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Representative microscopic images of the appearance of angiosarcomas. Low grade angiosarcoma demonstrated (A) irregular, anastomosing vascular channels (hematoxylin and eosin staining, original magnification, x200). (B) The ramifying channels were lined with atypical endothelial cells (hematoxylin and eosin staining, original magnification, x400). (C) High grade angiosarcoma exhibiting proliferation of high grade spindle tumor cells without clear vasoformation (hematoxylin and eosin staining, original magnification, x400). (D) High grade angiosarcoma with epithelioid appearance with abundant amphophilic to lightly eosinophilic cytoplasm (hematoxylin and eosin staining, original magnification, x400).
Source publication
Angiosarcoma is a rare soft tissue sarcoma, and the data about its clinicopathological features and prognostic factors are limited. The purpose of the present study was to report a large series of angiosarcoma at a single institution. Clinical data from 200 cases of angiosarcoma from the Shanghai Cancer Center (Shanghai, China) between March 2006 a...
Contexts in source publication
Context 1
... recorded in 107 cases (mean, 5.2 cm; median, 4.5 cm; range, 0.5-15 cm). According to the degree of vascular differentiation, cellular pleomorphism and mitotic activity, all cases were divided into low grade, and high grade (Table I). In low-grade cases, tumors contained irregular, complex anastomosing channels lined by atypical endothelial cells (Fig. 1A and B). In poorly differentiated areas, the high grade spindled tumor cells formed continuous sheets lacking vasoformation (Fig. 1C). In the present study, 37 cases (18.5%, 37/200) exhibited an epithelioid appearance, and tumors were composed of epithelioid endothelial cells, which were classified as 'epithelioid angiosarcoma' and included in ...
Context 2
... pleomorphism and mitotic activity, all cases were divided into low grade, and high grade (Table I). In low-grade cases, tumors contained irregular, complex anastomosing channels lined by atypical endothelial cells (Fig. 1A and B). In poorly differentiated areas, the high grade spindled tumor cells formed continuous sheets lacking vasoformation (Fig. 1C). In the present study, 37 cases (18.5%, 37/200) exhibited an epithelioid appearance, and tumors were composed of epithelioid endothelial cells, which were classified as 'epithelioid angiosarcoma' and included in the high-grade group (Fig. 1D). However, the epithelioid appearance was not observed in the angiosarcoma of breast, spleen, ...
Context 3
... differentiated areas, the high grade spindled tumor cells formed continuous sheets lacking vasoformation (Fig. 1C). In the present study, 37 cases (18.5%, 37/200) exhibited an epithelioid appearance, and tumors were composed of epithelioid endothelial cells, which were classified as 'epithelioid angiosarcoma' and included in the high-grade group (Fig. 1D). However, the epithelioid appearance was not observed in the angiosarcoma of breast, spleen, liver and heart (Table I). Immunohistochemistry is important in confirming the diagnosis of angiosarcoma, particularly for poorly differ- entiated cases. CD31 protein was detected in 105/110 tested cases (95.5%) ( Fig. 2A and B). CD34 staining ...
Citations
... 1 Most angiosarcoma cases, when diagnosed, are advanced and unresectable; chemotherapy and/or radiotherapy are the mainstream treatments. [2][3][4][5] Hence, early detection and complete tumor resection are essential for the long-term survival of patients with an angiosarcoma. ...
... A primary angiosarcoma of the thorax is extremely rare, and there have been no previous reports of its origin from the azygos vein. [1][2][3][4][5] In the present case, it was difficult to visualize the distal end of the azygos vein through a median sternotomy, even if thoracoscopy was used, because of the presence of the tumor itself. In rationale, VATS in the lateral position allows visualization more clearly with an easier and safer resection and is useful for the safe and reliable complete resection of a primary tumor arising from the azygos vein. ...
A 63‐year‐old woman was admitted to our department for the investigation of superior vena cava (SVC) syndrome. Computed tomography revealed an azygos tumor extending into the SVC. Video‐assisted thoracic surgery (VATS) was performed to remove the distal end of the azygos vein in the left lateral position, followed by complete resection of the entire tumor under median sternotomy in the supine position. The histological diagnosis was a primary angiosarcoma of the azygos vein. The patient was discharged without any complications and is now alive and tumor‐free 24 months after surgery. In addition, contrast‐enhanced computed tomography revealed no graft occlusion in the two reconstructed brachiocephalic veins. Thoracoscopic surgery in the lateral position is useful for safe and reliable complete resection of a tumor arising from the azygos vein.
... In a later cohort study, seven soft-tissue sarcomas were found, and sarcoma risk was higher for the MoM cohort than the non-MoM cohort (16). An angiosarcoma (AS) registry study (22) and cohort studies involving AS patients (23,24) found no associations between AS and orthopedic implants. ...
Total hip arthroplasty (THA) is a common treatment for osteoarthritis and is also
performed for other conditions, such as secondary arthritis due to
developmental dysplasia of the hip. Various THA types may be complicated by
osteolysis and an inflammatory pseudotumor due to an adverse reaction to
metal debris. Rarely, THA has been associated with malignant tumors, but their
causality remains unclear. In this case report, we describe a female patient with
developmental dysplasia of the hip. She had undergone left metal-onpolyethylene THA, acetabular revision of the THA, and left total knee
arthroplasty. In addition, she had a history of dyslipidemia and telangiectasia of
the eyes, anemia, hiatal hernia, and pleuritis. A THA-associated mass (suspected
to be a pseudotumor) had been detected during a previous hospital admission
due to pleuritis. She was hospitalized due to swelling in her left lower limb,
fatigue, and bruises. A clinical examination revealed anemia, thrombocytopenia,
and growth of the suspected pseudotumor. Within 6 weeks, she presented with
bleeding of the oral mucosa, hemoptysis, melena, severe thrombocytopenia that
did not respond to treatment, elevated D-dimer and C-reactive protein levels,
severe pain, increased osteolysis, and fractures around the THA. Infection or
malignancy was suspected, but two trocar biopsies suggested an inflammatory
pseudotumor. Since her anemia and thrombocytopenia were considered to
have been caused by an inflammatory process within the suspected
pseudotumor, her suspected pseudotumor and all THA components were
surgically removed. However, she developed severe alveolar hemorrhaging and
hypoxia and died 2 weeks after her surgery. Histopathological analysis of her
surgical and autopsy samples revealed highly malignant angiosarcoma. Although
individual cases of malignancies associated with THA have been reported, the
literature lacks a clear association between THA and increased cancer risk. Most
pseudotumors are non-malignant. The patient’s case presented in this report
exemplifies the challenges to the differential diagnosis of a THA-associated
pseudotumor and rare angiosarcoma. Atypically rapid tumor growth, severe
osteolysis, and deterioration in the general wellbeing suggest a malignant disease.
... Finally, angiosarcoma is often diagnosed at an advanced stage, and it is associated with a poor prognosis. The reported 5-year survival rates range from 12% to 35% [26]. Outcomes vary considerably and are dependent on the site, size, resectability, tumor type, and presence of metastases. ...
... Soft tissue sarcomas are a heterogeneous group of cancers that represent <1 % of all adult neoplasms [1]. Angiosarcoma is a very rare but highly aggressive malignant vascular tumor [2][3][4][5] accounting for 1 % to 4 % of all soft tissue sarcomas [2][3][4][5][6]. Localized angiosarcoma of the abdominal wall is so infrequent that only few anecdotal cases were reported in the literature [3,[7][8][9][10][11][12][13][14]. ...
... Soft tissue sarcomas represent respectively 0.72 % and 0.49 % of all expected cancers in United States [1] and Canada [17]. Angiosarcomas account for only 1-4 % of all soft tissue sarcomas [2][3][4][5][6]. The cutaneous form of angiosarcomas is the most common, and represents about half of all tumors, with the head and neck being the most frequently involved region [2,[5][6][7], followed by the extremities and the breast [7]. ...
... Angiosarcomas account for only 1-4 % of all soft tissue sarcomas [2][3][4][5][6]. The cutaneous form of angiosarcomas is the most common, and represents about half of all tumors, with the head and neck being the most frequently involved region [2,[5][6][7], followed by the extremities and the breast [7]. Other documented sarcomas of the abdominal wall include undifferentiated pleomorphic sarcomas, fibrosarcomas, and synovial sarcomas [4]. ...
Introduction
Angiosarcoma is a very rare but highly aggressive malignant vascular tumor. Sporadic cases that develop in the abdominal wall are almost exclusively observed in obese patients. The underlying causes remain unclear. Liraglutide, a glucagon-like peptid-1 agonist receptor treatment, effective for weight management and glycemic control in type 2 diabetes, is not known to be associated with the occurrence of angiosarcoma.
Case presentation
A 62-year-old woman developed a very aggressive, rapidly recurrent angiosarcoma of the abdominal wall. The angiosarcoma developed while she was taking liraglutide injections. The patient died within few months.
Discussion
Angiosarcoma, particularly of the abdominal wall, is very rare, and occurs mainly in obese patients. This cancer had a highly aggressive behavior. The association with liraglutide and angiosarcoma cannot be established nor eliminated from this case and literature review.
Conclusion
Angiosarcoma is a rare and highly aggressive cancer that occasionally originates in the abdominal wall. In this case, in addition to the underlying obesity, a possible association of her liraglutide subcutaneous injections cannot be excluded. In the future, if other cases of abdominal walls angiosarcomas associated with liraglutide subcutaneous injections were to be reported, a possible causality should be further investigated.
... Angiosarcoma is an aggressive neoplasm of the endothelial cells of blood or lymphatic vessels with a 5-year overall survival of 41-43%, affecting patients mostly in their sixth decade of life (1)(2)(3). It is a rare malignancy comprising only 4% of all soft tissue sarcomas (3,4). ...
... It is a rare malignancy comprising only 4% of all soft tissue sarcomas (3,4). These tumors can occur at any anatomical location but are most commonly found in the head, neck skin, and breast regions of the body (1). Risk factors for angiosarcoma include radiation, chronic lymphedema, radiotherapy, certain chemical exposure, and immunosuppression (5). ...
... Due to the rarity of angiosarcoma, the sample sizes of large case series studies remain small with only around 200 patients reported (1,2). Operation, radiotherapy, and chemotherapy are the main treatments for this disease, with the core treatment being surgical resection if possible (6). ...
Objective
Angiosarcoma is a rare malignant vascular tumor, and the management and outcome of this disease are not well-described. The aim of this study was to report the incidence, patient demographics, and outcomes of angiosarcoma based on national data.
Methods
Data on patients with angiosarcoma were obtained from the Surveillance, Epidemiology, and End Results (SEER) database. Inverse probability treatment weights (IPTW) were used to assess the survival benefit of operation with additional chemo or radiation therapy compared to operation alone. These variables were further compared against patients who did not receive an operation despite being initially offered one. Cox regression was used to assess survival. Statistical analyses were performed on RStudio.
Results
For this study, 5,135 patients (46% men; median age 69, range 0–102) with angiosarcoma were identified in the SEER database between 1975 and 2016. The age-adjusted incidence rate was 1–4%. Patients were mostly non-Hispanic Caucasian (75.4%). The average tumor size was 4.7 cm, range (.1–98.9). Tumor grades were high at presentation (Grade III 17.2, Grade IV 19, and unknown 50.6%), but half were considered localized tumors. Most patients underwent an operation (66.1%). In 5.6% of patients, the operation was recommended but not performed. The overall 5-year survival was 26.7% (95% CI 25.4–28.1%). IPTW with adjusted Cox proportional hazard model demonstrated worse survival, showing that operation compared to no operation and operation with chemo/radiation compared to operation alone had worse survival between months 0 and 25 but had improved survival after month 25.
Conclusions
The incidence of angiosarcoma is low and long-term survival is poor. Multimodal therapy in the form of neoadjuvant or adjuvant chemo/radiation therapy offers significant long-term survival benefits over operation alone.
... Epithelioid ASs may express cytokeratin, CD30, and EMA. D2-40 (also known as podoplanin, a lymphatic marker) is variably expressed, and its positivity suggests lymphatic differentiation (Wang et al., 2017). ...
Angiosarcomas are uncommon malignant mesenchymal neoplasms of endothelial origin. They may be primary or secondary to radiation exposure, chronic lymphedema or to other associated risk factors. They can occur anywhere in the body, with the most common location being the skin of the head and neck. Radiation-induced angiosarcomas of the gynecologic tract are very rare with only few cases reported in the literature. We report a case of a 54-year-old lady who developed angiosarcoma of the vagina and vulva 9 years following radiotherapy for cervical cancer. She was treated with chemoradiotherapy and died nine months following the diagnosis of angiosarcoma. We also performed a literature review of the radiation-induced angiosarcomas arising in the vagina and vulva. Angiosarcomas should always be considered in the differential diagnosis when dealing with a tumor located in a previously irradiated area, as they may clinically mimic recurrence of the original tumor the patient had.
... [1,2] Tumors can occur in any part of the body, but the most common primary sites are cutaneous lesions (approximately 60% of cases), particularly in the head and neck; although, they can also present within the soft tissues, visceral organs, bone, and retroperitoneum. [2][3][4] There are major differences in the characteristics, behaviors, and prognosis of angiosarcoma among primary sites. [3] Primary pleural angiosarcoma (PPA), a highly malignant disease, is an extremely rare malignancy, and the literature is limited to case reports. ...
... [2][3][4] There are major differences in the characteristics, behaviors, and prognosis of angiosarcoma among primary sites. [3] Primary pleural angiosarcoma (PPA), a highly malignant disease, is an extremely rare malignancy, and the literature is limited to case reports. [5][6][7][8] Delayed diagnosis and the rarity of these tumors contribute to difficulties in determining the best treatment and prognostic factors. ...
Primary pleural angiosarcoma (PPA) is an extremely rare malignancy for which there is no consensus on treatment. The clinical course of PPA is usually quickly fatal, regardless of the treatment used.
We summarized and evaluated a relatively large population of published PPA cases to assess prognostic factors, diagnostic approaches, treatment methods and clinical outcomes. Using the CNKI, Embase, and PubMed databases, literature published in English and Chinese from 1988 through 2020 was searched using the terms “primary pleural angiosarcoma,” “pleural angiosarcoma,” and “pleuropulmonary angiosarcoma.”
A total of 43 patients with PPA were identified in retrospective case series and case reports. The median age at diagnosis was 64 years (range 24–87 years), and the median overall survival was 4 months (range 0.1–180 months). Approximately 80% of patients died from PPA within 10 months of diagnosis, and the 2-year survival rate was approximately 4.4%. In univariate analyses, the presence of pleural effusion and hemothorax were significant predictors of decreased survival, with hazard ratios (HRs) of 2.7 (P = .04) and 3.3 (P = .006), respectively. Sixteen patients received no therapy, and their prognosis was worse than patients who did receive therapy (P = .019). Radiation therapy improved survival more than no radiation therapy (P = .007). Patients appeared to derive clinical benefit from chemotherapy (P = .048). However, tumor resection did not seem to provide a survival benefit (P = .051). In multivariate analysis, tumor resection, and radiation were independent, statistically significant, positive predictors of better survival, with HRs of 0.3 (P = .017) and 0.1 (P = .006), respectively. The presence of hemothorax was an independent predictor of worse prognosis (P = .006).
Primary angiosarcoma of the pleura is a rare, poorly understood malignancy with a poor prognosis; hence, the clinical spectrum of PPA is not completely defined. By multivariate analysis, this retrospective study showed a survival benefit of tumor resection or radiation therapy, and the presence of hemothorax was a significant prognostic factor for poor outcomes.
... Our results demonstrate several unique features of angiosarcomas within this cohort of Asian patients. Confirming previous reports, most cases originated from the head and neck, in particular from the scalp and face 5,23 . This is in contrast to Western populations, in which AS-HN are less common, accounting for only 26.3% (US SEER database) 24 , 17% (Netherlands Cancer Registry) 25 and 10.6% (French Sarcoma Group 1 of angiosarcomas as per various studies. ...
Angiosarcomas are a rare subtype of soft-tissue sarcomas which exhibit aggressive clinical phenotypes with limited treatment options and poor outcomes. In this study, we investigated the clinical relevance of the peripheral blood neutrophil-to-lymphocyte ratio (NLR) as a marker of systemic immune response, as well as its correlation with intra-tumoral immune profiles in a subgroup of cases ( n = 35) using the NanoString PanCancer IO360 panel and multiplex immunohistochemistry. In the overall cohort ( n = 150), angiosarcomas of the head and neck (AS-HN) comprised most cases (58.7%) and median overall survival (OS) was 1.1 year. NLR, classified as high in 78 of 112 (70%) evaluable patients, was independently correlated with worse OS (HR 1.84, 95%CI 1.18–2.87, p = 0.0073). Peripheral blood NLR was positively correlated with intra-tumoral NLR (tNLR) (Spearman’s rho 0.450, p = 0.0067). Visualization of tumor-infiltrating immune cells confirmed that tNLR scores correlated directly with both neutrophil (CD15 ⁺ cells, rho 0.398, p = 0.0198) and macrophage (CD68 ⁺ cells, rho 0.515, p = 0.0018) cell counts. Interestingly, tNLR correlated positively with oncogenic pathway scores including angiogenesis, matrix remodeling and metastasis, and cytokine and chemokine signaling, as well as myeloid compartment scores (all p < 0.001). In patients with documented response assessment to first-line chemotherapy, these pathway scores were all significantly higher in non-responders (47%) compared to responders. In conclusion, systemic and local immune responses may inform chemotherapy response and clinical outcomes in angiosarcomas.
... The head and neck is the commonest primary anatomic site, accounting for 27% of all angiosarcomas [1]. Head and neck angiosarcomas most commonly occur in the elderly with a peak presentation in the seventh decade and are more common in men [1,3,7,21]. Previous data have indicated an association between excessive UV light exposure and cutaneous angiosarcomas as they are frequently seen in the sun-exposed face and scalp of elderly Caucasian men [11,12]. ...
... 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 [3,[5][6][7]. ...
Angiosarcomas are rare, aggressive soft tissue sarcomas originating from endothelial cells of lymphatic or vascular origin and associated with a poor prognosis. The clinical and imaging features of angiosarcomas are heterogeneous with a wide spectrum of findings involving any site of the body, but these most commonly present as cutaneous disease in the head and neck of elderly men. MRI and CT are complementary imaging techniques in assessing the extent of disease, focality and involvement of adjacent anatomical structures at the primary site of disease. CT plays an important role in the evaluation of metastatic disease. Given the wide range of imaging findings, correlation with clinical findings, specific risk factors and patterns of metastatic disease can help narrow the differential diagnosis. The final diagnosis should be confirmed with histopathology and immunohistochemistry in combination with clinical and imaging findings in a multidisciplinary setting with specialist sarcoma expertise. The purpose of this review is to describe the clinical and imaging features of primary sites and metastatic patterns of angiosarcomas utilising CT and MRI.
... A French retrospective multicenter study of 161 patients reported that visceral (heart, liver, and spleen) and primary bone sites were associated with worse prognosis [13]. In a study of 200 AS cases from China that also showed biological differences, the worst prognosis was seen in H/N-AS (5-year OS of 28%), followed by visceral (37%), and B-AS (87%) [14]. Evidence shows that patients with secondary B-AS have a more aggressive tumor phenotype and worse survival outcome than patients with primary B-AS [14,15]. ...
... In a study of 200 AS cases from China that also showed biological differences, the worst prognosis was seen in H/N-AS (5-year OS of 28%), followed by visceral (37%), and B-AS (87%) [14]. Evidence shows that patients with secondary B-AS have a more aggressive tumor phenotype and worse survival outcome than patients with primary B-AS [14,15]. A study of over 470 patients extracted from the SEER database described that secondary B-AS appears in older patients and presents with more locally advanced stage (57% vs. 18%) and high grade (58% vs. 32%). ...
We performed a retrospective analysis of angiosarcoma (AS) genomic biomarkers and their associations with the site of origin in a cohort of 143 cases. Primary sites were head and neck (31%), breast (22%), extremity (11%), viscera (20%), skin at other locations (8%), and unknown (9%). All cases had Next Generation Sequencing (NGS) data with a 592 gene panel, and 53 cases had Whole Exome Sequencing (WES) data, which we used to study the microenvironment phenotype. The immunotherapy (IO) response biomarkers Tumor Mutation Burden (TMB), Microsatellite Instability (MSI), and PD-L1 status were the most frequently encountered alteration, present in 36.4% of the cohort and 65% of head and neck AS (H/N-AS) (p < 0.0001). In H/N-AS, TMB-High was seen in 63.4% of cases (p < 0.0001) and PDL-1 positivity in 33% of cases. The most common genetic alterations were TP53 (29%), MYC amplification (23%), ARID1A (17%), POT1 (16%), and ATRX (13%). H/N-AS cases had predominantly mutations in TP53 (50.0%, p = 0.0004), POT1 (40.5%, p < 0.0001), and ARID1A (33.3%, p = 0.5875). In breast AS, leading alterations were MYC amplification (63.3%, p < 0.0001), HRAS (16.1%, p = 0.0377), and PIK3CA (16.1%, p = 0.2352). At other sites, conclusions are difficult to generate due to the small number of cases. A microenvironment with a high immune signature, previously associated with IO response, was evenly distributed in 13% of the cases at different primary sites. Our findings can facilitate the design and optimization of therapeutic strategies for AS.
