future population-based study conducts a detailed histologic review of CBCL in the US and our findings are confirmed.

• #35

  https://link.springer.com/article/10.1007/s40257-022-00704-0

  Primary cutaneous lymphomas are a rare group of diseases, with an estimated incidence of 0.51 case per 100,000 people per year. Primary cutaneous B-cell lymphomas (pCBCLs) represent 25-30% of all primary cutaneous lymphomas. […] To date, treatment recommendations for cutaneous B-cell lymphomas have been largely based on small retrospective studies and institutional experience. […] The pharmacotherapeutic landscape has recently expanded to include drugs that may modify the underlying disease pathology. […] Primary cutaneous B-cell lymphomas have a broad spectrum of clinical presentations, which makes diagnostic and therapeutic strategies challenging. […] Incidence is estimated at 1 per 100,000 persons/year and increases with age. […] Overall prognosis is favorable, with a 5-year survival rate of 95-100%.

• #36 Clinical Features, Epidemiology, Management and Prognosis of Patients With Primary Cutaneous Marginal Zone Lymphoma: An Observational Study | Actas Dermo-Sifiliográficas

  https://www.actasdermo.org/es-clinical-features-epidemiology-management-prognosis-articulo-S0001731023006919

  Primary cutaneous marginal zone lymphoma (PCMZL) is a low-grade B cell primary skin lymphoma, usually found in male adults around 50-55 years old. This disease can present as single or multiple erythematous macules, plaques, or tumors. The most common locations are the trunk and upper limbs. […] Treatment of this disease is based on only a limited number of short case series, and well-designed clinical trials are lacking; therefore, strong evidence is scarce. Currently, solitary lesions are treated via surgery or local radiotherapy, whereas multiple lesions are treated with radiotherapy, intravenous administration of rituximab, or watchful follow-up. The prognosis of this type of lymphoma is excellent, with a five-year survival rate of 95-100%. Nevertheless, PCMZL skin relapses are common (44-50%).

• #37 Primary Cutaneous B-Cell Lymphomas

  https://atlasgeneticsoncology.org/haematological/1851/primary-cutaneous-b-cell-lymphomas

  It is primarily a disease of middle aged to older patients (median age at onset is 51) and a male to female ratio of approximately 1.5:1. […] PCLBCL, leg type comprises 5% of all cutaneous lymphomas and 20% of all PCBCLs. […] The median age at presentation is in the mid to late seventies and is more common in females than in males with a male to female ratio of 1:3-4. […] In contrast to the previous 2 entities, PCDLBCL-leg type is an aggressive disease and is associated with poor prognosis. […] Subclassification and the extent of cutaneous involvement were identified as the most relevant prognostic factors in CBCL. […] PCFCL and PCMZL are indolent types of cutaneous lymphoma with excellent prognosis. […] The 5 year survival rate is 90-95%. […] However, like their nodal counterparts, they have tendency to recur in up to 40% of cases but dissemination to extracutaneous sites is rare. […] Both PCFCL and PCMZL can transform to DLBCL with a more aggressive behavior. […] The PCDLBCL, LT on the other hand is an aggressive disease with a 5 year survival of 20-50%.

• #38 Cutaneous lymphoma incidence patterns in the United States: a population-based study of 3884 cases

  https://pmc.ncbi.nlm.nih.gov/articles/PMC2686177/

  Overall 5-year relative survival rates for patients with CTCL and CBCL were high (85% and 87%, respectively). CTCL survival rates ranged from 91% for patients with MF to 40% for patients with Szary syndrome. CBCL survival rates ranged from 93% to 96% for patients with cutaneous follicle center lymphoma or cutaneous marginal B-cell lymphoma. Survival rates were much lower among patients with pcDLBCL-leg (46%) than pcDLBCL-other (81%). Five-year relative survival rates were similar among males and females with CTCL (84% and 88%, respectively) and CBCL (both 86%). Overall CTCL and CBCL survival rates were highest among whites (both 87%) and lowest in blacks (82% and 83%, respectively). […] In conclusion, our result of primary cutaneous follicular center lymphoma and pcDLCL should be interpreted with caution until a future population-based study conducts a detailed histologic review of CBCL in the US and our findings are confirmed.

• #39 Cutaneous B-Cell Lymphoma: Overview, Pathophysiology, Epidemiology

  https://emedicine.medscape.com/article/1099540-overview

  The frequency of cutaneous lymphomas is 0.3 case per 100,000 population per year, with 10% (in the United States) to 20% (in Europe) being cutaneous B-cell lymphomas (CBCLs), marginal zone lymphomas, or follicle center lymphomas (FCLs). One study reported a slightly lower rate of cutaneous B-cell lymphoma in Argentina compared with the United States and Europe. The 5-year overall survival rate for most cases of CBCL is greater than 90%, except in diffuse large B-cell lymphoma (DLBCL), for which the 5-year survival rate is 20-50%. […] No significant statistical data are available for CBCL with regard to any predisposition based on race. However, with regard to sex and age, DLBCL is predominantly seen in elderly women.

• #40 Primary Cutaneous B-Cell Lymphomas

  https://atlasgeneticsoncology.org/haematological/1851/primary-cutaneous-b-cell-lymphomas

  It is primarily a disease of middle aged to older patients (median age at onset is 51) and a male to female ratio of approximately 1.5:1. […] PCLBCL, leg type comprises 5% of all cutaneous lymphomas and 20% of all PCBCLs. […] The median age at presentation is in the mid to late seventies and is more common in females than in males with a male to female ratio of 1:3-4. […] In contrast to the previous 2 entities, PCDLBCL-leg type is an aggressive disease and is associated with poor prognosis. […] Subclassification and the extent of cutaneous involvement were identified as the most relevant prognostic factors in CBCL. […] PCFCL and PCMZL are indolent types of cutaneous lymphoma with excellent prognosis. […] The 5 year survival rate is 90-95%. […] However, like their nodal counterparts, they have tendency to recur in up to 40% of cases but dissemination to extracutaneous sites is rare. […] Both PCFCL and PCMZL can transform to DLBCL with a more aggressive behavior. […] The PCDLBCL, LT on the other hand is an aggressive disease with a 5 year survival of 20-50%.

• #41 Cutaneous lymphoma incidence patterns in the United States: a population-based study of 3884 cases

  https://pmc.ncbi.nlm.nih.gov/articles/PMC2686177/

  Overall 5-year relative survival rates for patients with CTCL and CBCL were high (85% and 87%, respectively). CTCL survival rates ranged from 91% for patients with MF to 40% for patients with Szary syndrome. CBCL survival rates ranged from 93% to 96% for patients with cutaneous follicle center lymphoma or cutaneous marginal B-cell lymphoma. Survival rates were much lower among patients with pcDLBCL-leg (46%) than pcDLBCL-other (81%). Five-year relative survival rates were similar among males and females with CTCL (84% and 88%, respectively) and CBCL (both 86%). Overall CTCL and CBCL survival rates were highest among whites (both 87%) and lowest in blacks (82% and 83%, respectively). […] In conclusion, our result of primary cutaneous follicular center lymphoma and pcDLCL should be interpreted with caution until a future population-based study conducts a detailed histologic review of CBCL in the US and our findings are confirmed.

• #42

  https://link.springer.com/article/10.1007/s40257-022-00704-0

  While local recurrence is common, with cutaneous relapse in up to 50% of patients, extracutaneous dissemination is rare and reported in approximately 4% of patients. […] Primary cutaneous follicle center lymphoma is the most common type of pCBCL, representing 13% of all primary cutaneous lymphomas. […] Overall prognosis is good, with a 5-year survival rate of 95%, although localization to the lower extremities is associated with a worse prognosis and a 5-year survival of 41%. […] Primary cutaneous diffuse large B-cell lymphoma, leg type is the least common variant of pCBCL, accounting for 2% of all primary cutaneous lymphomas. […] The 5-year survival rate is 56% and extracutaneous dissemination occurs in 10% of cases.

• #43 Clinical Features, Epidemiology, Management and Prognosis of Patients With Primary Cutaneous Marginal Zone Lymphoma: An Observational Study | Actas Dermo-Sifiliográficas

  https://www.actasdermo.org/es-clinical-features-epidemiology-management-prognosis-articulo-S0001731023006919

  Primary cutaneous marginal zone lymphoma (PCMZL) is a low-grade B cell primary skin lymphoma, usually found in male adults around 50-55 years old. This disease can present as single or multiple erythematous macules, plaques, or tumors. The most common locations are the trunk and upper limbs. […] Treatment of this disease is based on only a limited number of short case series, and well-designed clinical trials are lacking; therefore, strong evidence is scarce. Currently, solitary lesions are treated via surgery or local radiotherapy, whereas multiple lesions are treated with radiotherapy, intravenous administration of rituximab, or watchful follow-up. The prognosis of this type of lymphoma is excellent, with a five-year survival rate of 95-100%. Nevertheless, PCMZL skin relapses are common (44-50%).

• #44

  https://link.springer.com/article/10.1007/s40257-022-00704-0

  While local recurrence is common, with cutaneous relapse in up to 50% of patients, extracutaneous dissemination is rare and reported in approximately 4% of patients. […] Primary cutaneous follicle center lymphoma is the most common type of pCBCL, representing 13% of all primary cutaneous lymphomas. […] Overall prognosis is good, with a 5-year survival rate of 95%, although localization to the lower extremities is associated with a worse prognosis and a 5-year survival of 41%. […] Primary cutaneous diffuse large B-cell lymphoma, leg type is the least common variant of pCBCL, accounting for 2% of all primary cutaneous lymphomas. […] The 5-year survival rate is 56% and extracutaneous dissemination occurs in 10% of cases.

• #45

  https://link.springer.com/article/10.1007/s40257-022-00704-0

  While local recurrence is common, with cutaneous relapse in up to 50% of patients, extracutaneous dissemination is rare and reported in approximately 4% of patients. […] Primary cutaneous follicle center lymphoma is the most common type of pCBCL, representing 13% of all primary cutaneous lymphomas. […] Overall prognosis is good, with a 5-year survival rate of 95%, although localization to the lower extremities is associated with a worse prognosis and a 5-year survival of 41%. […] Primary cutaneous diffuse large B-cell lymphoma, leg type is the least common variant of pCBCL, accounting for 2% of all primary cutaneous lymphomas. […] The 5-year survival rate is 56% and extracutaneous dissemination occurs in 10% of cases.

• #46 Cutaneous lymphoma incidence patterns in the United States: a population-based study of 3884 cases

  https://pmc.ncbi.nlm.nih.gov/articles/PMC2686177/

  Overall 5-year relative survival rates for patients with CTCL and CBCL were high (85% and 87%, respectively). CTCL survival rates ranged from 91% for patients with MF to 40% for patients with Szary syndrome. CBCL survival rates ranged from 93% to 96% for patients with cutaneous follicle center lymphoma or cutaneous marginal B-cell lymphoma. Survival rates were much lower among patients with pcDLBCL-leg (46%) than pcDLBCL-other (81%). Five-year relative survival rates were similar among males and females with CTCL (84% and 88%, respectively) and CBCL (both 86%). Overall CTCL and CBCL survival rates were highest among whites (both 87%) and lowest in blacks (82% and 83%, respectively). […] In conclusion, our result of primary cutaneous follicular center lymphoma and pcDLCL should be interpreted with caution until a future population-based study conducts a detailed histologic review of CBCL in the US and our findings are confirmed.

• #47 Cutaneous lymphoma incidence patterns in the United States: a population-based study of 3884 cases

  https://pmc.ncbi.nlm.nih.gov/articles/PMC2686177/

  Overall 5-year relative survival rates for patients with CTCL and CBCL were high (85% and 87%, respectively). CTCL survival rates ranged from 91% for patients with MF to 40% for patients with Szary syndrome. CBCL survival rates ranged from 93% to 96% for patients with cutaneous follicle center lymphoma or cutaneous marginal B-cell lymphoma. Survival rates were much lower among patients with pcDLBCL-leg (46%) than pcDLBCL-other (81%). Five-year relative survival rates were similar among males and females with CTCL (84% and 88%, respectively) and CBCL (both 86%). Overall CTCL and CBCL survival rates were highest among whites (both 87%) and lowest in blacks (82% and 83%, respectively). […] In conclusion, our result of primary cutaneous follicular center lymphoma and pcDLCL should be interpreted with caution until a future population-based study conducts a detailed histologic review of CBCL in the US and our findings are confirmed.

• #48 Primary cutaneous B cell lymphoma: Clinical features, diagnosis and treatment

  https://www.wjgnet.com/2218-6190/full/v4/i1/50.htm

  The diagnosis of the disease can be established by histopathologic examination of the skin biopsy and exclusion of a systemic disease. Excisional biopsy is preferred but if an excisional biopsy is not appropriate, it can be substituted by a punch biopsy of an adequate length. […] The differentiation of PCMZL from reactive skin changes and other cutaneous lymphomas is important. Morphologic and immunophenotypic features and demonstration of clonality will aid in the differential diagnosis. […] Treatment depends on the symptoms of the patient, stage of the disease and the number of the lesions. The treatment options include antibiotics, rituximab, chemotherapy, intralesional interferon alfa, radiotherapy and excision. […] In patients with very extensive skin lesions, systemic rituximab is the first choice of treatment. Rituximab, systemic or intralesional, have been used in limited number of patients with PCFCL in the literature. CR has been achieved in 10/11 PCFCL patients treated with systemic rituximab while 3 patients had relapse after achieving CR. […] PCLBCL-LT is a more aggressive disease with extra-cutaneous progression, lower remission and higher relapse rates. Multiple skin lesions, bcl-2 expression, are independent prognostic factors in these patients.

• #49 Primary Cutaneous Diffuse Large B-cell Lymphoma, Leg Type

  https://www.webpathology.com/images/hematopathology/mature-b-cell-neoplasms—part-ii/primary-cutaneous-b-cell-lymphomas/37742

  Primary cutaneous diffuse large B-cell lymphoma, leg type (PCDLBCL, LT) is an aggressive tumor with poor prognosis. The 5-yr survival rate is 50% compared to 90% for cutaneous marginal zone lymphoma and 95% for primary cutaneous follicle center lymphoma. The treatment is similar to that for systemic diffuse large B-cell lymphoma and consists of anti-CD20 antibody Rituximab + CHOP-like regimen. Relapses occur in 55-69% of cases. With the addition of rituximab, the 5-yr disease-specific survival rate has increased from 50% to about 70%. Indicators of poor prognosis include: loss of CDKN2A by deletion or hypermethylation, MYD88 L265P mutation, and multifocal skin lesions. […] Primary cutaneous B-cell lymphomas (PCBCLs) are a heterogenous group of mostly low-grade B-cell lymphomas that involve skin with no evidence of extracutaneous disease at presentation. They are less common than cutaneous T-cell lymphomas and make up 20-25% of all primary cutaneous lymphomas. PCBCLs must be distinguished from systemic B-cell lymphomas with secondary skin involvement as the biologic behavior, treatment, and prognosis are completely different for the two groups.

• #50 Primary cutaneous B cell lymphoma: Clinical features, diagnosis and treatment

  https://www.wjgnet.com/2218-6190/full/v4/i1/50.htm

  PCBCL is defined as B cell lymphomas that present in the skin without any evidence of extra-cutaneous involvement at diagnosis. They are the second most common type of PCL, accounting for 25%-30%. Since the prognosis and treatment differ from systemic lymphomas involving the skin, differential diagnosis becomes crucial. […] PCBCL is a heterogeneous group of diseases consisting different B cell lymphomas with distinct treatment and prognosis. Their clinical presentation is relatively uniform, mostly manifested by nodules. The indolent forms, primary cutaneous marginal zone lymphoma (PCMZL) and primary cutaneous follicle center lymphoma (PCFCL) are often confined to the skin during the course of the disease. […] PCBCL are divided into 5 subclasses according to World Health Organization and European Organization of Research and Treatment of Cancer (WHO-EORTC) classification.

• #51 Secondary Cutaneous Diffuse Large B-cell Lymphoma has a Higher International Prognostic Index Score and Worse Prognosis Than Diffuse Large B-cell Lymphoma, Leg Type | HTML | Acta Dermato-Venereologica

  https://www.medicaljournals.se/acta/content/html/10.2340/00015555-2139

  Diffuse large B-cell lymphoma (DLBCL) can be separated into 2 groups: nodal and extranodal disease. […] A total of 44 patients with cutaneous DLBCL were classified as primary cutaneous DLBCL, leg type or cutaneous DLBCL secondary to primary disease. […] Secondary cutaneous DLBCL was more commonly associated with an advanced stage and higher International Prognostic Index score than DLBCL, leg type. […] Survival outcomes and prognostic factors differ depending on the primary tumour site of cutaneous DLBCL. […] The classification of primary cutaneous DLBCL was introduced to designate a specific subtype of lymphoma with characteristic clinicopathological, immunohistochemical, and evolutive features. […] Depending on the primary site of involvement, DLBCL with cutaneous involvement can be divided into 2 distinct subsets: (i) primary cutaneous DLBCL, which initially presents in the skin, and (ii) DLBCL accompanied by secondary spread to the skin.

• #52 Secondary Cutaneous Diffuse Large B-cell Lymphoma has a Higher International Prognostic Index Score and Worse Prognosis Than Diffuse Large B-cell Lymphoma, Leg Type | HTML | Acta Dermato-Venereologica

  https://www.medicaljournals.se/acta/content/html/10.2340/00015555-2139

  Secondary cutaneous involvement in systemic DLBCL suggests that the patient is at high risk of disease progression and poor prognosis, which is not affected by the prognostic factors of conventional DLBCL. […] In conclusion, the prognostic factors that influence survival differed depending on the primary tumour site of cutaneous DLBCL. […] Extensive cutaneous lesions and cutaneous involvement soon after initial diagnosis predicted a poorer OS in patients with secondary cutaneous DLBCL.

• #53 A Descriptive Study of 103 Primary Cutaneous B-Cell Lymphomas: Clinical and Pathological Characteristics and Treatment from the Spanish Lymphoma Oncology Group (GOTEL)

  https://www.mdpi.com/2072-6694/16/5/1034

  Primary cutaneous B-cell lymphomas account for approximately 25% of all cutaneous lymphomas and 2% of all non-Hodgkin’s lymphomas. […] The exact incidence of this group of lymphomas is difficult to determine, but population-based data from the US Surveillance, Epidemiology, and End Results (SEER) program show an adjusted incidence of 0.31 cases per 100,000 person-years (0.4 for men and 0.23 for women), with an M/F ratio of 1.76. […] Approximately 2% of NHLs are estimated to be primary cutaneous B-cell lymphomas (PCBCLs). […] The most common histology was PCMZL (66%), followed by PCFCL (26%) and PCDLBCL-LT (8%). […] The low incidence of PCBCL has led to great variability in therapeutic management, often extrapolated from patients’ lymph node counterparts. […] The vast majority of patients (98%) were diagnosed at localized stages (I-II), which corresponds to most of the studies published with PCBCLs. […] Disease-specific survival (DSS) at 10 years was 97.2% in the whole cohort, which is also in agreement with the data known to date, reinforcing the idea that, globally, PCBCL has a very good prognosis.

• #54

  https://link.springer.com/article/10.1007/s40257-022-00704-0

  Primary cutaneous lymphomas are a rare group of diseases, with an estimated incidence of 0.51 case per 100,000 people per year. Primary cutaneous B-cell lymphomas (pCBCLs) represent 25-30% of all primary cutaneous lymphomas. […] To date, treatment recommendations for cutaneous B-cell lymphomas have been largely based on small retrospective studies and institutional experience. […] The pharmacotherapeutic landscape has recently expanded to include drugs that may modify the underlying disease pathology. […] Primary cutaneous B-cell lymphomas have a broad spectrum of clinical presentations, which makes diagnostic and therapeutic strategies challenging. […] Incidence is estimated at 1 per 100,000 persons/year and increases with age. […] Overall prognosis is favorable, with a 5-year survival rate of 95-100%.

• #55 Clinical Features, Epidemiology, Management and Prognosis of Patients With Primary Cutaneous Marginal Zone Lymphoma: An Observational Study | Actas Dermo-Sifiliográficas

  https://www.actasdermo.org/es-clinical-features-epidemiology-management-prognosis-articulo-S0001731023006919

  Our experience with patients with PCLZM in our hospital is similar to other centers; lesions are more common in 50-60-year-old men with a relatively high recurrence rate but an excellent survival rate. Primary staging in patients with PCMZL should include a physical examination, laboratory tests with a complete blood count and lactate dehydrogenase levels, skin biopsy, and complete body CT or FDG-PET scan. A bone marrow biopsy is not indicated for PCMZL. […] Concerning treatment for localized lesions, local electron radiotherapy (20-35Gy and 1-1.5cm margin) or wide margin excision are indicated as the first-choice treatments. Some authors found no difference in recurrence rates between the two treatment groups, while others showed a higher relapse rate with excised lesions, although this difference was not statistically significant.

• #56 Clinical Features, Epidemiology, Management and Prognosis of Patients With Primary Cutaneous Marginal Zone Lymphoma: An Observational Study | Actas Dermo-Sifiliográficas

  https://www.actasdermo.org/es-clinical-features-epidemiology-management-prognosis-articulo-S0001731023006919

  Our experience with patients with PCLZM in our hospital is similar to other centers; lesions are more common in 50-60-year-old men with a relatively high recurrence rate but an excellent survival rate. Primary staging in patients with PCMZL should include a physical examination, laboratory tests with a complete blood count and lactate dehydrogenase levels, skin biopsy, and complete body CT or FDG-PET scan. A bone marrow biopsy is not indicated for PCMZL. […] Concerning treatment for localized lesions, local electron radiotherapy (20-35Gy and 1-1.5cm margin) or wide margin excision are indicated as the first-choice treatments. Some authors found no difference in recurrence rates between the two treatment groups, while others showed a higher relapse rate with excised lesions, although this difference was not statistically significant.

• #57 Clinical Features, Epidemiology, Management and Prognosis of Patients With Primary Cutaneous Marginal Zone Lymphoma: An Observational Study | Actas Dermo-Sifiliográficas

  https://www.actasdermo.org/es-clinical-features-epidemiology-management-prognosis-articulo-S0001731023006919

  The prognosis of patients with PCMZL is excellent, with a 5-year survival rate of 90% (93% and 95%). An extended follow-up period for patients with PCLZM, sometimes as long as five years after a complete response to a primary treatment, is recommended due to possible late relapse. The optimal management of these patients requires a multidisciplinary approach. Multicentric studies and clinical trials are needed to assess the best therapeutic approach and management of patients with PCMZL.

• #58

  https://link.springer.com/article/10.1007/s40257-022-00704-0

  While local recurrence is common, with cutaneous relapse in up to 50% of patients, extracutaneous dissemination is rare and reported in approximately 4% of patients. […] Primary cutaneous follicle center lymphoma is the most common type of pCBCL, representing 13% of all primary cutaneous lymphomas. […] Overall prognosis is good, with a 5-year survival rate of 95%, although localization to the lower extremities is associated with a worse prognosis and a 5-year survival of 41%. […] Primary cutaneous diffuse large B-cell lymphoma, leg type is the least common variant of pCBCL, accounting for 2% of all primary cutaneous lymphomas. […] The 5-year survival rate is 56% and extracutaneous dissemination occurs in 10% of cases.

• #59 Skin lymphoma | Lymphoma Action

  https://lymphoma-action.org.uk/types-lymphoma/skin-lymphoma

  Skin lymphomas are rare. In the UK, around seven people in every million develop a skin lymphoma each year. T-cell skin lymphomas make up about 7 in 10 cases of skin lymphoma. […] In general, skin lymphomas are slightly more common in men than women. They are usually diagnosed in older people, most often those aged between 50 and 74. Only about 1 in 5 skin lymphomas affect people under 50. Very rarely, some types of skin lymphoma can develop in children. […] Although skin lymphomas are a form of cancer, in many cases they are very slow growing and do not affect life expectancy. They behave more like a long-term (chronic) skin condition than a cancer. […] Many people with a slow-growing skin lymphoma don’t need treatment straightaway. Instead, the doctor monitors the condition. This is called active monitoring or watch and wait. It is often used for early-stage lymphoma that would not benefit from treatment. […] If you have been diagnosed with skin lymphoma, you can find more detail on the treatment you might have on our pages about specific skin lymphomas: cutaneous T-cell lymphomas (CTCLs), including mycosis fungoides and Szary syndrome, cutaneous B-cell lymphomas (CBCLs).

• #60 Clinical Features, Epidemiology, Management and Prognosis of Patients With Primary Cutaneous Marginal Zone Lymphoma: An Observational Study | Actas Dermo-Sifiliográficas

  https://www.actasdermo.org/es-clinical-features-epidemiology-management-prognosis-articulo-S0001731023006919

  The prognosis of patients with PCMZL is excellent, with a 5-year survival rate of 90% (93% and 95%). An extended follow-up period for patients with PCLZM, sometimes as long as five years after a complete response to a primary treatment, is recommended due to possible late relapse. The optimal management of these patients requires a multidisciplinary approach. Multicentric studies and clinical trials are needed to assess the best therapeutic approach and management of patients with PCMZL.

• #61 Cutaneous lymphoma incidence patterns in the United States: a population-based study of 3884 cases

  https://pmc.ncbi.nlm.nih.gov/articles/PMC2686177/

  There have been no prior large population-based studies focusing on cutaneous lymphomas (CL) in the United States. Using the Surveillance, Epidemiology and End Results (SEER) program data, we analyzed age-adjusted CL incidence rates (IRs) and survival rates by sex and race/ethnicity. There were 3884 CLs diagnosed during 2001-2005. Cutaneous T-cell lymphomas (CTCLs) accounted for 71% (age-adjusted incidence rate [IR] = 7.7/1000000 person-years), whereas cutaneous B-cell lymphomas (CBCLs) accounted for 29% (IR = 3.1/1000000 person-years). Males had a statistically significant higher IR of CL than females (14.0 vs 8.2/1000000 person-years, respectively; male-female IR ratio [M/F IRR] = 1.72; P .001). CL IRs were highest among blacks and non-Hispanic whites (both 11.5/1000000 person-years), followed by Hispanic whites (7.9) and Asian/Pacific Islanders (7.1). The CTCL IR was highest among blacks (10.0/1000000 person-years), whereas the CBCL IR was highest among non-Hispanic whites (3.5). Over the past 25 years, the CL IR increased from 5.0/1000000 person-years during 1980-1982 to 14.3 during 2001-2003. During 2004-2005, the CL IR was 12.7. This recent apparent change could be incomplete case ascertainment or potential leveling off of IRs. CLs rates vary markedly by race and sex, supporting the notion that they represent distinct disease entities.

• #62

  https://journals.lww.com/hemasphere/fulltext/2023/08003/p1109__epidemiology_of_cutaneous_t_cell_lymphomas_.1007.aspx

  Cutaneous T cell lymphomas (CTCL) represent a rare and heterogeneous group of lymphomas that present in the skin without extracutaneous manifestations at the time of diagnosis. […] Data on the epidemiology of CTCL are primarily based on patient cohorts seen in these expert centers, frequently influenced by referral bias. […] Several epidemiological studies have been reported, but no epidemiologic data are currently available in Italy. […] One of the main goals of the MDT was to register the number and types of cutaneous lymphomas across Marche in order to estimate the incidence as well as survival rate and other outcome data. […] During the study period (1995-2020), 492 patients with newly diagnosed CTCL were reported and evaluated. […] CTCL showed a moderately higher prevalence in men than in women (M: F = 2,3: 1) and median age at diagnosis was 62 years (range, 10-90), with significative differences reported between subtype. […] The real epidemiology of CTCL is still a matter of debate. […] Currently, the Italian Cutaneous Lymphoma Study-Group (ICLSG-named Commissione Linfomi Cutanei) effort is to create a national register for CTCL, based on MDT registry, to provide information from a national perspective.

• #63 Cutaneous lymphoma incidence patterns in the United States: a population-based study of 3884 cases

  https://pmc.ncbi.nlm.nih.gov/articles/PMC2686177/

  There have been no prior large population-based studies focusing on cutaneous lymphomas (CL) in the United States. Using the Surveillance, Epidemiology and End Results (SEER) program data, we analyzed age-adjusted CL incidence rates (IRs) and survival rates by sex and race/ethnicity. There were 3884 CLs diagnosed during 2001-2005. Cutaneous T-cell lymphomas (CTCLs) accounted for 71% (age-adjusted incidence rate [IR] = 7.7/1000000 person-years), whereas cutaneous B-cell lymphomas (CBCLs) accounted for 29% (IR = 3.1/1000000 person-years). Males had a statistically significant higher IR of CL than females (14.0 vs 8.2/1000000 person-years, respectively; male-female IR ratio [M/F IRR] = 1.72; P .001). CL IRs were highest among blacks and non-Hispanic whites (both 11.5/1000000 person-years), followed by Hispanic whites (7.9) and Asian/Pacific Islanders (7.1). The CTCL IR was highest among blacks (10.0/1000000 person-years), whereas the CBCL IR was highest among non-Hispanic whites (3.5). Over the past 25 years, the CL IR increased from 5.0/1000000 person-years during 1980-1982 to 14.3 during 2001-2003. During 2004-2005, the CL IR was 12.7. This recent apparent change could be incomplete case ascertainment or potential leveling off of IRs. CLs rates vary markedly by race and sex, supporting the notion that they represent distinct disease entities.

• #64 Cutaneous lymphoma incidence patterns in the United States: a population-based study of 3884 cases

  https://pmc.ncbi.nlm.nih.gov/articles/PMC2686177/

  Overall 5-year relative survival rates for patients with CTCL and CBCL were high (85% and 87%, respectively). CTCL survival rates ranged from 91% for patients with MF to 40% for patients with Szary syndrome. CBCL survival rates ranged from 93% to 96% for patients with cutaneous follicle center lymphoma or cutaneous marginal B-cell lymphoma. Survival rates were much lower among patients with pcDLBCL-leg (46%) than pcDLBCL-other (81%). Five-year relative survival rates were similar among males and females with CTCL (84% and 88%, respectively) and CBCL (both 86%). Overall CTCL and CBCL survival rates were highest among whites (both 87%) and lowest in blacks (82% and 83%, respectively). […] In conclusion, our result of primary cutaneous follicular center lymphoma and pcDLCL should be interpreted with caution until a future population-based study conducts a detailed histologic review of CBCL in the US and our findings are confirmed.

• #65

  https://journals.lww.com/hemasphere/fulltext/2023/08003/p1109__epidemiology_of_cutaneous_t_cell_lymphomas_.1007.aspx

  Cutaneous T cell lymphomas (CTCL) represent a rare and heterogeneous group of lymphomas that present in the skin without extracutaneous manifestations at the time of diagnosis. […] Data on the epidemiology of CTCL are primarily based on patient cohorts seen in these expert centers, frequently influenced by referral bias. […] Several epidemiological studies have been reported, but no epidemiologic data are currently available in Italy. […] One of the main goals of the MDT was to register the number and types of cutaneous lymphomas across Marche in order to estimate the incidence as well as survival rate and other outcome data. […] During the study period (1995-2020), 492 patients with newly diagnosed CTCL were reported and evaluated. […] CTCL showed a moderately higher prevalence in men than in women (M: F = 2,3: 1) and median age at diagnosis was 62 years (range, 10-90), with significative differences reported between subtype. […] The real epidemiology of CTCL is still a matter of debate. […] Currently, the Italian Cutaneous Lymphoma Study-Group (ICLSG-named Commissione Linfomi Cutanei) effort is to create a national register for CTCL, based on MDT registry, to provide information from a national perspective.

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