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% [2].

• According to a retrospective study conducted in Mexico [3], 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 [4], while cardiac HS may cause dyspnea, pericardial effusion and cardiac tamponade [5] [6]. Spontaneous tumor rupture has repeatedly been described and may lead to anemia, thrombocytopenia, and coagulopathy, or hemorrhagic shock [7] [8].
  

Hematogenous spread is common, and metastases most frequently develop in the lungs. Accordingly, patients may present with signs of lung cancer.

Additionally, patients may claim constitutive symptoms like fatigue, fever, night sweats and weight loss. Hemolytic anemia may be observed [9] [10].

• Lymphedema

  This syndrome is an unusual form of angiosarcoma occuring as a complication of lymphedema. [ncbi.nlm.nih.gov]

  This typically appears as a complication of a long course lymphedema located on … [scholar.google.it]

  Patients with long-standing lymphedema are at increased risk of developing angiosarcoma. [codes.iarc.fr]

  We present a case of cutaneous angiosarcoma on the leg in an elderly woman in the setting of a chronic nonhealing wound and lymphedema. We also discuss the differential diagnosis and treatment options. [mdedge.com]

• Swelling

  A case of a hemangiosarcoma involving the bones of the foot in a diabetic patient who had swelling of the left foot is reported. Radiographs demonstrated multiple lytic areas throughout the bones of the foot. [ncbi.nlm.nih.gov]

  Swelling caused by lymph vessel damage. Swelling caused by a backup of lymph fluid is called lymphedema. It happens when the lymphatic system gets blocked or damaged. Lymphedema can happen when lymph nodes are removed during surgery. [mayoclinic.org]

  To our surprise on intra-oral examination, we noticed another swelling on the left faucial pillar region apart from the rapid increase in size of the swelling on the right side. [omicsonline.org]

  (Lymphedema is swelling that can develop after surgery or radiation therapy to treat breast cancer.) It can cause skin changes and/or a lump in the breast. How is angiosarcoma treated? Angiosarcomas tend to grow and spread quickly. [cancer.org]

• Anemia

  Thyroid hemangiosarcoma can metastasize to the bone marrow and anemia may be an indicator of the advanced disease. In the differential diagnosis of the anemia, bone marrow metastasis and bone marrow biopsy should be considered in suspected cases. [ncbi.nlm.nih.gov]

• Asymptomatic

  Case No 2: Our second case was an asymptomatic 76-year-old female. Her sister died 10 years earlier of breast cancer and the patient had mild hypertension. [breastlink.com]

  Rapidly developing mass in asymptomatic man. Chest, 75 (1974), pp. 703-704 [3.] N. Martini, A.G. Huvos. Primary malignant tumors of the sternum. Surg Gynecol Obstet, 138 (1974), pp. 391-395 [4.] R.G. Brown, W.H. Fleming, M.J. Jurkiewicz. [archbronconeumol.org]

  Neither symptomatic nor asymptomatic recurrences were observed at 18-months follow-up, suggesting that ECT is a promising tool in the palliative care of patients presenting with angiosarcomas after breast surgery. Conflicts of interest None. [ncbi.nlm.nih.gov]

  Abdominal pain, fatigue, weight loss, and anorexia are common symptoms of HAS, but many cases can be asymptomatic.5 Examination findings include hepatomegaly, jaundice, ascites,17 and, rarely, hepatic bruits may be audible on auscultation due to the vascular [emjreviews.com]

  Genotypic characterization of Kaposi's sarcoma-associated herpesvirus in asymptomatic infected subjects from isolated populations. [scielo.br]

• Congestive Heart Failure

  An 8-year-old spayed Golden Retriever was examined because of recent onset of congestive heart failure complicated by ventricular arrhythmias. Echocardiography revealed a thickened, hypokinetic region of the left ventricular free wall. [ncbi.nlm.nih.gov]

  Case Report An 83-year-old woman with a medical history of advanced dementia, congestive heart failure, chronic obstructive pulmonary disease, chronic kidney disease, type 2 diabetes mellitus, hypertension, and chronic venous insufficiency with stasis [mdedge.com]

  […] is the cause of death in approximately 50% of patients and haemoperitoneum in 25% of patients, followed by metastatic disease, infection, and, rarely, renal failure and congestive heart failure (3% of deaths).3,17,18,25,29 The lung is the most common [emjreviews.com]

  She was found to have a myocardial infarction with mild congestive heart failure. She was treated medically and did well. [breastlink.com]

• Abdominal Pain

  Clinical symptoms include abdominal pain and left upper quadrant mass. Many clinical conditions have similar presenting symptoms and make diagnosis difficult. [ncbi.nlm.nih.gov]

• Jaundice

  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. [symptoma.com]

  Case 4 presented in July 1973 with hepatosplenomegaly, weight loss, and jaundice. Two liver biopsies were interpreted as showing severe cirrhosis. Autopsy in December 1973 revealed angiosarcoma. [cdc.gov]

  Abdominal pain, fatigue, weight loss, and anorexia are common symptoms of HAS, but many cases can be asymptomatic.5 Examination findings include hepatomegaly, jaundice, ascites,17 and, rarely, hepatic bruits may be audible on auscultation due to the vascular [emjreviews.com]

• Hepatomegaly

  Physical examination revealed hepatomegaly, ascites, and firm, huge splenomegaly. Ultrasonography showed many nodular structures characterized by hypoechogenic and hyperechogenic areas. [ncbi.nlm.nih.gov]

  Patients typically have non-specific abdominal symptoms and may present with an abdominal mass or hepatomegaly. Patients can also present with spontaneous rupture and hemoperitoneum 1,5. [radiopaedia.org]

  Abdominal pain, fatigue, weight loss, and anorexia are common symptoms of HAS, but many cases can be asymptomatic.5 Examination findings include hepatomegaly, jaundice, ascites,17 and, rarely, hepatic bruits may be audible on auscultation due to the vascular [emjreviews.com]

• Vascular Disease

  Initial clinical diagnostic impressions included pericarditis, vasculitis due to a collagen vascular disease, interstitial pneumonia, sarcoidosis, and metastatic adenocarcinoma ( n = 13). [nature.com]

• Skin Plaque

  Both may manifest with skin discoloration, skin plaques or nodules, a palpable mass, or a combination of these signs. Discoloration may be mistaken for bruising, thereby delaying diagnosis. Mammographic findings are nonspecific for angiosarcoma. [ajronline.org]

• Scalp Mass

  In June 2008, more than seven months after the surgery, the patient was readmitted to the Department of Neurosurgery presenting with multiple scalp masses ( Fig. 2 ). This was diagnosed as a post-operative recurrence of the scalp angiosarcoma. [spandidos-publications.com]

• Confusion

  However, they should not be confused with cherry hemangiomas. Most tumors of visceral blood and lymphatic vessel walls are cancerous (malignant). [en.wikipedia.org]

  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. [symptoma.com]

  Skin erythema, another common early finding, is often confused with infection. The initial presenting findings can also be confused with post-irradiation changes. [breastlink.com]

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 [1]. 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 [11] [6].

• Human Herpesvirus 8

  Absence of human herpesvirus-8 DNA in angiosarcoma. Br J Dermatol. 1999;140:170-1. [ Links ] 28 Fink-Puches R, Zöchling N, Wolf P, Bäck B, Kerl H, Soyer HP. No detection of human herpesvirus 8 in different types of cutaneous angiosarcoma. [scielo.br]

  Absence of Kaposi’s sarcoma-associated virus (human herpesvirus-8) sequences in angiosarcoma. Virchows Arch. 1999;434:51–56. [PubMed] [Google Scholar] 24. Tomita K, Yokogawa A, Oda Y, Terahata S. [ncbi.nlm.nih.gov]

• Lymphocytic Infiltrate

  A: Hyperkeratosis of the epidermis, mild lymphocytic infiltrate in the upper part of the dermis, and varying degrees of edema in the interstitial tissues. [ncbi.nlm.nih.gov]

  Skin punch biopsy revealed an atypical lymphocytic infiltrate with the suggestion of viral inclusions. Second opinion of the biopsy was interpreted as atypical lymphoreticular infiltrate suggestive of Ki-1 lymphoproliverative disorder. [breastlink.com]

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]

Treatment and Follow-up for Metastatic Disease In the situation where the tumor is recurrent or metastatic, in some cases radiation or another surgical resection can be offered, but usually treatment consists of intravenous chemotherapy directed against [web.archive.org]

Even with aggressive treatment, most patients have a survival rate of approximately 6 months. [knowcancer.com]

Both of these patients had complete and durable responses to the treatment and remained without evidence of disease at the time of publication of this study. [ascopost.com]

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]

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]

Etiology Most arise spontaneously, although hemochromatosis and neurofibromatosis type 1 have also been associated. [radiopaedia.org]

Breast sarcoma: Epidemiology, risk factors, clinical presentation, diagnosis, and staging. In Shah S, ed. UpToDate. Waltham, Mass.: UpToDate, 2021. https://www.uptodate.com. Last updated March 30, 2021. Accessed August 30, 2021. [cancer.org]

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.medscape.com]

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.medscape.com]

The sequestration of platelets and local intravascular destruction of clotting factors28 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 HAS [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]

It may work by preventing the cancer cells from dividing and eventually cause the tumor cells to die similar to other drugs that target microtubules [clinicaltrials.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]

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 [1]. 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 [2]. 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.

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