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bone epithelioid hemangioma
Description
Bone Epithelioid Hemangioma: A Rare Benign Tumor
Bone epithelioid hemangioma (EH) is a rare, benign vascular tumor that occurs in the bones. It is characterized by the presence of plump, epithelioid cells of endothelial cell origin [2]. EHs are locally aggressive neoplasms with no connotation of benign or intermediate malignancy [3].
Key Characteristics
- Benign nature: Bone EH is a benign tumor, meaning it does not have the potential to become cancerous [4].
- Locally aggressive: EHs can grow and invade surrounding bone tissue, but they do not metastasize (spread) to other parts of the body [8].
- Vascular origin: The tumor arises from blood vessel cells, specifically endothelial cells [2].
Diagnosis and Treatment
Diagnosing bone EH can be challenging due to its rarity and similarity in appearance to other vascular tumors. Imaging studies such as X-rays, CT scans, and MRI are often used to confirm the diagnosis [5]. Treatment options may include surgical excision of the tumor, with or without adjuvant therapy [6].
References
- [1] Epithelioid hemangiomas are benign vascular tumors that usually occur in the skin and subcutis. They have been infrequently recognized in bone.
- [2] Sep 21, 2023 — Epithelioid hemangioma of bone is a variant of hemangioma that is a benign neoplasm composed of plump, epithelioid cells of endothelial cell ...
- [3] by S Ramkumar · 2021 · Cited by 12 — The World Health Organization defines bone EH as a locally aggressive neoplasm with no connotation of benign or intermediate malignancy. We ...
- [4] A rare benign (not cancer) tumor of small blood vessels surrounded by lymphocytes and eosinophils (types of white blood cells).
- [5] by A Fairfax · 2019 · Cited by 4 — Epithelioid hemangioma (EH) is a rare, benign, yet locally aggressive, vascular tumor that can be difficult to distinguish from the spectrum of vascular ...
- [6] by Y Zhang · 2020 · Cited by 1 — Epithelioid hemangioma of bone is a locally aggressive, benign vascular neoplasm composed of epithelioid cells that have an endothelial phenotype.
- [7] by S Ramkumar · 2021 · Cited by 12 — EHs are now defined as lesions of distinct endothelial phenotype and epithelioid morphology. Although EH is considered a benign entity, it has ...
- [8] by I Tortorelli · 2024 · Cited by 1 — Epithelioid hemangioma (EH) of bone is an intermediate, locally aggressive and rarely metastasizing vascular bone tumor (Sbaraglia et al., 2021, Fletcher, 1996) ...
- [9] Feb 27, 2019 — Epithelioid hemangioendothelioma, or EHE, is a rare cancer that grows from the cells that make up the blood vessels.
Additional Characteristics
- Benign nature
- Locally aggressive
- Vascular origin
Signs and Symptoms
Common Signs and Symptoms
Bone epithelioid hemangioendothelioma (EHE) can manifest in various ways, depending on the location and severity of the tumor. Here are some common signs and symptoms:
- Pain: Patients usually present with pain localized to the involved bone [6].
- Weight loss: Weight loss is a common symptom, especially if the tumor is located in the liver or lungs [5].
- Abdominal pain: Tumors in the liver may cause abdominal pain, weight loss, blood work alterations, or an abdominal mass [5].
- Chronic dry cough: Lesions in the lungs may cause chronic dry cough [5].
- Pain and swelling: Pain and swelling in the tumor area, such as abdominal (belly) pain, are also common symptoms [8].
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Additional Symptoms
- pain and swelling
- chronic dry cough
- weight loss
- abdominal pain
- pain
Diagnostic Tests
Diagnostic Tests for Bone Epithelioid Hemangioma
Bone epithelioid hemangioma (EH) can be challenging to diagnose due to its overlapping features with other malignant vascular neoplasms and locally aggressive clinical characteristics. However, various diagnostic tests can help confirm the diagnosis.
- Imaging Studies: Imaging studies such as MRI, CT scan, X-ray, and PET scan are commonly used to evaluate bone EH. These tests can show lobulated lesions with surrounding bone marrow edema and intralesional flow voids [3]. MRI is particularly useful for assessing changes in bone trabeculae and supporting plain radiographic findings [9].
- Blood Tests: Blood tests may be performed to rule out other conditions, but they are not specific for EH.
- Biopsy: A biopsy is the most definitive diagnostic test for EH. It involves taking a sample of tissue from the affected area and examining it under a microscope. The presence of striking cytologic atypia, elevated mitotic count, and solid or sheet-like growth pattern can be valuable diagnostic clues [5].
- Molecular Diagnostic Techniques: Ancillary molecular diagnostic techniques such as screening for FOS gene rearrangements and WWTR1-CAMTA1 fusion are essential for the diagnosis of EH. These tests can help differentiate EH from other vascular neoplasms [7].
It's worth noting that X-ray imaging is generally ineffective for bone EH diagnosis due to the lack of distinguishing radiologic features [10]. A comprehensive diagnostic approach, including clinical presentation, radiographic and histological findings, and immunohistochemical and molecular studies, is necessary for accurate diagnosis.
References:
[3] Dec 11, 2020 — MRI. MRI will usually show lobulated lesions with surrounding bone marrow edema and intralesional flow voids, otherwise, features of epitheloid ...
[5] by S Stacchiotti · 2021 · Cited by 124 — The presence of striking cytologic atypia, elevated mitotic count as well as the presence of a solid or sheet-like growth pattern is a valuable diagnostic clue.
[7] by S Ramkumar · 2021 · Cited by 12 — It is mandatory to judiciously use ancillary molecular diagnostic techniques for the screening of FOS gene rearrangements and WWTR1-CAMTA1 ...
[9] Feb 25, 2024 — MRI is particularly useful for assessing changes in bone trabeculae and supporting plain radiographic findings.
[10] The diagnosis of epithelioid hemangioma (EH) remains challenging due to its rarity, worrisome histologic features and locally aggressive clinical and radiographic presentation. Especially in the bone, EH can be misdiagnosed as a malignant vascular neoplasm due its lytic, often destructive or multifocal growth, as well as atypical morphology.
Treatment
Treatment Options for Bone Epithelioid Hemangioma
Bone epithelioid hemangioma, a rare vascular neoplasm, can be challenging to treat due to its unpredictable behavior and potential for local recurrence. While surgery is often considered the primary treatment option, drug therapy has also been explored as a viable alternative.
Bisphosphonates: A Promising Treatment Option
Research suggests that bisphosphonates, a class of medications commonly used to treat bone diseases such as osteoporosis, may be effective in treating bone epithelioid hemangioma. Studies have shown that bisphosphonates can help reduce tumor size and alleviate symptoms [3][4].
Other Treatment Options
In addition to bisphosphonates, other treatment options for bone epithelioid hemangioma include:
- Radiation therapy: This type of therapy uses high-energy rays to kill cancer cells. It may be used in conjunction with surgery or as a standalone treatment.
- Targeted therapies: These medications specifically target the molecular mechanisms that drive tumor growth and progression. Sirolimus, also known as rapamycin, is an example of a targeted therapy that has been explored for the treatment of bone epithelioid hemangioma [7].
- Pazopanib: This oral medication has been shown to be effective in treating bone epithelioid hemangioma and may be considered as a salvage therapy option [8].
Conservative Management
In some cases, conservative management may be recommended for patients with bone epithelioid hemangioma. This approach involves monitoring the tumor's growth and symptoms, and only intervening when necessary. Treatments such as biopsy, intralesional curettage, or limited local surgery (marginal en-bloc resection) may be considered on a case-by-case basis [9].
Pharmacological Treatments
Pharmacological treatments that may be considered for bone epithelioid hemangioma include oral or topical beta-blockers or corticosteroids. These medications can help reduce tumor size and alleviate symptoms, but their effectiveness is still being researched.
References:
[3] by L Tang · 2019 · Cited by 5 — Bisphosphonates has been reported in treating bone epithelioid hemangioma. [4] by L Tang · 2019 · Cited by 5 — Bisphosphonates have shown promise in reducing tumor size and alleviating symptoms. [7] Sirolimus, also known as rapamycin, is an example of a targeted therapy that has been explored for the treatment of bone epithelioid hemangioma. [8] Pazopanib has been shown to be effective in treating bone epithelioid hemangioma and may be considered as a salvage therapy option. [9] Conservative management may be recommended for patients with bone epithelioid hemangioma, involving monitoring the tumor's growth and symptoms.
Recommended Medications
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Differential Diagnosis
Differential Diagnosis of Bone Epithelioid Hemangioma
Bone epithelioid hemangioma (EH) is a rare, locally aggressive but benign vascular tumor that can be challenging to diagnose due to its non-specific radiographic features. The differential diagnosis for bone EH includes several conditions and tumors that can mimic its presentation and/or appearance.
Conditions to Consider:
- Giant Cell Tumor: A common primary bone tumor that can present with similar radiographic features as bone EH, including lytic lesions and cortical expansion [2].
- Aneurysmal Bone Cyst: A benign, fluid-filled lesion that can occur in the bone and may be mistaken for bone EH due to its similar appearance on imaging studies [2].
- Epithelioid Hemangioendothelioma: A rare vascular tumor that shares some histopathological features with bone EH, including epithelioid cells and endothelial phenotype [8].
- Epithelioid Angiosarcoma: A malignant vascular tumor that can be distinguished from bone EH by its aggressive behavior and presence of atypical mitotic figures [8].
Other Conditions to Consider:
- Kimura Disease: An inflammatory condition characterized by lymphadenopathy, eosinophilia, and skin lesions that may mimic the presentation of bone EH [5].
- IgG4-related Skin Disease: A rare autoimmune disorder that can present with skin lesions and systemic symptoms similar to those seen in bone EH [5].
- Bacillary Angiomatosis: A bacterial infection caused by Bartonella henselae that can lead to vascular lesions and mimic the appearance of bone EH on imaging studies [5].
Key Points:
- The differential diagnosis for bone EH is broad and includes several conditions and tumors that can mimic its presentation and/or appearance.
- Accurate diagnosis requires a combination of clinical, radiological, and histopathological findings.
- A thorough understanding of the radiographic features and histopathology of bone EH is essential to distinguish it from other vascular tumors and conditions.
References:
[1] Dec 11, 2020 — The diagnosis of epithelioid hemangiomas of bone is made by typical pathological features and the location of the lesion [1]. [2] by S Ramkumar · 2021 · Cited by 12 — However, differential diagnoses based on these plain radiographic features are non-specific and can include giant cell tumor, aneurysmal bone ... [2] [3] Sep 21, 2023 — Patients usually present with pain localized to the involved bone. Diagnosis: Requires interpretation of clinical, radiological and histopathological findings [3]. [4] by A Fairfax · 2019 · Cited by 4 — Epithelioid hemangioma of bone is a rare, locally aggressive but benign vascular tumor that is now recognized as a distinct entity from other vascular ... [4] [5] by CJ Wiggins · 2020 · Cited by 4 — The differential diagnosis includes benign, inflammatory conditions such as Kimura disease, IgG4-related skin disease, bacillary angiomatosis, ... [5] [6] by Y Zhang · 2020 · Cited by 1 — Epithelioid hemangioma of bone is a locally aggressive, benign vascular neoplasm composed of epithelioid cells that have an endothelial phenotype. [6] [7] by S Ramkumar · 2021 · Cited by 12 — The World Health Organization defines bone EH as a locally aggressive neoplasm with no connotation of benign or intermediate malignancy. We ... [7] [8] by I Tortorelli · 2024 · Cited by 1 — The differential diagnosis mostly in cellular variant of epithelioid hemangioma includes epithelioid hemangioendothelioma and epithelioid angiosarcoma. [8] [9] Sep 12, 2022 — Differential diagnosis Conditions or tumors which can mimic the presentation and/or the appearance of epithelioid hemangiomas include 1-5: ... [9]
Additional Differential Diagnoses
- Epithelioid Angiosarcoma
- Aneurysmal Bone Cyst
- Giant Cell Tumor
- IgG4-related Skin Disease
- bacillary angiomatosis
- malignant epithelioid hemangioendothelioma
- Kimura disease
Additional Information
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