Materiały źródłowe

• #1 Etiology, pathogenesis, and management of angiosarcoma associated with implants and foreign body: Clinical cases and research updates

  https://pmc.ncbi.nlm.nih.gov/articles/PMC11062743/

  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.

• #2 Angiosarcoma – Symptoms & causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/angiosarcoma/symptoms-causes/syc-20350244

  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. […] The result is a buildup of cancer cells that can grow beyond the blood vessel or lymph vessel. The cancer cells can invade and destroy healthy body tissue. In time, cancer cells may break away and spread to other areas of the body.

• #3 Angiosarcoma: Practice Essentials, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/276512-overview

  Angiosarcomas arising at different sites and in different organs have some distinct features. Angiosarcomas may occur in any region of the body but are more frequent in skin and soft tissue. Angiosarcomas also can originate in the liver, breast, spleen, bone, or heart. […] The Angiosarcoma Project performed whole-exome sequencing of 47 tumors and found recurrent mutations of genes including KDR, TP53, and PIK3CA. PIK3CA-activating mutations were observed predominantly in primary breast angiosarcoma, while angiosarcoma of the head, neck, face, and scalp was associated with a high tumor mutation burden and a dominant ultraviolet damage mutational signature, suggesting that ultraviolet damage may be a causative factor and that immune checkpoint inhibition may be beneficial.

• #4 Imaging features of primary sites and metastatic patterns of angiosarcoma | Insights into Imaging | Full Text

  https://insightsimaging.springeropen.com/articles/10.1186/s13244-021-01129-9

  Angiosarcomas are rare, aggressive soft tissue sarcomas originating from endothelial cells of lymphatic or vascular origin and associated with a poor prognosis. […] The aetiology is unknown in most cases, but there are several risk factors that are associated with angiosarcomas. […] A previous study using data from the Surveillance, Epidemiology and End Results (SEER) registries has shown that radiotherapy is associated with an increased risk in the development of secondary sarcomas in breast cancer patients, with angiosarcomas being the most prevalent accounting for 56.8% of radiation-associated sarcomas. […] The relation between angiosarcoma and chronic lymphoedema (also known as StewartTreves syndrome) has been historically established in post-mastectomy patients with lymphoedema in the upper limb but in can be of any origin.

• #5 Etiology, pathogenesis, and management of angiosarcoma associated with implants and foreign body: Clinical cases and re…

  https://ouci.dntb.gov.ua/en/works/9ZRb6Dv9/

  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.

• #6 Angiosarcoma – Wikipedia

  https://en.wikipedia.org/wiki/Angiosarcoma

  Angiosarcoma is a rare and aggressive cancer that starts in the endothelial cells that line the walls of blood vessels or lymphatic vessels. […] 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. […] Ultraviolet radiation and localized immunodeficiency may play a role in pathogenesis of angiosarcoma. […] Angiosarcoma develops when damage to the DNA of endothelial cells causes them to divide uncontrollably and prevents programmed death. […] Accepted risk factors include lymphedema, radiation therapy, neurofibromatosis, Maffucci syndrome, Klippel-Trenaunay syndrome and chemicals such as arsenic, thorotrast and vinyl chloride. […] A 2020 study analyzing the genome of cutaneous angiosarcomas suggests that ultraviolet radiation may be a causative agent of angiosarcomas. […] Immunosuppression may play a role in the pathogenesis of angiosarcoma.

• #7 Angiosarcoma – SFA

  https://curesarcoma.org/sarcoma-subtypes/angiosarcoma/

  Angiosarcomas are genetically heterogeneous. Most harbor complex karyotypes, without recurrent chromosomal changes. Unlike other sarcomas with complex genomics, angiosarcomas show low levels of alterations in TP53 and PIK3CA/AKT/mTOR pathways. Genes related to angiogenesis and vascular-specific receptor tyrosine kinases (including TIE1, TEK, KDR [VEGFR2], and FLT4 [VEGFR3]) are usually upregulated compared with other sarcomas. […] A subset of cases, typically in younger patients, are associated with CIC gene abnormalities and inferior disease-free survival. About 40% harbor recurrent somatic mutations involving angiogenic signaling pathways (e.g., in KDR, PTPRB, and PLCG1), and rare mutations occur in RAS genes, PIK3CA, TP53, FLT4, and TIE1. […] High-level MYC gene amplifications (at 8q24) occur in almost all postirradiation and chronic lymphedema-associated angiosarcomas, and rarely in primary angiosarcomas; 25% of secondary angiosarcomas are associated with FLT4 (VEGFR3) coamplification at 5q35. PLCG1 and KDR mutations occur in subsets of both primary and secondary angiosarcomas, particularly in breast and bone/visceral sites, regardless of MYC status. KDR and PLCG1 mutations are mutually exclusive, with both genes involved in the angiogenesis signaling pathway. FLT4-amplified angiosarcomas lack PLCG1 or KDR mutations, occur predominantly in MYC-amplified tumors, and are associated with a poor prognosis.

• #8 Malignant vascular tumors—an update | Modern Pathology

  https://www.nature.com/articles/modpathol2013176

  The most common cause of angiosarcoma appears to be therapeutic radiation, which was a well-recognized cause of hepatic angiosarcoma in the era when the thorium containing contrast agent Thorotrast was employed. Presently, the breast is the most common anatomic site affected by radiation-induced angiosarcoma. […] Angiosarcomas may arise after exposure to vinyl chloride, although they remain rare tumors even in an exposed population. Angiosarcomas are also observed after lymphedema from any cause, be it surgical, filarial or congenital, and defined as Stewart-Treves syndrome. […] High level MYC amplification on 8q24 is a consistent hallmark of radiation-induced and lymphedema-associated angiosarcoma. […] Furthermore, high level MYC amplification on 8q24 is a consistent hallmark of radiation-induced and lymphedema-associated angiosarcoma.

• #9 Angiosarcoma : Molecular Genetics

  https://www.webpathology.com/images/soft-tissue/vascular-and-lymphatic/angiosarcoma/44510

  Angiosarcoma is genetically heterogenous with different clinical subtypes showing non-overlapping genetic abnormalities. Gene expression profiling studies have shown up-regulation of several genes linked to angiogenesis and endothelial differentiation. These include genes for vascular specific receptor tyrosine kinases: FLT1 (VEGFR1), KDR (VEGFR2), TIE1, SNRK, and TEK. Recurrent somatic mutations have been identified in KDR, PTPRB, and PLCG1 genes in about 40% of cases. These genes are involved in angiogenic signaling pathways. PTPRB mutations, with or without co-existing R707Q hotspot PLCG1 mutations, have been reported exclusively in secondary or radiation-associated/MYC-amplified angiosarcomas. Activating KDR mutations and PLCG1 mutations are seen in about 10% of angiosarcomas, both primary and secondary tumors, but restricted to breast, bone, and viscera. KDR and PLCG1 genes are involved in the VEGFR2 signaling pathway and their mutations appear to be mutually exclusive. Amplification of MYC oncogene (on 8q24.21) is a hallmark of post-radiation and chronic lymphedema-associated angiosarcomas, especially following treatment for breast cancer (91% of cases). This abnormality is seen in only a small percentage of angiosarcomas secondary to radiation for other tumor types (lymphoma, hemangiomatosis, salivary gland carcinoma, germ cell tumor, and prostate cancer) and in primary angiosarcomas (7% of cases). MYC amplification can be detected by fluorescence in-situ hybridization or by immunohistochemistry. MYC amplification is useful in separating post-radiation angiosarcomas (MYC amplified) from other radiation-induced conditions such as atypical vascular lesion (no MYC amplification). In about 25% of post-radiation angiosarcomas, there is co-amplification of FLT4 which codes for VEGFR3. FLT4-amplified angiosarcomas usually have solid, high-grade phenotype with epithelioid or spindle cell morphology.

• #10 Meningeal angiosarcoma: a case report and review of the literature – Cai – Translational Cancer Research

  https://tcr.amegroups.org/article/view/9509/html

  The pathogenetic mechanism of angiosarcoma may be related to many vascular growth factors. Lots of studies suggested that vascular endothelial growth factor (VEGF) and its receptors can be overexpressed in angiosarcomas, especially highly concentrated VEGF-A with its receptors found in tissues of angiosarcoma. […] Lack of VEGFR-2 expression in tumor tissues could herald a worse prognosis. Angiosarcoma may also be associated with the abnormal expression of TP53, Wilms tumor-1, and galectin-3. […] Studies have reported K-ras gene mutations in angiosarcoma of liver and cardiac tissue. Some researchers found that about 50% of angiosarcoma had positive C-kit gene expression, but showed no activating mutations on exon 11 or 17. […] Common chromosomal abnormalities of angiosarcoma included trisomy 5; deletions on the short arm of chromosome 7; varied abnormalities on chromosomes 8, 20, and 22; and loss of chromosome Y.

• #11 Expression of angiopoietin-TIE system components in angiosarcoma | Modern Pathology

  https://www.nature.com/articles/modpathol201343

  Angiosarcoma is an aggressive malignancy of endothelial differentiation. Potential roles of the endothelial angiopoietin-tunica interna endothelial cell kinase (ANGPT-TIE) system in angiosarcoma diagnosis, pathogenesis, prognosis and treatment are undefined. […] The ANGPT-TIE pathway is largely confined to vasculature and consists of two tyrosine kinase receptors, TIE1 and TEK (TIE2), and three corresponding ligands, angiopoietins-1, -2 and -4. […] Despite considerable examination of the roles of the ANGPT-TIE system in solid tumors, little information is available regarding the significance of this predominantly endothelium-limited signaling pathway in angiosarcoma. […] Our observation that increased expression of ANGPT-TIE system components is associated with both the more favorable vasoformative histological pattern and improved overall survival is consistent with a loss of endothelial markers with progression to more aggressive disease. […] TEK (TIE2) inhibitor therapy has recently been reported to inhibit angiosarcoma growth in two distinct murine models of this disease. […] Further studies on the mechanistic roles for this critical vascular signaling pathway in angiosarcomagenesis are certainly merited.

• #12 Clinical Pathophysiology and Research Highlights of Cardiac Angiosarcoma: Obligation for Immunogenetic Profiling to Understand Their Growth Pattern and Tailor Therapies

  https://www.mdpi.com/2673-3846/5/3/28

  The clinical presentation, growth pattern, treatment response, and clinical remission rate of cardiac angiosarcoma are quite variable, as seen in the clinical cases reported in the literature. […] In most cases, the growth pattern of the angiosarcoma is primarily dependent upon its inherent genetic expression, which provides the necessary drive for its aggressive growth pattern. […] Exploration to ascertain the genetic makeup and immunological markers of these intractable tumor tissues might unravel molecular targets, which might be the lurking and tangible catalysts for setting in motion pertinent downstream signaling events for sparking extravagant growth patterns typically perceived in these tumors. […] The underlying and intrinsic signaling mechanisms that foster incessant proliferation, persistent angiogenesis, and endless survival of angiosarcoma cancer cells are PI3K [Phosphoinositide 3-kinases]/AKT [Protein Kinase b]/PLCG1 [Phospholipase C, gamma 1]/PKC [Protein Kinase C], and RAS [proto-oncogene] pathways.

• #13 IL-13/IL-13Rα2 axis promotes proliferation of angiosarcoma cells | bioRxiv

  https://www.biorxiv.org/content/10.1101/2024.10.24.619789v1.full-text

  Angiosarcoma is a rare and aggressive soft tissue sarcoma with a poor prognosis and limited treatment options. The role of interleukin-13 (IL-13) and its receptors in angiosarcoma pathogenesis has been largely unknown. We detected high IL-13 receptor α2 (IL-13Rα2) expression in angiosarcoma cell lines and patient samples compared to other cell types and benign vascular tumors. […] Moreover, histological analysis showed the presence of IL-13 in the angiosarcoma microenvironment. Functional studies using angiosarcoma cell lines, MO-LAS-B cells, revealed the promoting effect of IL-13 on cell proliferation. The effect was inhibited by siRNA-mediated knockdown of IL13RA2 or neutralizing antibodies against IL-13, suggesting the direct impact of IL-13/IL-13Rα2 axis in the angiosarcoma proliferation.

• #14 IL-13/IL-13Rα2 axis promotes proliferation of angiosarcoma cells | bioRxiv

  https://www.biorxiv.org/content/10.1101/2024.10.24.619789v1.full-text

  These findings highlight the importance of the IL-13/IL-13Rα2 axis in angiosarcoma progression and its potential as a novel therapeutic target for this challenging malignancy. […] Our findings revealed that IL-13 is present in the angiosarcoma microenvironment, with strong expression observed in atypical tumor cells. Notably, angiosarcoma cell lines exhibited higher expression of IL-13Rα2 compared to other cell types, including immune cells and skin fibroblasts. […] Functional studies showed that IL-13 promotes angiosarcoma cell proliferation, an effect mediated primarily through IL-13Rα2. […] Knockdown experiments and neutralizing antibody studies corroborated the critical role of the IL-13-IL-13Rα2 axis in driving angiosarcoma cell proliferation. […] Furthermore, IL-13 stimulation increased IL-13Rα2 and VEGF-A expression, suggesting a positive feedback mechanism. This upregulation was attenuated by a STAT6 inhibitor, suggesting a potential role for STAT6 in this signaling pathway.

• #15

  https://hammer.purdue.edu/articles/thesis/_b_MicroRNA_mediated_tumor_suppression_in_angiosarcoma_b_/26343802

  Angiosarcoma (AS) is a rare, understudied cancer that arises from endothelial cells with an extremely poor prognosis. More research is necessary to understand AS pathogenesis, which will lead to the development of novel therapies to improve patient survival. […] Evidence from our lab highlights the importance of miRNAs in disease progression by demonstrating that endothelial cell-specific loss of mature miRNAs drives AS in mice. Furthermore, individual miRNAs have been characterized as tumor suppressors in AS. […] Taken together, this underscores the role of miRNAs in AS pathogenesis and suggests that they are an underexplored therapeutic strategy that could be efficacious in treating this cancer. […] Overall, this work further highlights the important roles miRNAs play in AS pathogenesis, and points toward miRNAs as an exciting therapy that should be explored further.

• #16 Malignant vascular tumors—an update | Modern Pathology

  https://www.nature.com/articles/modpathol2013176

  MYC has a crucial role in growth control, differentiation and apoptosis, and its aberrant expression is associated with several cancers. More recently, MYC was also implicated as having a major contribution in tumor angiogenesis, by upregulating one of its direct targets, the miR-17-92 cluster. […] Indeed, a significant upregulation of miR-1792 cluster is present in MYC-amplified angiosarcoma compared with angiosarcoma lacking MYC amplification and other vascular lesions. […] Thus, MYC amplification has a crucial role in the angiogenic phenotype of angiosarcoma through upregulation of the miR-17-92 cluster, which subsequently downregulates thrombospondin-1 (THBS1), a potent endogenous inhibitor of angiogenesis.

• #17 Etiology, pathogenesis, and management of angiosarcoma associated with implants and foreign body: Clinical cases and research updates

  https://pmc.ncbi.nlm.nih.gov/articles/PMC11062743/

  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. […] Various reports have also highlighted the association of AS with the presence of foreign body material in human and animal models. […] Chronic inflammation around these foreign materials could probably potentiate the pathogenic progression to the malignant transformation of surrounding soft tissues. […] 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.

• #18 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 is one of the rarest malignancies. Molecular pathways involved in angiosarcoma development have not been described clearly, yet some gene point mutations and protein altered expression levels have been detected. […] 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. […] The observed deregulation of p53/MDM-2 expression affects also differentiation and phenotype of endothelial cells up-regulating VEGF expression.

• #19 Imaging features of primary sites and metastatic patterns of angiosarcoma | Insights into Imaging | Full Text

  https://insightsimaging.springeropen.com/articles/10.1186/s13244-021-01129-9

  The pathogenesis of angiosarcoma in the setting of chronic lymphoedema is unclear. It has been proposed that the oedematous region behaves as an immunologically privileged site, whereby tumour development is able to take place at this site without triggering a sufficient immune response. […] Angiosarcomas spread haematogenously, most frequently to the lungs, followed by liver and bones, but spread to the brain, lymph nodes and spleen have also been reported.

• #20 Clinical Pathophysiology and Research Highlights of Cardiac Angiosarcoma: Obligation for Immunogenetic Profiling to Understand Their Growth Pattern and Tailor Therapies

  https://www.mdpi.com/2673-3846/5/3/28

  Cardiac angiosarcoma is the most common malignant tumor of the heart. […] The most common type of sarcoma is cardiac angiosarcoma, which primarily originates from the blood vessels of the heart. […] Although the exact cause is unknown, the relationship between some risk factors and the onset of soft tissue angiosarcoma was ascertained based on prior clinical studies. Some of the common etiologies speculated to trigger the onset of cardiac angiosarcoma are radiation therapy, environmental carcinogens (vinyl chloride, thorium dioxide, and chronic arsenic ingestion and Anabolic steroids), lymphedema, immunosuppression, and genetic syndromes (Neurofibromatosis NF-1, Maffucci syndrome, bilateral retinoblastoma, Ollier Disease, Xeroderma pigmentosa, and Klippel–Trenaunay syndrome). […] Genetic predisposition to the development of angiosarcoma was seen in some genetic syndromes, and it has been hypothesized that aberrations in the angiogenic pathways and tumor suppressor genes might be alleged to have caused its causation.

• #21 Liver angiosarcoma – Wikipedia

  https://en.wikipedia.org/wiki/Liver_angiosarcoma

  Liver angiosarcoma is a rare and rapidly fatal cancer arising from endothelial that line the blood vessels of the liver. […] Despite this, several things are associated with liver angiosarcoma. Exposure to vinyl chloride, arsenic, thorotrast, radium, phenylhydrazine and use of androgens is known to contribute to the pathogenesis of liver angiosarcoma. […] A study in Taiwan found no evidence to suggest that viral hepatitis a significant role in the pathogenesis of liver angiosarcoma.

• #22 Etiology, pathogenesis, and management of angiosarcoma associated with implants and foreign body: Clinical cases and research updates

  https://pmc.ncbi.nlm.nih.gov/articles/PMC11062743/

  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. […] Pathogenic progression of AS remains enigmatic, and the mechanism is poorly understood due to poor prognosis. […] It has been hypothesized that the solid-state carcinogenesis associated with the inert foreign material is mediated by fibrous capsule formation. […] This leads to granulation tissue response in which the site of the foreign body is infiltrated with neutrophils, monocytes, macrophages, and matrix proteins, adhering them to the inflammatory site.

• #23

  https://journals.lww.com/md-journal/fulltext/2024/05030/etiology,_pathogenesis,_and_management_of.73.aspx

  Foreign body tumorigenesis is a well-established phenomenon that has been experimentally validated in laboratory animals. […] 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. […] Pathogenic progression of AS remains enigmatic, and the mechanism is poorly understood due to poor prognosis. […] It has been hypothesized that the solid-state carcinogenesis associated with the inert foreign material is mediated by fibrous capsule formation. […] This leads to granulation tissue response in which the site of the foreign body is infiltrated with neutrophils, monocytes, macrophages, and matrix proteins, adhering them to the inflammatory site.

• #24 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 are always working to understand how cancer forms, but it can be hard to prove. We know that some risk factors for getting angiosarcoma include: […] Angiosarcoma can sometimes run in families. 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.

• #25 Soft tissue sarcoma – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/soft-tissue-sarcoma/symptoms-causes/syc-20377725

  Soft tissue sarcoma starts when a connective tissue cell gets changes in its DNA. A cell’s DNA holds the instructions that tell a cell what to do. The changes turn the connective tissue cells into cancer cells. The changes tell the cancer cells to grow and make more cells. Healthy cells die as part of their natural cycle, but cancer cells keep growing because they do not have instructions to stop. […] The type of cell with DNA changes is what determines the type of soft tissue sarcoma. For example, angiosarcoma begins in cells in the lining of blood vessels, while liposarcoma starts in fat cells.

• #26 Etiology, pathogenesis, and management of angiosarcoma associated with implants and foreign body: Clinical cases and research updates

  https://pmc.ncbi.nlm.nih.gov/articles/PMC11062743/

  AS initially can appear as inflammatory lesions and, if ignored, may eventually progress into a tumor mass with metastatic potentials. […] The long-standing granulation tissue response, venous ulceration, and hemorrhage further prognoses to the fibrotic stage through extensive tissue fibrosis evolving into a mature fibrous capsule surrounding the implant. […] The abnormal malignant mesenchymal cells undergo remodeling into neoplastic endothelial cells and fibroblast sequestered with vascular elements. […] However, direct evidence linking the foreign body tumorigenic potential to AS development remains baffled and, therefore, necessitates a detailed exploration of the molecular pathogenesis pathway.

• #27

  https://journals.lww.com/md-journal/fulltext/2024/05030/etiology,_pathogenesis,_and_management_of.73.aspx

  AS initially can appear as inflammatory lesions and, if ignored, may eventually progress into a tumor mass with metastatic potentials. […] The long-standing granulation tissue response, venous ulceration, and hemorrhage further prognoses to the fibrotic stage through extensive tissue fibrosis evolving into a mature fibrous capsule surrounding the implant. […] However, direct evidence linking the foreign body tumorigenic potential to AS development remains baffled and, therefore, necessitates a detailed exploration of the molecular pathogenesis pathway.

• #28

  https://journals.lww.com/md-journal/fulltext/2024/05030/etiology,_pathogenesis,_and_management_of.73.aspx

  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. […] Various reports have also highlighted the association of AS with the presence of foreign body material in human and animal models. […] Chronic inflammation around these foreign materials could probably potentiate the pathogenic progression to the malignant transformation of surrounding soft tissues. […] 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.

• #29 Radiation Oncology/Sarcoma/Angiosarcoma – Wikibooks, open books for an open world

  https://en.wikibooks.org/wiki/Radiation_Oncology/Sarcoma/Angiosarcoma

  Thought to arise from vascular endothelium […] Thought to originate from vascular endothelium […] Vascular endothelial factor D (VEGF-D) and angiopoietin 2 significantly elevated compared to controls […] Fas ligand is detected in 70% tumors, and number of Fas-L positive cells correlates inversely with survival. This suggests that Fas-L positive tumor cells may induce apoptosis in tumor-infiltrating lymphocytes and thus blunt immune response […] Frequently develops in areas previously treated with radiation or with chronic lymphedema Stewart-Treves syndrome […] Pathology distinguished from hemangioma by presence of „collagen dissection patterns”, anastomoses, and papillary formation […] Early lesions may in fact present as benign hemangiomas […] Most present as intermediate or high grade lesions, but typically have an aggressive course

• #30 Cutaneous Angiosarcoma: Clinical and Pathology Study of 16 Cases | Actas Dermo-Sifiliográficas

  https://www.actasdermo.org/en-cutaneous-angiosarcoma-clinical-pathology-study-articulo-S1578219017300926

  Primary cutaneous angiosarcoma is one of the most aggressive skin tumors and carries a very poor prognosis. Its initially indolent clinical presentation explains the frequently late diagnosis that, together with its typically multifocal pattern and poor delimitation, often makes surgery difficult. […] The major determinants of survival among patients with cutaneous angiosarcoma are tumor size and patient age. Other characteristics associated with a poor prognosis were infiltration of deep planes (muscle), a predominantly solid histologic pattern, and a larger number of mitoses. […] Cutaneous angiosarcoma has one of the worst prognoses among all cutaneous tumors. It is very aggressive and has high local recurrence rates. […] The histopathologic appearance of cutaneous angiosarcoma varies from relatively differentiated forms with recognizable vascular spaces covered by prominent endothelial cells with some atypia and an infiltrative pattern dissecting the collagen bundles to more solid highly undifferentiated forms composed of spindle or epithelioid cells with considerably greater atypia and pleomorphism and a higher number of mitoses.

• #31 Cutaneous Angiosarcoma: Clinical and Pathology Study of 16 Cases | Actas Dermo-Sifiliográficas

  https://actasdermo.org/en-cutaneous-angiosarcoma-clinical-pathology-study-articulo-S1578219017300926

  Primary cutaneous angiosarcoma is one of the most aggressive skin tumors and carries a very poor prognosis. […] The major determinants of survival among patients with cutaneous angiosarcoma are tumor size and patient age. Other characteristics associated with a poor prognosis were infiltration of deep planes (muscle), a predominantly solid histologic pattern, and a larger number of mitoses. […] Cutaneous angiosarcoma has one of the worst prognoses among all cutaneous tumors. It is very aggressive and has high local recurrence rates. […] The histopathologic appearance of cutaneous angiosarcoma varies from relatively differentiated forms with recognizable vascular spaces covered by prominent endothelial cells with some atypia and an infiltrative pattern dissecting the collagen bundles to more solid highly undifferentiated forms composed of spindle or epithelioid cells with considerably greater atypia and pleomorphism and a higher number of mitoses.

• #32 Radiation Oncology/Sarcoma/Angiosarcoma – Wikibooks, open books for an open world

  https://en.wikibooks.org/wiki/Radiation_Oncology/Sarcoma/Angiosarcoma

  Well differentiated tumors are composed of well-formed, irregular vascular channels anastomosing together, lined by atypical cells […] Poorly differentiated tumors may contain only pleomorphic spindle cells with subtle vascular lumens, or may still contain vaso-formative areas […] Grade is not a prognostic factor.

• #33

  https://journals.lww.com/ijpm/fulltext/2024/67030/angiosarcoma_of_the_head_and_neck__a.10.aspx

  Given the wide differential diagnosis of AS, a high index of suspicion and the application of an appropriate panel of IHC markers that includes CD31, CD34, CK, EMA, S100 protein, Melan A, HMB45, CD30, CD45, and other relevant markers will aid in the diagnosis. […] Evidence of vasoformation is the sine quo none of these tumors, and a diligent search for their identification is necessary for any superficial tumor in the head and neck area of elderly patients.

• #34 Angiosarcoma | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/angiosarcoma?lang=us

  Angiosarcomas (like hemangiopericytomas and hemangioendotheliomas) are tumors that arise from vascular structures. They are typically difficult to distinguish from one another on imaging alone. […] Angiosarcomas are the most aggressive of the three, frequently having metastases at the time of diagnosis (and therefore carrying an extremely poor prognosis), and often recur locally. […] The malignant cells express morphological and functional properties of endothelial cells. The tumors can be multicentric with hemorrhage and necrosis being common.

• #35 Cutaneous Angiosarcoma: Clinical and Pathology Study of 16 Cases | Actas Dermo-Sifiliográficas

  https://actasdermo.org/en-cutaneous-angiosarcoma-clinical-pathology-study-articulo-S1578219017300926

  Tumor size is currently the most widely accepted prognostic marker for cutaneous angiosarcoma, and it has been frequently reported that angiosarcomas measuring 5cm or more have a worse prognosis than smaller tumors. […] Other potentially high-risk histologic factors proposed in the literature are presence of necrosis, an epithelioid morphology, and a greater depth of invasion. […] The treatment of choice of cutaneous angiosarcoma is surgical excision with wide margins followed by radiation therapy. […] Despite the small number of cases in our series of cutaneous angiosarcoma, which impeded us from performing statistical analyses, we observed that larger tumor size and older age were both associated with worse prognosis. A less evident association was also observed between poor prognosis and the following histologic features: presence of necrosis, a predominance of epithelioid cells, invasion of deeper layers, and a larger number of mitoses.

• #36 Clinical Pathophysiology and Research Highlights of Cardiac Angiosarcoma: Obligation for Immunogenetic Profiling to Understand Their Growth Pattern and Tailor Therapies

  https://www.mdpi.com/2673-3846/5/3/28

  The tumor growth and their aggressive behavior can be subdued with a drug regimen including monoclonal antibodies, tyrosine kinase inhibitors, RAF [rapidly accelerated fibrosarcoma] inhibitors, PKC [Protein Kinase C] inhibitors, and P13K [Phosphoinositide 3-kinases]/mTOR [mammalian target of rapamycin] inhibitors, either alone or in combination.

• #37 Targeting Angiosarcoma Vascular and Metabolic Signaling by Blocking Beta Adrenergic Receptor Signaling – SFA

  https://curesarcoma.org/funded-research/targeting-angiosarcoma-vascular-and-metabolic-signaling-by-blocking-beta-adrenergic-receptor-signaling/

  Angiosarcomas are highly lethal tumors composed primarily of aberrant lymphatic and/or vascular endothelial cells. […] The mechanism by which these drugs preferentially target angiosarcomas while sparing normal endothelial cells is not known. […] Our data suggest that ADRB antagonism disrupts angiogenic signaling by blocking vascular cascades important for newly developing vessels and tumor vasculature. […] Our results also suggest that angiosarcomas rewire their metabolism to support the high metabolic demands of oncogenic growth, and that ADRB antagonism disrupts this critical process. […] Therefore, we hypothesize that ADRB antagonists preferentially block angiosarcoma tumor growth through targeting aberrant angiogenic and metabolic processes, exploited by the tumors for their own growth advantage.

• #38 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. […] Angiosarcoma is an aggressive sarcoma type and can quickly spread to other parts of the body. […] In angiosarcoma, researchers have been learning more about treatments known as targeted therapies and immunotherapies. […] 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.

• #39

  https://www.jci.org/articles/view/139080

  Our results confirm the presence of several of these somatic alterations in angiosarcomas. […] We observed a subgroup of cutaneous head and neck angiosarcomas with higher TMB and dominant UV signatures. […] Collectively, these findings indicate a genomic heterogeneity of angiosarcomas, even within those of similar (head and neck) origins. […] Our transcriptomic profiling results identified several actionable protein kinases in angiosarcoma, the efficacy of which was confirmed in vitro. […] A dissection of the cases by etiology and anatomical location revealed interesting disease patterns. […] The clinical implications of such findings are interesting, given recent reports that both high TMB and TIS scores independently conferred higher response rates to immune checkpoint inhibitors across various tumor types.

• #40 Etiology, pathogenesis, and management of angiosarcoma associated with implants and foreign body: Clinical cases and re…

  https://ouci.dntb.gov.ua/en/works/9ZRb6Dv9/

  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. Further research into the underlying molecular mechanisms could provide valuable insights for tailored management and developing novel targeted therapeutic strategies.

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