One percent of all (STS) soft-tissue sarcomas, which are a rare form of cancer, are angiosarcomas (AS). As ‘high-grade’ cancers by description, they develop from vascular and lymphatic endothelial and exhibit physical aggression. Despite the fact that AS can affect any area of the body, it typically manifests as a cutaneous condition in elderly white males or on the rib cage after getting radiation treatment (RT) for breast carcinoma.
Particularly if AS is discovered in the metastatic phase, the prognosis is dismal, and the therapy is quite difficult. A multidisciplinary cancer board setting offers the greatest treatment options for AS patients. The greatest outcomes in terms of overall lifespan are provided by surgical excision with a negative boundary, just like with any other soft-tissue sarcomas.
It has been shown that combining radiation treatment (RT) with weekly paclitaxel results in long-lasting results for cutaneous angiosarcomas. For metastatic or advanced illness, doxorubicin & paclitaxel are advised treatment regimens. Targeted treatment against vascular endothelial growth factor (VEGF) has gained popularity because of the increased vasculature of AS, but it has yet to be demonstrated in a prospective research.
Epidemiology
STS make for 1 percent of all cancers in total. Up to 2 percent of all STS & 5 to 4 percent of all dermal STS are caused by AS. Similar to how the sexes are distributed, older white men are more likely to have AS.
Though it can appear in any body part, cutaneous AS most frequently affects the head & neck, especially the scalp. Due to its more endothelial nature, AS can sometimes develop in the heart and major blood arteries.
Anatomy
Pathophysiology
Etiology
Although it has been documented that AS can develop from a benign vascular tumor, it typically does so spontaneously. Many breast carcinoma survivors who get RT to the chest wall experience radiation-induced AS. However, it can happen in any area of the body that has previously received radiation.
As described above, Stewart-Treves syndrome is characterised as AS when persistent lymphedema is present. Cancer, treatment for cancer, & diseases like filariasis (Milroy’s illness) can all cause long – term lymphedema. The DNA repair mutations BRCA2 and BRCA1 have been linked in some published studies to AS.
AS is also linked to familial diseases such Maffuci syndrome, neurofibromatosis, & Klippel-Trenaunay disorder. AS is also linked to substances like thorium dioxide, vinyl chloride, radium, arsenic, and anabolic steroids.
Genetics
Prognostic Factors
About 35% of AS survive after five years. About 60 percent of patients survive at five years, even with locally advanced illness amenable to resection with negative margins (median survival of seven months).
Poor outcomes have often been demonstrated to be predicted by metastatic illness at presentation, advanced age, & poor performance condition.
Additionally, AS in unusual places like the heart and liver as well as AS brought on by radiation therapy, have very bad prognoses. According to several research, tumours larger than 5 cm had a worse prognosis.
Clinical History
Clinical History
Because angiosarcomas are sneaky, symptoms can not appear until the condition has progressed significantly. The history should concentrate on determining risk factors, although the majority of patients lack these characteristics.
The following are risk factors:
Exposure to toxins and radiation treatment
Additional carcinogens (e.g., Dacron, bone wax, metal bodies)
Lymphedema
Although angiosarcomas that originate at various places and in various organs have certain unique characteristics, their clinical symptoms are dependent on how much organ tissue has been replaced, as continues to follow:
Anemia, pathologic fractures, or liver dysfunction
Other inherently carcinogenic vascular proliferation traits (e.g., disseminated intravascular coagulation, bleeding, or thrombocytopenia)
Pain from compression of nearby neurovascular structures
Physical Examination
Physical Examination
The results of a physical examination are frequently uninteresting, but more subtle findings might offer hints for early diagnosis.
Soft tissue angiosarcoma
Soft tissue angiosarcoma patients typically have a moderately developing tumour in their limbs when they first arrive; the disease’s sometimes-quickly diagnosable rapid improvement. The retroperitoneum and abdominal lining may potentially produce soft tissue angiosarcoma. Because the belly may accommodate malignancies, retroperitoneal angiosarcoma typically present as asymptomatic lumps & typically expand to huge sizes. Patients may exhibit neurologic symptoms as a result of lumbar or pelvic nerve compression.
Anemia, hemothoraxes, persistent hematomas, hemorrhagic ascites, and GI bleeding are examples of the signs of recent haemorrhage and coagulopathy that are present in about 33percent of patients. The neighbouring nodes are frequently enlarged, & node metastasis can occur up to 45 percent of the time.
Bone angiosarcoma
Any part of the skeleton can be affected by angiosarcomas; however, 33 percent of these malignancies develop in the skeletal system, 33 percent in long tubular bones, and the remaining 46 percent in the tiny bones of the feet and hands. These tumours can be multicentric, affecting multiple bones in the same extremities, or multifocal, affecting the same bone with multiple lesions. Although pain is regularly felt and the area is usually tender, patients do not typically come with specific signs and symptoms. The presentation may occasionally include swelling and enlargement of the afflicted limb due to the participation of a superficial bone or soft tissue expansion. 10% of cases of fractures are pathologic.
Cutaneous angiosarcomas
The four identified cutaneous angiosarcomas subtypes are as follows:
Angiosarcomas of the face and scalp
In the setting of lymphedema, angiosarcoma
Angiosarcoma brought on by radiation.
Epithelioid angiosarcomas
Angiosarcomas of the face and scalp
The most typical type of angiosarcoma is this one. The condition, also known as senile angiosarcoma, Wilson-Jones angiosarcomas, and malignant angioendothelioma, mostly affects the head and neck and typically affects elderly people. The majority of patients arrive with an ulceration that has not healed, a blue-black nodule, or an expanding bruise. Initial misdiagnosis of these lesions as infection, cellulitis, bruising, or edema might cause a delay in treatment. There could be pain and bleeding. The lesions’ clinical pattern can be nodular, ulcerated, or diffuse.
Angiosarcoma of the skin that is connected to lymphedema
Six patients with lymphedema following a mastectomy were the first to be diagnosed with lymphedema-associated angiosarcoma (LAS). Angiosarcoma appeared in the ipsilateral arm several years following mastectomy in each patient. Later, LAS was described in the context of chronic idiopathic lymphedema, congenital lymphedema, and filarial lymphedema, as well as after axillary node removal for melanoma. In the five years following a mastectomy, there is a 5% chance of getting LAS. The medial aspect of the upper arm is the most typical place. A violaceous plaque or nodule on top of a bulky, nonpitting edema is how LAS manifests. Ulceration may spread quickly.
Radiation-induced angiosarcomas
4 to 40 years after radiation exposure, these lesions appear in the radiation field. Thorotrast, a radioactive radiography contrasting agent used in the U.s until the 1950s, may cause hepatic angiosarcoma many years after exposure.
Epithelioid angiosarcoma
This variation has an uncommon, recently characterized aggressive course. 2 to 3 years after presentation, death happens.
Age group
Associated comorbidity
Associated activity
Acuity of presentation
Differential Diagnoses
Differential Diagnoses
Unknown Primary Site Metastatic Carcinoma
Kaposi Sarcomas
Hepatic Hemangioma
Hemangioblastoma
Phyllodes malignancy (Cystosarcomas Phyllodes)
Laboratory Studies
Imaging Studies
Procedures
Histologic Findings
Staging
Treatment Paradigm
A multidisciplinary strategy is beneficial for every patient with STS. The same is true for AS. Depending on the stage of manifestation, there are many types of treatment for AS (metastatic Vs non-metastatic).
Unmetastatic illness
The preferred course of treatment is local operation with R0 excision. A worse outcome is conferred by R1 & R2 resection. Wide boundaries are suggested because of AS’s invasive tendency.
High-grade neoplasm is AS. So, peri-operative radiotherapy is always advised. Small retrospective investigations have shown that local control improves survival.
No study has demonstrated a definite advantage for chemoradiotherapy. Neoadjuvant cancer treatment, however, may be an option for patients whose tumours are big and where obtaining negative margins may be difficult. Small retrospective trials have demonstrated the value of adding weekly paclitaxel to RT in those with locally advanced cutaneous-AS to increase local control percentages & lengthen life.
The condition known as localized visceral angiosarcomas has a significant morbidity and fatality rate. Surgery, radiation, & chemotherapy must all be used in a multimodal strategy. Recent studies have shown the value & safety of treating cardiac angiosarcomas with proton beam treatment and chemotherapy.
Metastatic illness: Chemotherapy is used to treat metastatic illness. In order to treat metastatic illness, cytotoxic medicines, targeted treatment, & immune checkpoint blockers are being investigated.
cancer-specific chemotherapy – When a patient has any STS, anthracycline-based treatments are typically used as the first line of therapy. Paclitaxel, however, is an effective medication with response levels similar to anthracyclines in individuals with AS.
Anthracyclines – The number of responses in STS patients ranges from 16 to 36%. In most situations, ifosfamide is combined with another medicine, which undoubtedly enhances toxicity but may not improve the outcome. In individuals with AS, liposomal doxorubicin has also had therapeutic effects.
Taxanes – Taxanes are advantageous for AS patients because of their antiangiogenic characteristics. A small stage II prospective research (ANGIOTAX study) with an ORR of 17 percent was conducted after multiple retrospective research showing improvements in AS. Two out of the five individuals who had reactions, though, went into surgery and had a fully pathological reaction. Since then, numerous studies have supported the weekly paclitaxel’s efficacy in AS patients. 80mg/m2 of paclitaxel is given on days 1, 8, & 15 of a 28-day cycle.
In individuals with AS, other cytotoxic medications such as gemcitabine, ifosfamide, and cisplatin are also effective; however, they are typically not used as the initial line of therapy.
Targeted treatment
Anti-VEGF substance – Being a cancer of the vascular endothelial cells, AS has drawn a lot of interest in anti-VEGF treatment, notably bevacizumab. A prospective stage II research, however, came up empty-handed.
TKI (Tyrosine Kinase inhibitors) – Individuals with AS have undergone testing for a number of TKI, including axitinib, sorafenib, and regorafenib, and pazopanib. The medications mostly target VEGF or PDGFRA, which are important for the development of AS. Unfortunately, neither of these medicines demonstrated a favorable response, despite therapeutic activity in individuals with AS.
ICI (Immune checkpoint inhibitors)
Recent studies imply that ICI may be working in AS patients. This has not yet been put to the test in an upcoming study.
One percent of all (STS) soft-tissue sarcomas, which are a rare form of cancer, are angiosarcomas (AS). As ‘high-grade’ cancers by description, they develop from vascular and lymphatic endothelial and exhibit physical aggression. Despite the fact that AS can affect any area of the body, it typically manifests as a cutaneous condition in elderly white males or on the rib cage after getting radiation treatment (RT) for breast carcinoma.
Particularly if AS is discovered in the metastatic phase, the prognosis is dismal, and the therapy is quite difficult. A multidisciplinary cancer board setting offers the greatest treatment options for AS patients. The greatest outcomes in terms of overall lifespan are provided by surgical excision with a negative boundary, just like with any other soft-tissue sarcomas.
It has been shown that combining radiation treatment (RT) with weekly paclitaxel results in long-lasting results for cutaneous angiosarcomas. For metastatic or advanced illness, doxorubicin & paclitaxel are advised treatment regimens. Targeted treatment against vascular endothelial growth factor (VEGF) has gained popularity because of the increased vasculature of AS, but it has yet to be demonstrated in a prospective research.
STS make for 1 percent of all cancers in total. Up to 2 percent of all STS & 5 to 4 percent of all dermal STS are caused by AS. Similar to how the sexes are distributed, older white men are more likely to have AS.
Though it can appear in any body part, cutaneous AS most frequently affects the head & neck, especially the scalp. Due to its more endothelial nature, AS can sometimes develop in the heart and major blood arteries.
Although it has been documented that AS can develop from a benign vascular tumor, it typically does so spontaneously. Many breast carcinoma survivors who get RT to the chest wall experience radiation-induced AS. However, it can happen in any area of the body that has previously received radiation.
As described above, Stewart-Treves syndrome is characterised as AS when persistent lymphedema is present. Cancer, treatment for cancer, & diseases like filariasis (Milroy’s illness) can all cause long – term lymphedema. The DNA repair mutations BRCA2 and BRCA1 have been linked in some published studies to AS.
AS is also linked to familial diseases such Maffuci syndrome, neurofibromatosis, & Klippel-Trenaunay disorder. AS is also linked to substances like thorium dioxide, vinyl chloride, radium, arsenic, and anabolic steroids.
About 35% of AS survive after five years. About 60 percent of patients survive at five years, even with locally advanced illness amenable to resection with negative margins (median survival of seven months).
Poor outcomes have often been demonstrated to be predicted by metastatic illness at presentation, advanced age, & poor performance condition.
Additionally, AS in unusual places like the heart and liver as well as AS brought on by radiation therapy, have very bad prognoses. According to several research, tumours larger than 5 cm had a worse prognosis.
Clinical History
Because angiosarcomas are sneaky, symptoms can not appear until the condition has progressed significantly. The history should concentrate on determining risk factors, although the majority of patients lack these characteristics.
The following are risk factors:
Exposure to toxins and radiation treatment
Additional carcinogens (e.g., Dacron, bone wax, metal bodies)
Lymphedema
Although angiosarcomas that originate at various places and in various organs have certain unique characteristics, their clinical symptoms are dependent on how much organ tissue has been replaced, as continues to follow:
Anemia, pathologic fractures, or liver dysfunction
Other inherently carcinogenic vascular proliferation traits (e.g., disseminated intravascular coagulation, bleeding, or thrombocytopenia)
Pain from compression of nearby neurovascular structures
Physical Examination
The results of a physical examination are frequently uninteresting, but more subtle findings might offer hints for early diagnosis.
Soft tissue angiosarcoma
Soft tissue angiosarcoma patients typically have a moderately developing tumour in their limbs when they first arrive; the disease’s sometimes-quickly diagnosable rapid improvement. The retroperitoneum and abdominal lining may potentially produce soft tissue angiosarcoma. Because the belly may accommodate malignancies, retroperitoneal angiosarcoma typically present as asymptomatic lumps & typically expand to huge sizes. Patients may exhibit neurologic symptoms as a result of lumbar or pelvic nerve compression.
Anemia, hemothoraxes, persistent hematomas, hemorrhagic ascites, and GI bleeding are examples of the signs of recent haemorrhage and coagulopathy that are present in about 33percent of patients. The neighbouring nodes are frequently enlarged, & node metastasis can occur up to 45 percent of the time.
Bone angiosarcoma
Any part of the skeleton can be affected by angiosarcomas; however, 33 percent of these malignancies develop in the skeletal system, 33 percent in long tubular bones, and the remaining 46 percent in the tiny bones of the feet and hands. These tumours can be multicentric, affecting multiple bones in the same extremities, or multifocal, affecting the same bone with multiple lesions. Although pain is regularly felt and the area is usually tender, patients do not typically come with specific signs and symptoms. The presentation may occasionally include swelling and enlargement of the afflicted limb due to the participation of a superficial bone or soft tissue expansion. 10% of cases of fractures are pathologic.
Cutaneous angiosarcomas
The four identified cutaneous angiosarcomas subtypes are as follows:
Angiosarcomas of the face and scalp
In the setting of lymphedema, angiosarcoma
Angiosarcoma brought on by radiation.
Epithelioid angiosarcomas
Angiosarcomas of the face and scalp
The most typical type of angiosarcoma is this one. The condition, also known as senile angiosarcoma, Wilson-Jones angiosarcomas, and malignant angioendothelioma, mostly affects the head and neck and typically affects elderly people. The majority of patients arrive with an ulceration that has not healed, a blue-black nodule, or an expanding bruise. Initial misdiagnosis of these lesions as infection, cellulitis, bruising, or edema might cause a delay in treatment. There could be pain and bleeding. The lesions’ clinical pattern can be nodular, ulcerated, or diffuse.
Angiosarcoma of the skin that is connected to lymphedema
Six patients with lymphedema following a mastectomy were the first to be diagnosed with lymphedema-associated angiosarcoma (LAS). Angiosarcoma appeared in the ipsilateral arm several years following mastectomy in each patient. Later, LAS was described in the context of chronic idiopathic lymphedema, congenital lymphedema, and filarial lymphedema, as well as after axillary node removal for melanoma. In the five years following a mastectomy, there is a 5% chance of getting LAS. The medial aspect of the upper arm is the most typical place. A violaceous plaque or nodule on top of a bulky, nonpitting edema is how LAS manifests. Ulceration may spread quickly.
Radiation-induced angiosarcomas
4 to 40 years after radiation exposure, these lesions appear in the radiation field. Thorotrast, a radioactive radiography contrasting agent used in the U.s until the 1950s, may cause hepatic angiosarcoma many years after exposure.
Epithelioid angiosarcoma
This variation has an uncommon, recently characterized aggressive course. 2 to 3 years after presentation, death happens.
Differential Diagnoses
Unknown Primary Site Metastatic Carcinoma
Kaposi Sarcomas
Hepatic Hemangioma
Hemangioblastoma
Phyllodes malignancy (Cystosarcomas Phyllodes)
A multidisciplinary strategy is beneficial for every patient with STS. The same is true for AS. Depending on the stage of manifestation, there are many types of treatment for AS (metastatic Vs non-metastatic).
Unmetastatic illness
The preferred course of treatment is local operation with R0 excision. A worse outcome is conferred by R1 & R2 resection. Wide boundaries are suggested because of AS’s invasive tendency.
High-grade neoplasm is AS. So, peri-operative radiotherapy is always advised. Small retrospective investigations have shown that local control improves survival.
No study has demonstrated a definite advantage for chemoradiotherapy. Neoadjuvant cancer treatment, however, may be an option for patients whose tumours are big and where obtaining negative margins may be difficult. Small retrospective trials have demonstrated the value of adding weekly paclitaxel to RT in those with locally advanced cutaneous-AS to increase local control percentages & lengthen life.
The condition known as localized visceral angiosarcomas has a significant morbidity and fatality rate. Surgery, radiation, & chemotherapy must all be used in a multimodal strategy. Recent studies have shown the value & safety of treating cardiac angiosarcomas with proton beam treatment and chemotherapy.
Metastatic illness: Chemotherapy is used to treat metastatic illness. In order to treat metastatic illness, cytotoxic medicines, targeted treatment, & immune checkpoint blockers are being investigated.
cancer-specific chemotherapy – When a patient has any STS, anthracycline-based treatments are typically used as the first line of therapy. Paclitaxel, however, is an effective medication with response levels similar to anthracyclines in individuals with AS.
Anthracyclines – The number of responses in STS patients ranges from 16 to 36%. In most situations, ifosfamide is combined with another medicine, which undoubtedly enhances toxicity but may not improve the outcome. In individuals with AS, liposomal doxorubicin has also had therapeutic effects.
Taxanes – Taxanes are advantageous for AS patients because of their antiangiogenic characteristics. A small stage II prospective research (ANGIOTAX study) with an ORR of 17 percent was conducted after multiple retrospective research showing improvements in AS. Two out of the five individuals who had reactions, though, went into surgery and had a fully pathological reaction. Since then, numerous studies have supported the weekly paclitaxel’s efficacy in AS patients. 80mg/m2 of paclitaxel is given on days 1, 8, & 15 of a 28-day cycle.
In individuals with AS, other cytotoxic medications such as gemcitabine, ifosfamide, and cisplatin are also effective; however, they are typically not used as the initial line of therapy.
Targeted treatment
Anti-VEGF substance – Being a cancer of the vascular endothelial cells, AS has drawn a lot of interest in anti-VEGF treatment, notably bevacizumab. A prospective stage II research, however, came up empty-handed.
TKI (Tyrosine Kinase inhibitors) – Individuals with AS have undergone testing for a number of TKI, including axitinib, sorafenib, and regorafenib, and pazopanib. The medications mostly target VEGF or PDGFRA, which are important for the development of AS. Unfortunately, neither of these medicines demonstrated a favorable response, despite therapeutic activity in individuals with AS.
ICI (Immune checkpoint inhibitors)
Recent studies imply that ICI may be working in AS patients. This has not yet been put to the test in an upcoming study.
One percent of all (STS) soft-tissue sarcomas, which are a rare form of cancer, are angiosarcomas (AS). As ‘high-grade’ cancers by description, they develop from vascular and lymphatic endothelial and exhibit physical aggression. Despite the fact that AS can affect any area of the body, it typically manifests as a cutaneous condition in elderly white males or on the rib cage after getting radiation treatment (RT) for breast carcinoma.
Particularly if AS is discovered in the metastatic phase, the prognosis is dismal, and the therapy is quite difficult. A multidisciplinary cancer board setting offers the greatest treatment options for AS patients. The greatest outcomes in terms of overall lifespan are provided by surgical excision with a negative boundary, just like with any other soft-tissue sarcomas.
It has been shown that combining radiation treatment (RT) with weekly paclitaxel results in long-lasting results for cutaneous angiosarcomas. For metastatic or advanced illness, doxorubicin & paclitaxel are advised treatment regimens. Targeted treatment against vascular endothelial growth factor (VEGF) has gained popularity because of the increased vasculature of AS, but it has yet to be demonstrated in a prospective research.
STS make for 1 percent of all cancers in total. Up to 2 percent of all STS & 5 to 4 percent of all dermal STS are caused by AS. Similar to how the sexes are distributed, older white men are more likely to have AS.
Though it can appear in any body part, cutaneous AS most frequently affects the head & neck, especially the scalp. Due to its more endothelial nature, AS can sometimes develop in the heart and major blood arteries.
Although it has been documented that AS can develop from a benign vascular tumor, it typically does so spontaneously. Many breast carcinoma survivors who get RT to the chest wall experience radiation-induced AS. However, it can happen in any area of the body that has previously received radiation.
As described above, Stewart-Treves syndrome is characterised as AS when persistent lymphedema is present. Cancer, treatment for cancer, & diseases like filariasis (Milroy’s illness) can all cause long – term lymphedema. The DNA repair mutations BRCA2 and BRCA1 have been linked in some published studies to AS.
AS is also linked to familial diseases such Maffuci syndrome, neurofibromatosis, & Klippel-Trenaunay disorder. AS is also linked to substances like thorium dioxide, vinyl chloride, radium, arsenic, and anabolic steroids.
About 35% of AS survive after five years. About 60 percent of patients survive at five years, even with locally advanced illness amenable to resection with negative margins (median survival of seven months).
Poor outcomes have often been demonstrated to be predicted by metastatic illness at presentation, advanced age, & poor performance condition.
Additionally, AS in unusual places like the heart and liver as well as AS brought on by radiation therapy, have very bad prognoses. According to several research, tumours larger than 5 cm had a worse prognosis.
Clinical History
Because angiosarcomas are sneaky, symptoms can not appear until the condition has progressed significantly. The history should concentrate on determining risk factors, although the majority of patients lack these characteristics.
The following are risk factors:
Exposure to toxins and radiation treatment
Additional carcinogens (e.g., Dacron, bone wax, metal bodies)
Lymphedema
Although angiosarcomas that originate at various places and in various organs have certain unique characteristics, their clinical symptoms are dependent on how much organ tissue has been replaced, as continues to follow:
Anemia, pathologic fractures, or liver dysfunction
Other inherently carcinogenic vascular proliferation traits (e.g., disseminated intravascular coagulation, bleeding, or thrombocytopenia)
Pain from compression of nearby neurovascular structures
Physical Examination
The results of a physical examination are frequently uninteresting, but more subtle findings might offer hints for early diagnosis.
Soft tissue angiosarcoma
Soft tissue angiosarcoma patients typically have a moderately developing tumour in their limbs when they first arrive; the disease’s sometimes-quickly diagnosable rapid improvement. The retroperitoneum and abdominal lining may potentially produce soft tissue angiosarcoma. Because the belly may accommodate malignancies, retroperitoneal angiosarcoma typically present as asymptomatic lumps & typically expand to huge sizes. Patients may exhibit neurologic symptoms as a result of lumbar or pelvic nerve compression.
Anemia, hemothoraxes, persistent hematomas, hemorrhagic ascites, and GI bleeding are examples of the signs of recent haemorrhage and coagulopathy that are present in about 33percent of patients. The neighbouring nodes are frequently enlarged, & node metastasis can occur up to 45 percent of the time.
Bone angiosarcoma
Any part of the skeleton can be affected by angiosarcomas; however, 33 percent of these malignancies develop in the skeletal system, 33 percent in long tubular bones, and the remaining 46 percent in the tiny bones of the feet and hands. These tumours can be multicentric, affecting multiple bones in the same extremities, or multifocal, affecting the same bone with multiple lesions. Although pain is regularly felt and the area is usually tender, patients do not typically come with specific signs and symptoms. The presentation may occasionally include swelling and enlargement of the afflicted limb due to the participation of a superficial bone or soft tissue expansion. 10% of cases of fractures are pathologic.
Cutaneous angiosarcomas
The four identified cutaneous angiosarcomas subtypes are as follows:
Angiosarcomas of the face and scalp
In the setting of lymphedema, angiosarcoma
Angiosarcoma brought on by radiation.
Epithelioid angiosarcomas
Angiosarcomas of the face and scalp
The most typical type of angiosarcoma is this one. The condition, also known as senile angiosarcoma, Wilson-Jones angiosarcomas, and malignant angioendothelioma, mostly affects the head and neck and typically affects elderly people. The majority of patients arrive with an ulceration that has not healed, a blue-black nodule, or an expanding bruise. Initial misdiagnosis of these lesions as infection, cellulitis, bruising, or edema might cause a delay in treatment. There could be pain and bleeding. The lesions’ clinical pattern can be nodular, ulcerated, or diffuse.
Angiosarcoma of the skin that is connected to lymphedema
Six patients with lymphedema following a mastectomy were the first to be diagnosed with lymphedema-associated angiosarcoma (LAS). Angiosarcoma appeared in the ipsilateral arm several years following mastectomy in each patient. Later, LAS was described in the context of chronic idiopathic lymphedema, congenital lymphedema, and filarial lymphedema, as well as after axillary node removal for melanoma. In the five years following a mastectomy, there is a 5% chance of getting LAS. The medial aspect of the upper arm is the most typical place. A violaceous plaque or nodule on top of a bulky, nonpitting edema is how LAS manifests. Ulceration may spread quickly.
Radiation-induced angiosarcomas
4 to 40 years after radiation exposure, these lesions appear in the radiation field. Thorotrast, a radioactive radiography contrasting agent used in the U.s until the 1950s, may cause hepatic angiosarcoma many years after exposure.
Epithelioid angiosarcoma
This variation has an uncommon, recently characterized aggressive course. 2 to 3 years after presentation, death happens.
Differential Diagnoses
Unknown Primary Site Metastatic Carcinoma
Kaposi Sarcomas
Hepatic Hemangioma
Hemangioblastoma
Phyllodes malignancy (Cystosarcomas Phyllodes)
A multidisciplinary strategy is beneficial for every patient with STS. The same is true for AS. Depending on the stage of manifestation, there are many types of treatment for AS (metastatic Vs non-metastatic).
Unmetastatic illness
The preferred course of treatment is local operation with R0 excision. A worse outcome is conferred by R1 & R2 resection. Wide boundaries are suggested because of AS’s invasive tendency.
High-grade neoplasm is AS. So, peri-operative radiotherapy is always advised. Small retrospective investigations have shown that local control improves survival.
No study has demonstrated a definite advantage for chemoradiotherapy. Neoadjuvant cancer treatment, however, may be an option for patients whose tumours are big and where obtaining negative margins may be difficult. Small retrospective trials have demonstrated the value of adding weekly paclitaxel to RT in those with locally advanced cutaneous-AS to increase local control percentages & lengthen life.
The condition known as localized visceral angiosarcomas has a significant morbidity and fatality rate. Surgery, radiation, & chemotherapy must all be used in a multimodal strategy. Recent studies have shown the value & safety of treating cardiac angiosarcomas with proton beam treatment and chemotherapy.
Metastatic illness: Chemotherapy is used to treat metastatic illness. In order to treat metastatic illness, cytotoxic medicines, targeted treatment, & immune checkpoint blockers are being investigated.
cancer-specific chemotherapy – When a patient has any STS, anthracycline-based treatments are typically used as the first line of therapy. Paclitaxel, however, is an effective medication with response levels similar to anthracyclines in individuals with AS.
Anthracyclines – The number of responses in STS patients ranges from 16 to 36%. In most situations, ifosfamide is combined with another medicine, which undoubtedly enhances toxicity but may not improve the outcome. In individuals with AS, liposomal doxorubicin has also had therapeutic effects.
Taxanes – Taxanes are advantageous for AS patients because of their antiangiogenic characteristics. A small stage II prospective research (ANGIOTAX study) with an ORR of 17 percent was conducted after multiple retrospective research showing improvements in AS. Two out of the five individuals who had reactions, though, went into surgery and had a fully pathological reaction. Since then, numerous studies have supported the weekly paclitaxel’s efficacy in AS patients. 80mg/m2 of paclitaxel is given on days 1, 8, & 15 of a 28-day cycle.
In individuals with AS, other cytotoxic medications such as gemcitabine, ifosfamide, and cisplatin are also effective; however, they are typically not used as the initial line of therapy.
Targeted treatment
Anti-VEGF substance – Being a cancer of the vascular endothelial cells, AS has drawn a lot of interest in anti-VEGF treatment, notably bevacizumab. A prospective stage II research, however, came up empty-handed.
TKI (Tyrosine Kinase inhibitors) – Individuals with AS have undergone testing for a number of TKI, including axitinib, sorafenib, and regorafenib, and pazopanib. The medications mostly target VEGF or PDGFRA, which are important for the development of AS. Unfortunately, neither of these medicines demonstrated a favorable response, despite therapeutic activity in individuals with AS.
ICI (Immune checkpoint inhibitors)
Recent studies imply that ICI may be working in AS patients. This has not yet been put to the test in an upcoming study.
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