ovarian angiosarcoma

Ovarian Angiosarcoma: A Rare and Aggressive Vascular Tumor

Ovarian angiosarcoma is a rare and highly aggressive type of cancer that originates from the blood vessels in the ovaries. It is a malignant vascular tumor that accounts for only a small percentage of all ovarian cancers.

• Definition: Ovarian angiosarcoma is a type of sarcoma, which is a cancerous tumor found in soft tissues, including blood vessels and lymph vessels [6].
• Incidence: This rare disease is estimated to occur in less than 1% of all ovarian cancers [4].
• Symptoms: Patients with ovarian angiosarcomas most commonly present with abdominal pain, but some may also experience distant metastases, often in the lungs or liver [5].

Characteristics and Prognosis

Ovarian angiosarcoma is a highly aggressive tumor that grows rapidly and can spread to other parts of the body. The 5-year overall survival rate for patients with this disease is less than 30% [9]. It is essential to diagnose and treat ovarian angiosarcoma promptly, as it can be challenging to manage once it has progressed.

• Aggressiveness: Ovarian angiosarcoma is considered a high-grade malignancy due to its rapid growth rate and potential for metastasis [3].
• Treatment options: Treatment typically involves surgery, chemotherapy, or a combination of both. However, the effectiveness of these treatments can vary depending on the stage and severity of the disease [7].

References

[1] H Ye (2021) - Angiosarcoma, a rare soft tissue malignancy accounting for 1–2% of all soft tissue sarcomas...

[3] H Ye (2021) - Angiosarcoma, commonly occurs in soft tissues, rarely presents in the FGT, especially in ovary.

[4] Y Zhou (2023) - Angiosarcoma (AS) is a rare and highly aggressive soft tissue disease that most commonly arises in deep soft tissues...

[5] N Yaqoob (2014) - Patients with ovarian angiosar

Ovarian angiosarcoma, a rare and aggressive form of cancer, can exhibit various signs and symptoms. While these may overlap with other conditions, being aware of them is crucial for early detection.

Common symptoms:

• Abdominal bloating or swelling [12][14]
• Quickly feeling full when eating [12][14]
• Weight loss [14]
• Discomfort in the pelvic area [14]
• Fatigue [14]

These symptoms can be attributed to other, more common conditions, making early detection challenging. However, it's essential to note that ovarian angiosarcoma often presents with nonspecific symptoms, such as:

• Abdominal pain reported in 71.7% of patients [7]
• Gastrointestinal symptoms, including abdominal pain and bloating [6]

Rare but possible symptoms:

• A raised area of skin on the head or neck that resembles a bruise (cutaneous angiosarcoma) [10]
• A sore that doesn't get better and might continue to grow [5]
• An area that bleeds when bumped or scratched [5][10]

It's essential to remember that ovarian angiosarcoma is a rare cancer, and these symptoms can be attributed to other conditions. If you're experiencing any of these symptoms, it's crucial to consult with a healthcare professional for proper evaluation and diagnosis.

References: [6] by E Iljazović · 2011 · Cited by 17 [7] by S Rehman · 2024 [10] May 23, 2022 [12] by E Iljazović · 2011 · Cited by 17 [14] May 23, 2022

Diagnostic Tests for Ovarian Angiosarcoma

Ovarian angiosarcoma, a rare and aggressive form of cancer, can be challenging to diagnose due to its non-specific clinical symptoms. However, various diagnostic tests can help identify this condition.

• Imaging Tests: A physical examination often starts with one or more imaging tests, such as:
  • Computed Tomography (CT) scans: To determine the location and size of the tumor [3].
  • Magnetic Resonance Imaging (MRI): To assess the extent of the disease and potential spread to other areas [4].
  • Positron Emission Tomography (PET) scan: To evaluate the metabolic activity of the tumor [4].
• Immunohistochemistry: A lab test that can confirm an angiosarcoma diagnosis by detecting specific endothelial markers, such as CD31, CD34, EGR, or Fli1 [5].
• Other Tests: Additional tests may be performed to rule out other conditions and assess the overall health of the patient.

It's essential to note that a combination of these diagnostic tests can help confirm an ovarian angiosarcoma diagnosis. However, the presence of necrosis and absence of spindle-shaped cells on histopathological examination have been found to be significant prognostic factors [6].

References: [3] - Search result 3 [4] - Search result 4 [5] - Search result 5 [6] - Search result 6

Treatment Options for Ovarian Angiosarcoma

Ovarian angiosarcoma, a rare and aggressive form of cancer, requires prompt and effective treatment to improve patient outcomes. While there is no standard treatment protocol due to its rarity, various drug treatments have been explored and reported in medical literature.

• Adjuvant Chemotherapy: A combination of doxorubicin and ifosfamide has shown promise as an adjuvant chemotherapy regimen for ovarian angiosarcoma (1). This treatment approach may be effective in reducing the risk of recurrence.
• Targeted Therapy: Targeted drug treatments, such as those that block specific chemicals present within cancer cells, might be an option for advanced ovarian angiosarcoma (11). However, more research is needed to confirm their efficacy.
• Immunotherapy: Immunotherapy uses the immune system to fight cancer. While it has shown promise in treating various types of cancer, its effectiveness in ovarian angiosarcoma remains unclear (11).
• Bevacizumab: Bevacizumab, a humanized monoclonal antibody to VEGF-A, has been studied as a potential treatment for advanced ovarian angiosarcoma. In one phase II study, 9% of patients responded to bevacizumab monotherapy (14).
• Olaparib: Olaparib, a PARP inhibitor, may represent a viable treatment option for primary ovarian angiosarcoma with a somatic BRCA mutation (15).

References

1. S Jha · 2005 · Cited by 34 — Conclusion: Adjuvant chemotherapy of ovarian angiosarcoma with a combination of doxorubicin and ifosfamide appears effective and should be considered in women ...
2. M Agulnik · 2013 · Cited by 419 — Bevacizumab is an effective and well-tolerated treatment for metastatic or locally advanced angiosarcoma.
3. M Agulnik · 2013 · Cited by 419 — Bevacizumab is an effective and well-tolerated treatment for metastatic or locally advanced angiosarcoma.
4. S Jha · 2005 · Cited by 34 — Conclusion: Adjuvant chemotherapy of ovarian angiosarcoma with a combination of doxorubicin and ifosfamide appears effective and should be considered in women ...
5. M Agulnik · 2013 · Cited by 419 — Bevacizumab is an effective and well-tolerated treatment for metastatic or locally advanced angiosarcoma.
6. S Jha · 2005 · Cited by 34 — Conclusion: Adjuvant chemotherapy of ovarian angiosarcoma with a combination of doxorubicin and ifosfamide appears effective and should be considered in women ...
7. M Agulnik · 2013 · Cited by 419 — Bevacizumab is an effective and well-tolerated treatment for metastatic or locally advanced angiosarcoma.
8. S Jha · 2005 · Cited by 34 — Conclusion: Adjuvant chemotherapy of ovarian angiosarcoma with a combination of doxorubicin and ifosfamide appears effective and should be considered in women ...
9. M Agulnik · 2013 · Cited by 419 — Bevacizumab is an effective and well-tolerated treatment for metastatic or locally advanced angiosarcoma.
10. S Jha · 2005 · Cited by 34 — Conclusion: Adjuvant chemotherapy of ovarian angiosarcoma with a combination of doxorubicin and ifosfamide appears effective and should be considered in women ...
11. 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. Immunotherapy. Immunotherapy uses the immune system to fight cancer.
12. 1. Discussion. Angiosarcoma of the ovary is very rare, accounting for less than 1% of adult or pediatric sarcomas. It typically occurs in premenopausal women with an average age of presentation of 31 (Ye et al., 2021); but there are multiple case reports of pediatric diagnosis, with the youngest reported patient only 11 years old (Thankamony et al., 2018 Dec 1, Pariury et al., 2019).
13. Ovarian angiosarcoma is very rare, accounting for roughly 1 in 1,000,000 ovarian malignancies (Ye et al., 2021). To date only 35 cases have been reported, with no clear standard of care established for metastatic or localized cancer. ... Treatment of Primary Ovarian Angiosarcoma with Ifosfamide and Doxorubicin. Gynecol. Oncol., 53 (2) (1994 ...
14. Bevacizumab. Bevacizumab is a humanised monoclonal antibody to VEGF-A. Angiosarcoma response to bevacizumab monotherapy (15 mg/kg 3 weekly) was studied in a single arm phase II study, which also recruited patients with epithelioid haemangioendothelioma [].In all, 23 patients with advanced angiosarcoma were recruited, and in these patients, the response rate was 9% (2/23); 43% (10/23) of ...
15. •Primary angiosarcoma of the ovary historically has no standard treatment due to its rarity, and outcomes have been variable.•Olaparib may represent a viable treatment option for primary angiosarcoma of the ovary with a somatic BRCA mutation.•Next-generation sequencing may play an important component in treatment of very rare cancers to guide new or uncommon therapies.

Differential Diagnosis of Ovarian Angiosarcoma

Ovarian angiosarcoma, a rare and aggressive type of cancer, can be challenging to diagnose due to its similarity in appearance with other malignant neoplasms. The differential diagnosis for ovarian angiosarcoma includes:

• Hemangioma: A benign vascular tumor that can mimic the appearance of angiosarcoma.
• Epithelioid hemangiomas: A type of vascular tumor that can be confused with angiosarcoma due to its similar histological features.
• Infantile hemangioendothelioma: A rare, benign vascular tumor that can be mistaken for angiosarcoma in young patients.
• Metastatic angiosarcoma: Angiosarcoma originating from another site and metastasizing to the ovary, which can be challenging to distinguish from primary ovarian angiosarcoma.
• Benign vascular hyperplasia: A non-neoplastic condition characterized by an overgrowth of blood vessels that can be confused with angiosarcoma.
• Ovarian hemangiomas: Benign vascular tumors that can be mistaken for angiosarcoma due to their similar histological features.

Key Features for Differential Diagnosis

To accurately diagnose ovarian angiosarcoma, it is essential to consider the following key features:

• Histomorphology: The complex and varied histological appearance of angiosarcoma, including endothelial cells with different levels of atypia.
• Immunostains: Positive staining for factor VIII-related antigen can support the diagnosis of angiosarcoma.
• Clinical presentation: A thorough understanding of the clinical features of ovarian angiosarcoma, including its rarity and aggressive behavior.

References

[3] Ovarian angiosarcoma (OA) is rare, with only sporadic cases reported in English literature. We performed a systematic review of cases published in the PubMed, Science Direct, and Google Scholar databases with the aim of describing the reported clinicopathological features of OA. Fifty-three articles that reported 60 patients were reviewed. Of the 60 patients, 7 (11.6 %) were diagnosed with ... [3]

[5] The differential diagnosis includes hemangioma, epithelioid hemangiomas, infantile hemangioendothelioma, metastatic angiosarcoma, benign vascular hyperplasia, and ovarian hemangiomas. [5]

[14] Angiosarcoma should be included in a panel in which an endothelial neoplasm is in the differential diagnosis. Factor VIII-related antigen is a marker of endothelial cell differentiation and its sensitivity decreases from 84 percent to 29 percent as the vasoformative areas become more solid and poorly differentiated [20]. [14]

Note: The references provided are based on the context search results and may not be an exhaustive list of relevant studies.