Hemangiosarcoma is a rare malignant neoplasm of vascular origin. It consists of atypical endothelial cells and undifferentiated sarcoma cells, proliferates rapidly, grows invasively and is associated with a poor prognosis.
About 75% of HS arise in the skin or soft tissues, with other organs being affected as follows: liver, 6%; heart, 5%; bones, 4%; spleen 3% .
- According to a retrospective study conducted in Mexico , cutaneous HS most commonly develop in the following regions of the human body: scalp and neck (37%), trunk (24%), face (20%). In the same study, tumor sizes ranging from a few millimeters to more than 30 cm have been reported. HS typically manifest in form of multiple, reddish blue or purple papules. As the disease progresses, these papules augment in size, their surface may ulcerate and become increasingly prone to bleed. Most patients diagnosed with this type of HS have a medical history of cancer, often of breast cancer treated with radiation therapy or by means of mastectomy.
- HS affecting soft tissues or internal organs may cause pain and exert local mass effects. Further symptoms may develop due to HS interfering with organ function, e.g., hepatic HS may be associated with jaundice and lead to hepatic failure , while cardiac HS may cause dyspnea, pericardial effusion and cardiac tamponade  . Spontaneous tumor rupture has repeatedly been described and may lead to anemia, thrombocytopenia, and coagulopathy, or hemorrhagic shock  .
Entire Body System
Fine-needle aspiration biopsy and histopathological analysis of obtained specimens will reveal characteristic features. On the one hand, there are epithelioid cells lining small cavities that distantly resemble blood vessels. On the other hand, spindle-shaped sarcoma cells are present. They are generally arranged in strands and form the backbone of vessel-like structures. With regards to nuclei, aberrant mitotic figures are frequently observed . There may be necrotic foci. Antibodies directed against vascular markers like CD31, CD34, and FLI1 may be used for immunohistochemical analyses to corroborate histological findings.
While this approach is feasible in case of superficial HS, a more extensive preparation is required to obtain tumor samples from other sites. Here, imaging techniques have to be applied first in order to confirm the presence of a local mass and to obtain information regarding its accessibility  .
Our case stresses the importance of multimodality treatment in centers for cancer treatment. Histopathologic evaluation is essential in the diagnosis of epitheloid hemangioendothelioma. [ncbi.nlm.nih.gov]
Early splenectomy will improve prognosis and survival. [ncbi.nlm.nih.gov]
BACKGROUND: The etiology of hemangiosarcoma remains incompletely understood. Its common occurrence in dogs suggests predisposing factors favor its development in this species. [ncbi.nlm.nih.gov]
Known etiological factors (seen in 20% of hepatic angiosarcomas) include occupational exposure to vinyl chloride monomer, arsenic, and androgenic-anabolic steroids. [codes.iarc.fr]
Little is known about the disease' etiology, but patients suffering from chronic lymphedema as well as those who received radiation therapy are known to be predisposed for HS. [symptoma.com]
Angiosarcomas also can originate in the liver, breast, spleen, bone, or heart. [1, 2, 3, 4] Epidemiology Frequency United States Angiosarcomas are rare neoplasms. [emedicine.com]
Epidemiology Angiosarcomas are relatively rare. They comprise 1-2% of sarcomas in the surgical database from 1982-present from Memorial Hospital. Like other sarcomas, the risk of recurrence depends on the stage of disease. [web.archive.org]
Epidemiology They account for less than 1% of all sarcomas and are more frequently seen in males (M:F 2:1) except when seen in the setting of post-mastectomy lymphedema which is clearly more common in women and is known as Stewart-Treves syndrome. [radiopaedia.org]
Moreover, the growth-factor environment of HSA is very similar to that of pathophysiological angiogenesis, which some homeobox genes regulate in the transcription of angiogenic molecules. [ncbi.nlm.nih.gov]
Pathophysiology 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. [emedicine.com]
The sequestration of platelets and local intravascular destruction of clotting factors 28 both contribute to the pathophysiologic process of DIC, which has been well-described in the literature. 28,29 Anaemia is another common finding in patients with [emjreviews.com]
The formation of vessel-like structures was blocked by nicotine, and restored by FK506, suggesting that 'nuclear factor of activated T cells' activity prevents differentiation of these cells. [ncbi.nlm.nih.gov]
These treatments can help prevent the tumor from coming back. Which combination of treatments is right for you depends on the unique characteristics of the tumor. [mskcc.org]
A SHE has been defined as "an unnecessary disease, disability, or untimely death whose occurrence signals a failure of prevention" (3). [cdc.gov]
Hence, surgical resection is preceded or followed by brachytherapy or external beam radiotherapy to prevent local recurrence. [omicsonline.org]
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Hemangiosarcoma (HS) is a rare malignant neoplasm originating from mesenchymal tissue, namely from endothelial cells. This neoplasm is often described as a spongy tumor composed of dysfunctional blood vessel cells that are positioned together by undifferentiated sarcoma cells arranged in strands. The tumor is well supplied with blood. It differs largely from its benign counterpart, the hemangioma; HS generally affect the elderly, grow rapidly and invasively, and metastasize early. Hemangioma is typically diagnosed in neonates, grows slowly and resolves spontaneously.
HS primarily develop in the skin, in superficial or deep soft tissues, but they may also affect the liver, spleen, heart, or any other organ . Little is known about the disease' etiology, but patients suffering from chronic lymphedema as well as those who received radiation therapy are known to be predisposed for HS . It has been suggested that the growing number of patients exposed to therapeutic radiation accounts for the observed increase in HS incidence rates.
Of note, HS is a rather common diagnosis in veterinary medicine. Dogs are most frequently affected, but this tumor is also diagnosed in other species of domestic and wild animals. In order to avoid confusion between human and non-human HS, the term angiosarcoma is sometimes preferred for the disease of men. In any case, research concerning human HS is complicated by the low incidence of the disease. This does not apply to veterinary medicine and research; efforts made in this discipline may eventually allow the design of target-oriented studies in humans.
- Geller RL, Hookim K, Sullivan HC, Stuart LN, Edgar MA, Reid MD. Cytologic features of angiosarcoma: A review of 26 cases diagnosed on FNA. Cancer Cytopathol. 2016;124(9):659-668.
- Young RJ, Brown NJ, Reed MW, Hughes D, Woll PJ. Angiosarcoma. Lancet Oncol. 2010;11(10):983-991.
- Albores-Saavedra J, Schwartz AM, Henson DE, et al. Cutaneous angiosarcoma. Analysis of 434 cases from the Surveillance, Epidemiology, and End Results Program, 1973-2007. Ann Diagn Pathol. 2011;15(2):93-97.
- Abegunde AT, Aisien E, Mba B, Chennuri R, Sekosan M. Fulminant hepatic failure secondary to primary hepatic angiosarcoma. Case Rep Gastrointest Med. 2015;2015:869746.
- Ge Y, Ro JY, Kim D, et al. Clinicopathologic and immunohistochemical characteristics of adult primary cardiac angiosarcomas: analysis of 10 cases. Ann Diagn Pathol. 2011;15(4):262-267.
- Patel SD, Peterson A, Bartczak A, et al. Primary cardiac angiosarcoma - a review. Med Sci Monit. 2014;20:103-109.
- Xu B, Xie X, Zhou X, Zhai M, Yang W. Spontaneous rupture of primary splenic angiosarcoma: A case report. Oncol Lett. 2015;10(5):3271-3273.
- Tang K, Shang QL, Zhou QC, Zhou JW, She XL, Zhang M. Primary cardiac angiosarcoma with spontaneous ruptures of the right atrium and right coronary artery. Echocardiography. 2013;30(6):E156-160.
- Dalal B, Al Riyami A, Yenson P. Microangiopathic haemolytic anaemia and hyposplenism in angiosarcoma. Br J Haematol. 2010;151(5):413.
- Fong CY, Low M. An unusual case of microangiopathic hemolytic anemia resulting from metastatic angiosarcoma. Blood. 2013;122(1):7.
- Koyama T, Fletcher JG, Johnson CD, Kuo MS, Notohara K, Burgart LJ. Primary hepatic angiosarcoma: findings at CT and MR imaging. Radiology. 2002;222(3):667-673.