Angiosarcoma

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Angiosarcoma
Metastatic angiosarcoma in the brain.jpg
MRI of a cardiac angiosarcoma that has metastasised to the brain.
Specialty Oncology
Symptoms Often asymptomatic, non-specific symptoms common
Usual onsetTypically 60-70 however all age groups are affected
Types Hemangiosarcoma, lymphangiosarcoma, parenchymal angiosarcoma
CausesUnknown
Risk factors Lymphedema, radiation therapy, various chemicals
Diagnostic method Imaging, biopsy
Treatment Chemotherapy, radiation therapy, surgery
Prognosis Depends on type, overall five-year survival ~ 30-38% [1] [2] [3]
Frequency1 per million people (US) [4]

Angiosarcoma is a rare and aggressive cancer that starts in the endothelial cells that line the walls of blood vessels or lymphatic vessels. Since they are made from vascular lining, they can appear anywhere and at any age, but older people are more commonly affected, and the skin is the most affected area, with approximately 60% of cases being cutaneous. Specifically, the scalp makes up ~50% of angiosarcoma cases, but this is still <0.1% of all head and neck tumors. [5] [6] [7] Since angiosarcoma is an umbrella term for many types of tumor that vary greatly in origin and location, many symptoms may occur, from completely asymptomatic to non-specific symptoms like skin lesions, ulceration, shortness of breath and abdominal pain. Multiple-organ involvement at time of diagnosis is common and makes it difficult to ascertain origin and how to treat it. [7]

Contents

The cause of angiosarcoma is not known, though several risk factors are known, such as chronic lymphedema, radiation therapy and various chemicals such as arsenic and vinyl chloride. Angiosarcomas have been reported in association with long standing foreign bodies. [8] [9] Infrequently they have occurred in association with breast implants. [10] Ultraviolet radiation [2] and localized immunodeficiency may play a role in pathogenesis of angiosarcoma. [3] Angiosarcoma can be seen on MRI, CT and ultrasound scans, but it is usually difficult to discern it from other cancers, requiring confirmation of diagnosis by biopsy and immunohistochemical analysis.

Treatment includes surgery, chemotherapy and radiation therapy, usually all three combined. Because these cancers arise from the cells lining the blood or lymphatic vessels, they can easily metastasize to distant sites, particularly the liver and lungs. [7] This makes them especially lethal, and an early diagnosis is usually necessary for survival. Even with treatment, prognosis is poor, with a five-year survival rate of 30–38%. [1] [3] This is even worse in cardiac angiosarcoma and angiosarcoma of the liver, where prognosis may be as low as three months. [7]

Angiosarcomas make up 1–2% of soft tissue sarcomas, which in turn make up less than 1% of adult cancer. [2] Due to this, no large studies have ever been published on the disease, with few exceeding even 100 patients; however, many case reports and small cohort studies have been published, and they cumulatively provide enough information to get a useful understanding of the disease. [7] The rate of angiosarcoma is increasing in the US. [6]

Classification

The classification of angiosarcoma is based on both its origin and the underlying risk factors that may have contributed. [7] This is not a comprehensive list and case reports often go into further depth. [3]

Primary cutaneous angiosarcoma

Angiosarcoma in the skin (usually the head or neck and specifically the scalp) with no underlying risk factors such as lymphedema or radiation therapy.[ citation needed ]

Lymphedema associated angiosarcoma

Also called Stewart-Treves syndrome, it is a lymphangiosarcoma resulting from chronic lymphedema, swelling due to the build up of lymphatic fluid. It typically happens in the breast following a mastectomy that removes the lymph nodes of the breast. However it can happen anywhere. [3] [7]

Parenchymal angiosarcoma

Angiosarcoma in parenchymal organs such as the liver, breast or heart. These makes up about 40% of angiosarcoma.[ citation needed ]

Primary breast angiosarcoma

This refers to angiosarcoma of the breast without underlying causes such as radiation therapy or lymphedema. It is rare and makes up only 0.04% of breast tumors and 8% of breast sarcomas. It is very aggressive, usually presents in younger females and is often noticeable by a palpable mass. Prognosis is poor with a five-year survival between 8 and 50%. [7]

Secondary breast angiosarcoma

These are angiosarcoma that arise in the breast from underlying causes such as lymphedema or radiation, usually from radiation therapy for breast cancer. The link between radiation therapy and angiosarcoma has been disputed by some who profess the angiosarcoma is caused by concurrent lymphedema. Secondary breast angiosarcoma tends to have a bad prognosis. Radiation-associated angiosarcoma has a five-year survival of 10 to 54%. Distant metastases are present in 27–42% of individuals and is associated with a far worse prognosis. Other prognostic factors include size of tumor, age and how much can be removed by surgery. [3] [7]

Primary brain angiosarcoma

Primary brain angiosarcoma are angiosarcoma that arise in the brain (i.e. not metastasised from elsewhere). They are exceedingly rare with only a few cases reported. Imaging is non specific and differential diagnosis includes most other brain tumors such as gliomas or cavernomas, [3] requiring biopsy to confirm diagnosis, usually after surgery. Prognosis is generally poor with a median survival rate of eight months. However it varies greatly depending on whether or not the tumor has metastasised, some individuals may be rid of the disease following surgery, chemotherapy and radiation therapy. [11]

Primary cardiac angiosarcoma

Primary cardiac angiosarcoma is an angiosarcoma that arises in the heart. Despite being very rare they are the most common malignant primary heart tumor with 10–25% [12] [3] of cases being angiosarcomas. Symptoms may include shortness of breath, chest pain (46% [12] ), hypotension and syncope. Superior vena cava syndrome is reportedly a complication of cardiac angiosarcoma. [7] Due to the non-specificity of symptoms and rarity of the disease it is often missed by doctors and initial diagnosis may be delayed. A 2012 study reported that 56% of patients presented with pericardial effusion with or without cardiac tamponade. [12] The most common finding on imaging is cardiomegaly. Prognosis is generally very poor with a mean survival rate of three months to four years following diagnosis. [7] Metastasis at time of diagnosis is common. [12]

Primary liver angiosarcoma

Primary angiosarcoma of the liver is the third most common type of liver cancer however it only makes up 0.1–2.0% of all liver cancers. It is rapidly fatal with most individuals dying within six months or a year even with surgical resection. Despite being associated with arsenic, vinyl chloride, thorotrast and other chemicals, 75% of liver angiosarcomas have no known cause. Men are more commonly affected in a ratio of 3–4:1 however in children girls are more often affected. If symptomatic it often presents with non-specific liver related symptoms such as abdominal pain, jaundice, fatigue, unintended weight loss and distension. [13]

Deep soft tissue angiosarcoma

Angiosarcoma in the deep soft tissue such as adipose tissue, ligaments or muscles. These make up about 10% of angiosarcoma.[ citation needed ]

Signs and symptoms

Three different types of scalp angiosarcoma. (a) ulcer type (b) bruise type (c) nodular type Three types of scalp angiosarcoma.jpg
Three different types of scalp angiosarcoma. (a) ulcer type (b) bruise type (c) nodular type

Cutaneous angiosarcoma may initially look like a bruise or a purplish-red papule. It may look like a benign tumor which can delay correct diagnosis and treatment. Fungation, ulceration and bleeding may develop. [3]

Cardiac angiosarcoma may present with shortness of breath, chest pain (46%), weight loss, anemia-like symptoms, [12] low blood pressure and syncope. [7]

Angiosarcomas principally metastasises through the blood with the most common site for metastasis being the lungs, this may present as pleural effusion, pneumothorax or other pleural disease. Other common sites of metastasis include the liver, bone and lymph nodes. [3]

Causes

Angiosarcoma develops when damage to the DNA of endothelial cells causes them to divide uncontrollably and prevents programmed death. This allows them to grow into a mass invading other parts of the body and consuming its resources. If part of the mass breaks off and enters the circulatory or lymphatic system (which is very common due to angiosarcomas arising from the lining of said systems) it allows them to lodge elsewhere in the body. This process is called metastasis and is a negative prognostic factor. [14] The underlying causes of DNA damage is not understood but several risk factors are widely accepted.[ citation needed ]

Accepted risk factors include lymphedema, radiation therapy, neurofibromatosis, Maffucci syndrome, Klippel-Trenaunay syndrome [3] and chemicals such as arsenic, thorotrast and vinyl chloride. [4]

A 2020 study analyzing the genome of cutaneous angiosarcomas suggests that ultraviolet radiation may be a causative agent of angiosarcomas. [2]

Immunosuppression may play a role in the pathogenesis of angiosarcoma. There have been reports of angiosarcoma in immunosuppressed individuals following kidney transplantation. The association between lymphedema and angiosarcoma may be due to localized immunodeficiency, however this hypothesis has not been confirmed. Some studies suggest a link between AIDS and angiosarcoma though this may be due to misdiagnosed Kaposi's sarcoma. [3]

Diagnosis

Immunostained CD31 in a cutaneous angiosarcoma Cutaneous angiosarcoma (4) CD31.jpg
Immunostained CD31 in a cutaneous angiosarcoma

The diagnosis of angiosarcoma can be difficult due to its asymptomatic nature or non-specific symptoms. Initial diagnosis is typically done by MRI, CT or ultrasound scan, however it is typically difficult to discern if a mass is an angiosarcoma or other type of tumor such as a melanoma or carcinoma. This means that while the initial diagnosis is typically done via imaging, conclusive diagnosis has to be performed via biopsy and subsequent histological and immunohistochemical analysis. [5]

The marker CD31 is often considered the gold standard for angiosarcoma diagnosis due to its high sensitivity and specificity. Other markers typically used include von Willebrand factor, CD34, and vascular endothelial growth factor. [5] The absence of markers present in melanomas such as S100 and HMB-45 can help in differentiating angiosarcomas from melanomas. [3]

Epidemiology

Angiosarcomas are rare with one in a million people being diagnosed with it each year in the US. [4] Older adults are more commonly affected and there is no gender bias, [1] except in angiosarcoma of the liver where males are affected in a ratio of 3-4:1 [13] and cutaneous angiosarcoma where males are again affected more commonly, particularly elderly white men. [3] [7]

Animals

In dogs, hemangiosarcoma is relatively common with the golden retriever, boxer and labrador retriever at higher risk of the disease than other breeds. It occurs primarily in the spleen, liver, heart, skin and subcutaneous layer of the skin. It is rapidly fatal and metastatic and can present with anything from nonspecific signs to imminent death from tumor rupture. [15]

See also

Related Research Articles

<span class="mw-page-title-main">Lymphedema</span> Medical condition

Lymphedema, also known as lymphoedema and lymphatic edema, is a condition of localized swelling caused by a compromised lymphatic system. The lymphatic system functions as a critical portion of the body's immune system and returns interstitial fluid to the bloodstream.

<span class="mw-page-title-main">Metastasis</span> Spread of a disease inside a body

Metastasis is a pathogenic agent's spread from an initial or primary site to a different or secondary site within the host's body; the term is typically used when referring to metastasis by a cancerous tumor. The newly pathological sites, then, are metastases (mets). It is generally distinguished from cancer invasion, which is the direct extension and penetration by cancer cells into neighboring tissues.

<span class="mw-page-title-main">Soft-tissue sarcoma</span> Medical condition

A soft-tissue sarcoma (STS) is a malignant tumor, a type of cancer, that develops in soft tissue. A soft-tissue sarcoma is often a painless mass that grows slowly over months or years. They may be superficial or deep-seated. Any such unexplained mass must be diagnosed by biopsy. Treatment may include surgery, radiotherapy, chemotherapy, and targeted drug therapy. Bone sarcomas are the other class of sarcomas.

<span class="mw-page-title-main">Sarcoma</span> Medical condition

A sarcoma is a malignant tumor, a type of cancer that arises from cells of mesenchymal origin. Connective tissue is a broad term that includes bone, cartilage, fat, vascular, or other structural tissues, and sarcomas can arise in any of these types of tissues. As a result, there are many subtypes of sarcoma, which are classified based on the specific tissue and type of cell from which the tumor originates. Sarcomas are primary connective tissue tumors, meaning that they arise in connective tissues. This is in contrast to secondary connective tissue tumors, which occur when a cancer from elsewhere in the body spreads to the connective tissue. Sarcomas are one of five different types of cancer, classified by the cell type from which they originate. The word sarcoma is derived from the Greek σάρκωμα sarkōma 'fleshy excrescence or substance', itself from σάρξsarx meaning 'flesh'.

<span class="mw-page-title-main">Uterine cancer</span> Medical condition

Uterine cancer, also known as womb cancer, includes two types of cancer that develop from the tissues of the uterus. Endometrial cancer forms from the lining of the uterus, and uterine sarcoma forms from the muscles or support tissue of the uterus. Endometrial cancer accounts for approximately 90% of all uterine cancers in the United States. Symptoms of endometrial cancer include changes in vaginal bleeding or pain in the pelvis. Symptoms of uterine sarcoma include unusual vaginal bleeding or a mass in the vagina.

<span class="mw-page-title-main">Endometrial cancer</span> Uterine cancer that is located in tissues lining the uterus

Endometrial cancer is a cancer that arises from the endometrium. It is the result of the abnormal growth of cells that have the ability to invade or spread to other parts of the body. The first sign is most often vaginal bleeding not associated with a menstrual period. Other symptoms include pain with urination, pain during sexual intercourse, or pelvic pain. Endometrial cancer occurs most commonly after menopause.

<span class="mw-page-title-main">Rhabdomyosarcoma</span> Medical condition

Rhabdomyosarcoma (RMS) is a highly aggressive form of cancer that develops from mesenchymal cells that have failed to fully differentiate into myocytes of skeletal muscle. Cells of the tumor are identified as rhabdomyoblasts.

Inflammatory breast cancer (IBC) is one of the most aggressive types of breast cancer. It can occur in women of any age. It is referred to as "inflammatory" due to its frequent presentation with symptoms resembling a skin inflammation, such as erysipelas.

<span class="mw-page-title-main">Invasive carcinoma of no special type</span> Medical condition

Invasive carcinoma of no special type, invasive breast carcinoma of no special type (IBC-NST), invasive ductal carcinoma (IDC), infiltrating ductal carcinoma (IDC) or invasive ductal carcinoma, not otherwise specified (NOS) is a disease. For international audiences this article will use "invasive carcinoma NST" because it is the preferred term of the World Health Organization (WHO).

<span class="mw-page-title-main">Malignant peripheral nerve sheath tumor</span> Medical condition

A malignant peripheral nerve sheath tumor (MPNST) is a form of cancer of the connective tissue surrounding nerves. Given its origin and behavior it is classified as a sarcoma. About half the cases are diagnosed in people with neurofibromatosis; the lifetime risk for an MPNST in patients with neurofibromatosis type 1 is 8–13%. MPNST with rhabdomyoblastomatous component are called malignant triton tumors.

<span class="mw-page-title-main">Desmoplastic small-round-cell tumor</span> Aggressive and rare cancer

Desmoplastic small-round-cell tumor (DSRCT) is an aggressive and rare cancer that primarily occurs as masses in the abdomen. Other areas affected may include the lymph nodes, the lining of the abdomen, diaphragm, spleen, liver, chest wall, skull, spinal cord, large intestine, small intestine, bladder, brain, lungs, testicles, ovaries, and the pelvis. Reported sites of metastatic spread include the liver, lungs, lymph nodes, brain, skull, and bones. It is characterized by the EWS-WT1 fusion protein.

<span class="mw-page-title-main">Hemangioendothelioma</span> Medical condition

Hemangioendotheliomas are a family of vascular neoplasms of intermediate malignancy.

Lymphangiosarcoma is a rare cancer which occurs in long-standing cases of primary or secondary lymphedema. It involves either the upper or lower lymphedematous extremities but is most common in upper extremities. Although its name implies lymphatic origin, it is believed to arise from endothelial cells and may be more accurately referred to as angiosarcoma.

Stewart–Treves syndrome refers to a lymphangiosarcoma, a rare disorder marked by the presence of an angiosarcoma in a person with chronic (long-term) lymphedema. Although it most commonly refers to malignancies associated with chronic lymphedema resulting from mastectomy and/or radiotherapy for breast cancer, it may also describe lymphangiosarcomas that result from congenital and other causes of chronic secondary lymphedema. Lymphangiosarcoma arising from cancer-related lymphedema has become much less common with better surgical techniques, radiation therapy, and conservative treatment. The prognosis, even with wide surgical excision and subsequent radiotherapy, is poor.

Heart cancer is an extremely rare form of cancer that is divided into primary tumors of the heart and secondary tumors of the heart.

<span class="mw-page-title-main">Ewing sarcoma</span> Type of cancer

Ewing sarcoma is a type of pediatric cancer that forms in bone or soft tissue. Symptoms may include swelling and pain at the site of the tumor, fever, and a bone fracture. The most common areas where it begins are the legs, pelvis, and chest wall. In about 25% of cases, the cancer has already spread to other parts of the body at the time of diagnosis. Complications may include a pleural effusion or paraplegia.

<span class="mw-page-title-main">Langerhans cell sarcoma</span> Medical condition

Langerhans cell sarcoma (LCS) is a rare form of malignant histiocytosis. It should not be confused with Langerhans cell histiocytosis, which is cytologically benign. It can present most commonly in the skin and lymphatic tissue, but may also present in the lung, liver, and bone marrow. Treatment is most commonly with surgery or chemotherapy.

<span class="mw-page-title-main">Brain metastasis</span> Cancer that has metastasized (spread) to the brain from another location in the body

A brain metastasis is a cancer that has metastasized (spread) to the brain from another location in the body and is therefore considered a secondary brain tumor. The metastasis typically shares a cancer cell type with the original site of the cancer. Metastasis is the most common cause of brain cancer, as primary tumors that originate in the brain are less common. The most common sites of primary cancer which metastasize to the brain are lung, breast, colon, kidney, and skin cancer. Brain metastases can occur in patients months or even years after their original cancer is treated. Brain metastases have a poor prognosis for cure, but modern treatments are allowing patients to live months and sometimes years after the diagnosis.

Carcinoma of the tonsil is a type of squamous cell carcinoma. The tonsil is the most common site of squamous cell carcinoma in the oropharynx. It comprises 23.1% of all malignancies of the oropharynx. The tumors frequently present at advanced stages, and around 70% of patients present with metastasis to the cervical lymph nodes. . The most reported complaints include sore throat, otalgia or dysphagia. Some patients may complain of feeling the presence of a lump in the throat. Approximately 20% patients present with a node in the neck as the only symptom.

<span class="mw-page-title-main">Liver angiosarcoma</span> Medical condition

Liver angiosarcoma also known as angiosarcoma of the liver or hepatic angiosarcoma is a rare and rapidly fatal cancer arising from endothelial that line the blood vessels of the liver. It is a type of angiosarcoma. Although very rare with around 200 cases diagnosed each year, it is still considered the third most common primary liver cancer, making up around 2% of all primary liver cancers. Liver angiosarcoma can be primary, meaning it arose in the liver, or secondary, meaning the angiosarcoma arose elsewhere and metastasized to the liver. This article covers PHA, however much is also applicable to secondary tumors.

References

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  9. ↑ Schneider, Tim; Renney, John; Hayman, John (August 1997). "Angiosarcoma occurring with chronic osteomyelitis and residual foreign material. A case report of a late World War wound complication". ANZ Journal of Surgery. 67 (8): 576–578. doi: 10.1111/j.1445-2197.1997.tb02045.x . ISSN   1445-1433.
  10. ↑ Russo, Daniela; Campanino, Maria Raffaela; Cepurnaite, Rima; Gencarelli, Annarita; De Rosa, Filippo; Corvino, Antonio; Menkulazi, Marsela; Tammaro, Vincenzo; Fuggi, Mariano; Insabato, Luigi (December 2020). "Primary High-Grade Angiosarcoma of the Breast in a Young Woman With Breast Implants: A Rare Case and a Review of Literature". International Journal of Surgical Pathology. 28 (8): 906–912. doi:10.1177/1066896920924087. ISSN   1066-8969.
  11. ↑ La Corte, Emanuele; Acerbi, Francesco; Schiariti, Marco; Broggi, Morgan; Maderna, Emanuela; Pollo, Bianca; Nunziata, Raffaele; Maccagnano, Elio; Ferroli, Paolo (April 2015). "Primary central nervous system angiosarcoma: a case report and literature review". Neuropathology. 35 (2): 184–191. doi:10.1111/neup.12178. ISSN   1440-1789. PMID   25388456. S2CID   21199506.
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