Extramedullary primary spinal cord tumor

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The article was written by MSc Vu Duy Dung - Doctor of Neurology, Department of General Internal Medicine - Vinmec Times City International Hospital

1. Primary spinal cord tumors located extramedullary and intradural

Meningiomas, endothelial mucinous papillomas, and schwannomas (schwannomas, neurofibromas) account for the majority of extramedullary and intradural myeloma.
1.1 Meningiomas 1.1.1 What is medulloblastoma? Meningeal tumors are the most common tumors of the spinal axis in adults. Meningiomas account for 25% of primary spinal cord tumors, and about 8% to 12% of all meningiomas are located within the spine. In the spine, the most common site is the thoracic segment, followed by the neck, and least commonly the lumbar region. Most medulloblastomas are benign. Meningiomas arise from arachnoid cells and attach to the inner surface of the dura mater. Therefore, the majority of medulloblastomas are extramedullary and intradural tumors. Meningiomas are common in patients with neurofibromatosis type 2. Loss of NF2 is seen in 40% to 60% of patients with multiple myeloma. 1.1.2 Treatment Methods Meningiomas are usually treated with surgical resection, and complete resection of the tumor usually offers a very good prognosis. Patients with atypical or undifferentiated myeloma may require adjuvant radiation therapy. The role of chemotherapy and targeted therapy in refractory medulloblastoma has not been clearly defined.

1.2 Schwannoma 1.2.1 What is Schwannoma? Schwannomas are benign nerve sheath tumors composed of highly differentiated Schwann cells. Schwannomas account for about 29% of radial radiculopathy.
Most schwannomas are outside the brain and spine. In the skull, the most common site is nerve VIII at the pontine-cerebellar angle. The schwannoma is usually solitary and the disease is sporadic. Multiple schwannomas may be seen in patients with neurofibromatosis type 2 and in patients with schwannomatosis.
1.2.2 Treatments Spinal nerve schwannomas usually present with radicular pain and other signs of nerve root compression. Schwannoma has a good prognosis, and is largely resolved after surgical resection.
1.3 Mucinous papilloma of the endothelial canal 1.3.1 What is a mucinous papilloma of the endothelial canal? The majority of intramedullary mucinous papillomas are extramedullary in the dura, although they can be intramedullary if they arise from the pulp cone and possibly epidural if they also originate in a bony spinal cord. amputate in the distal part of the neural tube.
On MRI, endothelial mucinous papillomas present as well-demarcated and smooth sausage-shaped lesions, co- or hypointense on T1, hyperintense on T2, and enhanced with gadolinium. Cysts and tunnels within the tumor may also be present in some patients. When there is hyperintensity on both T1 and T2, the reason is bleeding or mucus.
Patients often present with low back pain, leg weakness, numbness, or urinary dysfunction. Tumors are usually partially or completely encapsulated. If the tumor is resected without encroaching on the capsule, the local control rate is almost 100%, which can be considered as curative.

1.3.2 Treatments If the capsule is perforated, the mucinous endothelial papillomas can spread through the cerebrospinal fluid to enter the entire neural axis and often recur. Therefore, total tumor resection is strongly recommended.
Sometimes, total tumor resection is not possible because of anatomical location, and many centers choose adjuvant radiation therapy for patients with incomplete resection. Because these tumors grow slowly with a risk of spreading, long-term follow-up with full-axis MRI is indicated.

2. Epidural primary myeloma

Epidural medullomas originate from ectopic arachnoid cells around the nerve root canal. Meningiomas with extensive and diffuse dural involvement are often referred to as disseminated medullomas, in contrast to typical medullomas that are clearly attached to the dura.
Usually, epidural medullomas may present as scattered medulloblastomas and may resemble metastatic epidural tumours. On MRI, these medulloblastomas may be accompanied by calcification, bone destruction, and increased interstitial size.
This is a rare form of primary central nervous system lymphoma. There were no associated brain, spinal, or systemic lymphomas.
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