Cutaneous Angiosarcoma Research Articles

Facial angiosarcoma: A unique, non-purpuric presentation with good prognosis and unconventional management.

Cutaneous angiosarcoma is a rare, aggressive malignant neoplasm of vascular endothelial cell origin with a poor prognosis. The typical presentation of cutaneous angiosarcoma involves a progressive, bruise-like lesion on the head or neck.1 We present an atypical case of facial angiosarcoma in a 79-year-old white male arising as an asymmetric, non-purpuric swelling of the left nasal tip and ala, resembling an asymmetric rhinophyma. Several studies comparing face and scalp angiosarcomas have shown improved prognosis when angiosarcoma occurs on the face.2 Our case supports this trend noted in the literature. Angiosarcoma is typically treated with multiple modalities including surgery, radiotherapy, and chemotherapy. Our patient’s angiosarcoma was controlled with debulking surgery, radiation therapy, and Pembrolizumab (off-label use of immunotherapy). Patient is doing well 4 years later and will resume surgery or radiation if needed for any recurrence.

1734P Evaluation of nivolumab in cutaneous angiosarcoma management: The ANGIOCHECK study

1734P Evaluation of nivolumab in cutaneous angiosarcoma management: The ANGIOCHECK study

PD-L1 expression in cutaneous angiosarcoma : Systematic review with meta-anakysis

PD-L1 expression in cutaneous angiosarcoma : Systematic review with meta-anakysis

A commentary on 'Electrochemotherapy for advanced cutaneous angiosarcoma: A European register-based cohort study from the International Network for Sharing Practices of electrochemotherapy (InspECT)'.

A commentary on 'Electrochemotherapy for advanced cutaneous angiosarcoma: A European register-based cohort study from the International Network for Sharing Practices of electrochemotherapy (InspECT)'.

Cutaneous angiosarcoma arising over rhinophyma: an unusual variant of lymphoedema-associated angiosarcoma?

Cutaneous angiosarcoma is a rare tumour with poor prognosis. When arising on phymatous nose its diagnosis can be very deceptive and lead to delayed treatment as well as metastatic dissemination. Our case report highlights that some red flag as oedema, ulceration, unresponsiveness to conventional rosacea therapies or bluish vascular structures at dermoscopy should prompt biopsy to rule out malignancy.

Epithelioid Angiosarcoma: A Diagnostic Dilemma with Clinically and Histopathologically Overlapping Features

Cutaneous angiosarcoma is a rare malignancy of endothelial cell origin that commonly presents in the head and neck region of elderly individuals and is known for high rates of lymph node metastasis and mortality. There is significant variability in the histopathologic features, and immunohistochemistry is necessary to help differentiate epithelioid angiosarcoma from lesions it may clinically mimic. Here, we report a rare case of poorly differentiated epithelioid angiosarcoma on the trunk initially diagnosed as poorly differentiated squamous cell carcinoma (SCC) on shave biopsy. A 56-year-old man presented for a full-body skin examination significant for a 5.6 x 2.8 mm brown-black macule on the left superomedial posterior trunk and a 5.0 x 2.7 mm pink, ulcerated papule on the right inferior posterior trunk. On histopathology, one lesion was found to be melanoma in situ, and the other showed ulcerated and infiltrative, poorly differentiated SCC. Post-excision histologic analysis of the right inferior back lesion revealed tumor cells immunoreactive to vimentin, ERG, and CD31 (PECAM-1). These findings suggested that the lesion previously diagnosed as SCC was an invasive, poorly differentiated epithelioid angiosarcoma. The patient underwent 40 sessions of radiotherapy. Epithelioid angiosarcoma occurring outside of the conventional epidemiologic profile is believed to portend higher mortality rates. This case highlights the importance of thorough clinical evaluation and appropriate histopathologic diagnosis of an aggressive neoplasm unlikely to present in this location. Further studies are required to explore potential risk factors, effective treatments, and long-term outcomes in patients with epithelioid angiosarcoma.

Adolescent with multiple benign skin lesions, cutaneous angiosarcoma and multiple thyroid nodules. what is the diagnosis?

Adolescent with multiple benign skin lesions, cutaneous angiosarcoma and multiple thyroid nodules. what is the diagnosis?

An Update on Cutaneous Angiosarcoma Diagnosis and Treatment.

Cutaneous angiosarcoma (CAS) is a rare aggressive malignancy that most commonly manifests in White men older than 60 years and often appears as an enlarging ecchymosis on the head, neck, or scalp. Surgery with negative margins is the first-line treatment. The role of Mohs micrographic surgery (MMS) is uncertain but can be used in smaller, well-circumscribed lesions on the head and neck. The greatest impact that dermatologists can have in the management of CAS is through a thorough total-body skin examination and heightened awareness resulting in a shortened time to diagnosis. Until quality evidence allows for the creation of consensus guidelines, multidisciplinary care at a cancer center that specializes in rare difficult-to-treat tumors is essential in optimizing patient outcomes.

Incidence and Presenting Characteristics of Angiosarcoma in the US, 2001-2020

Angiosarcoma is an aggressive vascular malignant neoplasm presenting either as a primary or secondary cancer, often arising after radiotherapy or in the context of preexisting lymphedema. Comprehensive data describing its incidence and presentation patterns are needed. To describe the incidence, presenting characteristics, and change over time of angiosarcoma in the US. This retrospective cross-sectional study used data from the US Cancer Statistics (USCS) National Program of Cancer Registries-Surveillance, Epidemiology, and End Results Combined Database, which captures more than 99% of newly diagnosed cancers in the US. The study included all 19 289 patients in the US with a new diagnosis of angiosarcoma between 2001 and 2020 captured in the USCS database. Statistical analysis was performed from June to September 2023. Incidence of angiosarcoma, demographics of patients with angiosarcoma, and extent of disease at presentation. The study included 19 289 patients (median age, 71 years [IQR, 59-80 years]; 10 506 women [54.5%]) with a new diagnosis of angiosarcoma. The US incidence of angiosarcoma doubled between 2001 (657 cases) and 2019 (1312 cases), reflecting both an increase in the adjusted incidence rate of 1.6% per year (P = .001), to 3.3 cases per 1 000 000 person-years (95% CI, 3.1-3.5 cases per 1 000 000 person-years), and an increase in the population at risk. In 2020, the reported incidence rate (3.0 cases per 1 000 000 person-years) and cases of angiosarcoma (n = 1159) were modestly lower than in 2019. Overall, 72.3% of cases of angiosarcoma (n = 13 955) were cutaneous, subcutaneous, or breast angiosarcomas; 24.4% were visceral (n = 4701); and 3.3% were located in unknown or rare primary sites (n = 633). Secondary breast and chest wall angiosarcomas among women represented the largest contribution to increasing incidence. Among breast angiosarcomas, 99.2% (2684 of 2705) were in women and 71.9% (1944 of 2705) were secondary. A total of 80.4% of chest wall or thorax cases among women (1861 of 2316) were secondary vs 26.5% among men (112 of 422), and 63.9% of upper extremity cases among women (205 of 321) were secondary vs 26.8% (56 of 209) among men (P = .001). Rates of secondary angiosarcoma in the abdomen and lower extremities were similar between men and women. The incidence rate of visceral angiosarcoma was also found to be increasing (1.5% per year; P = .001). This cross-sectional study describes angiosarcoma presentation patterns and incidence rates in the US over a 20-year period and shows that the number of cases in men and women increased, with the greatest increase among women with secondary angiosarcoma of the chest, breast, and upper extremity. These data increase awareness of a rare but highly morbid disease and highlight the need for improved early detection of angiosarcoma among patients at high risk, such as women with a history of breast cancer.

Functional and molecular 3D mapping of angiosarcoma tumor using non-invasive laser speckle, hyperspectral, and photoacoustic imaging

ABSTRACT Purpose The gold standard for skin cancer diagnosis is surgical excisional biopsy and histopathological examination. Several non-invasive diagnostic techniques exist, although they have not yet translated into clinical use. This is a proof-of-concept study to assess the possibility of imaging an angiosarcoma in the periocular area. Methods We use laser speckle, hyperspectral, and photoacoustic imaging to monitor blood perfusion and oxygen saturation, as well as the molecular composition of the tissue. The information obtained from each imaging modality was combined in order to yield a more comprehensive picture of the function, as well as molecular composition of a rapidly growing cutaneous angiosarcoma in the periocular area. Results We found an increase in perfusion coupled with a reduction in oxygen saturation in the angiosarcoma. We could also extract the molecular composition of the angiosarcoma at a depth, depicting both the oxygen saturation and highlighting the presence of connective tissue via collagen. Conclusions We demonstrate the different physiological parameters that can be obtained with the different techniques and how these can be combined to provide detailed 3D maps of the functional and molecular properties of tumors useful in preoperative assessment.

Cutaneous Angiosarcoma Subtypes: A Quantitative Systematic Review of Demographics, Treatments, and Outcomes Within Published Patient-Level Cases.

Cutaneous angiosarcoma (cAS) is a highly aggressive malignancy arising from the vascular endothelium. Given its rarity, there is insufficient data detailing patient demographics, management, and survival outcomes. To systematically compile published patient-level cases of cAS and to quantify and analyze data on demographics, management, and outcomes while determining prognostic indicators. Searches of EBSCOhost, MEDLINE, EMBASE, and the Cochrane Library generated 1,500 cases of cAS with individual level data available. PRISMA guidelines were followed. Cutaneous angiosarcoma presented most often on the scalp of elderly men. Metastasis occurred in 36.3% of cases. Aggregate 5-year survival was 31.6% with the median survival of 25 months. The best 5-year survival was in the radiation-associated subtype (48.8%), whereas the worst was in the Stewart-Treves subtype (21.6%). Using multivariate analysis, gender, age group, disease subtype, treatment modality, and metastasis at presentation had significant effects on survival outcomes ( p < .05). The breadth of information obtained enables this study to serve as a resource that clinicians may reference when they encounter cAS.

Ambient UV radiation is associated with cutaneous angiosarcoma incidence in the United States, 1992 to 2020

Ambient UV radiation is associated with cutaneous angiosarcoma incidence in the United States, 1992 to 2020

Two cases of cutaneous angiosarcoma: diagnostic challenges on small or superficial biopsies

Two cases of cutaneous angiosarcoma: diagnostic challenges on small or superficial biopsies

Clinical utility of comprehensive genomic profiling for skin cancer: A cohort study of 522 cases in a Japanese real-world setting

BackgroundAdvanced cases of skin cancer sometimes represent a clinical challenge to treat due to limited approved treatments. Comprehensive genomic profiling (CGP) tests are indicated for metastatic or unresectable cases; however, their results and clinical utility in skin cancer remain unclear. MethodsThis is a retrospective cohort study analyzing the CGP test results of 522 cases of skin cancer submitted between June 2019 and November 2022 using a nationwide database recording clinical data and CGP test results in Japan. Results522 cases were registered. In cancer types, the most prevalent was cutaneous melanoma (CM, n=235), followed by cutaneous squamous cell carcinoma (cSCC, n=91), Extramammary Paget’s disease (EMPD, n=75), adnexal carcinoma (AC, n=58), cutaneous angiosarcoma (cAS, n=33), basal cell carcinoma (BCC, n=15), Merkel cell carcinoma (n=10) and dermatofibrosarcoma protuberans (n=5). The median TMB was highest in EMPD and cAS (5.0 mutations/Mb), whereas high TMB cases (≥10.0 mutations/Mb) were most prevalent in BCC (33 %), followed by cSCC (26 %). No cases were classified as MSI-high. NTRK1/2/3 fusions were identified in two cases: one CM and one AC. BRAF V600E mutations in non-melanoma skin cancer were observed in one case of AC. Actionable genetic alterations eligible for ongoing clinical trials utilizing unapproved drugs were found most frequently in EMPD (51 %), followed by BRAF V600E/K-negative CM (44 %). ConclusionsThis study provides an overview of CGP test results and identifies potential therapeutic targets for each type of skin cancer. These findings improve our understanding of CGP test outcomes and advance tailored therapies for skin cancer.

Misdiagnosis of Cutaneous Epithelioid Angiosarcoma as Diabetic Foot Ulcer: A Case Study

Background. AS is a malignant tumor that originates from vascular endothelial cells and is known for a high rate of local recurrence and metastasis. Case Report. A 48-year-old male presented with cutaneous epithelioid AS. Cutaneous AS of the foot is quite rare, especially in the absence of predisposing factors, and in this patient it was previously misdiagnosed as a DFU. Conclusion. Physicians should be aware of this rare presentation of cutaneous AS. The authors of the current report advise regular clinical reassessment of chronic ulcers and biopsies of nonhealing wounds, even when adequate wound treatment has been administered, with the goal of identifying ulcerated skin malignancies and preventing delay in providing appropriate treatment.

A Clinicopathologic Feature and Survival Analysis of Cutaneous Angiosarcoma: A Single Tertiary Center Study

A Clinicopathologic Feature and Survival Analysis of Cutaneous Angiosarcoma: A Single Tertiary Center Study

A case of facial angiosarcoma mimicking deep fungal infection

Cutaneous angiosarcoma is a rare, yet a highly aggressive skin and soft tissue tumor with a predilection to the head and neck region in white-skinned elderly males1.Cutaneous angiosarcomas have varied clinical presentations and could mimic several clinical entities making the diagnosis a challenge. Upon clinical suspicion, diagnosis is confirmed with histopathology examination and immunohistochemistry. Early diagnosis improves prognosis. We report a case of cutaneous angiosarcoma mimicking facial deep fungal infection.

Angiosarcoma of the scalp successfully treated with Nivolumab—A case report

AbstractAngiosarcomas are rare, aggressive tumours with a propensity for the head and neck region. Surgery is the mainstay of treatment of localised angiosarcoma, but in unresectable or metastatic disease, the treatment is much more challenging. Traditionally, chemotherapy (Paclitaxel in particular) has been the treatment of choice. Evidence from case reports and small trials shows that PD1 inhibitors have activity against angiosarcomas, despite the lack of a large clinical trial data demonstrating definitive efficacy. The present case exemplifies the susceptibility of this malignancy to anti‐PD1 agents. We report a 77‐year‐old male patient with an advanced, multifocal angiosarcoma of the frontotemporal and malar region. The patient was pretreated using the association of Propranolol and Endoxan and then Paclitaxel. Because of the lack of efficacy, the patient was started on Nivolumab as the third line of therapy. After five cycles of immunotherapy, an excellent response was achieved, with complete remission of the cutaneous lesions as well as a partial remission of the hepatic nodules. The duration of response is currently 14 months, the patient being still alive at the present time. This case suggests that immune checkpoint inhibitors may represent a viable therapeutic option that needs further exploration for the treatment of locally advanced, unresectable or metastatic cases of cutaneous angiosarcoma.

Genomic Landscape Comparison of Cardiac versus Extra-Cardiac Angiosarcomas.

Angiosarcomas (ASs) are rare malignant vascular entities that can affect several regions in our body, including the heart. Cardiac ASs comprise 25-40% of cardiac sarcomas and can cause death within months of diagnosis. Thus, our aim was to identify potential differences and/or similarities between cardiac and extra-cardiac ASs to enhance targeted therapies and, consequently, patients' prognosis. Whole-transcriptome analysis of three cardiac and eleven extra-cardiac non-cutaneous samples was performed to investigate differential gene expression and mutational events between the two groups. The gene signature of cardiac and extra-cardiac non-cutaneous ASs was also compared to that of cutaneous angiosarcomas (n = 9). H/N/K-RAS and TP53 alterations were more recurrent in extra-cardiac ASs, while POTE-gene family overexpression was peculiar to cardiac ASs. Additionally, in vitro functional analyses showed that POTEH upregulation conferred a growth advantage to recipient cells, partly supporting the cardiac AS aggressive phenotype and patients' scarce survival rate. These features should be considered when investigating alternative treatments.

Translated article] A Rare Case of Cutaneous Angiosarcoma Secondary to Chronic Lymphedema in a Patient on Hemodialysis

Translated article] A Rare Case of Cutaneous Angiosarcoma Secondary to Chronic Lymphedema in a Patient on Hemodialysis