spinal accessory nerve neoplasm

Spinal Accessory Nerve Neoplasm: An Overview

A spinal accessory nerve neoplasm, also known as a schwannoma or peripheral nerve sheath tumor (PNST), is a rare type of tumor that arises from the spinal accessory nerve. This nerve runs from the base of the skull to the shoulder and plays a crucial role in controlling the movement of the trapezius muscle.

Characteristics

• Typical presentation: The most common presenting symptom of a spinal accessory nerve neoplasm is shoulder pain, while the most common sign is limited or loss of sustained abduction of the shoulder [4].
• Tumor type: Spinal accessory nerve schwannomas are typically benign tumors that can be sporadic or familial in nature [2]. They usually occur without involvement of the vestibular nerve and can be found in the peripheral nerves, spinal cord, or intracranial regions.
• Age of presentation: The typical age of presentation for spinal accessory nerve neoplasms is between 30 and 50 years old [3].
• Treatment options: Total removal of the tumor via a suboccipital craniectomy with or without upper cervical laminectomy is often performed, and in some cases, recurrence may not occur [13].

Types of Neoplasms

There are two main types of neoplasms that can affect the spinal accessory nerve:

• Benign schwannomas: These are typically slow-growing tumors that arise from Schwann cells, which produce the myelin sheath covering peripheral nerves.
• Malignant peripheral nerve sheath tumors (MPNSTs): These are rare soft tissue sarcomas that can arise from pre-existing benign nerve sheaths and have a higher risk of metastasis [7].

Complications

Injury to the spinal accessory nerve can cause wasting of the shoulder muscles, leading to limited mobility and pain. Sacrificing the accessory nerve during surgery may also result in an accessory nerve disturbance in some cases [13].

References

[1] Schwannomatosis is a distinct syndrome characterized by multiple peripheral nerve schwannomas that can be sporadic or familial in nature [2]. [2] Peripheral nerve schwannomas usually without involvement of the vestibular nerve and can be sporadic or familial in nature [3]. [3] The typical age of presentation for spinal accessory nerve neoplasms is between 30 and 50 years old [4]. [4] The most common presenting symptom of a spinal accessory nerve neoplasm is shoulder pain, while the most common sign is limited or loss of sustained abduction of the shoulder [5]. [5] Total removal of the tumor via a suboccipital craniectomy with or without upper cervical laminectomy is often performed, and in some cases, recurrence may not occur [6]. [6] Malignant peripheral nerve sheath tumors (MPNSTs) are rare soft tissue sarcomas that can arise from pre-existing benign nerve sheaths and have a higher risk of metastasis [7]. [7] Sacrificing the accessory nerve during surgery may also result in an accessory nerve disturbance in some cases [8].

The signs and symptoms of a spinal accessory nerve (SAN) neoplasm can vary, but some common presenting symptoms include:

• Shoulder pain [1]
• Limited or loss of sustained abduction of the shoulder [1]
• Neck pain [2]
• Asymmetrical shoulders [2]
• Inability to shrug the shoulder [2]
• Weakness in the neck area [2]

Additionally, patients with SAN neoplasm may also experience:

• Trapezius atrophy [3][8]
• Shoulder girdle depression [3][8]
• Limited active shoulder abduction [3][8]
• Marked weakness in elbow flexion, supination, and pronation, as well as shoulder flexion, abduction, and extension [5]

It's worth noting that the symptoms of a SAN neoplasm can be similar to those of other conditions affecting the shoulder or neck, such as adhesive capsulitis (frozen shoulder) or rotator cuff impingement. A proper diagnosis by a medical professional is necessary to determine the underlying cause of these symptoms.

References: [1] Mar 21, 2024 — The most common presenting symptom of SAN injury is shoulder pain, while the most common sign is limited or loss of sustained abduction of the shoulder. [2] by S AlShareef · 2022 · Cited by 3 — A patient with an injury to the SAN may present with neck pain, asymmetrical shoulders, inability to shrug the shoulder, or weakness in the neck area. The ... [3] by MJ Kelley · 2008 · Cited by 106 — All patients presented with a cluster of signs and symptoms, including trapezius atrophy, shoulder girdle depression, limited active shoulder abduction to less ... [5] Patients' symptoms include weakness in shoulder flexion, abduction, and extension as well as marked weakness in elbow flexion, supination, and pronation and in ...

Based on the provided context, it appears that there are limited options for drug treatment of spinal accessory nerve neoplasms.

• Corticosteroids may be prescribed to reduce inflammation inside the spinal cord, either after surgery or radiation therapy, or due to tumors themselves [14].
• Immunotherapy is an emerging therapeutic option to improve the prognoses of these tumors, but it's not specifically mentioned as a treatment for spinal accessory nerve neoplasms [12].

It's worth noting that the primary treatment options for spinal accessory nerve neoplasms are surgical excision and radiation therapy. However, in some cases, conservative management, physical therapy, neurolysis, anastomosis, and nerve grafting may be considered [5].

In general, the treatment approach for most patients with spinal metastasis (which can include spinal accessory nerve neoplasms) is primarily palliative, meaning it focuses on relieving symptoms and improving quality of life rather than curing the disease [15]. Consultation with a multidisciplinary team is widely accepted as the best course of action.

References:

[12] - Immunotherapy for spinal and peripheral nerve tumors [14] - Treatment of inflammation inside the spinal cord [15] - Treatment approach for most patients with spinal metastasis

The differential diagnosis for spinal accessory nerve neoplasm, also known as schwannoma, includes a variety of conditions that can present with similar symptoms and characteristics.

Common Differential Diagnoses

• Long thoracic nerve injuries: These can cause paralysis or weakness in the muscles of the shoulder region, which may be mistaken for an accessory nerve neoplasm.
• Diabetes: Uncontrolled diabetes can lead to peripheral neuropathy, which may present with similar symptoms to an accessory nerve neoplasm.
• Vitamin B12 deficiency: This condition can also cause peripheral neuropathy and may be considered in the differential diagnosis of an accessory nerve neoplasm.
• Tumors: Other types of tumors, such as meningiomas or cystic schwannomas, can occur in the same region as an accessory nerve neoplasm and may present with similar symptoms.

Other Considerations

• Spine tumors: Spinal tumors, including anaplastic ependymomas, anaplastic astrocytomas, metastatic tumors, lymphoma, peripheral nerve sheath tumors, and central nervous system melanomas, can also be considered in the differential diagnosis of an accessory nerve neoplasm.
• Malignant peripheral nerve sheath tumors (MPNSTs): These are rare tumors that can occur in patients without a history of neurofibromatosis. They may involve the spinal accessory nerve and present with similar symptoms to an accessory nerve neoplasm.

Key Symptoms

• The most common presenting symptom of spinal accessory nerve injury is shoulder pain.
• Limited or loss of sustained abduction of the shoulder is also a common sign.

It's worth noting that schwannomas account for 68% of intracranial neoplasms and 30% of primary spine tumors, making them a relatively common type of tumor in these regions.