Angiosarcoma: A population-based cancer registry descriptive study of 45 consecutive cases diagnosed between 1979 and 2016

Colas, M.; Gérazime, Aurélie; Popescu, Dragos; Puzenat, E.; Chaigneau, L.; Woronoff, Anne Sophie; Dupond, A.-S.; Nardin, Charlée; Aubin, F.

doi:10.1177/2036361320979216

Cited by 10 publications

(5 citation statements)

References 10 publications

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“…A French registry study estimated the incidence of angiosarcoma to be 0.15 per 100 000 person-years but noted that the incidence between 2007 and 2016 was 0.26 per 100 000 person-years, higher than in the preceding decades. 8 A US-based registry study found a similar rate during that time period and also identified an increasing incidence over time specifically among older patients, most notably those older than 75 years. 9 Given the strong association of breast cancer treatment with the rare but serious later development of angiosarcoma, a previous study investigated the incidence of breast angiosarcoma in the US using the Surveillance, Epidemiology, and End Results (SEER) database across all ages and identified an increasing incidence of angiosarcoma among patients previously treated for breast cancer.…”

Section: Introductionmentioning

confidence: 79%

“…Despite this progress, the true incidence of angiosarcoma remains uncertain. A French registry study estimated the incidence of angiosarcoma to be 0.15 per 100 000 person-years but noted that the incidence between 2007 and 2016 was 0.26 per 100 000 person-years, higher than in the preceding decades . A US-based registry study found a similar rate during that time period and also identified an increasing incidence over time specifically among older patients, most notably those older than 75 years …”

Section: Introductionmentioning

confidence: 99%

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

Wagner,

Ravi,

Schaub

et al. 2024

JAMA Netw Open

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ImportanceAngiosarcoma 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.ObjectiveTo describe the incidence, presenting characteristics, and change over time of angiosarcoma in the US.Design, Setting, and ParticipantsThis 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.Main Outcomes and MeasuresIncidence of angiosarcoma, demographics of patients with angiosarcoma, and extent of disease at presentation.ResultsThe 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).Conclusions and RelevanceThis 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.

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Section: Introductionmentioning

confidence: 79%

Section: Introductionmentioning

confidence: 99%

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

Wagner,

Ravi,

Schaub

et al. 2024

JAMA Netw Open

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“…It mainly affects the proximal jejunum and the ileum. The most common clinical presentation is abdominal pain, obscure or occult gastrointestinal bleeding, anemia, and other nonspecific manifestations such as nausea, vomiting, and weight loss [ 7 ]. Acute presentation with gastrointestinal bleeding or melena is not uncommon, requiring treatment by blood transfusions (the typical presentation of our case report) [ 3 ].…”

Section: Discussionmentioning

confidence: 99%

Diagnostic and Therapeutic Benefits of Intra-operative Enteroscopy in Epithelioid Angiosarcoma of the Small Intestine

Abdulfattah¹

2023

Cureus

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Intestinal epithelioid angiosarcoma is an uncommon type of vascular tumor with unusually varied clinical presentations, including non-explained severe gastrointestinal bleeding, anemia, and abdominal pain. A 77year-old man presented with recurrent severe anemia caused by gastrointestinal bleeding. The diagnosis was jejunal angiosarcoma. The patient had three intestinal resections assisted by explorative intraoperative enteroscopy, which detected multifocal intestinal lesions. Intraoperative enteroscopy is a helpful diagnostic and prognostic approach that detects small intestinal tumors. Intraoperative enteroscopy could improve the outcome of intestinal angiosarcomas.

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“…Angiosarcoma is a rare malignancy involving lymphatic or vascular endothelial cells that represents 1% of all soft tissue sarcomas [ 1 ]. It has an estimated incidence of about one to two cases per million [ 2 ]. These highly aggressive tumors can arise anywhere in the body, although the most common sites are cutaneous lesions of the head and neck and occasional involvement of the spleen, liver, bone, and other visceral organs.…”

Section: Introductionmentioning

confidence: 99%

Primary Epithelioid Angiosarcoma of the Colon With Peritoneal Carcinomatosis

Do¹,

Farooqui²,

Parajuli³

2023

Cureus

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Angiosarcoma is a rare mesenchymal tissue neoplasm, typically involving lymphatic or vascular endothelial cells. The tumor can arise anywhere in the body, though it is most often found as cutaneous lesions in the head and neck region. Due to its rarity, a diagnosis can sometimes be missed, especially when the sarcoma involves an uncommon site like the gastrointestinal tract. In this case, we describe a male patient who was found to have primary epithelioid angiosarcoma of the colon. Initial biopsies with immunohistochemistry staining were weakly positive for anti-cytokeratin (CAM 5.2) and negative for SRY-Box transcription factor 10 (SOX-10) and B-cell-specific activator protein (PAX-5). He was misdiagnosed as having poorly differentiated carcinoma as a result. However, a more in-depth look at the colon specimen after tumor resection revealed CD-31 and factor VIII positivity, which established the diagnosis of epithelioid angiosarcoma of the colon. This case suggests the use of rare histopathology markers as part of the workup for colonic lesions to confirm the diagnosis, especially when tissue biopsy is limited.

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Angiosarcoma: A population-based cancer registry descriptive study of 45 consecutive cases diagnosed between 1979 and 2016

Cited by 10 publications

References 10 publications

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

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

Diagnostic and Therapeutic Benefits of Intra-operative Enteroscopy in Epithelioid Angiosarcoma of the Small Intestine

Primary Epithelioid Angiosarcoma of the Colon With Peritoneal Carcinomatosis

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