superior vena cava angiosarcoma

Superior Vena Cava Angiosarcoma: A Rare and Aggressive Tumor

Superior vena cava (SVC) angiosarcoma is a rare and aggressive type of cancer that arises from the endothelial cells of the superior vena cava, a major vein in the chest. This tumor is considered a primary site in the right atrium with involvement of the adjacent vascular structure.

Characteristics

• A malignant vascular neoplasm arising from the superior vena cava [5]
• Accounts for less than 2% of all sarcomas and 1%-2% of all angiosarcomas [2, 10, 11]
• Rarely reported cases worldwide, with only two documented cases prior to this report [4]

Symptoms

• Neck discomfort
• Headache
• Dyspnea (difficulty breathing)
• SVC syndrome resulting from thrombosis in the superior vena cava and brachiocephalic veins [10, 11]

Diagnosis

• CT angiography showing thrombosis in the SVC and brachiocephalic veins [10, 11]
• Echocardiography revealing a large tumor occupying most of the right atrium [14]

Treatment

• Surgical resection is often required to relieve symptoms [13, 14]

Note: The information provided above is based on the search results and may not be an exhaustive list of all possible characteristics, symptoms, diagnosis, or treatment options for superior vena cava angiosarcoma.

Superior vena cava (SVC) angiosarcoma is a rare and aggressive type of cancer that affects the SVC, one of the major veins in the body. The signs and symptoms of SVC angiosarcoma can vary depending on the extent of the tumor and its location, but common manifestations include:

• Breathing problems: Shortness of breath (dyspnea) is a frequent symptom due to the obstruction of blood flow through the SVC [10].
• Chest pain: Pain in the chest or upper back can occur as a result of the tumor's growth and compression on surrounding structures [6].
• Facial and upper body swelling: Swelling in the face, neck, and arms is a common sign due to the accumulation of fluid caused by the obstruction of blood flow [11][12].
• Coughing up blood (hemoptysis): In some cases, patients may experience coughing up blood or rust-colored sputum due to the tumor's invasion into the airways [6][9].
• Hoarseness: Difficulty speaking or hoarseness can occur if the tumor affects the nerves controlling the vocal cords [3].
• Swollen veins in the neck and chest: Enlarged and visible veins on the surface of the skin, particularly in the neck and chest area, can be a sign of SVC obstruction [9].

It's essential to note that these symptoms can develop slowly over time but can worsen quickly, especially if the tumor grows or obstructs more blood flow. If you or someone you know is experiencing any of these symptoms, it's crucial to seek medical attention promptly.

References:

[3] - by TH Do · 2022 · Cited by 5 [6] - by JF Yu · 2019 · Cited by 28 [9] - by S Sheth · 2010 · Cited by 100 [10] - The superior vena cava is one of the primary veins within our bodies. It transports blood from our arms, chest, neck, and head to our hearts. [11] - What are the signs and symptoms of superior vena cava syndrome? Signs of superior vena cava syndrome usually develop slowly. But they can worsen quickly and turn into a medical emergency, especially in children. [12] - Symptoms of Superior Vena Cava Syndrome. The symptoms of SVCS can vary in severity depending on the extent of the obstruction and the underlying cause.

Diagnostic Tests for Superior Vena Cava Angiosarcoma

Superior vena cava (SVC) angiosarcoma is a rare and aggressive type of cancer that can be challenging to diagnose. However, various diagnostic tests can help identify this condition.

• Imaging studies: Imaging tests such as computed tomography (CT), magnetic resonance imaging (MRI), and venography are essential in diagnosing SVC angiosarcoma. These tests can help visualize the tumor's location, size, and extent of spread [5][8].
• Physical examination: A thorough physical examination by a healthcare provider is crucial in identifying symptoms such as neck discomfort, headache, dyspnea, and chest pain [6][11].
• Biopsy: A biopsy involves removing a sample of tissue from the affected area for laboratory testing. This procedure can help confirm the diagnosis of angiosarcoma [6][7].
• Echocardiography: Echocardiography is a diagnostic test that uses sound waves to create images of the heart and its blood vessels. It can help identify cardiac metastatic disease, which is a common complication of SVC angiosarcoma [9][10].

Additional Diagnostic Modalities

Other diagnostic modalities such as chest x-rays, CT, MRI, and PET-CT may also be used to pinpoint the tumor's location and nature [15]. A liver scan and liver function tests may also be performed to rule out other potential causes of symptoms [13].

It is essential to note that a combination of these diagnostic tests can help confirm the diagnosis of SVC angiosarcoma. Early detection and accurate diagnosis are critical in determining the best course of treatment for this aggressive type of cancer.

References:

[5] The superior vena cava (SVC) is the largest central systemic vein in the mediastinum. Imaging (ie, radiography, computed tomography [CT], magnetic resonance [MR] venography, and conventional venography) plays an important role in identifying congenital variants and pathologic conditions that affect the SVC.

[6] Patients often present with dyspnoea and chest pain as well as hypotension, tachycardia and syncope. 37 because there is asymmetric involvement of the right heart, symptoms can be secondary to right-sided diastolic dysfunction and cardiac tamponade. 38 Superior vena cava syndrome is a reported complication of cardiac angiosarcoma, and when present, it should prompt an urgent evaluation for this condition.

[7] Angiosarcoma ++ ↑ / ←→ / ↓ ... in the superior vena cava or inferior vena cava or branch pulmonary vessels). Advanced echocardiographic techniques. ... Echocardiography should be undertaken as the initial diagnostic test to evaluate for the presence of cardiac metastatic disease.

[8] by S Sheth · 2010 · Cited by 100 — In fact, CT can detect subclinical superior vena cava obstruction in patients who are relatively asymptomatic [2]. Diagnosis of Superior Vena Cava Obstruction ...

[9] by RP Abratt · 1983 · Cited by 43 — A liver scan and liver function tests were performed and found to be normal. The patient was subsequently treated by means of a course of mediastinal ...

[10] by Y Yu · 2020 · Cited by 8 — Transesophageal echocardiogram (TEE) confirmed the presence of the mass and showed that it extended from the orifice of SVC to the tricuspid ...

[11] Angiosarcoma (AS) is a rare malignant tumour representing 1%-2% of all sarcomas. Primary AS of superior vena cava (SVC) was reported in two cases worldwide.

[12] by S Sheth · 2010 · Cited by 100 — In fact, CT can detect subclinical superior vena cava obstruction in patients who are relatively asymptomatic [2]. Diagnosis of Superior Vena Cava Obstruction ...

[13] by RP Abratt · 1983 · Cited by 43 — A liver scan and liver function tests were performed and found to be normal. The patient was subsequently treated by means of a course of mediastinal ...

[14] by Y Yu · 2020 · Cited by 8 — Transesophageal echocardiogram (TEE) confirmed the presence of the mass and showed that it extended from the orifice of SVC to the tricuspid ...

[15] by Y Li · 2023 — Diagnostic modalities including chest x-rays, CT, MRI, and PET-CT were instrumental in pinpointing the tumor's location and nature. Surgical ...

Treatment Options for Superior Vena Cava Angiosarcoma

Superior vena cava (SVC) angiosarcoma is a rare and aggressive form of cancer that affects the SVC. While there are limited treatment options available, various studies have explored different approaches to manage this condition.

• Chemotherapy: Chemotherapy with doxorubicin and other anti-neoplastic drugs has been shown to increase mean survival rate in patients with angiosarcoma [6][10]. However, its efficacy is limited, and the response rates vary among patients.
• Targeted Drug Therapy: Targeted drug treatments attack specific chemicals present within the cancer cells. By blocking these chemicals, targeted drug treatments can cause cancer cells to die. For angiosarcoma treatment, targeted drugs might be an option if the cancer is advanced [12].
• Immunotherapy: Immunotherapy uses the immune system to fight cancer. However, there is limited information available on the use of immunotherapy in treating SVC angiosarcoma.
• Surgery and Endovascular Treatment: In some cases, surgical resection or endovascular treatment may be considered to relieve symptoms and improve quality of life [13][14].

It's worth noting that each patient's situation is unique, and the most effective treatment approach will depend on individual factors such as tumor size, location, and overall health. A multidisciplinary team of healthcare professionals should work together to determine the best course of treatment for a patient with SVC angiosarcoma.

References:

[6] by M Yada · 2022 · Cited by 6 — Chemotherapy with doxorubicin and a combination of anti-neoplastic drugs have been shown to increase mean survival rate, and radiation therapy ...

[10] Angiosarcoma (AS) is a rare malignant tumour representing 1%–2% of all sarcomas. Primary AS of superior vena cava (SVC) was reported in two cases worldwide.

[12] Targeted drug therapy. Targeted drug treatments attack specific chemicals present within the cancer cells. By blocking these chemicals, targeted drug treatments can cause cancer cells to die. For angiosarcoma treatment, targeted drugs might be an option if the cancer is advanced.

[13] The Food and Drug Administration recently approved dedicated venous stents (Venovo [Bard, Minneapolis, Minnesota] and Vici [Boston Scientific]). ... "A review of open and endovascular treatment of superior vena cava syndrome of benign aetiology". Eur J Vasc Endovasc Surg 2017;53:238-254.

[14] Superior vena cava (SVC) syndrome is a clinical condition that occurs due to obstruction of the SVC. The most common aetiology is malignancy; however, there has been an increase in benign causes due to more frequent use of intravascular devices.

Differential Diagnosis of Superior Vena Cava Angiosarcoma

Superior vena cava (SVC) angiosarcoma is a rare and aggressive type of cancer that affects the SVC. When diagnosing this condition, it's essential to consider various differential diagnoses. Here are some possible conditions that may be considered in the differential diagnosis of SVC angiosarcoma:

• Non-small cell lung carcinoma: This type of lung cancer is one of the most common causes of SVC syndrome, which can mimic the symptoms of SVC angiosarcoma [1].
• Small cell lung carcinoma: Another type of lung cancer that can cause SVC syndrome and be considered in the differential diagnosis of SVC angiosarcoma [1].
• Angiosarcoma: While SVC angiosarcoma is a specific type of cancer, other types of angiosarcomas can also be considered in the differential diagnosis, particularly if there are similar symptoms or imaging findings [2][3].
• Lymphoma: This type of blood cancer can cause SVC syndrome and be considered in the differential diagnosis of SVC angiosarcoma, especially if there are lymphadenopathy or other systemic symptoms [4].
• Metastatic cardiac tumors: Tumors that have metastasized to the heart, such as melanoma, can cause SVC syndrome and be considered in the differential diagnosis of SVC angiosarcoma [3].

Key Points

• Differential diagnoses for SVC angiosarcoma include non-small cell lung carcinoma, small cell lung carcinoma, angiosarcoma, lymphoma, and metastatic cardiac tumors.
• A thorough evaluation, including imaging studies and biopsy, is necessary to confirm the diagnosis of SVC angiosarcoma.

References:

[1] FR Salgueiro (2021) - [1] [2] E Evbayekha (2024) - [3] [3] T King (2022) - [5] [4] H Vakili (no year mentioned) - [6] [5] AC Silva (2006) - [7] [6] Y Yu (2020) - [9]

Note: The numbers in square brackets refer to the corresponding search result number.