conventional angiosarcoma

Conventional Angiosarcoma: A Rare and Aggressive Malignant Tumor

Conventional angiosarcoma is a rare and highly aggressive malignant tumor that originates from the endothelial cells of blood vessels. It is characterized by rapidly proliferating, extensively infiltrating anaplastic cells [3]. The tumor is composed of anastomosed irregular vascular channels lined by neoplastic endothelial cells, which infiltrate surrounding tissues [4].

Key Features:

• Origin: Conventional angiosarcoma arises from the endothelial cells of blood vessels.
• Cellular Characteristics: The tumor is characterized by rapidly proliferating, extensively infiltrating anaplastic cells.
• Histological Appearance: Microscopically, it is composed of anastomosed irregular vascular channels lined by neoplastic endothelial cells.

Prognosis and Treatment

Unfortunately, conventional angiosarcoma is a highly aggressive tumor with no genuinely effective therapy [5]. Most patients die within 6 months of diagnosis. The prognosis is generally poor due to the tumor's rapid growth and infiltration into surrounding tissues.

References:

• [1] Definition from NCI.
• [2] Angiosarcoma (AS) is a rare and highly aggressive malignant tumor, originating from lymphatic or vascular endothelial-cell [from Cao et al., 2019].
• [3] An angiosarcoma (AS) is an uncommon malignant neoplasm characterized by rapidly proliferating, extensively infiltrating anaplastic cells derived from blood vessels.
• [4] Angiosarcoma: a high-grade malignant vascular tumor comprising anastomosed irregular vascular channels lined by neoplastic endothelial cells and infiltrating surrounding tissues.
• [5] Angiosarcoma is a highly aggressive tumor with no genuinely effective therapy, and most patients die within 6 months of diagnosis.

Common Signs and Symptoms of Conventional Angiosarcoma

Conventional angiosarcoma, a rare and aggressive form of cancer, can manifest in various ways depending on its location. Here are some common signs and symptoms associated with this condition:

• Swelling at the affected site: This is often accompanied by pain and tenderness (1).
• Pain near the tumor: As the tumor grows, it can cause discomfort or pain in the surrounding area (2).
• Bruised area on the skin that looks purple: Cutaneous angiosarcoma can present as a single or multiple lesions with a characteristic blue-purple discoloration (4, 8).
• Abdominal discomfort, nausea, vomiting, and altered bowel habits: When angiosarcoma affects the abdominal region, patients may experience gastrointestinal symptoms (4).
• Right heart failure and/or cardiac tamponade: In cases where the tumor metastasizes to the heart, patients may exhibit signs of cardiovascular distress (5).

Additional Symptoms

Other possible signs and symptoms of conventional angiosarcoma include:

• Hepatomegaly (enlarged liver): Patients with liver involvement may experience abdominal swelling or tenderness (6).
• Ascites (fluid accumulation in the abdomen), jaundice, thrombocytopenia (low platelet count), hemoperitoneum (blood in the abdominal cavity), and liver failure can also occur (6).

Keep in mind: The signs and symptoms of angiosarcoma can vary depending on the location and stage of the tumor. If you suspect you or someone else has this condition, it's essential to consult a medical professional for proper diagnosis and treatment.

References:

[1] - Context result 1 [2] - Context result 2 [4] - Context result 4 [5] - Context result 5 [6] - Context result 6

Diagnostic Tests for Conventional Angiosarcoma

Conventional angiosarcoma, a rare and aggressive type of cancer, can be challenging to diagnose. However, various diagnostic tests are used to confirm the presence of this disease.

• Imaging Studies: Imaging studies such as computed tomography (CT) scans, magnetic resonance imaging (MRI), and positron emission tomography (PET) scans are commonly used to evaluate the extent of tumor spread and identify potential metastases [1][2].
• Ultrasound: Ultrasound is also a diagnostic tool that can be used to assess the size and location of the tumor, as well as its relationship with surrounding structures [3].
• Angiography: Angiography, which involves injecting a contrast agent into the bloodstream to visualize blood vessels, may be performed to evaluate the tumor's vascular supply and identify any potential metastases [4].
• Biopsy: A biopsy, which involves removing a sample of tissue from the tumor for examination under a microscope, is essential for confirming the diagnosis of conventional angiosarcoma. The biopsy sample should be examined by a pathologist who can confirm the presence of malignant cells associated with vascular structures [5].

Additional Diagnostic Tools

• Contrast-Enhanced CT: Contrast-enhanced CT scans are particularly useful in evaluating the liver, as they can help identify any potential metastases or tumor spread [6].
• Immunostaining: Immunostaining for factor VIII has been consistently positive among studies, which can be used to confirm the diagnosis of conventional angiosarcoma [7].

Importance of Early Detection and Accurate Diagnosis

Early detection and accurate diagnosis are crucial in managing conventional angiosarcoma effectively. A team-based interprofessional care approach is essential for affected patients, involving a multidisciplinary team of healthcare professionals, including surgeons, radiologists, pathologists, and medical oncologists [8].

References:

[1] Context 3 [2] Context 5 [3] Context 4 [4] Context 5 [5] Context 6 [6] Context 5 [7] Context 7 [8] Context 11

Conventional Angiosarcoma Drug Treatment

The treatment of conventional angiosarcoma typically involves a combination of therapies, including surgery, radiotherapy, and chemotherapy.

• Chemotherapy: Chemotherapy is often used to treat advanced or metastatic angiosarcoma. The most common chemotherapeutic drugs used in the treatment of angiosarcoma include:
  • Adriamycin (doxorubicin) [9]
  • Ifosfamide
  • Cyclophosphamide
  • Vincristine
  • Paclitaxel [3, 10]
• Targeted Therapy: Targeted therapy drugs may be an option for treating advanced angiosarcoma. These drugs work by blocking specific chemicals present within the cancer cells, causing them to die.
• Immunotherapy: Immunotherapy uses the immune system to fight cancer. It is a promising area of research in the treatment of angiosarcoma.

It's worth noting that the effectiveness of these treatments can vary depending on the individual case and the stage of the disease. A multidisciplinary approach, involving a team of healthcare providers, may be necessary to determine the best course of treatment for each patient.

References:

[9] by E Esposito · 2019 · Cited by 27 — The most common chemotherapeutic drugs are adriamycin, ifosfamide, cyclophosphamide, vincristine, and paclitaxel, typically administered weekly whereas kinase ...

[10] 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.

[3] by ME Weidema · 2019 · Cited by 35 — Patients with locally advanced or metastatic disease are treated with systemic therapy, either paclitaxel or doxorubicin. In case of cutaneous angiosarcoma, a ...

Differential Diagnosis of Conventional Angiosarcoma

Conventional angiosarcoma, a rare and aggressive type of cancer, can be challenging to diagnose due to its similarity in appearance with other vascular tumors. The differential diagnosis for conventional angiosarcoma includes:

• Atypical fibroxanthoma: A type of skin cancer that can mimic the appearance of angiosarcoma on imaging studies [7].
• Rhabdomyosarcoma: A malignant tumor of skeletal muscle origin, which can present with similar imaging characteristics to angiosarcoma [13].
• Mesothelioma: A rare cancer affecting the lining of the lungs and chest cavity, which can be difficult to distinguish from angiosarcoma on imaging studies [14].
• Metastatic disease: Angiosarcoma can metastasize to various parts of the body, making it challenging to differentiate from other types of metastatic tumors.
• Thrombus or myxoma: In some cases, angiosarcoma may be mistaken for a thrombus (blood clot) or a benign tumor called a myxoma [13].

Imaging Studies and Diagnostic Markers

Imaging studies such as CT scans, MRI, and PET scans can help in the differential diagnosis of conventional angiosarcoma. However, these studies may not always provide a definitive diagnosis due to overlapping features with other vascular tumors.

Diagnostic markers such as CD31, CD34, factor VIII-related antigen, Fli-1, and ERG positivity are typically expressed in angiosarcoma [6]. However, these markers can also be present in other types of vascular tumors, making them less reliable for differential diagnosis.

Clinical Presentation

The clinical presentation of conventional angiosarcoma can vary depending on the location and size of the tumor. In some cases, patients may experience symptoms such as pain, swelling, or bleeding at the site of the tumor [4].

In conclusion, the differential diagnosis of conventional angiosarcoma is complex and requires a comprehensive evaluation of clinical presentation, imaging studies, and diagnostic markers.

References:

[1] Angiosarcoma (AS) comprises 1% of all soft-tissue sarcoma (STS), which are themselves a rare malignancy. [10] [4] Pathological features, diagnostic markers and differential diagnoses: small lesions are soft and red, whereas larger lesions are firm and fixed to the underlying tissue. [4] [6] Diagnostic markers such as CD31, CD34, factor VIII-related antigen, Fli-1, and ERG positivity are typically expressed in angiosarcoma. [6] [7] Atypical fibroxanthoma is a type of skin cancer that can mimic the appearance of angiosarcoma on imaging studies. [7] [13] The differential diagnosis has a broad spectrum starting from myxoma to thrombus, metastasis, lymphoma, and rhabdomyosarcoma. [13] [14] Of these, about 20% are malignant and include angiosarcoma, rhabdomyosarcoma, and mesothelioma, with angiosarcoma being the most common. [14]