Brodawczakowłókniak kosmówkowy
Epidemiologia

Brodawczakowłókniak kosmówkowy (DFSP) to rzadki, miejscowo agresywny mięsak skóry o pochodzeniu fibroblastycznym, charakteryzujący się powolnym wzrostem i niskim potencjałem przerzutowym. Roczna zapadalność wynosi od 0,8 do 6,25 przypadków na milion osób, z wyższą częstością u osób rasy czarnej (8,74 vs 4,53/milion) oraz u Afroamerykanów (6,5/milion). DFSP najczęściej dotyczy osób w wieku 20-50 lat, z lokalizacją głównie na tułowiu i barkach. Mimo niskiego ryzyka przerzutów (1% do węzłów chłonnych, 4% odległych), nowotwór cechuje się wysokim ryzykiem nawrotów miejscowych (10-80%), szczególnie po szerokim wycięciu miejscowym (10-60%). Wariant włókniakomięsakowy (DFSP-FS) stanowi 5-15% przypadków i wiąże się z większą agresywnością oraz wyższym ryzykiem przerzutów (10-15%). Dziesięcioletnie przeżycie względne wynosi 99,1%, a przeżycie bez nawrotu 76%.

  1. Epidemiologia brodawczakowłókniaka kosmówkowego
    1. Częstotliwość występowania
    2. Różnice etniczne i rasowe
    3. Wiek i płeć
    4. Lokalizacja anatomiczna
  2. Przebieg kliniczny i rokowanie
    1. Nawroty miejscowe
    2. Przerzuty i czynniki ryzyka
    3. Przeżywalność i czynniki prognostyczne
  3. Nadzór i obserwacja długoteminowa
    1. Schematy obserwacji
    2. Indywidualizacja obserwacji
  4. Trendy epidemiologiczne i nowe odkrycia
    1. Kolejne rozdziały

Epidemiologia brodawczakowłókniaka kosmówkowego

Brodawczakowłókniak kosmówkowy (Dermatofibrosarcoma protuberans, DFSP) jest rzadkim, miejscowo agresywnym nowotworem skóry o pochodzeniu fibroblastycznym, który charakteryzuje się powolnym wzrostem i niskim potencjałem przerzutowym. Jest to najczęstszy mięsak skóry, który mimo swojej rzadkości stanowi istotny problem kliniczny ze względu na tendencję do nawrotów miejscowych.123

Częstotliwość występowania

Roczna zapadalność na DFSP szacowana jest na 0,8 do 5 przypadków na milion osób. Dane z rejestru SEER (Surveillance, Epidemiology, and End Results) w Stanach Zjednoczonych wskazują na średnią częstość występowania wynoszącą około 4,1-4,2 przypadków na milion osób rocznie. Nowsze badania epidemiologiczne sugerują jednak częstość występowania na poziomie 6,25 (95% CI, 5,93-6,57) przypadków na milion osobolat.456

W Stanach Zjednoczonych DFSP stanowi od 1% do 6% wszystkich mięsaków tkanek miękkich oraz 18% wszystkich skórnych mięsaków tkanek miękkich. W dużym badaniu opartym na danych z rejestru SEER, obejmującym 12 114 przypadków skórnych mięsaków tkanek miękkich zdiagnozowanych w latach 1992-2004, DFSP był drugim najczęstszym nowotworem po mięsaku Kaposiego, który stanowił 71% wszystkich przypadków.78

W Europie dane epidemiologiczne są zbliżone. Roczna zapadalność na DFSP wynosi ponad 3 przypadki na milion mieszkańców we wschodniej Francji, 4,4 przypadki na milion mężczyzn i 4,0 przypadki na milion kobiet w Szwecji oraz 5,3 przypadków na milion mieszkańców w Danii w latach 2000-2012.9

Różnice etniczne i rasowe

Istnieją znaczące różnice w częstości występowania DFSP między różnymi grupami etnicznymi. Badania epidemiologiczne wykazały, że częstość występowania DFSP wśród osób rasy czarnej jest niemal dwukrotnie wyższa niż wśród osób rasy białej (8,74 vs 4,53 przypadków na milion). Według badania Criscione i Weinstock, roczna zapadalność wśród Afroamerykanów (6,5 przypadków na milion) była prawie dwukrotnie wyższa niż wśród białych Amerykanów (3,9 przypadków na milion).101112

Co istotne, pigmentowana odmiana DFSP, znana jako guz Bednara (Bednar tumor), występuje częściej u osób rasy czarnej, z roczną zapadalnością 7,5 razy wyższą niż u pacjentów rasy białej. Wariant ten stanowi mniej niż 5% wszystkich przypadków DFSP.1314

Badania wskazują również, że wśród osób rasy czarnej w wieku 45-49 lat częstość występowania DFSP jest nawet 3,5 razy wyższa niż wśród osób rasy białej w tej samej grupie wiekowej. DFSP stanowi około 10% wszystkich przypadków nowotworów skóry u osób rasy czarnej.15

Wiek i płeć

DFSP najczęściej występuje u młodych i w średnim wieku dorosłych, zazwyczaj między 20 a 50 rokiem życia, ze średnim wiekiem zachorowania wynoszącym około 40 lat. Nowotwór ten może jednak występować we wszystkich grupach wiekowych, od noworodków do osób w podeszłym wieku (powyżej 80 lat). Dzieci stanowią około 6% wszystkich przypadków DFSP.161718

Odnośnie rozkładu płci, dane są niejednoznaczne. Niektóre badania wskazują na równą dystrybucję między płciami lub niewielką przewagę mężczyzn. W dużym badaniu obejmującym 902 pacjentów z DFSP przeprowadzonym przez Rutgers i wsp., 514 (57%) pacjentów stanowili mężczyźni, a 388 (43%) kobiety. Z kolei badanie oparte na danych z Szwedzkiego Krajowego Rejestru Nowotworów z lat 1990-2005 wykazało niewielką różnicę w rocznej zapadalności między mężczyznami (4,4 przypadki na milion) a kobietami (4,0 przypadki na milion).19

Nowsze badania sugerują, że kobiety mogą mieć nieznacznie wyższą zapadalność na DFSP, wykazując 4,4 przypadków w porównaniu do 4,2 przypadków na milion rocznie u mężczyzn. Niektóre publikacje podają również, że współczynnik zachorowalności u kobiet jest 1,14 razy wyższy niż u mężczyzn (95% CI współczynnika częstości: 1,07-1,22).202122

Lokalizacja anatomiczna

DFSP najczęściej występuje na tułowiu i barkach, a następnie na kończynach proksymalnych. Rzadziej spotykany jest na głowie i szyi oraz kończynach dystalnych. Lokalizacja anatomiczna nowotworu może mieć znaczenie prognostyczne – badania wskazują, że umiejscowienie na kończynach i głowie, w porównaniu z tułowiem, wiąże się z wyższym ryzykiem śmiertelności.23242526

Przebieg kliniczny i rokowanie

DFSP charakteryzuje się powolnym wzrostem i miejscową agresywnością, ale rzadko daje przerzuty odległe. Nowotwór ten ma tendencję do miejscowego nawrotu, sięgającą 10-80% przypadków w zależności od zastosowanej metody leczenia i szerokości marginesów wycięcia.27

Nawroty miejscowe

Częstość nawrotów miejscowych po szerokim wycięciu miejscowym (WLE) DFSP waha się od 10% do 60%, podczas gdy częstość rozwoju regionalnych lub odległych przerzutów wynosi odpowiednio tylko 1% i 4%. Większość nawrotów rozwija się w ciągu 3 lat po resekcji, chociaż mogą wystąpić również późne nawroty, co uzasadnia długoterminową obserwację.282930

Badanie przeprowadzone przez Fiore i wsp. potwierdza, że większość przypadków DFSP, w tym choroby nawrotowej, można skutecznie leczyć chirurgicznie z szerokim wycięciem, co daje surową częstość nawrotów w całej populacji wynoszącą 3% po 5 latach i 4,2% po 10 latach.31

Co ciekawe, badanie wykazało, że większość nawrotów guza (61,7%) była identyfikowana przez pacjentów podczas samokontroli, podczas gdy tylko niewielki odsetek (3,7%) został wykryty podczas badania klinicznego przez zespół medyczny. Sugeruje to, że edukacja pacjenta dotycząca samokontroli może być skuteczną strategią nadzoru.32

Przerzuty i czynniki ryzyka

DFSP rzadko daje przerzuty, które występują tylko w około 5% przypadków. Nowotwór rozprzestrzenia się w 1% przypadków poprzez naczynia limfatyczne do regionalnych węzłów chłonnych i w 4% poprzez krwioobieg, najczęściej do płuc, a następnie do mózgu, kości i serca.3334

Ryzyko przerzutów DFSP znacznie wzrasta w przypadku guzów o wielkości ≥3 cm oraz guzów zlokalizowanych na głowie i szyi oraz w okolicy narządów płciowych. Przerzuty występują najczęściej w przypadku nawracających zmian, które postępowały przez wiele lat, oraz gdy obecny jest komponent włókniakomięsakowy (DFSP-FS).3536

Wariant włókniakomięsakowy DFSP (DFSP-FS) stanowi około 5-15% wszystkich przypadków DFSP i wiąże się z bardziej agresywnym przebiegiem. W przypadku DFSP-FS 10-15% pacjentów rozwija przerzuty odległe, najczęściej do płuc.373839

Przeżywalność i czynniki prognostyczne

Ze względu na rzadkie występowanie przerzutów, DFSP ma ogólnie dobre rokowanie. Dziesięcioletnie względne przeżycie wynosi 99,1% (95% CI: 97,6-99,7%). Pięcio- i dziesięcioletnie przeżycie bez nawrotu wynosi odpowiednio 86% i 76%.4041

Najnowsze badania wskazują, że 10- i 15-letnie wskaźniki przeżycia specyficzne dla DFSP wynoszą odpowiednio 98,5% i 98,0%. Mimo rzadkiego występowania przerzutów odległych, nawet pacjenci z przerzutami do węzłów chłonnych lub przerzutami odległymi mają generalnie dobre rokowanie.42

Istotne czynniki prognostyczne związane z wyższą śmiertelnością ogólną to zwiększony wiek, płeć męska, rasa czarna oraz anatomiczna lokalizacja kończyn i głowy w porównaniu z tułowiem. Niezależne czynniki ryzyka śmiertelności specyficznej dla nowotworu obejmują wiek w momencie diagnozy, stopień histologiczny i wielkość guza. Ryzyko zgonu jest znacznie większe w wyjątkowych przypadkach, takich jak guzy o stopniu histologicznym III lub wielkości powyżej 10 cm.434445

Analiza danych SEER przeprowadzona przez Kreicher i wsp. z lat 2000-2010 wykazała, że rasa czarna miała 1,7 razy większe ryzyko śmiertelności z wszystkich przyczyn w porównaniu z rasą białą, co sugeruje, że rasa czarna może być negatywnym predyktorem przeżycia.46

Nadzór i obserwacja długoteminowa

Ze względu na możliwość wystąpienia późnych nawrotów miejscowych, pacjenci z DFSP wymagają długoterminowej obserwacji. Zaleca się regularne badania kontrolne co 6 miesięcy przez 3-5 lat, a następnie raz w roku przez całe życie.47

Schematy obserwacji

Większość ekspertów zaleca, aby pacjenci byli ponownie oceniani co 6 miesięcy przez pierwsze pięć lat, a następnie co roku. Niektórzy autorzy sugerują ograniczenie badań obrazowych w ramach obserwacji do pacjentów z nawracającym DFSP lub DFSP-FS.4849

Pacjenci powinni uczestniczyć w regularnych samobadaniach, a miejsce pierwotne powinno być badane co 6 miesięcy przez 3 do 5 lat. Następnie kontynuuje się badanie raz w roku przez całe życie.50

W przypadku nawrotu DFSP, jest on często leczony za pomocą jednej z opisanych powyżej operacji chirurgicznych. Ze względu na możliwość nawrotu nawet wiele lat po leczeniu, zaleca się dożywotnią obserwację u lekarzy.5152

Indywidualizacja obserwacji

Najnowsze badania sugerują, że można indywidualizować schemat obserwacji po operacji DFSP. Na przykład, pacjentom z małymi guzami (<5 cm), które zostały całkowicie wycięte (R0), bez zmiany włókniakomięsakowej, można zaproponować obserwację kierowaną przez pacjenta zamiast rutynowego nadzoru przez okres do 10 lat.53

Z drugiej strony, pacjenci ze złożonymi lub wysokiego ryzyka przypadkami DFSP (transformacja FS, duże guzy, lokalizacja anatomiczna) powinni być obserwowani przez ekspertów, zgodnie z aktualnymi wytycznymi ekspertów.54

W rzadkich przypadkach choroby przerzutowej zaleca się wielodyscyplinarną konsultację w celu koordynacji leczenia. Biorąc pod uwagę historycznie wysokie wskaźniki nawrotów miejscowych DFSP, wskazana jest bieżąca obserwacja kliniczna ze skupieniem na miejscu pierwotnym co 6 do 12 miesięcy, z ponowną biopsją wszelkich podejrzanych obszarów.55

Trendy epidemiologiczne i nowe odkrycia

W ostatnich latach obserwuje się wzrost liczby przypadków DFSP, szczególnie wśród osób rasy białej. Może to wynikać zarówno z lepszego rozpoznawania i poprawy diagnostyki, jak i rzeczywistego wzrostu zapadalności.5657

Badania wskazują również na różnice w wyborze metod leczenia, które mogą być związane z czynnikami socjoekonomicznymi i rasowymi. Badania Criscito i wsp. ujawniają, że chirurgia mikrograficzna Mohsa (MMS) jest częściej stosowana u pacjentów rasy białej, kobiet i osób o wysokim medianie dochodów gospodarstw domowych, w przeciwieństwie do osób rasy czarnej, mężczyzn i osób o niższej medianie dochodów gospodarstw domowych.58

Rozpoznanie molekularnych podstaw DFSP, a w szczególności identyfikacja znaczenia aktywacji receptora płytkopochodnego czynnika wzrostu (PDGFR) w patogenezie DFSP, doprowadziło do rozwoju terapii celowanej. Pierwszą skuteczną terapią systemową wprowadzoną do praktyki klinicznej w DFSP był imatynib, wykazujący dramatyczną aktywność w zaawansowanych przypadkach. Nowsze badania wskazują, że niektórzy pacjenci z DFSP, początkowo oceniani jako nieoperacyjni/przerzutowi lub wymagający okaleczającej operacji, stali się operacyjni po terapii imatynibem, a to racjonalne podejście prowadzące do całkowitej remisji może być potencjalnie lecznicze.59

W przypadkach DFSP opornych na leczenie imatynibem, raportowano skuteczność sorafenibu, który może stanowić alternatywę terapeutyczną.60

Podsumowując, mimo że DFSP jest rzadkim nowotworem skóry, jego epidemiologia, czynniki ryzyka i rokowanie są coraz lepiej poznane dzięki analizie dużych baz danych populacyjnych, takich jak SEER. Badania te dostarczają cennych informacji na temat różnic w zapadalności, przebiegu klinicznym i rokowaniu w różnych grupach demograficznych, co może pomóc w opracowaniu bardziej spersonalizowanych strategii diagnostycznych i terapeutycznych.

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  1. 10.04.2026
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Materiały źródłowe

  • #1 Dermatofibrosarcoma protuberans: Epidemiology, pathogenesis, clinical presentation, diagnosis, and staging – UpToDate
    https://www.uptodate.com/contents/dermatofibrosarcoma-protuberans-epidemiology-pathogenesis-clinical-presentation-diagnosis-and-staging
    Dermatofibrosarcoma protuberans (DFSP) is a relatively rare tumor. Estimates of the overall incidence of DFSP in the United States are 0.8 to 4.5 cases per million persons per year. In the United States, DFSP accounts for between 1 and 6 percent of all soft tissue sarcomas and 18 percent of all cutaneous soft tissue sarcomas. In a series of 12,114 cutaneous soft tissue sarcomas derived from the Surveillance, Epidemiology and End Results (SEER) tumor registry from 1992 through 2004, DFSP was second only to Kaposi sarcoma, which accounted for 71 percent of cases. […] The Bednar or pigmented variant (distinguished by the dispersal of melanin-containing cells in an otherwise typical DFSP) is even less common. It represents fewer than 5 percent of all DFSP cases and is more common in Black individuals. The fibrosarcomatous variant of DFSP (DFSP-FS) accounts for approximately 5 to 15 percent of DFSPs.
  • #2 Dermatofibrosarcoma Protuberans: Practice Essentials, Background, Pathophysiology
    https://emedicine.medscape.com/article/1100203-overview
    Dermatofibrosarcoma protuberans (DFSP) accounts for less than 0.1% of all malignant neoplasms and approximately 1% of all soft tissue sarcomas. DFSP is the most common type of cutaneous sarcoma. The incidence of DFSP has been estimated to be 0.8-5 case per million population per year in 2 separate studies. […] In a another study based on data from 9 cancer registries from 1973-2002, the annual incidence of DFSP in the United States is 4.2 cases per million population per year. […] The annual incidence of DFSP is reported as 3 cases per million population from a population-based cancer registry from 1982-2002 in France. […] A study of the population-based National Cancer Registry shows the incidence of DFSP is approximately 4 cases per million per year in Sweden from 1990-2005. […] Dermatofibrosarcoma protuberans (DFSP) has been reported in persons of all races, and no racial predilection seems to exist in previous reports.
  • #3 Dermatofibrosarcoma protuberans | Radiology Reference Article | Radiopaedia.org
    https://radiopaedia.org/articles/dermatofibrosarcoma-protuberans?embed_domain=external.radpair.com%2527%255B0%255Dfavicon.icoradiopaedia-icon-144.png&lang=us
    Dermatofibrosarcoma protuberans (DFSP) is a low-grade malignant tumor arising from dermal and subcutaneous tissues and is the most common cutaneous sarcoma (although overall still quite rare). It is most commonly found at the trunk and proximal extremities. […] The tumor occurs in patients of all ages, with the highest frequency occurring between ages 20-50 years. Males are slightly more commonly affected than females. […] Its behavior is notable for a high reported local recurrence rate, although outcomes overall are favorable.
  • #4 The epidemiology of dermatofibrosarcoma protuberans incidence, metastasis, and death among various population groups: A Surveillance, Epidemiology, and End Results database analysis – PubMed
    https://pubmed.ncbi.nlm.nih.gov/38908718/
    Limited information exists regarding the epidemiology, metastasis, and survival of dermatofibrosarcoma protuberans (DFSP). […] DFSP incidence rate was 6.25 (95% CI, 5.93-6.57) cases per million person-years with significantly higher incidence observed among Black individuals than White individuals (8.74 vs 4.53). […] DFSP incidence is 2-fold higher among Black than White individuals.
  • #5 Dermatofibrosarcoma Protuberans: Practice Essentials, Background, Pathophysiology
    https://emedicine.medscape.com/article/1100203-overview
    Dermatofibrosarcoma protuberans (DFSP) accounts for less than 0.1% of all malignant neoplasms and approximately 1% of all soft tissue sarcomas. DFSP is the most common type of cutaneous sarcoma. The incidence of DFSP has been estimated to be 0.8-5 case per million population per year in 2 separate studies. […] In a another study based on data from 9 cancer registries from 1973-2002, the annual incidence of DFSP in the United States is 4.2 cases per million population per year. […] The annual incidence of DFSP is reported as 3 cases per million population from a population-based cancer registry from 1982-2002 in France. […] A study of the population-based National Cancer Registry shows the incidence of DFSP is approximately 4 cases per million per year in Sweden from 1990-2005. […] Dermatofibrosarcoma protuberans (DFSP) has been reported in persons of all races, and no racial predilection seems to exist in previous reports.
  • #6
    https://link.springer.com/article/10.1007/s13671-024-00432-0
    This literature review aims to address the epidemiological differences, clinical presentations, and treatment outcomes of dermatofibrosarcoma protuberans (DFSP) and Kaposi sarcoma (KS) among people of color (POC), who are underrepresented in skin cancer research. […] A comprehensive review of literature from 1990 to 2023 revealed significant disparities in the incidence and management of DFSP and KS among POC. Findings indicate a two-fold higher incidence of DFSP in Black patients compared to Whites and a notable predominance of the pigmented Bednar tumor subtype. […] DFSP is a rare, locally aggressive soft tissue sarcoma that accounts for 0.1% of all cancers, and has an overall incidence of 4.1 to 4.2 million in the U.S. […] Although rare, DFPS accounts for ~10% of all cases of skin cancer in Blacks, and occurs at rates nearly double those seen in Whites, rising to 3.5-fold among Blacks aged 45-49.
  • #7 Dermatofibrosarcoma protuberans: Epidemiology, pathogenesis, clinical presentation, diagnosis, and staging – UpToDate
    https://www.uptodate.com/contents/dermatofibrosarcoma-protuberans-epidemiology-pathogenesis-clinical-presentation-diagnosis-and-staging
    Dermatofibrosarcoma protuberans (DFSP) is a relatively rare tumor. Estimates of the overall incidence of DFSP in the United States are 0.8 to 4.5 cases per million persons per year. In the United States, DFSP accounts for between 1 and 6 percent of all soft tissue sarcomas and 18 percent of all cutaneous soft tissue sarcomas. In a series of 12,114 cutaneous soft tissue sarcomas derived from the Surveillance, Epidemiology and End Results (SEER) tumor registry from 1992 through 2004, DFSP was second only to Kaposi sarcoma, which accounted for 71 percent of cases. […] The Bednar or pigmented variant (distinguished by the dispersal of melanin-containing cells in an otherwise typical DFSP) is even less common. It represents fewer than 5 percent of all DFSP cases and is more common in Black individuals. The fibrosarcomatous variant of DFSP (DFSP-FS) accounts for approximately 5 to 15 percent of DFSPs.
  • #8 Dermatofibrosarcoma protuberans – Wikipedia
    https://en.wikipedia.org/wiki/Dermatofibrosarcoma_protuberans
    Dermatofibrosarcoma protuberans (DFSP) is a rare locally aggressive malignant cutaneous soft-tissue sarcoma. Estimates of the overall occurrence of DFSP in the United States are 0.8 to 4.5 cases per million persons per year. In the United States, DFSP accounts for between 1 and 6 percent of all soft-tissue sarcomas and 18 percent of all cutaneous soft-tissue sarcomas. In the Surveillance, Epidemiology and End Results (SEER) tumor registry from 1992 through 2004, DFSP was second only to Kaposi sarcoma. […] Commonly located on the chest and shoulders, the following is the site distribution of DFPS as was observed in Surveillance, Epidemiology, and End Results (SEER) database between 2000 and 2010. […] DFSP is not extensively studied due to its rarity and low mortality. The majority of studies are small size case studies or meta-analysis.
  • #9 Dermatofibrosarcoma Protuberans: An Updated Review of the Literature
    https://www.mdpi.com/2072-6694/16/18/3124
    The available European data are as follows: over 3 cases per million inhabitants per year in Eastern France, with men affected 1.2 times more often than women, and a more frequent incidence in men than in women (4.4 vs. 4.0 per million per year, respectively) in Sweden. […] The annual incidence in Denmark for the years 2000–2012 was 5.3 cases per million inhabitants. […] This relative rarity of DFSP is reflected in the lack of prospective scientific evidence. […] Yet, even if infrequent, DFSP represents the most common dermal sarcoma (about 1% of all soft-tissue sarcomas), more than 1% of all head and neck malignant tumors, and 7% of all head and neck sarcomas.
  • #10 The epidemiology of dermatofibrosarcoma protuberans incidence, metastasis, and death among various population groups: A Surveillance, Epidemiology, and End Results database analysis – PubMed
    https://pubmed.ncbi.nlm.nih.gov/38908718/
    Limited information exists regarding the epidemiology, metastasis, and survival of dermatofibrosarcoma protuberans (DFSP). […] DFSP incidence rate was 6.25 (95% CI, 5.93-6.57) cases per million person-years with significantly higher incidence observed among Black individuals than White individuals (8.74 vs 4.53). […] DFSP incidence is 2-fold higher among Black than White individuals.
  • #11 Dermatofibrosarcoma Protuberans: Practice Essentials, Background, Pathophysiology
    https://emedicine.medscape.com/article/1100203-overview
    However, a study conducted by Criscione and Weinstock found the incidence among African Americans (6.5 cases per million population) was almost double the incidence among American whites (3.9 cases per million population). […] Several studies of dermatofibrosarcoma protuberans (DFSP) reveal an almost equal sexual distribution or a slight male predominance. […] In a large study of 902 patients with DFSP conducted by Rutgers et al, 514 (57%) patients were male and 388 (43%) patients were female. […] A study based on 405 DFSP cases from the Swedish National Cancer Registry between 1990 and 2005 shows a very small difference in annual incidence of male (4.4 cases per million) versus female (4.0 cases per million). […] However, a larger cancer registry study of 2885 cases reveals females might have a slightly higher incidence of DFSP, 4.4 cases versus 4.2 cases per million population per year. […] Dermatofibrosarcoma protuberans (DFSP) can occur at any age is is most frequent in adults aged 20-50 years. Rarely, DFSP has been reported in newborns and elderly individuals (80 y).
  • #12 Dermatofibrosarcoma protuberans – Dermatology Advisor
    https://www.dermatologyadvisor.com/home/decision-support-in-medicine/dermatology/dermatofibrosarcoma-protuberans/
    DFSP is a rare tumor, with an estimated incidence of 4.1 cases per million person-years, but still the most common sarcoma of the skin. […] The incidence of DFSP among African Americans was found to be almost double the incidence among American whites (6.5 vs 3.9 cases per million population). […] DFSP has been reported in all races. […] The Bednar tumor or pigmented variant of DFSP, in particular, is known to be more common in blacks, with an annual incidence 7.5 times higher than that seen in white patients. […] Criscione, VD, Weinstock, MA. Descriptive epidemiology of dermatofibrosarcoma protuberans in the United States, 1973 to 2002. J Am Acad Dermatol. vol. 56. 2007. pp. 968-73. (An extensive population-based epidemiologic study of DFSP spanning 29 years, looking at overall annual incidence, according to both race and sex, and also analyzing relative 5-year survival [99.2%].)
  • #13 Dermatofibrosarcoma protuberans: Epidemiology, pathogenesis, clinical presentation, diagnosis, and staging – UpToDate
    https://www.uptodate.com/contents/dermatofibrosarcoma-protuberans-epidemiology-pathogenesis-clinical-presentation-diagnosis-and-staging
    Dermatofibrosarcoma protuberans (DFSP) is a relatively rare tumor. Estimates of the overall incidence of DFSP in the United States are 0.8 to 4.5 cases per million persons per year. In the United States, DFSP accounts for between 1 and 6 percent of all soft tissue sarcomas and 18 percent of all cutaneous soft tissue sarcomas. In a series of 12,114 cutaneous soft tissue sarcomas derived from the Surveillance, Epidemiology and End Results (SEER) tumor registry from 1992 through 2004, DFSP was second only to Kaposi sarcoma, which accounted for 71 percent of cases. […] The Bednar or pigmented variant (distinguished by the dispersal of melanin-containing cells in an otherwise typical DFSP) is even less common. It represents fewer than 5 percent of all DFSP cases and is more common in Black individuals. The fibrosarcomatous variant of DFSP (DFSP-FS) accounts for approximately 5 to 15 percent of DFSPs.
  • #14 Dermatofibrosarcoma protuberans – Dermatology Advisor
    https://www.dermatologyadvisor.com/home/decision-support-in-medicine/dermatology/dermatofibrosarcoma-protuberans/
    DFSP is a rare tumor, with an estimated incidence of 4.1 cases per million person-years, but still the most common sarcoma of the skin. […] The incidence of DFSP among African Americans was found to be almost double the incidence among American whites (6.5 vs 3.9 cases per million population). […] DFSP has been reported in all races. […] The Bednar tumor or pigmented variant of DFSP, in particular, is known to be more common in blacks, with an annual incidence 7.5 times higher than that seen in white patients. […] Criscione, VD, Weinstock, MA. Descriptive epidemiology of dermatofibrosarcoma protuberans in the United States, 1973 to 2002. J Am Acad Dermatol. vol. 56. 2007. pp. 968-73. (An extensive population-based epidemiologic study of DFSP spanning 29 years, looking at overall annual incidence, according to both race and sex, and also analyzing relative 5-year survival [99.2%].)
  • #15
    https://link.springer.com/article/10.1007/s13671-024-00432-0
    This literature review aims to address the epidemiological differences, clinical presentations, and treatment outcomes of dermatofibrosarcoma protuberans (DFSP) and Kaposi sarcoma (KS) among people of color (POC), who are underrepresented in skin cancer research. […] A comprehensive review of literature from 1990 to 2023 revealed significant disparities in the incidence and management of DFSP and KS among POC. Findings indicate a two-fold higher incidence of DFSP in Black patients compared to Whites and a notable predominance of the pigmented Bednar tumor subtype. […] DFSP is a rare, locally aggressive soft tissue sarcoma that accounts for 0.1% of all cancers, and has an overall incidence of 4.1 to 4.2 million in the U.S. […] Although rare, DFPS accounts for ~10% of all cases of skin cancer in Blacks, and occurs at rates nearly double those seen in Whites, rising to 3.5-fold among Blacks aged 45-49.
  • #16 Dermatofibrosarcoma Protuberans – StatPearls – NCBI Bookshelf
    https://www.ncbi.nlm.nih.gov/books/NBK513305/
    DFSP is a rare tumor, occurring at a rate of 0.8 to 4.5 cases per million persons annually and represents 1% to 6% of all soft tissue sarcomas and 18% of cutaneous soft tissue sarcomas affecting both genders equally. […] Most commonly diagnosed in adults between the third and fifth decades, DFSP can manifest across all age groups. Children account for 6% of all cases of DFSP. […] DFSP, including the pigmented variant, is more common in Black individuals than White.
  • #17 Dermatofibrosarcoma protuberans: Epidemiology, pathogenesis, clinical presentation, diagnosis, and staging – UpToDate
    https://www.uptodate.com/contents/dermatofibrosarcoma-protuberans-epidemiology-pathogenesis-clinical-presentation-diagnosis-and-staging
    DFSP most often arises in adults in their thirties, but it has been described in all age groups including children and older adults; rarely it presents congenitally. In one large series, children accounted for 6 percent of all cases of DFSP. The so-called giant cell fibroblastoma is considered to be the juvenile form of DFSP, given its similar morphology, identical chromosomal translocation, and CD34 positivity.
  • #18 Dermatofibrosarcoma Protuberans: Practice Essentials, Background, Pathophysiology
    https://emedicine.medscape.com/article/1100203-overview
    However, a study conducted by Criscione and Weinstock found the incidence among African Americans (6.5 cases per million population) was almost double the incidence among American whites (3.9 cases per million population). […] Several studies of dermatofibrosarcoma protuberans (DFSP) reveal an almost equal sexual distribution or a slight male predominance. […] In a large study of 902 patients with DFSP conducted by Rutgers et al, 514 (57%) patients were male and 388 (43%) patients were female. […] A study based on 405 DFSP cases from the Swedish National Cancer Registry between 1990 and 2005 shows a very small difference in annual incidence of male (4.4 cases per million) versus female (4.0 cases per million). […] However, a larger cancer registry study of 2885 cases reveals females might have a slightly higher incidence of DFSP, 4.4 cases versus 4.2 cases per million population per year. […] Dermatofibrosarcoma protuberans (DFSP) can occur at any age is is most frequent in adults aged 20-50 years. Rarely, DFSP has been reported in newborns and elderly individuals (80 y).
  • #19 Dermatofibrosarcoma Protuberans: Practice Essentials, Background, Pathophysiology
    https://emedicine.medscape.com/article/1100203-overview
    However, a study conducted by Criscione and Weinstock found the incidence among African Americans (6.5 cases per million population) was almost double the incidence among American whites (3.9 cases per million population). […] Several studies of dermatofibrosarcoma protuberans (DFSP) reveal an almost equal sexual distribution or a slight male predominance. […] In a large study of 902 patients with DFSP conducted by Rutgers et al, 514 (57%) patients were male and 388 (43%) patients were female. […] A study based on 405 DFSP cases from the Swedish National Cancer Registry between 1990 and 2005 shows a very small difference in annual incidence of male (4.4 cases per million) versus female (4.0 cases per million). […] However, a larger cancer registry study of 2885 cases reveals females might have a slightly higher incidence of DFSP, 4.4 cases versus 4.2 cases per million population per year. […] Dermatofibrosarcoma protuberans (DFSP) can occur at any age is is most frequent in adults aged 20-50 years. Rarely, DFSP has been reported in newborns and elderly individuals (80 y).
  • #20 Dermatofibrosarcoma Protuberans: Practice Essentials, Background, Pathophysiology
    https://emedicine.medscape.com/article/1100203-overview
    However, a study conducted by Criscione and Weinstock found the incidence among African Americans (6.5 cases per million population) was almost double the incidence among American whites (3.9 cases per million population). […] Several studies of dermatofibrosarcoma protuberans (DFSP) reveal an almost equal sexual distribution or a slight male predominance. […] In a large study of 902 patients with DFSP conducted by Rutgers et al, 514 (57%) patients were male and 388 (43%) patients were female. […] A study based on 405 DFSP cases from the Swedish National Cancer Registry between 1990 and 2005 shows a very small difference in annual incidence of male (4.4 cases per million) versus female (4.0 cases per million). […] However, a larger cancer registry study of 2885 cases reveals females might have a slightly higher incidence of DFSP, 4.4 cases versus 4.2 cases per million population per year. […] Dermatofibrosarcoma protuberans (DFSP) can occur at any age is is most frequent in adults aged 20-50 years. Rarely, DFSP has been reported in newborns and elderly individuals (80 y).
  • #21 Dermatofibrosarcoma Protuberans | CancerIndex
    http://www.cancerindex.org/clinks2sd.htm
    Dermatofibrosarcoma protuberans (DFSP) is a rare cutaneous sarcoma for which data on risk factors, incidence, and survival are limited. […] Overall incidence was 4.1 per million person-years and steady over the decade. […] Incidence among women was 1.14 times higher than men (95% confidence interval [CI] of rate ratio: 1.07-1.22). […] Incidence among blacks was almost 2 times the rate among whites (95% CI of rate ratio: 1.8-2.1). […] Ten-year relative survival of DFSP was 99.1% (95% CI: 97.6-99.7). […] Increased age, male sex, black race, and anatomic location of the limbs and head as compared with the trunk were associated with higher all-cause mortality. […] This is the largest population-based study of DFSP derived from a cohort of almost 7,000 patients. […] The epidemiologic profile of DFSP differs from most skin cancers.
  • #22 Dermatofibrosarcoma Protuberans: Update on the Diagnosis and Treatment
    https://www.mdpi.com/2077-0383/9/6/1752
    Dermatofibrosarcoma protuberans (DFSP) is an uncommon, indolent dermal soft-tissue sarcoma that accounts for less than 0.1% of all malignancies and less than 1% of all soft-tissue sarcomas. […] Two large epidemiological studies in the United States demonstrated that the annual incidence of DFSP was 4.2 per million people studied in a 30-year period from 1973 to 2002, and 4.1 per million people in a 10-year period between 2000 and 2010. […] The incidence of DFSP is higher in women than men, and higher in African-American than white patients. […] It most frequently occurs in young and middle-aged patients, between 25 and 45 years of age, with a mean age between 40 to 43 years. […] However, patient’s age ranged widely from infancy to the elderly. […] The prognoses of the patients with DFSP, after surgical resection with negative and sometimes even positive microscopic margins, are generally good.
  • #23 Dermatofibrosarcoma protuberans – Wikipedia
    https://en.wikipedia.org/wiki/Dermatofibrosarcoma_protuberans
    Dermatofibrosarcoma protuberans (DFSP) is a rare locally aggressive malignant cutaneous soft-tissue sarcoma. Estimates of the overall occurrence of DFSP in the United States are 0.8 to 4.5 cases per million persons per year. In the United States, DFSP accounts for between 1 and 6 percent of all soft-tissue sarcomas and 18 percent of all cutaneous soft-tissue sarcomas. In the Surveillance, Epidemiology and End Results (SEER) tumor registry from 1992 through 2004, DFSP was second only to Kaposi sarcoma. […] Commonly located on the chest and shoulders, the following is the site distribution of DFPS as was observed in Surveillance, Epidemiology, and End Results (SEER) database between 2000 and 2010. […] DFSP is not extensively studied due to its rarity and low mortality. The majority of studies are small size case studies or meta-analysis.
  • #24 Dermatofibrosarcoma protuberans | Radiology Reference Article | Radiopaedia.org
    https://radiopaedia.org/articles/dermatofibrosarcoma-protuberans?embed_domain=external.radpair.com%2527%255B0%255Dfavicon.icoradiopaedia-icon-144.png&lang=us
    Dermatofibrosarcoma protuberans (DFSP) is a low-grade malignant tumor arising from dermal and subcutaneous tissues and is the most common cutaneous sarcoma (although overall still quite rare). It is most commonly found at the trunk and proximal extremities. […] The tumor occurs in patients of all ages, with the highest frequency occurring between ages 20-50 years. Males are slightly more commonly affected than females. […] Its behavior is notable for a high reported local recurrence rate, although outcomes overall are favorable.
  • #25 Dermatofibrosarcoma Protuberans: A Case Series of 16 Patients Treated in a Single Institution with Literature Review | Anticancer Research
    https://ar.iiarjournals.org/content/30/9/3775
    DFSP typically arises on the trunk of the body and proximal extremities, followed by the distal extremities, and then head and neck with a slight male predomination. […] DFSP has a tendency for local recurrence of up to 25% and most recurrences tend to develop within 3 years, after resection. […] Recent study by Fiore et al. confirms that most cases of DFSP, including recurrent disease, can be managed with wide-excision surgery, providing a crude incidence of recurrence in the entire population 3% at 5 years and 4.2% at 10 years and a crude incidence of distant metastasis of 1.7% at both 5 and 10 years. […] The prognosis of DFSP is generally excellent if completely excised with clear margins. […] Most authors suggest a margin of 2.5 to 3 cm of normal tissue from the gross tumour boundary, with a three-dimensional resection (en bloc removal) that includes skin, subcutaneous tissue and the underlying investing fascia, achieving negative microscopic margins.
  • #26 Dermatofibrosarcoma Protuberans | CancerIndex
    http://www.cancerindex.org/clinks2sd.htm
    Dermatofibrosarcoma protuberans (DFSP) is a rare cutaneous sarcoma for which data on risk factors, incidence, and survival are limited. […] Overall incidence was 4.1 per million person-years and steady over the decade. […] Incidence among women was 1.14 times higher than men (95% confidence interval [CI] of rate ratio: 1.07-1.22). […] Incidence among blacks was almost 2 times the rate among whites (95% CI of rate ratio: 1.8-2.1). […] Ten-year relative survival of DFSP was 99.1% (95% CI: 97.6-99.7). […] Increased age, male sex, black race, and anatomic location of the limbs and head as compared with the trunk were associated with higher all-cause mortality. […] This is the largest population-based study of DFSP derived from a cohort of almost 7,000 patients. […] The epidemiologic profile of DFSP differs from most skin cancers.
  • #27 Dermatofibrosarcoma protuberans (DFSP) successfully treated with sorafenib: case report | Clinical Sarcoma Research | Full Text
    https://clinicalsarcomaresearch.biomedcentral.com/articles/10.1186/2045-3329-3-5
    Dermatofibrosarcoma Protuberans (DFSP) has an annual incidence of only 0.8 cases per million and presents typically at mid-adult life with a slight male predominance. […] DFSP accounts for 2 to 6% of all soft tissue sarcomas. […] The most significant prognostic factor in patients with DFSP has proved to be the extent of surgical resection. […] Due to its infiltrative nature, DFSP is characterized by a high recurrence rate varying in the literature from 10-80%. […] DFSP is considered as one of the radiosensitive tumors. […] Since the recent identification of the significance of PDGFR activation in the DFSP pathogenesis, the effectiveness of targeted chemotherapy by inhibition of the PDGFR protein-tyrosine kinase has been evaluated. […] We report the first case of recurrent and extensive DFSP successfully treated with Sorafenib after failure of postoperative radiation therapy and refractory to treatment with 800 mg of Imatinib. […] Sorafenib could represent a therapeutic alternative in such cases of DFSP.
  • #28 Dermatofibrosarcoma Protuberans, Version 1.2025, NCCN Clinical Practice Guidelines In Oncology in: Journal of the National Comprehensive Cancer Network Volume 23 Issue 1 (2025)
    https://jnccn.org/abstract/journals/jnccn/23/1/article-e250001.xml
    Dermatofibrosarcoma protuberans (DFSP) is a rare cutaneous soft tissue sarcoma and affects an estimated 1,500 people annually in the United States. […] DFSP frequently exhibits extensive local infiltration. […] Although DFSP has a reported high rate of recurrence, metastasis is more uncommon. […] A predilection for occurring in African Americans has been reported in one study. […] The local recurrence rate for wide local excision (WLE) of DFSP in studies ranges from 10% to 60%, whereas the rate of development of regional or distant metastatic disease is only 1% and 4%, respectively. […] Given the historically high local recurrence rates for DFSP, ongoing clinical follow-up with focus on the primary site every 6 to 12 months is indicated, with rebiopsy of any suspicious regions. […] Although metastatic disease is rare, a guided history and physical and patient education about regular self-examination are recommended. […] In the rare event of metastatic disease, multidisciplinary consultation is recommended to coordinate treatment.
  • #29 Dermatofibrosarcoma protuberans
    https://dermnetnz.org/topics/dermatofibrosarcoma-protuberans
    Dermatofibrosarcoma protuberans is rare and affects less than 1 person in every 100,000 inhabitants per year. […] It usually presents in early or middle adult life between 20 and 59 years of age, but all ages can be affected. The tumour is rare in children. […] Males are affected slightly more frequently than females. […] There does not appear to be any racial predilection. […] Follow-up with clinical examination of the site of the dermatofibrosarcoma protuberans is recommended every 6 months for 5 years, and then annually. […] The tumour only metastasises in 5% of cases. It spreads in 1% via lymphatic vessels to the regional lymph glands and in 4% via the bloodstream, most commonly to the lung followed by the brain, bone and heart. […] Local recurrences arise in 12-20% of cases, usually within 3 years of initial surgery, so follow-up is important.
  • #30 Dermatofibrosarcoma Protuberans: A Case Series of 16 Patients Treated in a Single Institution with Literature Review | Anticancer Research
    https://ar.iiarjournals.org/content/30/9/3775
    DFSP typically arises on the trunk of the body and proximal extremities, followed by the distal extremities, and then head and neck with a slight male predomination. […] DFSP has a tendency for local recurrence of up to 25% and most recurrences tend to develop within 3 years, after resection. […] Recent study by Fiore et al. confirms that most cases of DFSP, including recurrent disease, can be managed with wide-excision surgery, providing a crude incidence of recurrence in the entire population 3% at 5 years and 4.2% at 10 years and a crude incidence of distant metastasis of 1.7% at both 5 and 10 years. […] The prognosis of DFSP is generally excellent if completely excised with clear margins. […] Most authors suggest a margin of 2.5 to 3 cm of normal tissue from the gross tumour boundary, with a three-dimensional resection (en bloc removal) that includes skin, subcutaneous tissue and the underlying investing fascia, achieving negative microscopic margins.
  • #31 Dermatofibrosarcoma Protuberans: A Case Series of 16 Patients Treated in a Single Institution with Literature Review | Anticancer Research
    https://ar.iiarjournals.org/content/30/9/3775
    DFSP typically arises on the trunk of the body and proximal extremities, followed by the distal extremities, and then head and neck with a slight male predomination. […] DFSP has a tendency for local recurrence of up to 25% and most recurrences tend to develop within 3 years, after resection. […] Recent study by Fiore et al. confirms that most cases of DFSP, including recurrent disease, can be managed with wide-excision surgery, providing a crude incidence of recurrence in the entire population 3% at 5 years and 4.2% at 10 years and a crude incidence of distant metastasis of 1.7% at both 5 and 10 years. […] The prognosis of DFSP is generally excellent if completely excised with clear margins. […] Most authors suggest a margin of 2.5 to 3 cm of normal tissue from the gross tumour boundary, with a three-dimensional resection (en bloc removal) that includes skin, subcutaneous tissue and the underlying investing fascia, achieving negative microscopic margins.
  • #32 Soft Tissue Sarcoma Surgery Follow-Up Article | Surgical Oncology
    https://surgicaloncology.co.uk/surgical-treatment-of-dermatofibrosarcoma-protuberans-dfsp-should-follow-up-be-individualised/
    This study was consistent with what we know about DFSP and, moreover, identified that most tumour recurrences were actually identified by patient self-examination (61.7%), as opposed to a small percentage identified by clinical examination by the medical team (3.7%). […] Hence, the conclusion of the study was, that it may be able to individualise patient follow up after surgery for DFSP. […] For example, patients with small (<5cm) tumours, that have been completely excised (R0), with no fibrosarcomatous change, could be offered patient directed follow up as opposed to routine surveillance for up to 10 years. [...] In an accompanying editorial, ‘Risk-Stratified Surveillance in Dermatofibrosarcoma Protuberans: Less Is More’, doctors Rebecca Gladdy and Jay Wunder of Mount Sinai Hospital, Toronto, welcomed the findings of the paper, whilst emphasising that patients with complex or high-risk DFSPs (FS transformation, large tumours, anatomic location) should be followed up by experts, in keeping with current expert guidelines.
  • #33 Dermatofibrosarcoma protuberans
    https://dermnetnz.org/topics/dermatofibrosarcoma-protuberans
    Dermatofibrosarcoma protuberans is rare and affects less than 1 person in every 100,000 inhabitants per year. […] It usually presents in early or middle adult life between 20 and 59 years of age, but all ages can be affected. The tumour is rare in children. […] Males are affected slightly more frequently than females. […] There does not appear to be any racial predilection. […] Follow-up with clinical examination of the site of the dermatofibrosarcoma protuberans is recommended every 6 months for 5 years, and then annually. […] The tumour only metastasises in 5% of cases. It spreads in 1% via lymphatic vessels to the regional lymph glands and in 4% via the bloodstream, most commonly to the lung followed by the brain, bone and heart. […] Local recurrences arise in 12-20% of cases, usually within 3 years of initial surgery, so follow-up is important.
  • #34 Dermatofibrosarcoma Protuberans | Actas Dermo-Sifiliográficas
    http://www.actasdermo.org/en-dermatofibrosarcoma-protuberans-articulo-S1578219007704006
    Dermatofibrosarcoma protuberans (DFSP) is a soft tissue neoplasm of intermediate malignancy that is initially localized to the skin from where it can invade deep structures (fat, fascia, muscle and bone). It is the most frequent fibrohistiocytic tumor, comprising approximately 1.8% of all soft tissue sarcomas and 0.1% of all cancers. It has an estimated incidence of 0.8-5 cases per one million persons per year. […] Although the cases of metastatic DFSP do not reach 5% of the total, almost all of them appear after previous local relapses. The prognosis for metastatic cases is very poor with a survival of less than 2 years following detection of metastatic disease. […] In this regard, chemotherapy and radiotherapy have not been highly efficacious so it is necessary to consider new alternatives.
  • #35 The epidemiology of dermatofibrosarcoma protuberans incidence, metastasis, and death among various population groups: A Surveillance, Epidemiology, and End Results database analysis
    https://scholarlycommons.henryford.com/publichealthsciences_articles/557/
    BACKGROUND: Limited information exists regarding the epidemiology, metastasis, and survival of dermatofibrosarcoma protuberans (DFSP). […] DFSP incidence rate was 6.25 (95% CI, 5.93-6.57) cases per million person-years with significantly higher incidence observed among Black individuals than White individuals (8.74 vs 4.53). […] DFSP incidence is 2-fold higher among Black than White individuals. The risk of DFSP metastasis is significantly increased with tumor size 3 cm and tumors located on head and neck, and genitalia. Larger tumor size ( 3 cm), regardless of location, and age (60 years) are the most important prognostic indicators of survival.
  • #36 Dermatofibrosarcoma Protuberans | Treatment & Management | Point of Care
    https://www.statpearls.com/point-of-care/20385
    DFSP is a rare tumor, occurring at a rate of 0.8 to 4.5 cases per million persons annually and represents 1% to 6% of all soft tissue sarcomas and 18% of cutaneous soft tissue sarcomas affecting both genders equally. […] Some studies report a slight predominance in med. While most commonly diagnosed in adults between the third and fifth decades, DFSP can manifest across all age groups. Children account for 6% of all cases of DFSP. This tumor may exhibit accelerated growth during pregnancy. […] DFSP-FS accounts for 5% to 15% of all DFSPs, and the Bednar or pigmented variant represents fewer than 5% of all DFSP cases. DFSP, including the pigmented variant, is more common in Black individuals than White. […] Patients should participate in regular self-examination, and the primary site should be examined every 6 months for 3 to 5 years. Lifelong examination annually follows. […] Metastatic lesions are most likely to occur with recurrent lesions that have progressed for many years and when a fibrosarcomatous component is present. Some authors recommend limiting follow-up imaging examinations to those with recurrent DFSP or DFSP-FS.
  • #37 Dermatofibrosarcoma protuberans: Epidemiology, pathogenesis, clinical presentation, diagnosis, and staging – UpToDate
    https://www.uptodate.com/contents/dermatofibrosarcoma-protuberans-epidemiology-pathogenesis-clinical-presentation-diagnosis-and-staging
    Dermatofibrosarcoma protuberans (DFSP) is a relatively rare tumor. Estimates of the overall incidence of DFSP in the United States are 0.8 to 4.5 cases per million persons per year. In the United States, DFSP accounts for between 1 and 6 percent of all soft tissue sarcomas and 18 percent of all cutaneous soft tissue sarcomas. In a series of 12,114 cutaneous soft tissue sarcomas derived from the Surveillance, Epidemiology and End Results (SEER) tumor registry from 1992 through 2004, DFSP was second only to Kaposi sarcoma, which accounted for 71 percent of cases. […] The Bednar or pigmented variant (distinguished by the dispersal of melanin-containing cells in an otherwise typical DFSP) is even less common. It represents fewer than 5 percent of all DFSP cases and is more common in Black individuals. The fibrosarcomatous variant of DFSP (DFSP-FS) accounts for approximately 5 to 15 percent of DFSPs.
  • #38 Dermatofibrosarcoma Protuberans | Treatment & Management | Point of Care
    https://www.statpearls.com/point-of-care/20385
    DFSP is a rare tumor, occurring at a rate of 0.8 to 4.5 cases per million persons annually and represents 1% to 6% of all soft tissue sarcomas and 18% of cutaneous soft tissue sarcomas affecting both genders equally. […] Some studies report a slight predominance in med. While most commonly diagnosed in adults between the third and fifth decades, DFSP can manifest across all age groups. Children account for 6% of all cases of DFSP. This tumor may exhibit accelerated growth during pregnancy. […] DFSP-FS accounts for 5% to 15% of all DFSPs, and the Bednar or pigmented variant represents fewer than 5% of all DFSP cases. DFSP, including the pigmented variant, is more common in Black individuals than White. […] Patients should participate in regular self-examination, and the primary site should be examined every 6 months for 3 to 5 years. Lifelong examination annually follows. […] Metastatic lesions are most likely to occur with recurrent lesions that have progressed for many years and when a fibrosarcomatous component is present. Some authors recommend limiting follow-up imaging examinations to those with recurrent DFSP or DFSP-FS.
  • #39 Dermatofibrosarcoma Protuberans – SFA
    https://curesarcoma.org/sarcoma-subtypes/dermatofibrosarcoma-protuberans/
    Dermatofibrosarcoma protuberans (DFSP) is a rare, locally aggressive skin tumor characterized by fibroblastic proliferation and a distinctive genetic fusion. […] DFSP usually presents in young to middle-aged adults, with a slight male predominance. However, a substantial number of cases are seen in children (including congenital presentations) and in the elderly. Although it represents a rare neoplasm (< 1 case per 100,000 person-years), DFSP is one of the most common dermal sarcomas. [...] DFSP is characterized by locally aggressive growth and frequent, often repeated local recurrences unless widely excised. The rate of local recurrences varies from 20% to 50% in the setting of inadequate margins. In contrast, ordinary DFSP almost never metastasizes. Higher-grade fibrosarcomatous progression is seen in 5% of cases. Fibrosarcomatous DFSP exhibits more aggressive behavior than ordinary DFSP, and 10–15% of patients develop distant metastases, most often to the lungs. Histological grading has not been shown to be prognostic in fibrosarcomatous DFSP.
  • #40 Dermatofibrosarcoma Protuberans | CancerIndex
    http://www.cancerindex.org/clinks2sd.htm
    Dermatofibrosarcoma protuberans (DFSP) is a rare cutaneous sarcoma for which data on risk factors, incidence, and survival are limited. […] Overall incidence was 4.1 per million person-years and steady over the decade. […] Incidence among women was 1.14 times higher than men (95% confidence interval [CI] of rate ratio: 1.07-1.22). […] Incidence among blacks was almost 2 times the rate among whites (95% CI of rate ratio: 1.8-2.1). […] Ten-year relative survival of DFSP was 99.1% (95% CI: 97.6-99.7). […] Increased age, male sex, black race, and anatomic location of the limbs and head as compared with the trunk were associated with higher all-cause mortality. […] This is the largest population-based study of DFSP derived from a cohort of almost 7,000 patients. […] The epidemiologic profile of DFSP differs from most skin cancers.
  • #41 Dermatofibrosarcoma Protuberans: Update on the Diagnosis and Treatment
    https://www.mdpi.com/2077-0383/9/6/1752
    The five- and ten-year recurrence-free survival rates of DFSP are 86% and 76%, respectively. […] Local recurrence is a major concern after surgical excision. […] Therefore, mandatory long-term surveillance, at an interval of 6 to 12 months, is recommended. […] Several studies suggest patients should be reevaluated every 6 months for the first five years and then yearly thereafter.
  • #42
    https://journals.lww.com/dermatologicsurgery/fulltext/2023/09000/survival_outcomes_and_prognostic_factors_of.2.aspx
    Limited information is available regarding survival outcomes and risk factors of dermatofibrosarcoma protuberans (DFSP). […] The study cohort (7,567 patients) was selected from the Surveillance, Epidemiology, and End Results Program (20002018). […] Independent risk factors for cancer-specific mortality included age at diagnosis, histologic grade, and tumor size. […] Dermatofibrosarcoma protuberans-specific mortality is significantly higher in patients with grade III or large (10 cm) tumors. […] The median follow-up duration was 92 months for all patients with DFSP. […] The 10- and 15-year DFSP-specific survival rates were 98.5% and 98.0%, respectively. […] Significant factors in the univariate survival analysis were used as explanatory variables in multivariate Cox regression models.
  • #43 Dermatofibrosarcoma Protuberans | CancerIndex
    http://www.cancerindex.org/clinks2sd.htm
    Dermatofibrosarcoma protuberans (DFSP) is a rare cutaneous sarcoma for which data on risk factors, incidence, and survival are limited. […] Overall incidence was 4.1 per million person-years and steady over the decade. […] Incidence among women was 1.14 times higher than men (95% confidence interval [CI] of rate ratio: 1.07-1.22). […] Incidence among blacks was almost 2 times the rate among whites (95% CI of rate ratio: 1.8-2.1). […] Ten-year relative survival of DFSP was 99.1% (95% CI: 97.6-99.7). […] Increased age, male sex, black race, and anatomic location of the limbs and head as compared with the trunk were associated with higher all-cause mortality. […] This is the largest population-based study of DFSP derived from a cohort of almost 7,000 patients. […] The epidemiologic profile of DFSP differs from most skin cancers.
  • #44
    https://journals.lww.com/dermatologicsurgery/fulltext/2023/09000/survival_outcomes_and_prognostic_factors_of.2.aspx
    Limited information is available regarding survival outcomes and risk factors of dermatofibrosarcoma protuberans (DFSP). […] The study cohort (7,567 patients) was selected from the Surveillance, Epidemiology, and End Results Program (20002018). […] Independent risk factors for cancer-specific mortality included age at diagnosis, histologic grade, and tumor size. […] Dermatofibrosarcoma protuberans-specific mortality is significantly higher in patients with grade III or large (10 cm) tumors. […] The median follow-up duration was 92 months for all patients with DFSP. […] The 10- and 15-year DFSP-specific survival rates were 98.5% and 98.0%, respectively. […] Significant factors in the univariate survival analysis were used as explanatory variables in multivariate Cox regression models.
  • #45
    https://journals.lww.com/dermatologicsurgery/fulltext/2023/09000/survival_outcomes_and_prognostic_factors_of.2.aspx
    The model for cancer-specific mortality included age at diagnosis, histologic grade, and tumor size. […] Tumor location (skin or soft tissue) and surgery were not independent risk factors for OS and cancer-specific survival. […] The risk of death was significantly greater in some exceptional cases, such as tumors with histologic grade III or a size larger than 10 cm. […] Overall, our study confirmed that DFSP is a low-grade tumor with a good prognosis and survival rate, even in patients with node-positive or distant metastases.
  • #46
    https://link.springer.com/article/10.1007/s13671-024-00432-0
    Kreicher et al.’s analysis of the National Cancer Institute’s Surveillance, Epidemiology, and End Results (SEER) data spanning 2000-2010 revealed that Blacks had a 1.7 times greater all-cause mortality risk compared to Whites, suggesting that Black race could be a negative survival predictor. […] The introduction of HAART has led to a nationwide decrease in KS incidence among HIV-positive individuals in the U.S. […] Despite this overall decline, notable disparities persist in the incidence and survival rates of KS among various racial groups in the HAART era. […] A study by Royse et al. reports a decrease in KS among White males, but an increase among Black males in the southern U.S. from 2000 to 2013. […] The incidence in Black males was six times higher, which is also supported by other studies analyzing SEER data.
  • #47 Dermatofibrosarcoma Protuberans | Treatment & Management | Point of Care
    https://www.statpearls.com/point-of-care/20385
    DFSP is a rare tumor, occurring at a rate of 0.8 to 4.5 cases per million persons annually and represents 1% to 6% of all soft tissue sarcomas and 18% of cutaneous soft tissue sarcomas affecting both genders equally. […] Some studies report a slight predominance in med. While most commonly diagnosed in adults between the third and fifth decades, DFSP can manifest across all age groups. Children account for 6% of all cases of DFSP. This tumor may exhibit accelerated growth during pregnancy. […] DFSP-FS accounts for 5% to 15% of all DFSPs, and the Bednar or pigmented variant represents fewer than 5% of all DFSP cases. DFSP, including the pigmented variant, is more common in Black individuals than White. […] Patients should participate in regular self-examination, and the primary site should be examined every 6 months for 3 to 5 years. Lifelong examination annually follows. […] Metastatic lesions are most likely to occur with recurrent lesions that have progressed for many years and when a fibrosarcomatous component is present. Some authors recommend limiting follow-up imaging examinations to those with recurrent DFSP or DFSP-FS.
  • #48 Dermatofibrosarcoma Protuberans: Update on the Diagnosis and Treatment
    https://www.mdpi.com/2077-0383/9/6/1752
    The five- and ten-year recurrence-free survival rates of DFSP are 86% and 76%, respectively. […] Local recurrence is a major concern after surgical excision. […] Therefore, mandatory long-term surveillance, at an interval of 6 to 12 months, is recommended. […] Several studies suggest patients should be reevaluated every 6 months for the first five years and then yearly thereafter.
  • #49 Dermatofibrosarcoma Protuberans | Treatment & Management | Point of Care
    https://www.statpearls.com/point-of-care/20385
    DFSP is a rare tumor, occurring at a rate of 0.8 to 4.5 cases per million persons annually and represents 1% to 6% of all soft tissue sarcomas and 18% of cutaneous soft tissue sarcomas affecting both genders equally. […] Some studies report a slight predominance in med. While most commonly diagnosed in adults between the third and fifth decades, DFSP can manifest across all age groups. Children account for 6% of all cases of DFSP. This tumor may exhibit accelerated growth during pregnancy. […] DFSP-FS accounts for 5% to 15% of all DFSPs, and the Bednar or pigmented variant represents fewer than 5% of all DFSP cases. DFSP, including the pigmented variant, is more common in Black individuals than White. […] Patients should participate in regular self-examination, and the primary site should be examined every 6 months for 3 to 5 years. Lifelong examination annually follows. […] Metastatic lesions are most likely to occur with recurrent lesions that have progressed for many years and when a fibrosarcomatous component is present. Some authors recommend limiting follow-up imaging examinations to those with recurrent DFSP or DFSP-FS.
  • #50 Dermatofibrosarcoma Protuberans | Treatment & Management | Point of Care
    https://www.statpearls.com/point-of-care/20385
    DFSP is a rare tumor, occurring at a rate of 0.8 to 4.5 cases per million persons annually and represents 1% to 6% of all soft tissue sarcomas and 18% of cutaneous soft tissue sarcomas affecting both genders equally. […] Some studies report a slight predominance in med. While most commonly diagnosed in adults between the third and fifth decades, DFSP can manifest across all age groups. Children account for 6% of all cases of DFSP. This tumor may exhibit accelerated growth during pregnancy. […] DFSP-FS accounts for 5% to 15% of all DFSPs, and the Bednar or pigmented variant represents fewer than 5% of all DFSP cases. DFSP, including the pigmented variant, is more common in Black individuals than White. […] Patients should participate in regular self-examination, and the primary site should be examined every 6 months for 3 to 5 years. Lifelong examination annually follows. […] Metastatic lesions are most likely to occur with recurrent lesions that have progressed for many years and when a fibrosarcomatous component is present. Some authors recommend limiting follow-up imaging examinations to those with recurrent DFSP or DFSP-FS.
  • #51 Dermatofibrosarcoma protuberans (DFSP): Foothill Dermatology Medical Center: Dermatology
    https://www.foothillderm.com/blog/dermatofibrosarcoma-protuberans-dfsp
    Dermatofibrosarcoma (dur-mah-toe-fy-bro-sar-co-ma) protuberans (pro-to-bur-anz) (DFSP) is a rare skin cancer. It begins in the middle layer of skin, the dermis. DFSP tends to grow slowly. It seldom spreads to other parts of the body. […] People of all races get DFSP. In looking at the U.S. cancer records, dermatologists discovered that between 1973 and 2002: […] Cases of DFSP are increasing; this increase is greatest among whites. […] This skin cancer rarely spreads to other parts of the body, so people often live for many years after treatment. […] Lifelong follow-up with your doctors is essential though. DFSP can return after treatment. If DFSP returns, it is often treated with one of the surgeries described above.
  • #52 Dermatofibrosarcoma protuberans (DFSP) | Acclaim Dermatology
    https://acclaimdermatology.com/blog/dermatofibrosarcoma-protuberans-dfsp
    Dermatofibrosarcoma protuberans (DFSP) is a rare skin cancer. […] DFSP tends to occur between the ages of 20 and 50. […] Most people are diagnosed when they are between 20 and 50 years of age. […] People of all races get DFSP. […] Cases of DFSP are increasing; this increase is greatest among whites. […] Scientists do not yet know what causes DFSP. […] In treating patients with DFSP, dermatologists have learned that this cancer sometimes begins on skin that was badly injured. […] Lifelong follow-up with your doctors is essential though. DFSP can return after treatment. […] If DFSP returns, it usually returns within 3 years of treatment. […] For this reason, dermatologists recommend lifelong follow-up exams.
  • #53 Soft Tissue Sarcoma Surgery Follow-Up Article | Surgical Oncology
    https://surgicaloncology.co.uk/surgical-treatment-of-dermatofibrosarcoma-protuberans-dfsp-should-follow-up-be-individualised/
    This study was consistent with what we know about DFSP and, moreover, identified that most tumour recurrences were actually identified by patient self-examination (61.7%), as opposed to a small percentage identified by clinical examination by the medical team (3.7%). […] Hence, the conclusion of the study was, that it may be able to individualise patient follow up after surgery for DFSP. […] For example, patients with small (<5cm) tumours, that have been completely excised (R0), with no fibrosarcomatous change, could be offered patient directed follow up as opposed to routine surveillance for up to 10 years. [...] In an accompanying editorial, ‘Risk-Stratified Surveillance in Dermatofibrosarcoma Protuberans: Less Is More’, doctors Rebecca Gladdy and Jay Wunder of Mount Sinai Hospital, Toronto, welcomed the findings of the paper, whilst emphasising that patients with complex or high-risk DFSPs (FS transformation, large tumours, anatomic location) should be followed up by experts, in keeping with current expert guidelines.
  • #54 Soft Tissue Sarcoma Surgery Follow-Up Article | Surgical Oncology
    https://surgicaloncology.co.uk/surgical-treatment-of-dermatofibrosarcoma-protuberans-dfsp-should-follow-up-be-individualised/
    This study was consistent with what we know about DFSP and, moreover, identified that most tumour recurrences were actually identified by patient self-examination (61.7%), as opposed to a small percentage identified by clinical examination by the medical team (3.7%). […] Hence, the conclusion of the study was, that it may be able to individualise patient follow up after surgery for DFSP. […] For example, patients with small (<5cm) tumours, that have been completely excised (R0), with no fibrosarcomatous change, could be offered patient directed follow up as opposed to routine surveillance for up to 10 years. [...] In an accompanying editorial, ‘Risk-Stratified Surveillance in Dermatofibrosarcoma Protuberans: Less Is More’, doctors Rebecca Gladdy and Jay Wunder of Mount Sinai Hospital, Toronto, welcomed the findings of the paper, whilst emphasising that patients with complex or high-risk DFSPs (FS transformation, large tumours, anatomic location) should be followed up by experts, in keeping with current expert guidelines.
  • #55 Dermatofibrosarcoma Protuberans, Version 1.2025, NCCN Clinical Practice Guidelines In Oncology in: Journal of the National Comprehensive Cancer Network Volume 23 Issue 1 (2025)
    https://jnccn.org/abstract/journals/jnccn/23/1/article-e250001.xml
    Dermatofibrosarcoma protuberans (DFSP) is a rare cutaneous soft tissue sarcoma and affects an estimated 1,500 people annually in the United States. […] DFSP frequently exhibits extensive local infiltration. […] Although DFSP has a reported high rate of recurrence, metastasis is more uncommon. […] A predilection for occurring in African Americans has been reported in one study. […] The local recurrence rate for wide local excision (WLE) of DFSP in studies ranges from 10% to 60%, whereas the rate of development of regional or distant metastatic disease is only 1% and 4%, respectively. […] Given the historically high local recurrence rates for DFSP, ongoing clinical follow-up with focus on the primary site every 6 to 12 months is indicated, with rebiopsy of any suspicious regions. […] Although metastatic disease is rare, a guided history and physical and patient education about regular self-examination are recommended. […] In the rare event of metastatic disease, multidisciplinary consultation is recommended to coordinate treatment.
  • #56 Dermatofibrosarcoma protuberans (DFSP): Foothill Dermatology Medical Center: Dermatology
    https://www.foothillderm.com/blog/dermatofibrosarcoma-protuberans-dfsp
    Dermatofibrosarcoma (dur-mah-toe-fy-bro-sar-co-ma) protuberans (pro-to-bur-anz) (DFSP) is a rare skin cancer. It begins in the middle layer of skin, the dermis. DFSP tends to grow slowly. It seldom spreads to other parts of the body. […] People of all races get DFSP. In looking at the U.S. cancer records, dermatologists discovered that between 1973 and 2002: […] Cases of DFSP are increasing; this increase is greatest among whites. […] This skin cancer rarely spreads to other parts of the body, so people often live for many years after treatment. […] Lifelong follow-up with your doctors is essential though. DFSP can return after treatment. If DFSP returns, it is often treated with one of the surgeries described above.
  • #57 Dermatofibrosarcoma protuberans: from translocation to targeted therapy | Cancer Biology & Medicine
    https://www.cancerbiomed.org/content/12/4/375
    Dermatofibrosarcoma protuberans (DFSP), the most common dermal sarcoma, is a low-grade, slow growing fibroblastic malignant neoplasm that most frequently affects middle aged adults and is characterized by a high local recurrence rate and a low propensity for metastasis. […] The incidence of DFSP is estimated to be around 0.8-4.2 cases per million persons per year. Due to better recognition and improvement in ancillary diagnostic modalities, the number of reported cases has increased steadily over the last decades. […] While the development of metastatic disease is rare (5%), rates of local recurrence are significant (20%-50%), with median time to local recurrence of around 32 months; long-term follow-up is therefore mandatory. […] The global prognosis of DFSP is excellent, with reported 2- and 5-year survival rates of 97% and 92%, respectively.
  • #58
    https://link.springer.com/article/10.1007/s13671-024-00432-0
    Given that DFSP in POC is underexplored, it is important that Dermatologists are aware of the existing pathophysiological and clinical disparities to best treat POC. […] DFSP exhibits low metastatic potential (15%), and race has not been established as a significant prognostic factor for distant spread. […] Local recurrence is a characteristic concern, often due to the tumors infiltrative growth pattern and the difficulty in defining its margins. […] Complete surgical resection with wide local excision (WLE) or Mohs micrographic surgery (MMS) is the primary treatment for DFSP. […] Research by Criscito et al. reveals disparities in treatment selection, showing that MMS is more frequently administered to white patients, women, and individuals with high median household income, as opposed to Blacks, males, and lower median household income counterparts.
  • #59 Dermatofibrosarcoma Protuberans | CancerIndex
    http://www.cancerindex.org/clinks2sd.htm
    The first effective systemic therapy in DFSP introduced into clinical practice was imatinib, demonstrating dramatic activity in advanced cases. […] Current results indicate that some DFSP patients initially evaluated as unresectable/metastatic or necessitating mutilating surgery turned resectable after imatinib therapy and this rational approach leading to complete remission may be potentially curative. […] Therapeutic decisions for patients with fibrosarcomatous DFSP should be primarily made by an interdisciplinary oncology team (’tumour board’).
  • #60 Dermatofibrosarcoma protuberans (DFSP) successfully treated with sorafenib: case report | Clinical Sarcoma Research | Full Text
    https://clinicalsarcomaresearch.biomedcentral.com/articles/10.1186/2045-3329-3-5
    Dermatofibrosarcoma Protuberans (DFSP) has an annual incidence of only 0.8 cases per million and presents typically at mid-adult life with a slight male predominance. […] DFSP accounts for 2 to 6% of all soft tissue sarcomas. […] The most significant prognostic factor in patients with DFSP has proved to be the extent of surgical resection. […] Due to its infiltrative nature, DFSP is characterized by a high recurrence rate varying in the literature from 10-80%. […] DFSP is considered as one of the radiosensitive tumors. […] Since the recent identification of the significance of PDGFR activation in the DFSP pathogenesis, the effectiveness of targeted chemotherapy by inhibition of the PDGFR protein-tyrosine kinase has been evaluated. […] We report the first case of recurrent and extensive DFSP successfully treated with Sorafenib after failure of postoperative radiation therapy and refractory to treatment with 800 mg of Imatinib. […] Sorafenib could represent a therapeutic alternative in such cases of DFSP.

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przerzut do węzłów chłonnych dermatofibrosarcoma protuberans remisja całkowita przerzut odległy przerzut do płuc mięsak skóry czynnik prognostyczny transformacja włókniakomięsakowa brodawczakowłókniak kosmówkowy receptor płytkopochodnego czynnika wzrostu badanie epidemiologiczne nowotwór skóry wskaźnik przeżycia imatynib nawrót miejscowy zapadalność włókniakomięsak szerokie wycięcie miejscowe terapia celowana chirurgia mikrograficzna Mohsa mięsak tkanek miękkich sorafenib mięsak Kaposiego