obsolete malignant bone vascular tumor

Hemangioendothelioma: An Obsolete Malignant Bone Vascular Tumor

Hemangioendothelioma, also known as epithelioid hemangioendothelioma (EHE), is a rare and malignant vascular tumor that was once considered a distinct entity. However, it has been replaced by a broader spectrum of different vascular tumor entities [6].

Characteristics and Behavior

Hemangioendothelioma was characterized by its potential to be locally aggressive and rarely metastatic. It was often associated with multiple synchronous lesions, making it a part of the group of tumors that exhibit this feature [1][3].

Comparison with Other Vascular Tumors

In contrast to other vascular tumors like angiosarcoma, hemangioendothelioma had a relatively lower malignant potential and metastatic rate. However, its behavior was still considered aggressive compared to benign vascular tumors [5].

Current Understanding and Classification

The term "hemangioendothelioma" is no longer used in modern pathology, as it has been subsumed by more specific and accurate classifications of vascular tumors. The current understanding of these tumors emphasizes their heterogeneity and the need for precise diagnosis and treatment [6][9].

References:

• [1] Oct 23, 2020 — This group of tumors is characterized by the frequent presence of multiple synchronous lesions and includes different histopathologic entities ...
• [3] by E Palmerini · 2020 · Cited by 32 — This group of tumors is characterized by the frequent presence of multiple synchronous lesions and includes different histopathologic entities ...
• [5] by H Yamashita · 2012 · Cited by 10 — Angiosarcoma of bone is a rare malignant neoplasm and represents the most malignant end of the spectrum of vascular tumors. Approximately 6% of ...
• [6] by S Boriani · 2019 · Cited by 9 — The term Hemangioendothelioma is now obsolete: It is replaced by a broad spectrum of different vascular tumor entities, with relative ...
• [9] Sep 16, 2024 — Vascular tumors are proliferative tumors that can be benign or malignant. Growth and/or expansion of vascular tumors can cause clinical ...

Diagnostic Tests for Obsolete Malignant Bone Vascular Tumors

Obtaining an accurate diagnosis for malignant bone vascular tumors can be challenging due to their rarity and histologic overlap with other mimics. However, various diagnostic tests can help healthcare professionals identify these tumors.

• Imaging Tests: Imaging tests play a crucial role in diagnosing bone cancer by providing detailed images of the affected areas and helping healthcare professionals identify potential tumors. These tests include:
  • X-rays: A chest radiograph should be requested if malignant disease is suspected [12].
  • CT scans, MRI scans, and bone scans: These tests are essential tools in the diagnostic process, enabling early detection and appropriate treatment [7].
• Biopsy: If imaging tests are not adequate to diagnose your tumor, you may require a biopsy. A piece of tissue might be removed and tested for cancer cells [15].
• Molecular Ancillary Tests: Molecular ancillary tests have long been needed for a more objective diagnosis and classification of malignant vascular tumors, particularly within the epithelioid phenotype [11].

Understanding the ISSVA Classification

The International Society for the Study of Vascular Anomalies (ISSVA) classification is a comprehensive histology-based scheme that was updated in 2018. It is essential for cardiothoracic imagers to understand this classification to ensure accurate terminology and avoid archaic terms when describing vascular lesions [6].

Pathological Classification

Pathologically, vascular tumors show increased proliferation rates of endothelial and vascular cells and are classified as benign, locally aggressive or borderline, and malignant [8]. Some tumors may be congenital and present at birth.

These diagnostic tests can help healthcare professionals accurately diagnose obsolete malignant bone vascular tumors. However, it is essential to note that each case may require a tailored approach, taking into account the patient's medical history, physical exam, and imaging results.

References: [6] The International Society for the Study of Vascular Anomalies (ISSVA) classification. [7] Overall, imaging tests play a crucial role in diagnosing bone cancer by providing detailed images of the affected areas and helping healthcare professionals identify potential tumors. [8] Pathologically, vascular tumors show increased proliferation rates of endothelial and vascular cells and are classified as benign, locally aggressive or borderline, and malignant (3,5,7). [11] Molecular ancillary tests have long been needed for a more objective diagnosis and classification of malignant vascular tumors, particularly within the epithelioid phenotype. [12] A chest radiograph should be requested if malignant disease is suspected. [15] If these tests are not adequate to diagnose your tumor, you may require a biopsy.

Treatment Options for Malignant Bone Vascular Tumors

Malignant bone vascular tumors are rare and aggressive cancers that can be challenging to treat. While there is no cure for these tumors, various treatment options are available to manage the disease and improve quality of life.

• Chemotherapy: Chemotherapy is a common treatment approach for malignant bone vascular tumors. It involves using medications to kill cancer cells or stop them from dividing. The chemotherapy drugs used to treat these tumors include doxorubicin, high-dose methotrexate, cisplatin, and ifosfamide [2].
• Targeted Therapy: Targeted therapy is a type of treatment that uses medications to specifically target cancer cells. For malignant bone vascular tumors, targeted therapies such as denosumab have shown promise in treating patients with malignancy bone metastases [8].
• Bisphosphonates: Bisphosphonates are a group of drugs that help strengthen bones and can be used to treat some benign tumors. However, they may cause adverse effects such as atypical femoral fractures and esophageal cancer after long-term use [13].

Emerging Treatments

Newer treatments for malignant bone vascular tumors include:

• PI3K Inhibitors: A 2018 study reported promising level C evidence for the use of the PI3K inhibitor BYL719 (alpelisib) to treat patients who have lesions with a specific genetic mutation [7].
• Anlotinib: Anlotinib has demonstrated promising antitumor activities in recurrent or metastatic primary malignant bone tumors with manageable adverse effects [5].

Fertility Preservation

For young patients undergoing treatment for malignant bone and soft tissue tumors, fertility preservation is crucial. This includes preserving sperm prior to the initiation of treatment, which can help maintain reproductive health [11].

References:

[1] Righi et al., 2020 - Vascular tumors of bone are extremely rare, accounting for ∼1% to 2% of all bone neoplasms.

[2] by AE Bădilă · 2021 · Cited by 56 — The current chemotherapy possibilities include the usage of doxorubicin, high-dose methotrexate, cisplatin, and ifosfamide.

[5] Anlotinib has demonstrated promising antitumor activities in recurrent or metastatic primary malignant bone tumors with manageable adverse effects.

[7] A 2018 study reported promising level C evidence for the use of the PI3K inhibitor BYL719 (alpelisib) to treat patients who have lesions with a specific genetic mutation.

[8] Denosumab has shown promise in treating patients with malignancy bone metastases.

[11] Fertility preservation is crucial for young patients undergoing treatment for malignant bone and soft tissue tumors.

[13] Bisphosphonates may cause adverse effects such as atypical femoral fractures and esophageal cancer after long-term use.

The differential diagnosis for malignant vascular tumors of bone (MVTB) is rather broad and can be challenging due to their rarity and histologic overlap with diverse mimics.

According to the literature, the differential diagnosis for MVTB includes:

• Hemangioendothelioma: This is a type of benign tumor that can mimic the appearance of malignant vascular tumors. It is characterized by its endothelial differentiation and variable capacity to form vessels.
• Angiosarcoma: This is a highly aggressive and malignant tumor that can be difficult to distinguish from MVTB. It is characterized by its rapid growth and potential for metastasis.
• Epithelioid hemangioendothelioma (EHE): This is a rare and intermediate-grade tumor that can mimic the appearance of MVTB. It is characterized by its epithelioid morphology and variable capacity to form vessels.

Other conditions that may be considered in the differential diagnosis for MVTB include:

• Osteosarcoma: A type of primary malignant bone tumor that can be difficult to distinguish from MVTB.
• Ewing sarcoma: A highly aggressive and malignant tumor that can be challenging to diagnose due to its rarity and histologic overlap with other conditions.

It's worth noting that the differential diagnosis for MVTB can vary depending on the age of the patient, with different conditions being more likely in children versus adults.