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Current Opinion in Hematology Mar 2020Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and aggressive hematologic malignancy with historically poor outcomes. It typically manifests as... (Review)
Review
PURPOSE OF REVIEW
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and aggressive hematologic malignancy with historically poor outcomes. It typically manifests as asymptomatic skin lesions and cytopenias, which result from bone marrow involvement. Less commonly, it will present in lymph nodes or visceral organs as well. Although rare, BPDCN has been discussed more frequently in recent years as new drugs have been developed that could be effective at treating this disease.
RECENT FINDINGS
Until recently, treatment for BPDCN commonly included intensive chemotherapy regimens, which are generally reserved for management of acute myeloid leukemia or acute lymphoblastic leukemia. However, in 2018 tagraxofusp (SL-401) was approved as the only treatment specifically indicated for BPDCN. Additional clinical trials are ongoing evaluating the efficacy of newer agents, which could potentially further improve the long-term outcomes for patients with BPDCN.
SUMMARY
This manuscript reviews the diagnosis, manifestations and treatment of BPDCN.
Topics: Dendritic Cells; Humans; Skin Neoplasms; Soft Tissue Neoplasms
PubMed: 31972688
DOI: 10.1097/MOH.0000000000000569 -
Medicine Feb 2023Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a highly aggressive and extremely rare hematologic disease with a poor prognosis, involving mainly the skin and... (Review)
Review
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a highly aggressive and extremely rare hematologic disease with a poor prognosis, involving mainly the skin and bone marrow. The immunophenotype of these tumor cells is characterized by the expression of CD4, CD56, CD123, TCL-1, and CD303. To date, no consensus has been reached on the standard of care for BPDCN. Currently, clinical treatment is mainly based on high-dose chemotherapy combined with hematopoietic stem cell transplantation. However, this treatment method has limitations for elderly, frail, and relapsed/refractory patients. In recent years, breakthroughs in molecular biology and genetics have not only provided new ideas for the diagnosis of BPDCN but also helped develop targeted treatment strategies for this disease. The emergence of targeted drugs has filled the gap left by traditional therapies and shown great clinical promise. This article focuses on the latest advances in genetics and targeted therapies for BPDCN, especially the emerging therapies that may provide new ideas for the clinical treatment of BPDCN.
Topics: Humans; Dendritic Cells; Hematologic Neoplasms; Hematopoietic Stem Cell Transplantation; Myeloproliferative Disorders; Skin Neoplasms
PubMed: 36800625
DOI: 10.1097/MD.0000000000032904 -
Journal of the American Academy of... Aug 2021Skin cancer is the most common cancer worldwide.
BACKGROUND
Skin cancer is the most common cancer worldwide.
OBJECTIVE
To evaluate the burden of skin cancer in the US from 1990 to 2019.
METHODS
Age-standardized incidence, prevalence, disability-adjusted life years (DALY), and mortality rates from skin cancer in the US were evaluated from the Global Burden of Disease 2019.
RESULTS
Incidence, prevalence, DALY, and mortality rates for melanoma per 100,000 persons in 2019 were 17.0, 138, 64.8, and 2.2, respectively; for squamous cell carcinoma, rates were 262, 314, 26.6, and 0.8, respectively; and for basal cell carcinoma, rates were 525, 51.2, 0.2, and zero, respectively. Incidence and prevalence rates of melanoma and non-melanoma skin cancer (NMSC) have increased since 1990, while mortality rates have remained fairly stable. Males have had higher incidence, prevalence, DALY, and mortality rates from melanoma and NMSC every year since 1990. Incidence and prevalence of melanoma was relatively higher in the northern half of the US than in the southern half.
LIMITATIONS
Global Burden of Disease is derived from estimation and mathematical modeling.
CONCLUSIONS
Health care professionals can utilize differences and trends noted in this study to guide allocation of resources to reduce incidence and morbidity from skin cancer.
Topics: Adult; Aged; Aged, 80 and over; Carcinoma, Basal Cell; Carcinoma, Squamous Cell; Cost of Illness; Disability Evaluation; Female; Humans; Incidence; Male; Melanoma; Middle Aged; Prevalence; Quality-Adjusted Life Years; Skin Neoplasms; Time Factors; United States
PubMed: 33852922
DOI: 10.1016/j.jaad.2021.03.109 -
Journal Der Deutschen Dermatologischen... Apr 2023Squamous cell carcinoma of the skin (cSCC) is one of the most common cancers of the Caucasian population and accounts for 20% of all cutaneous tumors. An S3 guideline... (Review)
Review
Squamous cell carcinoma of the skin (cSCC) is one of the most common cancers of the Caucasian population and accounts for 20% of all cutaneous tumors. An S3 guideline from the German Guideline Program in Oncology has been available since 2019 and was updated in 2022. The diagnosis of cSCC is based on clinical examination. Excision and histological confirmation are required for clinically suspicious lesions to allow for prognostic assessment and correct treatment. The treatment of first choice is excision with complete histological assessment of the surgical margins. Adjuvant radiation therapy may be considered if there is a high risk of recurrence. The immune checkpoint inhibitor cemiplimab is approved and recommended as the treatment of first choice for locally advanced or metastatic cSCC in Europe. If contraindications are present, chemotherapy, EGFR inhibitors, or palliative radiation therapy may be used. Surveillance should be performed in a risk-stratified manner and includes a dermatological control supplemented by sonography examinations in high-risk patients. Much research is still needed for solid organ transplant patients, concomitant hematologic diseases, and cSCC showing primary or acquired resistance to immunotherapies. Current developments include new drug combinations, intralesional therapies alone or in combination with immune checkpoint inhibitors, and neoadjuvant approaches.
Topics: Humans; Carcinoma, Squamous Cell; Skin Neoplasms; Neoplasm Staging; Skin; Prognosis
PubMed: 36999577
DOI: 10.1111/ddg.15052 -
The British Journal of Dermatology May 2023Research has linked homelessness with an increased risk of skin conditions. However, representative studies of diagnosis-specific information on skin conditions in...
BACKGROUND
Research has linked homelessness with an increased risk of skin conditions. However, representative studies of diagnosis-specific information on skin conditions in people experiencing homelessness are lacking.
OBJECTIVES
To examine the association between homelessness and diagnosed skin conditions, prescribed medication and type of -consultation.
METHODS
This cohort study included data from the Danish nationwide health, social and administrative registers from 1 January 1999 to 31 December 2018. All people of Danish origin living in Denmark and aged at least 15 years at some point during the study period were included. Homelessness, measured by homeless shelter contacts, was the exposure. The outcome was any diagnosis of a skin disorder and specific skin disorders recorded in the Danish National Patient Register. Information on diagnostic consultation type (i.e. dermatological, nondermatological and emergency room) and dermatological prescriptions was studied. We estimated adjusted incidence rate ratio (aIRR) (adjusted for sex, age and calendar year) and cumulative incidence.
RESULTS
In total, 5 054 238 individuals (50.6% female) were included in the study population, accounting for 73 477 258 person-years at risk, with a start mean (SD) age of 39.4 (21.1) years. Of the total number of individuals, 759 991 (15.0%) received a skin diagnosis and 38 071 (0.7%) experienced homelessness. A 2.31-times [95% confidence interval (CI) 2.25-2.36] higher IRR of any diagnosed skin condition was associated with homelessness, higher for nondermatological and emergency room consultations. Homelessness was associated with a reduced IRR of a skin neoplasm diagnosis (aIRR 0.76, 95% CI 0.71-8.82) compared with no homelessness. By the end of follow-up, 2.8% (95% CI 2.5-3.0) of individuals experiencing homelessness had a skin neoplasm diagnosis vs. 5.1% (95% CI 4.9-5.3) of individuals not experiencing homelessness. Five or more shelter contacts during the first year from first contact was associated with the highest aIRR of any diagnosed skin condition (7.33, 95% CI 5.57-9.65) compared with no contacts.
CONCLUSIONS
Individuals experiencing homelessness have high rates of most diagnosed skin conditions, but a lower occurrence of skin cancer diagnosis. Diagnostic and medical patterns for skin disorders differed clearly between people experiencing homelessness and individuals without these experiences. The time after first homeless shelter contact is an important window of opportunity for mitigating and preventing skin disorders.
Topics: Humans; Female; Male; Cohort Studies; Registries; Ill-Housed Persons; Skin Neoplasms; Denmark
PubMed: 36810657
DOI: 10.1093/bjd/ljad040 -
JNMA; Journal of the Nepal Medical... Nov 2021Skin tumors are relatively uncommon malignancies worldwide, but its incidence has been progressively increased over the last few decades. Skin tumor belongs to a diverse...
INTRODUCTION
Skin tumors are relatively uncommon malignancies worldwide, but its incidence has been progressively increased over the last few decades. Skin tumor belongs to a diverse group of neoplasms arising from the epidermis, adnexal structures and dermis rendering the classification difficult. The study aims to find out the prevalence of benign skin neoplasm among the histopathological specimens of skin neoplasm of a teaching hospital.
METHODS
A descriptive cross-sectional study among the hospital records of histopathological samples of skin neoplasm in the Department of Pathology of a tertiary care center from January 2017 to December 2020. Ethical approval was taken from the Institutional Review Committee (Ref: MEMG/IRC/427/GA). Convenient sampling was done. Data were entered in Microsoft Excel and analyzed using Statistical Package for the Social Sciences version 21 software. Point estimate at 95% Confidence Interval was calculated with frequency and descriptive statistics.
RESULTS
Out of total skin neoplasm samples, 121 (57.34%) (50.67-64.01 at 95% Confidence Interval) benign skin neoplasms were present. Among them, the majority were keratinocytic tumor 81 (66.9%) followed by skin appendageal 23 (19.0%) and melanocytic tumors 17 (14.0%). Acrochordan 18 (14.9%) and pilomatricoma 12 (9.9%) were the predominant keratinocytic and appendageal neoplasms respectively. Most of the cases occurred in head and neck region 64 (52.9%).
CONCLUSIONS
The study concluded that the prevalence of benign skin neoplasm was slightly lower compared to the other studies. Most of the benign skin neoplasms were keratinocytic tumors followed by appendageal and melanocytic tumors. Acrochordan was the commonest benign keratinocytic tumor.
Topics: Cross-Sectional Studies; Hospitals, Teaching; Humans; Skin; Skin Neoplasms; Tertiary Care Centers
PubMed: 35199769
DOI: 10.31729/jnma.6086 -
Scientific Reports Feb 2021Non-melanoma skin cancers (NMSCs) are the most common malignancies diagnosed in Caucasian populations. Basal cell carcinoma (BCC) is the most frequent skin cancer,...
Non-melanoma skin cancers (NMSCs) are the most common malignancies diagnosed in Caucasian populations. Basal cell carcinoma (BCC) is the most frequent skin cancer, followed by squamous cell carcinoma (SCC). Unfortunately, most European cancer registries do not record individual types of NMSC. To evaluate the incidence of primary BCCs and SCCs regarding age, sex, tumour site and tumour subtype to determine trends in epidemiology of both cancers. Retrospective analysis of BCCs and SCCs diagnosed and treated across seven sites in Poland from 1999 to 2019. We recorded 13,913 NMSCs occurring in 10,083 patients. BCC represented 85.2% of all cases. SCC patients were older than BCC patients (77.1 ± 11.3 years vs. 70.1 ± 12.3 years, p < 0.01). The nodular subtype was the most common subtype of BCC, followed by the superficial and infiltrative subtypes. The superficial BCC subtype was more common on photoprotected areas (p < 0.01), whereas the nodular BCC subtype occurred on the face (p < 0.01). The high-risk SCC subtypes were more common on face compared to low-risk SCC subtypes (p < 0.01). BCC and SCC are common malignancies developing at various ages and anatomical sites. These data underline the need for better registration policies regarding NMSC in order to improve prevention and treatment strategies for these tumours.
Topics: Adult; Age Factors; Aged; Aged, 80 and over; Female; Humans; Incidence; Male; Middle Aged; Neoplasm Grading; Neoplasm Staging; Poland; Public Health Surveillance; Registries; Sex Factors; Skin Neoplasms; Young Adult
PubMed: 33619293
DOI: 10.1038/s41598-021-83502-8 -
International Journal of Dermatology Feb 2020Leiomyosarcoma (LMS) of the skin is rare, and no management guideline currently exists. Although LMS is historically classified as either dermal (cutaneous) or... (Review)
Review
Leiomyosarcoma (LMS) of the skin is rare, and no management guideline currently exists. Although LMS is historically classified as either dermal (cutaneous) or subcutaneous, definition for its classfication is inconsistent in the literature. Studies on the managenent of LMS are scarce, and there is no consensus on the appropriate surgical margin for the treatment of LMS. While a 1 cm margin may be sufficient in cutaneous LMS, wider margins may be required for subcutaneous tumors. Mohs micrographic surgery is a promising surgical modality for the treatment of cutaneous LMS. In this review, current knowledge on LMS is summarized and a practical approach to the management of this rare neoplasm is proposed.
Topics: Algorithms; Chemotherapy, Adjuvant; Humans; Leiomyosarcoma; Margins of Excision; Mohs Surgery; Neoplasm Staging; Prognosis; Skin Neoplasms
PubMed: 31729020
DOI: 10.1111/ijd.14705 -
Veterinary Pathology Nov 2023Cutaneous mastocytosis (CM) is a rare condition in young dogs characterized by multicentric cutaneous proliferation of neoplastic mast cells. Clinical data from 8 dogs... (Review)
Review
Cutaneous mastocytosis (CM) is a rare condition in young dogs characterized by multicentric cutaneous proliferation of neoplastic mast cells. Clinical data from 8 dogs that met inclusion criteria (age of onset less than 1.5 years, greater than 3 lesions) were obtained via a standardized survey. Biopsy samples were classified by the Kiupel/Patnaik grading systems and analyzed for mutations. The median age of onset was 6 months (range: 2-17 months). Dogs had 5 to more than 50 lesions characterized as nodules, plaques, and papules. Seven dogs were pruritic. Clinical staging in 2 dogs did not reveal visceral involvement. No dogs had systemic illnesses at diagnosis. Histologically, CM was similar to cutaneous mast cell tumor (cMCT). Two dogs had neoplasms classified as high-grade/grade II while 6 dogs had low-grade/grade II neoplasms. No dogs had mutations in exons 8 and 11. Treatment included antihistamines (8/8), corticosteroids (7/8), lokivetmab (3/8), and toceranib (1/8). Six dogs were alive with lesions at the end of the study with a median follow-up time of 898 days, while 2 dogs were euthanized. In dogs with high-grade/grade II neoplasms, one continued to develop lesions at 1922 days post-diagnosis, while the other dog was euthanized at 56 days post-diagnosis. One dog was euthanized 621 days post-diagnosis due to rupture of a neoplasm. CM occurs in young dogs and is histologically indistinguishable from cMCT. Current histologic grading systems did not apply uniformly to the dogs of the study and further studies are needed.
Topics: Dogs; Animals; Mastocytosis, Cutaneous; Skin; Skin Neoplasms; CME-Carbodiimide; Dog Diseases; Mast Cells
PubMed: 37222130
DOI: 10.1177/03009858231174452 -
Current Opinion in Oncology Mar 2020Cutaneous squamous cell carcinoma (cSCC) is a highly prevalent malignancy frequently occurring on body surfaces chronically exposed to ultraviolet radiation. While a... (Review)
Review
PURPOSE OF REVIEW
Cutaneous squamous cell carcinoma (cSCC) is a highly prevalent malignancy frequently occurring on body surfaces chronically exposed to ultraviolet radiation. While a large majority of tumors remain localized to the skin and immediate subcutaneous tissue and are cured with surgical excision, a small subset of patients with cSCC will develop metastatic disease. Risk stratification for cSCC is performed using clinical staging systems, but given a high mutational burden and advances in targeted and immunotherapy, there is growing interest in molecular predictors of high-risk disease.
RECENT FINDINGS
Recent literature on the risk for metastasis in cSCC includes notable findings in genes involved in cell-cycle regulation, tumor suppression, tissue invasion and microenvironment, interactions with the host-immune system, and epigenetic regulation.
SUMMARY
cSCC is a highly mutated tumor with complex carcinogenesis. Regulators of tumor growth and local invasion are numerous and increasingly well-understood but drivers of metastasis are less established. Areas of importance include central system regulators (NOTCH, miRNAs), proteins involved in tissue invasion (podoplanin, E-cadherin), and targets of existing and emerging therapeutics (PD-1, epidermal growth factor receptor). Given the complexity of cSCC carcinogenesis, the use of machine learning algorithms and computational genomics may provide ultimate insight and prospective studies are needed to verify clinical relevance.
Topics: Carcinoma, Squamous Cell; Epigenesis, Genetic; Humans; Neoplasm Metastasis; Skin Neoplasms
PubMed: 31850970
DOI: 10.1097/CCO.0000000000000609