Endometrial tubular medullomas

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

Endometrial tumors originate from the cells lining the endothelial canal (central canal) of the spinal cord. This is the most common primary intramedullary tumor of the spinal cord. Endometrial tubular medullomas (called meningiomas on the brain) are rare primary tumors of the central nervous system that are most common in children and young adults.

Endothelial medullomas are derived from radial glial stem cells. According to the 2007 World Health Organization (WHO) classification of central nervous system tumors, endothelial medullomas include the following:
Stage I (mucinous papilloma of the intramedullary canal, inferior tumor) endothelial membranous tumours), Grade II (endometrial medullomas), Grade III (undifferentiated endothelial tumors).

The majority of endothelial medullomas are grade II endothelial medullomas (70%), followed by mucinous endothelial papillomas (grade I) (28%) and nodular endothelial medullomas. differentiated (grade III) (2%).
The classic histological features of grade II or III endothelial medullomas are perivascular pseudo-rosacea. In which tumor cells are arranged around the blood vessels in a spokes pattern with a perivascular nonnucleated region, and true endothelial membranous rosettes, in which tumor cells are arranged around a central cavity. heart.
Grade II or III endothelial tumors are well-defined when compared with diffuse gliomas and usually do not infiltrate normal spinal cord tissue. Genetic studies of endothelial medullomas have revealed NF2 mutations, loss of chromosome 10q or 22q, duplication of chromosome 18, high levels of homeobox B5 (HOXB5), and group 5 phospholipase A2 (PLA2G5) and express the inter-α-trypsin inhibitor heavy chain 2 (TIH2).

Endometrial medullomas usually appear between the ages of 20 and 40. The symptoms of endothelial medullomas are decreased sensation and sensory disturbances.
In addition, other common symptoms when presenting the disease are motor weakness, gait ataxia and circular muscle disorder, invasion of cerebrospinal fluid.
Patients with endothelial medulloblastoma should be staged by whole-neuraxial imaging and lumbar puncture testing for cerebrospinal fluid. The majority of intramedullary medullomas recur locally; however, tumor extension into the skull has been reported. When tumor cells are present in the CSF, especially when an endothelial mucinous papilloma is partially resected, it can lead to a poor neurological outcome. Patients with pleural metastases from endothelial medullomas may present with headache, cranial nerve palsies, diplopia, visual disturbances, cognitive disturbances, radicular pain, or back pain.

In a study by the Endometrial Tumor Collaborative Network Fund (CERN) study, the majority of patients with spinal cord tumors had to undergo surgery to resolve the tumor and release nerve compression, because neoplasms have a non-stop growth and get bigger and bigger, putting a lot of pressure on the marrow and nervous system. Maximum surgical resection and radiation therapy are the mainstays of treatment. Studies have shown that: Proximal resection of the tumor reduces the local recurrence rate and prolongs survival than the partial tumor resection group. For metastatic cases, the patient's ability to recover will be poor and the remaining survival time will not be long. Therefore, in this case, the doctor will have to consider whether to operate. If surgery is not beneficial, the doctor may assign the patient to undergo chemotherapy and radiation. Cerebrospinal radiation therapy is indicated when there are signs of diffuse tumor. At the same time, these patients are often treated conservatively after surgery. Vinmec International General Hospital is one of the hospitals that not only ensures professional quality with a team of leading doctors, modern equipment and technology, but also stands out for its examination and consulting services. and comprehensive, professional medical treatment; civilized, polite, safe and sterile medical examination and treatment space. Customers when choosing to perform tests and treat diseases here can be completely assured of the accuracy and high efficiency in the treatment process.

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