Naczyniakomłoniak
Etiologia i przyczyny

Naczyniakomłoniak (angiosarcoma) to rzadki, wysoce złośliwy mięsak tkanek miękkich, wywodzący się z komórek endotelialnych naczyń krwionośnych lub limfatycznych, stanowiący 1-2% wszystkich mięsaków. Etiologia jest w większości przypadków nieznana, jednak kluczową rolę odgrywają mutacje genetyczne, takie jak utrata funkcji genu p53, nadekspresja MDM2, VEGF, amplifikacje MYC oraz zaburzenia szlaku PIK3CA/AKT/mTOR. Do istotnych czynników ryzyka należą wcześniejsza ekspozycja na promieniowanie jonizujące (zwykle 5-10 lat po radioterapii), przewlekły obrzęk limfatyczny (np. zespół Stewart-Treves), narażenie na toksyczne substancje chemiczne (chlorek winylu, arsen, dwutlenek toru, sterydy anaboliczne) oraz dziedziczne zespoły genetyczne (np. nerwiakowłókniakowatość typu 1, zespół Maffucciego). W patogenezie istotne są także czynniki immunologiczne i środowiskowe, takie jak immunosupresja, promieniowanie UV, ciała obce czy przewlekłe zapalenie.

Etiologia naczyniakomłoniaka (Angiosarcoma Etiology)

Naczyniakomłoniak (angiosarcoma) jest rzadkim, wysoce złośliwym nowotworem wywodzącym się z komórek endotelialnych wyściełających naczynia krwionośne lub limfatyczne. Stanowi on zaledwie około 1-2% wszystkich mięsaków tkanek miękkich.12 Mimo że dokładna etiologia naczyniakomłoniaka pozostaje w większości przypadków nieznana, badania naukowe pozwoliły zidentyfikować szereg czynników ryzyka związanych z rozwojem tego nowotworu.34

Patogeneza molekularna

Naczyniakomłoniak powstaje, gdy komórki wyściełające naczynia krwionośne lub limfatyczne rozwijają zmiany w swoim DNA. Mutacje te powodują, że komórki mnożą się w niekontrolowany sposób i nie ulegają naturalnej śmierci komórkowej, co prowadzi do gromadzenia się nieprawidłowych komórek tworzących guz.56 Zmiany genetyczne odgrywają kluczową rolę w rozwoju naczyniakomłoniaka, zarówno te nabyte w ciągu życia, jak i dziedziczone.7

Badania wykazały, że w rozwoju naczyniakomłoniaka mogą uczestniczyć liczne szlaki molekularne, w tym:89

  • Utrata funkcji genu p53
  • Nadekspresja MDM2 (mouse double minute 2)
  • Nadekspresja VEGF (vascular endothelial growth factor)
  • Amplifikacje genu MYC (szczególnie w naczyniakomłoniakach wtórnych)
  • Zaburzenia szlaku PIK3CA/AKT/mTOR
  • Mutacje w genach PTPRB, PLCG1, CIC, KDR i FLT410

Czynniki ryzyka naczyniakomłoniaka

Ekspozycja na promieniowanie

Jednym z najlepiej udokumentowanych czynników ryzyka naczyniakomłoniaka jest wcześniejsza ekspozycja na promieniowanie jonizujące.11 Naczyniakomłoniak indukowany promieniowaniem zwykle rozwija się w obszarze poddanym wcześniej radioterapii, najczęściej 5-10 lat po leczeniu.1213 Przypadki te są szczególnie często obserwowane u osób leczonych z powodu nowotworów piersi, szyjki macicy, jajnika, endometrium oraz chłoniaka Hodgkina.14

Uważa się, że osoby z mutacjami w genach naprawy DNA, jak BRCA1 i BRCA2, mogą być bardziej podatne na rozwój naczyniakomłoniaka indukowanego promieniowaniem, ponieważ utrata funkcji tych genów uniemożliwia skuteczną ochronę przed uszkodzeniami DNA wywołanymi promieniowaniem.15

Przewlekły obrzęk limfatyczny

Przewlekły obrzęk limfatyczny (limfoedema) jest najszerzej rozpoznawanym czynnikiem ryzyka rozwoju naczyniakomłoniaka, szczególnie w przypadku guzów skóry i tkanek miękkich.16 Klasycznym przykładem jest zespół Stewart-Treves, w którym naczyniakomłoniak rozwija się u kobiet po mastektomii z przewlekłym obrzękiem limfatycznym ramienia.17 Podobne przypadki opisano u pacjentów z obrzękiem kończyny dolnej po radykalnym usunięciu węzłów chłonnych pachwinowych z powodu przerzutów czerniaka złośliwego (zespół Kettlesa).18

Około 5% wszystkich naczyniakomłoniaków jest związanych z przewlekłym obrzękiem limfatycznym.19 Mechanizm rozwoju naczyniakomłoniaka w tym kontekście może być związany z lokalnym niedoborem odporności w obszarze dotkniętym obrzękiem limfatycznym, choć hipoteza ta wymaga dalszych badań.20

Ekspozycja na substancje chemiczne

Narażenie na niektóre substancje chemiczne jest znanym czynnikiem ryzyka naczyniakomłoniaka, szczególnie w przypadku naczyniakomłoniaka wątroby. Do najlepiej udokumentowanych związków chemicznych należą:212223

  • Chlorek winylu – używany w produkcji tworzyw sztucznych2425
  • Arsen26
  • Dwutlenek toru (Thorotrast) – dawniej stosowany jako środek kontrastowy w radiologii27
  • Steroidy anaboliczne28

Naczyniakomłoniak wątroby związany z narażeniem na substancje chemiczne może rozwijać się przez długi okres, z objawami pojawiającymi się nawet 10-40 lat po ekspozycji.29 Warto zauważyć, że po wprowadzeniu ścisłej kontroli nad stosowaniem tych substancji, liczba przypadków naczyniakomłoniaka wątroby związanego z narażeniem zawodowym znacznie spadła.30

Predyspozycje genetyczne

Około 3-5% przypadków naczyniakomłoniaka jest związanych z dziedzicznymi zespołami genetycznymi.3132 Zespoły te obejmują:

  • Nerwiakowłókniakowatość typu 1 (choroba von Recklinghausena)3334
  • Zespół Maffucciego35
  • Zespół Klippel-Trenaunaya36
  • Obustronne siatkówczaki37
  • Choroba Olliera38
  • Choroba von Hippel-Lindaua39

Badania wykazały również związek naczyniakomłoniaka z mutacjami w genach BRCA1 i BRCA2, znanych z podwyższonego ryzyka nowotworów piersi i jajnika.4041 W przypadku naczyniakomłoniaka serca zidentyfikowano mutacje w genie POT1, które mogą być dziedziczone.4243

Inne czynniki ryzyka

Badania wskazują również na inne potencjalne czynniki przyczyniające się do rozwoju naczyniakomłoniaka:

  • Promieniowanie ultrafioletowe – badanie z 2020 roku sugeruje, że promieniowanie UV może być czynnikiem sprawczym naczyniakomłoniaka skóry4445
  • Immunosupresja – opisano przypadki naczyniakomłoniaka u osób z obniżoną odpornością, np. po przeszczepie nerki4647
  • Ciała obce – implantowane materiały, w tym syntetyczne przeszczepy, mogą w rzadkich przypadkach prowadzić do rozwoju naczyniakomłoniaka4849
  • Wcześniejsze urazy lub zabiegi chirurgiczne w danym obszarze50
  • Istniejące wcześniej zmiany naczyniowe, takie jak naczyniaki lub malformacje naczyniowe51
  • Przewlekłe zapalenie52

Patogeneza naczyniakomłoniaka

Rozwój naczyniakomłoniaka obejmuje złożone współdziałanie czynników genetycznych, immunologicznych i środowiskowych. Mechanizmy patogenetyczne różnią się w zależności od typu naczyniakomłoniaka i czynników etiologicznych.53

W przypadku naczyniakomłoniaka indukowanego promieniowaniem, uszkodzenie DNA wywołane przez promieniowanie jonizujące prowadzi do mutacji, które mogą zakłócać normalne funkcjonowanie komórek endotelialnych. Osoby z upośledzonymi mechanizmami naprawy DNA są szczególnie narażone na tego typu uszkodzenia.54

W naczyniakomłoniaku związanym z przewlekłym obrzękiem limfatycznym, głównym mechanizmem może być miejscowe upośledzenie odporności, co tworzy mikrośrodowisko sprzyjające transformacji nowotworowej. Przewlekłe zapalenie i przebudowa tkanki w obszarach z zaburzonym drenażem limfatycznym mogą również przyczyniać się do tego procesu.5556

W naczyniakomłoniaku wątroby związanym z ekspozycją na substancje chemiczne, mechanizm niewydolności wątroby jest wieloczynnikowy. Obejmuje on niedokrwienie miąższu wątroby, zakrzepicę żyły wrotnej spowodowaną przez masy nowotworowe oraz niezakrzepowe zawały wątroby spowodowane wstrząsem lub posocznicą. Zastąpienie hepatocytów przez komórki nowotworowe prowadzi do wtórnej martwicy.5758

Typy naczyniakomłoniaka w zależności od etiologii

W zależności od czynników etiologicznych, naczyniakomłoniaka można podzielić na kilka typów:5960

  • Naczyniakomłoniak pierwotny (idiopatyczny) – powstaje spontanicznie, bez znanej przyczyny
  • Naczyniakomłoniak wtórny do radioterapii – rozwija się w obszarze wcześniej poddanym napromienianiu
  • Naczyniakomłoniak związany z obrzękiem limfatycznym (zespół Stewart-Treves)
  • Naczyniakomłoniak indukowany chemicznie – szczególnie w wątrobie, związany z narażeniem na substancje toksyczne
  • Naczyniakomłoniak w zespołach genetycznych – związany z dziedzicznymi zaburzeniami
  • Naczyniakomłoniak związany z ciałami obcymi – występujący w miejscu implantowanych materiałów

Nowe kierunki badań

Badania nad naczyniakomłoniakiem koncentrują się obecnie na lepszym zrozumieniu mechanizmów molekularnych leżących u podstaw rozwoju tego nowotworu. Identyfikacja specyficznych szlaków sygnałowych i mutacji genetycznych może prowadzić do opracowania ukierunkowanych terapii.61

Obiecujące wyniki w badaniach klinicznych wykazują terapie celowane i immunoterapie w leczeniu naczyniakomłoniaka.62 Leki ukierunkowane na VEGF i jego receptory są badane ze względu na kluczową rolę angiogenezy w rozwoju naczyniakomłoniaka.63

Modele zwierzęce naczyniakomłoniaka, takie jak myszy z delecją Cdkn2a i ekspresją HrasG12V, umożliwiają lepsze zrozumienie patogenezy choroby i testowanie nowych metod leczenia.6465

Podsumowanie etiologii

Etiologia naczyniakomłoniaka pozostaje w znacznej mierze nieznana, przy czym większość przypadków powstaje spontanicznie bez wyraźnej przyczyny. Zidentyfikowane czynniki ryzyka obejmują wcześniejszą radioterapię, przewlekły obrzęk limfatyczny, ekspozycję na określone substancje chemiczne oraz predyspozycje genetyczne.6667

Patogeneza naczyniakomłoniaka obejmuje złożoną interakcję między uszkodzeniami genetycznymi, zmianami mikrośrodowiska i czynnikami immunologicznymi. Dalsze badania są niezbędne, aby w pełni zrozumieć mechanizmy prowadzące do rozwoju tego agresywnego nowotworu i opracować skuteczniejsze strategie diagnostyczne i terapeutyczne.6869

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  1. 16.04.2026
  2. www.leksykon.com.pl

Materiały źródłowe

  • #1 Angiosarcoma // Middlesex Health
    https://middlesexhealth.org/learning-center/diseases-and-conditions/angiosarcoma
    Angiosarcoma is a rare type of cancer that forms in the lining of the blood vessels and lymph vessels. […] It’s not clear what causes most angiosarcomas. Researchers have identified factors that may increase the risk of the disease. […] Angiosarcoma happens when cells in the lining of a blood vessel or lymph vessel develop changes in their DNA. A cell’s DNA contains the instructions that tell the cell what to do. The changes, which doctors call mutations, tell the cells to multiply quickly. The changes cause the cells to keep living when healthy cells would die. […] Factors that may increase the risk of angiosarcoma include: […] Treatment with radiation for cancer or other conditions may increase the risk of angiosarcoma. Angiosarcoma is a rare side effect of radiation therapy. […] Liver angiosarcoma is linked to exposure to several chemicals. Examples of these chemicals include vinyl chloride and arsenic. […] Certain gene changes that people can be born with can raise the risk of having angiosarcoma. Examples include the gene changes that cause neurofibromatosis, Maffucci syndrome, or Klippel-Trenaunay syndrome, and the BRCA1 and BRCA2 genes.
  • #2 Angiosarcoma of the lungs, liver, and bones in a 27-year-old male patient | Mikołajczyk-Solińska | Medical Research Journal
    https://journals.viamedica.pl/medical_research_journal/article/view/90721
    Angiosarcoma is a rare (from 1% to 2% of all soft tissue sarcomas), highly aggressive endothelial tumor that may affect any organ. […] The etiology is unclear; however, there are definite risk factors including chronic lymphedema, radiation therapy, familial syndromes, and exposure to environmental chemical toxins and foreign bodies.
  • #3
    https://journals.lww.com/md-journal/fulltext/2024/05030/etiology,_pathogenesis,_and_management_of.73.aspx
    Angiosarcomas are rare and highly malignant soft tissue sarcomas originating from endothelial cells lining the lymphatic or vascular system. The etiology of angiosarcoma remains elusive in most clinical cases. Nevertheless, several prognosis risk factors play a pivotal role, including chronic lymphedema, therapeutic irradiation, environmental carcinogens, familial syndromes, and the presence of foreign materials like metallic objects and biomedical implants. […] The etiology of the disease is not well defined, and the diagnosis remains challenging due to rarity and poor prognosis. AS may arise spontaneously de novo without predisposing conditions or systemic syndromes. Nevertheless, several reports have identified potential predisposing risk factors such as radiation therapy, chronic lymphedema, familial genetic syndromes, environmental carcinogens, and retained foreign body material responsible for the pathogenesis of AS.
  • #4 What Is Angiosarcoma | Angiosarcoma Awareness Inc
    https://www.cureasc.org/what-is-angiosarcoma/
    Angiosarcoma is a rare cancer that originates in the endothelial cells lining blood or lymph vessels. […] While the precise cause of angiosarcoma remains elusive, research has identified several risk factors that may contribute to its development. […] Radiation Exposure: Previous radiation therapy, especially at high doses, is linked to an elevated risk of angiosarcoma. […] Chemical Contact: Exposure to certain chemicals, such as vinyl chloride, arsenic, and herbicides, has been associated with a higher incidence of this cancer. […] Chronic Lymphedema: Prolonged swelling due to lymphatic fluid accumulation, often following surgery, can lead to lymphangiosarcoma, a form of angiosarcoma. […] Genetic Predisposition: Although rare, genetic mutations in specific genes may predispose individuals to angiosarcoma. […] Despite these known risk factors, angiosarcoma can also appear spontaneously, without any identifiable cause. This complexity underscores the need for ongoing research to better understand the mechanisms behind this aggressive cancer.
  • #5 Angiosarcoma // Middlesex Health
    https://middlesexhealth.org/learning-center/diseases-and-conditions/angiosarcoma
    Angiosarcoma is a rare type of cancer that forms in the lining of the blood vessels and lymph vessels. […] It’s not clear what causes most angiosarcomas. Researchers have identified factors that may increase the risk of the disease. […] Angiosarcoma happens when cells in the lining of a blood vessel or lymph vessel develop changes in their DNA. A cell’s DNA contains the instructions that tell the cell what to do. The changes, which doctors call mutations, tell the cells to multiply quickly. The changes cause the cells to keep living when healthy cells would die. […] Factors that may increase the risk of angiosarcoma include: […] Treatment with radiation for cancer or other conditions may increase the risk of angiosarcoma. Angiosarcoma is a rare side effect of radiation therapy. […] Liver angiosarcoma is linked to exposure to several chemicals. Examples of these chemicals include vinyl chloride and arsenic. […] Certain gene changes that people can be born with can raise the risk of having angiosarcoma. Examples include the gene changes that cause neurofibromatosis, Maffucci syndrome, or Klippel-Trenaunay syndrome, and the BRCA1 and BRCA2 genes.
  • #6 Angiosarcoma Cancer: Symptoms, Treatment, Outlook, and More
    https://www.healthline.com/health/cancer/angiosarcoma-cancer
    Angiosarcoma begins due to a mutation (an error in your DNA) in one of the endothelial cells in your blood or lymph vessels. This causes the unhealthy cell to grow and multiply quickly, eventually creating a tumor. […] Researchers dont know what exactly causes these mutations. But they do know certain risk factors can increase the risk of angiosarcoma, such as: radiation therapy for previous cancer and other conditions, lymphedema, or swelling of the body from lymphatic system dysfunction, exposure to certain chemicals, such as vinyl chloride, arsenic, or thorium dioxide, certain genetic syndromes, such as neurofibromatosis, Maffucci syndrome, or Klippel-Trenaunay syndrome. […] There are several risk factors for this condition, including genetics and exposure to radiation and certain chemicals.
  • #7 What causes angiosarcoma?  – Acibadem Health Point – ACIBADEM Hospitals – Acibadem Health Group
    https://www.acibademhealthpoint.com/what-causes-angiosarcoma/
    Environmental triggers also play a role in causing angiosarcoma. Exposure to harmful chemicals can be a big factor here as well as radiation from treatments or other sources around us every day. People who have been through radiation therapy for other types of cancer are sometimes at higher risk for this one later on. […] Genetic changes can play a big role in the development of angiosarcoma. These changes may happen over time or be inherited from family members. Some changes make cells grow out of control. This is how cancer starts. […] Certain genes are linked to higher risks for this rare cancer. If you have these genes your chance of getting angiosarcoma goes up. Family history matters too. If someone in your family had it you might be at risk as well. Mutations in specific genes can also trigger angiosarcoma.
  • #8 Angiosarcoma of the breast, the unknown—a review of the current literature – Esposito – Translational Cancer Research
    https://tcr.amegroups.org/article/view/30432/html
    Angiosarcoma of the breast can be classified into primary when arising de novo and secondary to chronic lymphoedema or breast irradiation. […] Although the aetiology of the primary angiosarcoma of the breast remains unknown, the possible risk factors include trauma, radiation and lymphedema, but there is no definitive data to support this claim. […] Point mutations in BRCA2 are supposed to be causes of some secondary breast angiosarcomas. […] The loss of function of BRCA mutated prevents to exert protection against radiation-induced DNA damage hence it is reasonable supposing a role in developing this kind of tumour. […] p53 loss of function, mouse double minute 2 (MDM2) and vascular endothelial growing factor (VEGF) overexpression are alterations of molecular pathways which have been found involved in angiosarcoma origin.
  • #9 Angiosarcoma of the breast, the unknown—a review of the current literature – Esposito – Translational Cancer Research
    https://tcr.amegroups.org/article/view/30432/html
    The observed deregulation of p53/MDM-2 expression affects also differentiation and phenotype of endothelial cells up-regulating VEGF expression. […] MYC high-level gene amplifications were observed in all secondary angiosarcoma cases but not in primary ones, suggesting that, despite their identical morphology, secondary angiosarcomas are genetically different from primary ones. […] PIK3CA/AKT/mTOR pathway is either directly or indirectly involved in breast angiosarcoma onset. […] Aetiology and molecular pathways are only partially known and still under investigation.
  • #10 Bone: Angiosarcoma
    https://atlasgeneticsoncology.org/solid-tumor/5359/bone-angiosarcoma
    Although there is in a very small percentage an association between angiosarcoma and radiation, the etiology of the majority of angiosarcomas remains unknown. […] Angiosarcoma is extremely rare and represent less than 1% of primary malignant bone tumours. […] Recently mutations and amplifications have been described for angiosarcoma, most of these aberrations occur in the tyrosine-kinase pathways specific for vascular receptors. Currently PTPRB, PLCG1, CIC, KDR, and FLT4 mutations and MYC amplifications have been described for angiosarcoma.
  • #11 Angiosarcoma: Symptoms, Types & Treatment
    https://my.clevelandclinic.org/health/diseases/22778-angiosarcoma
    Angiosarcoma happens when the cells in the inner lining of your blood vessels change from normal to abnormal cells. Unlike normal cells, the cancerous cells dont die. Instead, they continue to make more cells that eventually become tumors. The cancerous cells keep on growing from your affected blood vessels. Often, they spread to other areas of your body. […] Researchers have identified several risk factors that may increase the chance youll develop angiosarcoma. These risk factors are: […] Angiosarcoma is sometimes a secondary cancer that people develop several years (usually around eight to 10) after getting radiation treatment for a different cancer type, most commonly breast cancer. […] Liver (hepatic) angiosarcoma is associated with exposure to polyvinyl chloride, arsenic and thorium dioxide. Sometimes, angiosarcomas dont show up until 10 to 40 years after exposure.
  • #12 Angiosarcoma: Practice Essentials, Pathophysiology, Etiology
    https://emedicine.medscape.com/article/276512-overview
    Radiation-induced angiosarcomas occur in the absence of chronic lymphedema after radiotherapy for carcinoma of the cervix, ovary, endometrium, or breast and Hodgkin lymphoma. […] Some angiosarcomas are associated with foreign material introduced in the body, either iatrogenically or accidentally. […] Exposure to arsenic may increase the risk of angiosarcoma of the liver. […] Approximately 3% of primary angiosarcomas are associated with hereditary diseases, such as congenital retinoblastoma, neurofibromatosis type 1, Ollier disease, Maffuci disease, xeroderma pigmentosum.
  • #13 Angiosarcoma (AS) Specimens – Bay Biosciences
    https://baybiosciences.com/angiosarcoma-as-specimens/
    Angiosarcoma (AS) is a rare type of cancer that forms in the lining of the blood vessels and lymph vessels. […] Its not clear what causes most angiosarcomas, though doctors have identified factors that may increase the risk of the disease. Researchers know that something happens that causes a cell in the lining of a blood vessel or lymph vessel to develop an error (mutation) in its genetic code. The mutation tells the cell to grow quickly, making more abnormal cells. The abnormal cells continue living when other cells would die. The result is a buildup of abnormal cells that grows from the affected blood vessel or lymphatic vessels. With time, cells may break off and spread (metastasize) to other areas of the body. […] Factors that may increase the risk of Angiosarcoma include: Radiation therapy: Treatment with radiation for cancer or other conditions may increase the risk of angiosarcoma. A rare complication of radiation therapy, angiosarcoma typically occurs five to 10 years after treatment. […] Chemicals: Liver angiosarcoma has been linked to exposure to several chemicals, including vinyl chloride and arsenic.
  • #14 Angiosarcoma: Practice Essentials, Pathophysiology, Etiology
    https://emedicine.medscape.com/article/276512-overview
    Radiation-induced angiosarcomas occur in the absence of chronic lymphedema after radiotherapy for carcinoma of the cervix, ovary, endometrium, or breast and Hodgkin lymphoma. […] Some angiosarcomas are associated with foreign material introduced in the body, either iatrogenically or accidentally. […] Exposure to arsenic may increase the risk of angiosarcoma of the liver. […] Approximately 3% of primary angiosarcomas are associated with hereditary diseases, such as congenital retinoblastoma, neurofibromatosis type 1, Ollier disease, Maffuci disease, xeroderma pigmentosum.
  • #15 Angiosarcoma of the breast, the unknown—a review of the current literature – Esposito – Translational Cancer Research
    https://tcr.amegroups.org/article/view/30432/html
    Angiosarcoma of the breast can be classified into primary when arising de novo and secondary to chronic lymphoedema or breast irradiation. […] Although the aetiology of the primary angiosarcoma of the breast remains unknown, the possible risk factors include trauma, radiation and lymphedema, but there is no definitive data to support this claim. […] Point mutations in BRCA2 are supposed to be causes of some secondary breast angiosarcomas. […] The loss of function of BRCA mutated prevents to exert protection against radiation-induced DNA damage hence it is reasonable supposing a role in developing this kind of tumour. […] p53 loss of function, mouse double minute 2 (MDM2) and vascular endothelial growing factor (VEGF) overexpression are alterations of molecular pathways which have been found involved in angiosarcoma origin.
  • #16 Angiosarcoma: Practice Essentials, Pathophysiology, Etiology
    https://emedicine.medscape.com/article/276512-overview
    The etiology of most cases of angiosarcoma is unknown. The tumors may develop as a complication of a preexisting condition. The following factors may be associated with tumor development: Radical mastectomy, Radiotherapy, Foreign materials, Environmental carcinogens, AIDS, Preexisting benign lesions (eg, bone infarct, pagetoid bone, chronic osteomyelitis), Genetic disorders. […] Chronic lymphedema is the most widely recognized risk factor, especially in angiosarcomas of the skin and soft tissue. Typically, lymphedema-associated angiosarcomas occur in women who have undergone radical mastectomy for breast carcinoma and have had chronic lymphedema for many years (Stewart-Treves syndrome) or in the leg of patients as a consequence of radical inguinal lymphadenectomy for metastases from malignant melanoma (Kettles syndrome).
  • #17 Angiosarcoma: Symptoms, Types & Treatment
    https://my.clevelandclinic.org/health/diseases/22778-angiosarcoma
    Approximately 5% of all angiosarcomas are linked to a form of chronic (long-term) lymphedema called Stewart-Treves syndrome. It most often affects people who had a mastectomy and lymph node removal during breast cancer treatment. […] About 3% of all angiosarcomas affect people who have conditions caused by genetic disorders. Specific conditions include bilateral retinoblastoma, Maffucci syndrome, neurofibromatosis and Klippel-Trenaunay syndrome.
  • #18 Angiosarcoma: Practice Essentials, Pathophysiology, Etiology
    https://emedicine.medscape.com/article/276512-overview
    The etiology of most cases of angiosarcoma is unknown. The tumors may develop as a complication of a preexisting condition. The following factors may be associated with tumor development: Radical mastectomy, Radiotherapy, Foreign materials, Environmental carcinogens, AIDS, Preexisting benign lesions (eg, bone infarct, pagetoid bone, chronic osteomyelitis), Genetic disorders. […] Chronic lymphedema is the most widely recognized risk factor, especially in angiosarcomas of the skin and soft tissue. Typically, lymphedema-associated angiosarcomas occur in women who have undergone radical mastectomy for breast carcinoma and have had chronic lymphedema for many years (Stewart-Treves syndrome) or in the leg of patients as a consequence of radical inguinal lymphadenectomy for metastases from malignant melanoma (Kettles syndrome).
  • #19 Angiosarcoma: Symptoms, Types & Treatment
    https://my.clevelandclinic.org/health/diseases/22778-angiosarcoma
    Approximately 5% of all angiosarcomas are linked to a form of chronic (long-term) lymphedema called Stewart-Treves syndrome. It most often affects people who had a mastectomy and lymph node removal during breast cancer treatment. […] About 3% of all angiosarcomas affect people who have conditions caused by genetic disorders. Specific conditions include bilateral retinoblastoma, Maffucci syndrome, neurofibromatosis and Klippel-Trenaunay syndrome.
  • #20 Angiosarcoma – Wikipedia
    https://en.wikipedia.org/wiki/Angiosarcoma
    The cause of angiosarcoma is not known, though several risk factors are known, such as chronic lymphedema, radiation therapy and various chemicals such as arsenic and vinyl chloride. Angiosarcomas have been reported in association with long standing foreign bodies. […] A 2020 study analyzing the genome of cutaneous angiosarcomas suggests that ultraviolet radiation may be a causative agent of angiosarcomas. […] Accepted risk factors include lymphedema, radiation therapy, neurofibromatosis, Maffucci syndrome, Klippel-Trenaunay syndrome and chemicals such as arsenic, thorotrast and vinyl chloride. […] Immunosuppression may play a role in the pathogenesis of angiosarcoma. There have been reports of angiosarcoma in immunosuppressed individuals following kidney transplantation. The association between lymphedema and angiosarcoma may be due to localized immunodeficiency, however this hypothesis has not been confirmed. Some studies suggest a link between AIDS and angiosarcoma though this may be due to misdiagnosed Kaposi’s sarcoma.
  • #21 Angiosarcoma // Middlesex Health
    https://middlesexhealth.org/learning-center/diseases-and-conditions/angiosarcoma
    Angiosarcoma is a rare type of cancer that forms in the lining of the blood vessels and lymph vessels. […] It’s not clear what causes most angiosarcomas. Researchers have identified factors that may increase the risk of the disease. […] Angiosarcoma happens when cells in the lining of a blood vessel or lymph vessel develop changes in their DNA. A cell’s DNA contains the instructions that tell the cell what to do. The changes, which doctors call mutations, tell the cells to multiply quickly. The changes cause the cells to keep living when healthy cells would die. […] Factors that may increase the risk of angiosarcoma include: […] Treatment with radiation for cancer or other conditions may increase the risk of angiosarcoma. Angiosarcoma is a rare side effect of radiation therapy. […] Liver angiosarcoma is linked to exposure to several chemicals. Examples of these chemicals include vinyl chloride and arsenic. […] Certain gene changes that people can be born with can raise the risk of having angiosarcoma. Examples include the gene changes that cause neurofibromatosis, Maffucci syndrome, or Klippel-Trenaunay syndrome, and the BRCA1 and BRCA2 genes.
  • #22 Angiosarcoma: Symptoms, Types & Treatment
    https://my.clevelandclinic.org/health/diseases/22778-angiosarcoma
    Angiosarcoma happens when the cells in the inner lining of your blood vessels change from normal to abnormal cells. Unlike normal cells, the cancerous cells dont die. Instead, they continue to make more cells that eventually become tumors. The cancerous cells keep on growing from your affected blood vessels. Often, they spread to other areas of your body. […] Researchers have identified several risk factors that may increase the chance youll develop angiosarcoma. These risk factors are: […] Angiosarcoma is sometimes a secondary cancer that people develop several years (usually around eight to 10) after getting radiation treatment for a different cancer type, most commonly breast cancer. […] Liver (hepatic) angiosarcoma is associated with exposure to polyvinyl chloride, arsenic and thorium dioxide. Sometimes, angiosarcomas dont show up until 10 to 40 years after exposure.
  • #23 Angiosarcoma: Symptoms, Causes, Diagnosis, Treatment
    https://www.webmd.com/cancer/what-is-angiosarcoma
    In most cases, the cause of an angiosarcoma isn’t known. But there are some things that can make you more likely to get it: […] Treatment with radiation therapy […] Lymphedema, a swelling caused by damage to lymph vessels […] Exposure to some chemicals, including arsenic, vinyl chloride, and thorium dioxide […] Genetic conditions such as neurofibromatosis, Klippel-Trenaunay syndrome, and Maffuci syndrome.
  • #24 What causes angiosarcoma?  – Acibadem Health Point – ACIBADEM Hospitals – Acibadem Health Group
    https://www.acibademhealthpoint.com/what-causes-angiosarcoma/
    Exposure to certain chemicals can trigger angiosarcoma. These chemicals are often found in industrial settings. Vinyl chloride is one such chemical used in making plastics. People who work with this substance have higher risks. […] Radiation exposure is another big factor here. If you had radiation therapy for other cancers it might later lead to angiosarcoma. Even small doses of radiation over long periods could be harmful. […] Did you know that past cancer treatments can sometimes cause angiosarcoma? It’s true. Radiation therapy, often used to treat other cancers, is a common trigger. […] Chemotherapy also plays a role here. Some drugs used in chemotherapy can damage healthy cells along with cancerous ones. Over time this damage might turn into angiosarcoma. […] Angiosarcoma causes include genetic changes, exposure to certain chemicals, and past cancer treatments. […] Yes, risk factors include a family history of similar cancers, exposure to harmful chemicals like vinyl chloride, and previous radiation therapy.
  • #25
    https://www.cdc.gov/mmwr/preview/mmwrhtml/lmrk103.htm
    Between September 1967 and December 1973, 4 cases of angiosarcoma of the liver were diagnosed among men employed in the polyvinyl chloride polymerization section of a B.F. Goodrich plant near Louisville, Kentucky. […] None of the patients gave histories of prolonged alcohol use or exposure to hepatotoxin outside their work place. In particular, none had ever had exposure to thorium dioxide or to arsenic, two materials known specifically to induce hepatic angiosarcoma in man. […] Four cases, therefore, among a small number of workers at a single plant is a most unusual event, and one which raises the possibility of some work-related carcinogen, conceivably vinyl chloride itself. […] Epidemiologic studies have started to determine the extent of the problem in the United States, with respect both to angiosarcoma of the liver and to its possible relationship to post-toxic cirrhosis.
  • #26 Angiosarcoma: Practice Essentials, Pathophysiology, Etiology
    https://emedicine.medscape.com/article/276512-overview
    Radiation-induced angiosarcomas occur in the absence of chronic lymphedema after radiotherapy for carcinoma of the cervix, ovary, endometrium, or breast and Hodgkin lymphoma. […] Some angiosarcomas are associated with foreign material introduced in the body, either iatrogenically or accidentally. […] Exposure to arsenic may increase the risk of angiosarcoma of the liver. […] Approximately 3% of primary angiosarcomas are associated with hereditary diseases, such as congenital retinoblastoma, neurofibromatosis type 1, Ollier disease, Maffuci disease, xeroderma pigmentosum.
  • #27 Hepatic angiosarcoma | Radiology Reference Article | Radiopaedia.org
    https://radiopaedia.org/articles/hepatic-angiosarcoma?lang=us
    Most hepatic angiosarcomas arise spontaneously, although hemochromatosis and neurofibromatosis type 1 have also been associated. […] Environmental exposure to Thorotrast, arsenic, ionizing radiation, and vinyl chloride have been implicated as risk factors, but such exposure is now rare.
  • #28
    https://step1.medbullets.com/oncology/109088/angiosarcoma
    hepatic angiosarcoma is a rare, high-grade malignant vascular neoplasm […] majority of cases are of unknown etiology […] risk factors include vinyl chloride, arsenic, anabolic steroids, radiation, and thorium dioxide.
  • #29 Angiosarcoma: Symptoms, Types & Treatment
    https://my.clevelandclinic.org/health/diseases/22778-angiosarcoma
    Angiosarcoma happens when the cells in the inner lining of your blood vessels change from normal to abnormal cells. Unlike normal cells, the cancerous cells dont die. Instead, they continue to make more cells that eventually become tumors. The cancerous cells keep on growing from your affected blood vessels. Often, they spread to other areas of your body. […] Researchers have identified several risk factors that may increase the chance youll develop angiosarcoma. These risk factors are: […] Angiosarcoma is sometimes a secondary cancer that people develop several years (usually around eight to 10) after getting radiation treatment for a different cancer type, most commonly breast cancer. […] Liver (hepatic) angiosarcoma is associated with exposure to polyvinyl chloride, arsenic and thorium dioxide. Sometimes, angiosarcomas dont show up until 10 to 40 years after exposure.
  • #30
    https://www.cdc.gov/mmwr/preview/mmwrhtml/lmrk103.htm
    Studies in Germany have suggested a link between hepatic damage and occupational exposure to vinyl chloride, while Italian workers have suggested that vinyl chloride may cause a wide variety of tumors in animals. […] This report marked the first recognition of the carcinogenicity to humans of vinyl chloride monomer (VCM), the highly reactive gas to which workers were exposed in the B.F. Goodrich plant near Louisville, Kentucky. […] VCM is now universally considered to be a highly potent chemical carcinogen. […] To prevent future cases of VCM-associated angiosarcoma, the Occupational Safety and Health Administration in 1974 proposed a 500-fold reduction in the occupational exposure standard for VCM gas — from 500 parts per million (ppm) in air to 1 ppm. […] New cases of hepatic angiosarcoma in vinyl chloride polymerization workers have been virtually eliminated.
  • #31 Angiosarcoma: Symptoms, Types & Treatment
    https://my.clevelandclinic.org/health/diseases/22778-angiosarcoma
    Approximately 5% of all angiosarcomas are linked to a form of chronic (long-term) lymphedema called Stewart-Treves syndrome. It most often affects people who had a mastectomy and lymph node removal during breast cancer treatment. […] About 3% of all angiosarcomas affect people who have conditions caused by genetic disorders. Specific conditions include bilateral retinoblastoma, Maffucci syndrome, neurofibromatosis and Klippel-Trenaunay syndrome.
  • #32 Angiosarcoma: Practice Essentials, Pathophysiology, Etiology
    https://emedicine.medscape.com/article/276512-overview
    Radiation-induced angiosarcomas occur in the absence of chronic lymphedema after radiotherapy for carcinoma of the cervix, ovary, endometrium, or breast and Hodgkin lymphoma. […] Some angiosarcomas are associated with foreign material introduced in the body, either iatrogenically or accidentally. […] Exposure to arsenic may increase the risk of angiosarcoma of the liver. […] Approximately 3% of primary angiosarcomas are associated with hereditary diseases, such as congenital retinoblastoma, neurofibromatosis type 1, Ollier disease, Maffuci disease, xeroderma pigmentosum.
  • #33 Angiosarcoma // Middlesex Health
    https://middlesexhealth.org/learning-center/diseases-and-conditions/angiosarcoma
    Angiosarcoma is a rare type of cancer that forms in the lining of the blood vessels and lymph vessels. […] It’s not clear what causes most angiosarcomas. Researchers have identified factors that may increase the risk of the disease. […] Angiosarcoma happens when cells in the lining of a blood vessel or lymph vessel develop changes in their DNA. A cell’s DNA contains the instructions that tell the cell what to do. The changes, which doctors call mutations, tell the cells to multiply quickly. The changes cause the cells to keep living when healthy cells would die. […] Factors that may increase the risk of angiosarcoma include: […] Treatment with radiation for cancer or other conditions may increase the risk of angiosarcoma. Angiosarcoma is a rare side effect of radiation therapy. […] Liver angiosarcoma is linked to exposure to several chemicals. Examples of these chemicals include vinyl chloride and arsenic. […] Certain gene changes that people can be born with can raise the risk of having angiosarcoma. Examples include the gene changes that cause neurofibromatosis, Maffucci syndrome, or Klippel-Trenaunay syndrome, and the BRCA1 and BRCA2 genes.
  • #34 Angiosarcoma – SFA
    https://curesarcoma.org/sarcoma-subtypes/angiosarcoma/
    The etiology is unknown in most cases, although a minority arise after radiation exposure or longstanding lymphedema. […] Smaller numbers occur in association with implanted foreign material (including synthetic grafts), in the vicinity of arteriovenous fistulas, in a pre-existing haemangioma/vascular malformation, in regions of prior trauma or surgery, in certain syndromes (e.g., neurofibromatosis and Maffucci syndrome), and rarely as heterologous components of other neoplasms (e.g., in benign or malignant nerve sheath tumors).
  • #35 Angiosarcoma: Symptoms, Causes, Diagnosis, Treatment
    https://www.webmd.com/cancer/what-is-angiosarcoma
    In most cases, the cause of an angiosarcoma isn’t known. But there are some things that can make you more likely to get it: […] Treatment with radiation therapy […] Lymphedema, a swelling caused by damage to lymph vessels […] Exposure to some chemicals, including arsenic, vinyl chloride, and thorium dioxide […] Genetic conditions such as neurofibromatosis, Klippel-Trenaunay syndrome, and Maffuci syndrome.
  • #36 Angiosarcoma: Symptoms, Causes, and Treatment Options
    https://www.tgh.org/institutes-and-services/conditions/angiosarcoma
    The causes of angiosarcoma are unclear, but researchers have linked the condition to several risk factors, such as prior radiation treatment and lymphedema. […] Certain genetic syndromes are also associated with angiosarcoma, including breast cancer gene 1 (BRCA1), breast cancer gene 2 (BRCA2), and the gene mutations that lead to the development of neurofibromatosis, Maffucci syndrome or Klippel-Trenaunay syndrome.
  • #37 Angiosarcoma: Practice Essentials, Pathophysiology, Etiology
    https://emedicine.medscape.com/article/276512-overview
    Radiation-induced angiosarcomas occur in the absence of chronic lymphedema after radiotherapy for carcinoma of the cervix, ovary, endometrium, or breast and Hodgkin lymphoma. […] Some angiosarcomas are associated with foreign material introduced in the body, either iatrogenically or accidentally. […] Exposure to arsenic may increase the risk of angiosarcoma of the liver. […] Approximately 3% of primary angiosarcomas are associated with hereditary diseases, such as congenital retinoblastoma, neurofibromatosis type 1, Ollier disease, Maffuci disease, xeroderma pigmentosum.
  • #38 Angiosarcoma Causes, Symptoms and Treatment
    https://www.netmeds.com/health-library/post/angiosarcoma-causes-symptoms-and-treatment?srsltid=AfmBOooFDYQvKPwd59FYCTdBEvEhOusggk5FNstUp_vYuZqMDGisQi-y
    Just like any other form of sarcoma, the exact cause of Angiosarcoma is yet unknown, but recent researches showcase that it chiefly happens when there is a change or mutation in the genetic matter or DNA of the blood vessel and lymph node cells. A cell’s DNA includes the instructions that tell the cell what to do. These changes in the DNA instruct the cell to grow abnormally in size and number without dying. And ultimately the unstable cells amass in large numbers to form a clump of tissue or tumour within the organ. They can even break away from the original site and invade near and distant tissues and organs to spread the infection. […] Certain gene changes that children are diagnosed with at birth can raise the risk of having Angiosarcoma later in life. Some of the genetic conditions that might lead to Angiosarcoma include Maffucci syndrome, or Klippel- syndrome, Bilateral retinoblastoma, Ollier disease, Von HippelLindau disease, Von Recklinghausens neurofibromatosis, and the BRCA1 and BRCA2 genes.
  • #39 Angiosarcoma Causes, Symptoms and Treatment
    https://www.netmeds.com/health-library/post/angiosarcoma-causes-symptoms-and-treatment?srsltid=AfmBOooFDYQvKPwd59FYCTdBEvEhOusggk5FNstUp_vYuZqMDGisQi-y
    Just like any other form of sarcoma, the exact cause of Angiosarcoma is yet unknown, but recent researches showcase that it chiefly happens when there is a change or mutation in the genetic matter or DNA of the blood vessel and lymph node cells. A cell’s DNA includes the instructions that tell the cell what to do. These changes in the DNA instruct the cell to grow abnormally in size and number without dying. And ultimately the unstable cells amass in large numbers to form a clump of tissue or tumour within the organ. They can even break away from the original site and invade near and distant tissues and organs to spread the infection. […] Certain gene changes that children are diagnosed with at birth can raise the risk of having Angiosarcoma later in life. Some of the genetic conditions that might lead to Angiosarcoma include Maffucci syndrome, or Klippel- syndrome, Bilateral retinoblastoma, Ollier disease, Von HippelLindau disease, Von Recklinghausens neurofibromatosis, and the BRCA1 and BRCA2 genes.
  • #40 Angiosarcoma – StatPearls – NCBI Bookshelf
    https://www.ncbi.nlm.nih.gov/books/NBK441983/
    Angiosarcoma is mostly a spontaneous tumor; however, a transformation from a benign vascular lesion has been reported previously. […] Radiation-induced AS is commonly seen in breast cancer survivors who receive RT to the chest wall. […] A few case reports suggest an association of AS with mutation in the DNA repair genes BRCA1 and BRCA2. […] Familial syndromes like neurofibromatosis, Maffuci syndrome, Klippel-Trenaunay syndrome are also associated with AS. […] Chemicals like vinyl chloride, thorium dioxide, arsenic, radium, anabolic steroids are also associated with AS.
  • #41 Angiosarcoma: Symptoms, Causes, and Treatment Options
    https://www.tgh.org/institutes-and-services/conditions/angiosarcoma
    The causes of angiosarcoma are unclear, but researchers have linked the condition to several risk factors, such as prior radiation treatment and lymphedema. […] Certain genetic syndromes are also associated with angiosarcoma, including breast cancer gene 1 (BRCA1), breast cancer gene 2 (BRCA2), and the gene mutations that lead to the development of neurofibromatosis, Maffucci syndrome or Klippel-Trenaunay syndrome.
  • #42 Angiosarcoma | Cause, Symptoms, Diagnosis & Treatment
    https://www.cincinnatichildrens.org/health/a/angiosarcoma
    Angiosarcoma can sometimes run in families and can be linked to a gene called POTI. Risk factors include lymphedema (when extra fluid builds up), previous radiation and exposure to chemicals. […] Angiosarcoma is a form of cancer that develops in the inner lining of the blood and lymph vessels.
  • #43 Angiosarcoma – NCI
    https://www.cancer.gov/pediatric-adult-rare-tumor/rare-tumors/rare-vascular-tumors/angiosarcoma
    Angiosarcoma is a rare cancer that develops in the inner lining of blood vessels and lymph vessels. […] Scientists have found that people with a change in a gene called POT1 may develop angiosarcoma of the heart, and this change can be passed on to their children. […] We know that some risk factors for getting angiosarcoma include: Lymphedema, a problem in which extra lymph fluid builds up in tissues, causing swelling, usually in the arms or legs; Past radiation therapy, such as treatment for breast cancer or Hodgkin lymphoma; Exposure to some cancer-causing chemicals.
  • #44 Angiosarcoma – Wikipedia
    https://en.wikipedia.org/wiki/Angiosarcoma
    The cause of angiosarcoma is not known, though several risk factors are known, such as chronic lymphedema, radiation therapy and various chemicals such as arsenic and vinyl chloride. Angiosarcomas have been reported in association with long standing foreign bodies. […] A 2020 study analyzing the genome of cutaneous angiosarcomas suggests that ultraviolet radiation may be a causative agent of angiosarcomas. […] Accepted risk factors include lymphedema, radiation therapy, neurofibromatosis, Maffucci syndrome, Klippel-Trenaunay syndrome and chemicals such as arsenic, thorotrast and vinyl chloride. […] Immunosuppression may play a role in the pathogenesis of angiosarcoma. There have been reports of angiosarcoma in immunosuppressed individuals following kidney transplantation. The association between lymphedema and angiosarcoma may be due to localized immunodeficiency, however this hypothesis has not been confirmed. Some studies suggest a link between AIDS and angiosarcoma though this may be due to misdiagnosed Kaposi’s sarcoma.
  • #45 Angiosarcoma of the Scalp: Practice Essentials, Etiology, Clinical Presentation
    https://emedicine.medscape.com/article/1296442-overview
    The cause of angiosarcoma of the scalp is unknown, although several associations have been reported. Angiosarcoma developing in persons with chronic lymphedema is well described. However, lymph stasis is probably not involved significantly in angiosarcoma of the scalp. The most frequent association with angiosarcoma of the scalp is prior radiation for either a malignant or a benign condition. The mechanism for radiation-induced angiosarcoma is unknown. Radiation-induced angiosarcoma typically presents 5-10 years after irradiation, while radiation-induced tumors of the head and neck have shown greater latency periods. […] Risk factors described for angiosarcoma at other locations include exposure to vinyl chloride and thorium dioxide (Thorotrast). Because most angiosarcoma occurs on the scalp and face of Whites and usually in nonhair-bearing areas, ultraviolet light exposure has also been suggested as a contributing cause. However, Lydiatt et al investigated the prevalence of metachronous skin cancers as an expression of solar damage and found that only three of 18 patients had a history of basal or squamous skin cancer. […] Finally, Lydiatt et al suggested trauma as a causative agent, but trauma seems more likely to just be the reason the lesion is noticed by the patient.
  • #46 Angiosarcoma – Wikipedia
    https://en.wikipedia.org/wiki/Angiosarcoma
    The cause of angiosarcoma is not known, though several risk factors are known, such as chronic lymphedema, radiation therapy and various chemicals such as arsenic and vinyl chloride. Angiosarcomas have been reported in association with long standing foreign bodies. […] A 2020 study analyzing the genome of cutaneous angiosarcomas suggests that ultraviolet radiation may be a causative agent of angiosarcomas. […] Accepted risk factors include lymphedema, radiation therapy, neurofibromatosis, Maffucci syndrome, Klippel-Trenaunay syndrome and chemicals such as arsenic, thorotrast and vinyl chloride. […] Immunosuppression may play a role in the pathogenesis of angiosarcoma. There have been reports of angiosarcoma in immunosuppressed individuals following kidney transplantation. The association between lymphedema and angiosarcoma may be due to localized immunodeficiency, however this hypothesis has not been confirmed. Some studies suggest a link between AIDS and angiosarcoma though this may be due to misdiagnosed Kaposi’s sarcoma.
  • #47 Cutaneous Angiosarcoma in a Psoriasis Patient on Infliximab and Methotrexate Therapy
    https://clinmedjournals.org/articles/iaim/international-archives-of-internal-medicine-iaim-2-006.php?jid=iaim
    It has been theorized that the vascularity of the head and neck, with its dense arrangement of vessels, may preclude it to oncogenic development in the setting of UV radiation. […] Tumor necrosis factor (TNF)-alpha inhibitors like infliximab have shown mixed evidence in regard to increased risk of overall malignancy stemming from their use. […] Methotrexate has also been implicated in increasing risk via activation of lytic EBV infection, releasing infectious virus into the host cells, allowing the virus to collaborate with the medication’s immunosuppressive effects to induce EBV lymphomas in patients with rheumatoid arthritis and polymyositis. […] Angiosarcomas have been reported in immunosuppressed patients following renal transplants specifically. […] However, the etiology of angiosarcoma in this setting as in our case also remains unclear.
  • #48 Etiology, pathogenesis, and management of angiosarcoma associated with implants and foreign body: Clinical cases and research updates – PubMed
    https://pubmed.ncbi.nlm.nih.gov/38701315/
    Angiosarcomas are rare and highly malignant soft tissue sarcomas originating from endothelial cells lining the lymphatic or vascular system. […] The etiology of angiosarcoma remains elusive in most clinical cases. Nevertheless, several prognosis risk factors play a pivotal role, including chronic lymphedema, therapeutic irradiation, environmental carcinogens, familial syndromes, and the presence of foreign materials like metallic objects and biomedical implants. […] Despite evidence implicating retained foreign material in angiosarcoma development, understanding its prognosis and pathogenesis remains limited. […] The pathogenesis of angiosarcoma appears to involve a complex interplay of chronic inflammation, tissue remodeling, and genetic factors that create a conducive microenvironment for malignant transformation. […] This comprehensive review delves into the potential etiological and pathogenic roles of foreign materials, such as metallic objects, biomedical implants, and biomaterials, in the development of angiosarcoma.
  • #49 Angiosarcoma – SFA
    https://curesarcoma.org/sarcoma-subtypes/angiosarcoma/
    The etiology is unknown in most cases, although a minority arise after radiation exposure or longstanding lymphedema. […] Smaller numbers occur in association with implanted foreign material (including synthetic grafts), in the vicinity of arteriovenous fistulas, in a pre-existing haemangioma/vascular malformation, in regions of prior trauma or surgery, in certain syndromes (e.g., neurofibromatosis and Maffucci syndrome), and rarely as heterologous components of other neoplasms (e.g., in benign or malignant nerve sheath tumors).
  • #50 Angiosarcoma – SFA
    https://curesarcoma.org/sarcoma-subtypes/angiosarcoma/
    The etiology is unknown in most cases, although a minority arise after radiation exposure or longstanding lymphedema. […] Smaller numbers occur in association with implanted foreign material (including synthetic grafts), in the vicinity of arteriovenous fistulas, in a pre-existing haemangioma/vascular malformation, in regions of prior trauma or surgery, in certain syndromes (e.g., neurofibromatosis and Maffucci syndrome), and rarely as heterologous components of other neoplasms (e.g., in benign or malignant nerve sheath tumors).
  • #51 Angiosarcoma | Boston Children’s Hospital
    https://www.childrenshospital.org/conditions/angiosarcoma
    Angiosarcoma is a type of cancer that begins in the cells that line the blood vessels or lymph vessels. […] The cause of angiosarcoma is currently unknown, although genetic mutations recently have been identified in adult patients; scientists are now searching for therapies that target these mutations. In adults, angiosarcoma can arise after exposure to chemicals or radiation, or in long-standing ulcerations and lymphedema. […] Though very rare, angiosarcomas in children have been reported to arise in vascular anomalies and hemangiomas of the liver.
  • #52
    https://journals.lww.com/md-journal/fulltext/2024/05030/etiology,_pathogenesis,_and_management_of.73.aspx
    The exact etiology of AS remains multifaceted and, in many cases, elusive. While several potential risk factors have been identified, a direct pathogenic mechanism is often challenging to pinpoint. […] Foreign body tumorigenesis is a well-established phenomenon that has been experimentally validated in laboratory animals. However, due to poor prognosis, direct evidence of such an event in humans remains enigmatic. […] The tested potentials of foreign body in evoking sarcoma development has enlisted them as an independent etiological risk factor. […] The vicinal association of the foreign body to the AS development indicates their significant involvement in the pathogenesis of the disease. […] Chronic inflammation around these foreign materials could probably potentiate the pathogenic progression to the malignant transformation of surrounding soft tissues. However, a direct connection between the development of AS and the presence of foreign material at those sites has not been established in humans. […] The primary treatment modalities currently include surgery, radiotherapy, and chemotherapy, but recent advances in targeted immunotherapy and gene therapy hold promise for more effective approaches.
  • #53
    https://journals.lww.com/md-journal/fulltext/2024/05030/etiology,_pathogenesis,_and_management_of.73.aspx
    The exact etiology of AS remains multifaceted and, in many cases, elusive. While several potential risk factors have been identified, a direct pathogenic mechanism is often challenging to pinpoint. […] Foreign body tumorigenesis is a well-established phenomenon that has been experimentally validated in laboratory animals. However, due to poor prognosis, direct evidence of such an event in humans remains enigmatic. […] The tested potentials of foreign body in evoking sarcoma development has enlisted them as an independent etiological risk factor. […] The vicinal association of the foreign body to the AS development indicates their significant involvement in the pathogenesis of the disease. […] Chronic inflammation around these foreign materials could probably potentiate the pathogenic progression to the malignant transformation of surrounding soft tissues. However, a direct connection between the development of AS and the presence of foreign material at those sites has not been established in humans. […] The primary treatment modalities currently include surgery, radiotherapy, and chemotherapy, but recent advances in targeted immunotherapy and gene therapy hold promise for more effective approaches.
  • #54 Angiosarcoma of the breast, the unknown—a review of the current literature – Esposito – Translational Cancer Research
    https://tcr.amegroups.org/article/view/30432/html
    Angiosarcoma of the breast can be classified into primary when arising de novo and secondary to chronic lymphoedema or breast irradiation. […] Although the aetiology of the primary angiosarcoma of the breast remains unknown, the possible risk factors include trauma, radiation and lymphedema, but there is no definitive data to support this claim. […] Point mutations in BRCA2 are supposed to be causes of some secondary breast angiosarcomas. […] The loss of function of BRCA mutated prevents to exert protection against radiation-induced DNA damage hence it is reasonable supposing a role in developing this kind of tumour. […] p53 loss of function, mouse double minute 2 (MDM2) and vascular endothelial growing factor (VEGF) overexpression are alterations of molecular pathways which have been found involved in angiosarcoma origin.
  • #55
    https://journals.lww.com/md-journal/fulltext/2024/05030/etiology,_pathogenesis,_and_management_of.73.aspx
    The exact etiology of AS remains multifaceted and, in many cases, elusive. While several potential risk factors have been identified, a direct pathogenic mechanism is often challenging to pinpoint. […] Foreign body tumorigenesis is a well-established phenomenon that has been experimentally validated in laboratory animals. However, due to poor prognosis, direct evidence of such an event in humans remains enigmatic. […] The tested potentials of foreign body in evoking sarcoma development has enlisted them as an independent etiological risk factor. […] The vicinal association of the foreign body to the AS development indicates their significant involvement in the pathogenesis of the disease. […] Chronic inflammation around these foreign materials could probably potentiate the pathogenic progression to the malignant transformation of surrounding soft tissues. However, a direct connection between the development of AS and the presence of foreign material at those sites has not been established in humans. […] The primary treatment modalities currently include surgery, radiotherapy, and chemotherapy, but recent advances in targeted immunotherapy and gene therapy hold promise for more effective approaches.
  • #56 Angiosarcoma in Chronic Lymphedema (Stewart-Treves Syndrome) | Actas Dermo-Sifiliográficas
    https://www.actasdermo.org/es-angiosarcoma-in-chronic-lymphedema-stewart-treves-articulo-S1578219012002004
    Angiosarcoma is a malignant endothelial tumor that can form in any part of the body, including the skin. It is known as hemangiosarcoma or lymphangiosarcoma, depending on whether it derives from the endothelium of the blood vessels or the lymph vessels, respectively. […] Angiosarcoma that develops on a limb with chronic lymphedema or Stewart-Treves syndrome is a rare complication with a rapid and aggressive course. No cure exists to date and the disease is associated with poor prognosis and considerable morbidity. When the disease occurs in the context of a mastectomy, removal of the axillary lymph nodes and radiotherapy are thought to be predisposing factors. […] The limbs are the most commonly affected areas, but lymphangiosarcoma has even been reported after abdominoplasty in obese patients. Furthermore, according to published research, the mean time between development of lymphedema and diagnosis of angiosarcoma is between 8 and 10 years; it is thus important to carry out long-term follow-up of patients. […] Prevention of lymphedema of the limbs, when treatable secondary causes exist, should be considered essential and existing medical measures to achieve this goal should be optimized.
  • #57 Acute liver failure due to primary angiosarcoma: A case report and review of literature | World Journal of Surgical Oncology | Full Text
    https://wjso.biomedcentral.com/articles/10.1186/1477-7819-6-104
    Hepatic angiosarcoma is a primary sarcoma of the liver, accounting for only 2% of all primary hepatic malignancies. […] Angiosarcoma is associated with environmental or occupational exposure to carcinogens (thorium dioxide, vinyl chloride, arsenic and radiation). There is also an association with hemochromatosis and von Recklinghausen disease. […] In most cases of primary hepatic angiosarcoma, no obvious risk factor can be identified. […] The mechanism of liver failure is multifactorial. Evidence suggests a combination of hepatic ischaemia leading to parenchymal infarction, vascular occlusion of portal vein by tumour thrombi and nonocclusive infarction of liver due to shock from secondary causes such as sepsis or cardiac dysfunction plays an important role in these patients. […] In this patient, replacement of hepatocytes by malignant cells, leading to secondary necrosis of hepatocytes played a significant role in development of liver failure. […] Angiosarcoma has very limited treatment options, without treatment the majority of patients die within 6 months of diagnosis.
  • #58 Hepatic Angiosarcoma: A Rare Cause of Acute Liver Failure Case Report and Review of the Literature
    http://www.fortunejournals.com/articles/hepatic-angiosarcoma-a-rare-cause-of-acute-liver-failure-case-report-and-review-of-the-literature.html
    Hepatic Angiosarcoma is a rare, high grade, aggressive mesenchymal tumor. […] Some hepatic Angiosarcoma were associated with exposure to anabolic steroids, radiation, thorium dioxide, arsenic, vinyl chloride and use of oral contraceptives, but most cases are now considered idiopathic. […] HAS is a rare and aggressive mesenchymal tumor associated with rapid liver failure and high mortality rate. It was associated with environmental exposure to carcinogens but etiology still unknown in the majority of the cases. […] Although hepatic angiosarcoma was associated with the use of oral contraceptives, exposure to environmental carcinogens, including radiation, thorium dioxide, arsenic or vinyl chloride, most cases arise without any causative associated risk factors. […] In HAS, the mechanism of liver failure is multifactorial. It is suggested that it be related to ischemia of the hepatic parenchyma, tumour thrombi due to portal vein occlusion and non occlusive infarction of liver due to sepsis or cardiac shock and the replacement of the hepatocytes by malignant cells that lead to secondary necrosis. […] This unusual combination of clinical characteristics was part of the case we reported. Our patient had a progressively worsening clinical course and died within four months of diagnosis. This demonstrates the rapid progression, poor prognosis, and the need to find a therapy that improves survival.
  • #59 Angiosarcoma | Quirónsalud
    https://www.quironsalud.com/en/enfermedades-sintomas/angiosarcoma
    Angiosarcoma occurs due to abnormal and uncontrolled growth of endothelial cells in blood or lymphatic vessels. This growth is triggered by genetic alterations or mutations that affect the process of cell growth and division. These mutations may arise without an apparent cause (primary angiosarcoma) or be associated with certain risk factors (secondary angiosarcoma). […] The following conditions increase the risk of developing angiosarcomas: Chronic lymphedema: a disease characterized by the accumulation of lymphatic fluid in tissues due to damage to the lymphatic system. Previous radiation therapy treatment. Exposure to chemicals such as arsenic and vinyl chloride. This is linked to liver angiosarcoma. Genetic disorders, such as neurofibromatosis, Maffucci syndrome, or Klippel-Trenaunay syndrome. Inherited mutations in the BRCA1 and BRCA2 genes, which cause abnormal cell growth.
  • #60 Cutaneous Angiosarcoma in a Psoriasis Patient on Infliximab and Methotrexate Therapy
    https://clinmedjournals.org/articles/iaim/international-archives-of-internal-medicine-iaim-2-006.php?jid=iaim
    Angiosarcoma is a soft tissue sarcoma of endothelial origin that can arise in any organ, with an incidence of up to 5% of all malignant cutaneous tumors. […] Risk factors for cutaneous angiosarcoma have not been clearly established. […] Whether there is a specific effect of these drugs on the development of the patient’s angiosarcoma remains uncertain. […] Angiosarcomas are frequently categorized as follows: Primary/idiopathic, soft tissue (uncommonly seen in the deep soft tissues of the lower extremities and abdominal wall; Often in patients with familial syndromes), lymphedema-associated (usually seen in post-mastectomy patients within chronic lymphedema), and radiation-induced angiosarcoma (within or adjacent to the radiation field, commonly seen in breast cancer patients). […] However, the etiology of cutaneous angiosarcoma occurring on the scalp or face, as in our patient, has yet to be elucidated.
  • #61 Angiosarcoma Awareness Inc
    https://www.cureasc.org/
    Angiosarcoma is a rare type of cancer that affects the inner lining of blood vessels or lymph vessels. There is ongoing research on angiosarcoma, aimed at improving the understanding of its causes, developing new diagnostic methods, and finding more effective treatments. […] Scientists are studying the genetic mutations that may contribute to the development of angiosarcoma.
  • #62 Angiosarcoma | Sarcoma UK
    https://sarcoma.org.uk/about-sarcoma/what-is-sarcoma/types-of-sarcoma/angiosarcoma/
    Angiosarcoma is a rare type of sarcoma that develops from the cells lining the blood vessels. […] The cause of most angiosarcoma is unknown. But, exposure to radiation has been shown to cause some angiosarcoma. Also, swelling in the lymph nodes, known as lymphoedema, chemical exposure and genetic diseases are known risk factors for angiosarcoma. Researchers are still trying to learn more about the causes of angiosarcoma. […] Angiosarcomas of the breast are more likely to be found in women who have had radiotherapy to treat breast cancer. This is called radiation-induced sarcoma. […] Researchers have found that some targeted therapies and immunotherapies worked well in clinical trials of angiosarcoma. But, more research and trials are needed before these treatments are approved.
  • #63 What is cardiac angiosarcoma? | MD Anderson Cancer Center
    https://www.mdanderson.org/cancerwise/heart-cancer-cardiac-tumors-insights-on-diagnosis–treatment-and-research.h00-159543690.html
    Cardiac angiosarcoma is the most common primary cardiac sarcoma. […] Although there are about 1.7 million new cancer cases yearly, only about 25 are cardiac angiosarcoma diagnoses. Its extraordinarily rare, Ravi says. Its also typically seen in adults younger than age 65. […] Its hypothesized that tumors on the right side of the heart may be fueled by vascular endothelial growth factor (VEGF), Ravi says. VEGF is a protein that helps grow blood vessels. […] Unfortunately, because of the rarity of these tumors and the challenges with taking tumor tissue from the heart, there isnt a lot of research explaining why these tumors grow more in the right atrium and what growth regulatory genes promote tumor growth. That’s the work in front of us, he says.
  • #64 Mouse genetic background influences whether Hras G12V expression plus Cdkn2a knockdown causes angiosarcoma or undifferentiated pleomorphic sarcoma | Oncotarget
    https://www.oncotarget.com/article/24831/text/
    Soft tissue sarcomas are rare mesenchymal tumours accounting for 1% of adult malignancies and are fatal in approximately one third of patients. […] Another clinically aggressive subtype of high-grade soft tissue sarcoma is angiosarcoma. These tumours represent rare malignancies of endothelial differentiation that account for approximately 1% of all soft tissue sarcomas. Angiosarcomas show a wide anatomic distribution and arise spontaneously or secondarily to radiation, toxic chemicals (e.g. vinyl chloride) or chronic lymphoedema (Stewart-Treves syndrome). Treatment options are limited and the prognosis is poor. Genetic mutations and amplifications of VEGF, MDM2, TP53, CDKN2A, KRAS and MYC have been described in angiosarcoma patients. […] Several in vivo mouse studies showed the involvement of loss of function of the p53 tumour suppressor in angiosarcoma development.
  • #65 Mouse genetic background influences whether Hras G12V expression plus Cdkn2a knockdown causes angiosarcoma or undifferentiated pleomorphic sarcoma | Oncotarget
    https://www.oncotarget.com/article/24831/text/
    In addition, the in vivo deletion of Cdkn2a in mice lead to the development of lesions which recapitulate human angiosarcoma, however, only 30% of the mice displayed angiosarcomas within 100 days. […] We conclude that we have developed a rapid autochthonous mouse model of angiosarcoma that is trackable via live animal imaging and that reflects the frequent genetic alterations that arise in human angiosarcoma tumours.
  • #66
    https://journals.lww.com/md-journal/fulltext/2024/05030/etiology,_pathogenesis,_and_management_of.73.aspx
    Angiosarcomas are rare and highly malignant soft tissue sarcomas originating from endothelial cells lining the lymphatic or vascular system. The etiology of angiosarcoma remains elusive in most clinical cases. Nevertheless, several prognosis risk factors play a pivotal role, including chronic lymphedema, therapeutic irradiation, environmental carcinogens, familial syndromes, and the presence of foreign materials like metallic objects and biomedical implants. […] The etiology of the disease is not well defined, and the diagnosis remains challenging due to rarity and poor prognosis. AS may arise spontaneously de novo without predisposing conditions or systemic syndromes. Nevertheless, several reports have identified potential predisposing risk factors such as radiation therapy, chronic lymphedema, familial genetic syndromes, environmental carcinogens, and retained foreign body material responsible for the pathogenesis of AS.
  • #67 Angiosarcoma – SFA
    https://curesarcoma.org/sarcoma-subtypes/angiosarcoma/
    The etiology is unknown in most cases, although a minority arise after radiation exposure or longstanding lymphedema. […] Smaller numbers occur in association with implanted foreign material (including synthetic grafts), in the vicinity of arteriovenous fistulas, in a pre-existing haemangioma/vascular malformation, in regions of prior trauma or surgery, in certain syndromes (e.g., neurofibromatosis and Maffucci syndrome), and rarely as heterologous components of other neoplasms (e.g., in benign or malignant nerve sheath tumors).
  • #68 Angiosarcoma of the breast, the unknown—a review of the current literature – Esposito – Translational Cancer Research
    https://tcr.amegroups.org/article/view/30432/html
    The observed deregulation of p53/MDM-2 expression affects also differentiation and phenotype of endothelial cells up-regulating VEGF expression. […] MYC high-level gene amplifications were observed in all secondary angiosarcoma cases but not in primary ones, suggesting that, despite their identical morphology, secondary angiosarcomas are genetically different from primary ones. […] PIK3CA/AKT/mTOR pathway is either directly or indirectly involved in breast angiosarcoma onset. […] Aetiology and molecular pathways are only partially known and still under investigation.
  • #69 Pericardial Angiosarcoma: Status Quo
    https://www.acc.org/latest-in-cardiology/articles/2019/09/04/06/43/pericardial-angiosarcoma
    Angiosarcoma originating from the heart, although exceptionally rare, is the most common cardiac primary malignant tumor. It has diverse clinical presentations and histological appearances, making early diagnosis challenging. […] To date, no specific etiology and risk factors for the development of pericardial angiosarcoma are known. However, exposure to vinyl chloride, namely plastics, is believed to be associated with angiosarcoma. It has also been reported that mediastinal radiation makes a contribution to the development of this entity. […] Presenting symptoms of this tumor frequently appear late, resulting in unresectable tumors in most cases. Consequently, available treatment options for this malignancy are only palliative. The length of survival for this group of neoplasms is dismal, ranging between 6-11 months after the onset of symptoms. […] When the etiology of recurrent bloody pericardial effusion remains unknown, underlying malignancy should be suspected and early invasive diagnostic interventions, such as surgical exploration, should be considered for targeted therapy.