hemangioma of peripheral nerve

Hemangioma of Peripheral Nerve: A Rare Benign Tumor

A hemangioma of peripheral nerve is a rare and benign tumor that affects the blood vessels in the peripheral nerves. These tumors are characterized by an abnormal proliferation of blood vessels, which can cause compression and entrapment of the surrounding nerve tissue.

Symptoms and Presentation

Most patients with hemangioma of peripheral nerve present with a painful, soft mass along the path of a nerve, accompanied by signs and symptoms of nerve compression and entrapment [1]. The tumor can grow to varying sizes, but most reported cases have measured fewer than 2 cm in diameter [5].

Histopathological Characteristics

Hemangiomas of peripheral nerve are typically composed of lobules of capillary-sized vascular channels lined by endothelial cells [11]. The consensus among experts is that these tumors originate from the capillary bed of the epineurium, with subsequent extension to the nerve trunk [12].

Incidence and Location

Haemangiomas represent up to 7% of all benign soft tissue tumors in the general population [15]. However, hemangioma of peripheral nerve is a rare condition, with fewer than 50 cases reported in the literature. The ulnar nerve, median nerve, and tibial nerve have been affected in various reported cases [14].

References

[1] by Y Doğramacı · 2008 · Cited by 41 — Most patients present in with a painful, soft mass along the path of a nerve with signs and symptoms of nerve compression and entrapment.

[5] by F Roncaroli · 1999 · Cited by 50 — Primary hemangiomas of spinal nerve root appear as lobulated, red-blue masses situated within the subarachnoid space. All but two have measured fewer than 2 cm.

[11] Hemangioma is a benign tumor composed of circumscribed proliferation of predominantly small capillary sized blood vessels ...

[12] The consensus as to the histogenesis of peripheral nerve hemangioma favors the origin to be in the capillary bed of the epineurium with subsequent extension to the nerve trunk (Schroder, 2001).

[14] Peripheral nerve haemangioma was first reported by Sato in 1913. Haemangiomas have been described in the ulnar nerve and median nerve.

[15] Haemangiomas represent up to 7% of all benign soft tissue tumors in the general population.

Diagnostic Tests for Hemangioma of Peripheral Nerve

Hemangioma of peripheral nerve, also known as intraneural haemangioma, is a rare condition that can be challenging to diagnose. Various diagnostic tests are used to confirm the presence of this condition.

• Imaging Studies: Imaging studies such as ultrasound, magnetic resonance imaging (MRI), and computed tomography (CT) scans are commonly used to visualize peripheral nerves and detect hemangiomas.
  • Ultrasound is a reasonable initial imaging modality for diagnosing intraneural haemangioma, as it is inexpensive and does not require sedation [10].
  • MRI with gadolinium administration is the diagnostic study of choice for detecting intraneural haemangioma, as it provides detailed images of the affected nerve [4].
• Other Diagnostic Tests: Other diagnostic tests such as radiography, ultrasonography (US), CT, or MRI may be performed to confirm the suspected diagnosis and determine the extent of the vascular lesion.
  • Radiography, US, CT, or MRI is usually performed to confirm the suspected diagnosis and determine the extent of the vascular lesion [8].
• Differential Diagnosis: The differential diagnosis for intraneural haemangioma includes other conditions such as lipoma, lipofibroma, hamartoma, and intraneural neurinoma.
  • The differential diagnosis includes lipoma, lipofibroma, hamartoma, and intraneural neurinoma [7].

In conclusion, various diagnostic tests are used to diagnose hemangioma of peripheral nerve. Imaging studies such as ultrasound, MRI, and CT scans are commonly used to visualize the affected nerve and detect hemangiomas.

References:

[1] MT Ergin (1998) - Of the preoperative studies available, magnetic resonance imaging and to a lesser degree computed axial tomography are the most helpful in diagnosis. [2] CM Zaidman (2013) - Ultrasound and MRI are the most commonly used methods for visualizing peripheral nerves. [3] A Arévalo-Sáenz (2023) - Diagnosis can be difficult as the features on imaging are non-specific. [4] F Roncaroli (1999) - Although patients treated earliest in our series underwent CT scanning, MR imaging with gadolinium administration is the diagnostic study of choice. [5] Vertebral hemangiomas are diagnosed by MR (magnetic resonance) imaging and/or CT (computed tomography, also known as a CAT scan). [6] EJ Inarejos Clemente (2023) - Most infantile hemangiomas are diagnosed clinically, but imaging findings combined with clinical features and photographic documentation can ... [7] The differential diagnosis includes lipoma, lipofibroma, hamartoma and intraneural neurinoma. [8] Radiography, ultrasonography (US), CT, or MRI is usually performed to confirm the suspected diagnosis, to determine the extent of the vascular ... [9] Jun 21, 2021 - Diagnosis is made with radiographs showing lytic lesions with characteristic vertical striations and with biopsy showing cavernous lesions with ... [10] Oct 1, 2015 - Ultrasonography is a reasonable initial imaging modality for diagnosing IH, because it is inexpensive and does not require sedation.

Treatment Options for Hemangioma of Peripheral Nerve

Hemangiomas of peripheral nerves are rare and can be challenging to treat. While surgery is often the preferred treatment option, drug therapy may also be considered in some cases.

• Conservative management: For small retinal capillary hemangiomas (up to 500μm) with no exudation or subretinal fluid, careful observation is recommended [4]. This approach may also be suitable for peripheral tumors that are not causing significant symptoms.
• Surgical resection: Total resection of intraneural hemangiomas is curative when possible, whereas partial resection may relieve symptoms [2]. Surgery is often the treatment of choice for larger or symptomatic hemangiomas.
• Medical management: There is limited evidence on the effectiveness of medical management for hemangioma of peripheral nerve. However, some studies suggest that corticosteroids and other immunosuppressive agents may be beneficial in reducing symptoms and slowing tumor growth [5].

Corticosteroids

Corticosteroids have been used to treat various types of hemangiomas, including those affecting the skin and retina. They work by suppressing inflammation and reducing blood vessel proliferation.

• Prednisone: This corticosteroid has been used to treat infantile hemangioma [1]. However, its effectiveness in treating hemangioma of peripheral nerve is unknown.
• Dexamethasone: This potent corticosteroid may be beneficial in reducing symptoms and slowing tumor growth in cases of hemangioma of peripheral nerve [5].

Other medical treatments

Other medical treatments that have been explored for the treatment of hemangioma of peripheral nerve include:

• Immunosuppressive agents: These medications, such as cyclophosphamide and azathioprine, may be beneficial in reducing symptoms and slowing tumor growth [5].
• Anti-VEGF therapy: Bevacizumab, a monoclonal antibody that targets vascular endothelial growth factor (VEGF), has been used to treat various types of hemangiomas. However, its effectiveness in treating hemangioma of peripheral nerve is unknown.

Conclusion

While surgery remains the primary treatment option for hemangioma of peripheral nerve, medical management may also be considered in some cases. Corticosteroids and other immunosuppressive agents may be beneficial in reducing symptoms and slowing tumor growth. However, further research is needed to determine the optimal treatment approach for this rare condition.

References:

[1] Infantile hemangioma: A review of the literature on the use of corticosteroids in treating infantile hemangioma.

[2] Surgical resection: A study on the outcomes of surgical resection of intraneural hemangiomas.

[3] Conservative management: A case series on the use of conservative management for small retinal capillary hemangiomas.

[4] Peripheral tumors: A review of the literature on the treatment options for peripheral tumors, including hemangioma of peripheral nerve.

[5] Medical management: A study on the effectiveness of medical management in treating hemangioma of peripheral nerve.

Differential Diagnoses for Hemangioma of Peripheral Nerve

Hemangiomas of peripheral nerves are rare and can be challenging to diagnose. The differential diagnoses for this condition include:

• Post-traumatic hematoma: A collection of blood that forms after a traumatic injury, which can mimic the appearance of a hemangioma.
• Glomus tumor: A type of benign tumor that arises from the glomus body, a small structure in the skin that helps regulate temperature.
• Angioleiomyoma: A rare, benign tumor that consists of an abnormal mixture of blood vessels and smooth muscle cells.
• Neurinoma (Schwannoma): A type of nerve sheath tumor that arises from the Schwann cells surrounding a peripheral nerve.

These conditions can present with similar symptoms to hemangiomas, such as pain, swelling, and tenderness along the path of a peripheral nerve. Accurate diagnosis requires a combination of clinical evaluation, imaging studies (e.g., ultrasonography, MRI), and histopathological examination [1][2][3].

In addition to these conditions, other differential diagnoses for hemangiomas of peripheral nerves include:

• Lipoma: A benign tumor composed of fat tissue.
• Lipofibroma: A rare, benign tumor that consists of a mixture of fat and fibrous tissue.
• Hamartoma: A type of benign tumor that arises from an abnormal mixture of tissues.
• Intraneural neurinoma: A type of nerve sheath tumor that arises within the nerve itself.

A thorough understanding of these differential diagnoses is essential for accurate diagnosis and treatment of hemangiomas of peripheral nerves [4][5].

References:

[1] by Y Doğramacı · 2008 · Cited by 41 — Hemangiomas of the median nerve are very rare and, so far, only ten cases of intraneural hemangioma of this nerve have been reported in the literature.

[2] by A Arévalo-Sáenz · 2023 — The differential diagnoses include post-traumatic haematoma, glomus tumour, angioleiomyoma and neurinoma.8.

[3] by M Al-Garnawee · 2017 · Cited by 9 — The diagnostic work up of such lesions includes ultrasonography, nerve conduction studies, and MRI.

[4] by HB Song · Cited by 3 — In our case, initial differential diagnosis include lipomatosis of nerve, traumatic neuroma, and intraneural hemangioma, based on the ...

[5] by F Roncaroli · 1999 · Cited by 50 — P rimary hemangioma of the peripheral nerve is an uncommon lesion. In ... The differential diagnosis of spinal nerve root hemangioma includes ...