What Is a Brainstem Glioma?

Brainstem gliomas account for 10% to 20% of all central nervous system tumors in children. They mainly include diffuse brainstem gliomas (most common in the pontine) and focal brainstem gliomas (mostly located in the midbrain and Medulla). The former is the leading cause of death in children with brain tumors. Steroids can significantly alleviate the symptoms caused by peritumoral edema and are an initial treatment for highly malignant brainstem gliomas. Radiographic and chemotherapy are the main treatments for patients with diffuse brainstem glioma typical of imaging, and can be started directly without biopsy. Focal brainstem tumors can be treated surgically (shunt surgery and / or tumor resection) to obtain a good prognosis.

Brainstem gliomas account for 10% to 20% of all central nervous system tumors in children. They mainly include diffuse brainstem gliomas (most commonly in the pontine) and focal brainstem gliomas (mostly in the midbrain and Medulla). The former is the leading cause of death in children with brain tumors. Steroids can significantly alleviate the symptoms caused by peritumoral edema and are an initial treatment for highly malignant brainstem gliomas. Radiographic and chemotherapy are the main treatments for patients with diffuse brainstem glioma typical of imaging, and can be started directly without biopsy. Focal brainstem tumors can be treated surgically (shunt surgery and / or tumor resection) to obtain a good prognosis.

Chinese name

Brainstem glioma

Foreign name

glioma of brain stem

Brain Stem Glioma I. Clinical Symptoms

The tumor location and growth pattern determine the clinical manifestations of the patient. The hidden clinical process cannot be ignored: patients with frequent vomiting may only manifest as stunting in the end; school age children's academic performance is reduced due to hydrocephaly caused by hydrocephalus; children with cranial nerve involvement may not be affected by patients and parents Find. Frequent upper respiratory tract infections caused by slight aspirations may be caused by dysphagia caused by brain stem lesions. When focal brainstem gliomas are diagnosed, most patients have mild symptoms and signs for a long time, and some have as long as 14 years. Most patients may develop a stiff neck or discomfort. What's more, the patient presented with torticollis. Unlike other brainstem tumors, hydrocephalus is present in more than 90% of glioma patients

Brain Stem Glioma II. Differential Diagnosis

The diagnosis of diffuse brainstem glioma is supported by cranial nerve palsy, symptoms less than 6 months before diagnosis, and lack of enhancement on MRI. Longer symptoms, lack of cranial nerve palsy, and the presence of intensive foci on MRI support the diagnosis of non-diffusive brainstem gliomas

Differential diagnosis is needed: vascular malformations, encephalitis, rare parasitic cysts, demyelinating disease (multiple sclerosis), and hamartomas in adult neurofibromatosis are the most common non-neoplastic brain stem diseases. Rare metastatic tumors from extracranial cancers can also be found in the pontine.

Brain stem glioma III. Principles of treatment

1. Surgical intervention

The operation of brain stem tumors is more challenging, which is mainly reflected in the choice of surgical indications and the scope of tumor resection. Indications for surgery In theory, as long as the tumor is relatively limited, it can be treated surgically. For tumor resection, in principle, nerve function is preserved. Cut as far as possible under the premise, that is, the safest resection. The degree of surgical resection will affect the patient's prognosis and survival.

2. Radiation therapy

Radiation therapy for brainstem gliomas is still one of the main treatments today and usually can relieve clinical symptoms, especially in children over 3 years of age. After radiotherapy, the time for symptom relief varies. Most of the symptoms are relieved within 1 to 2 months after irradiation, but there are also exacerbations of clinical symptoms. Therefore, pay attention to the local changes of the tumor and systemic response, whether there is brainstem edema and imbalance of water and electrolyte balance, and adjust the treatment plan according to the patient's situation.

3. Chemotherapy

Research on chemotherapy drugs for gliomas has made great progress in recent years. But unfortunately, no chemotherapeutic protocol has been proven to have a definitive effect on brainstem gliomas; research is limited to phase I or II clinical studies. Due to the inconsistent chemotherapy regimens and inaccurate curative effects, the question of whether chemotherapy is needed and how old children are for chemotherapy has been controversial.

4.Other

Immunotherapy and gene-targeted therapies are still being researched, but it is expected to become a powerful tool for brain stem glioma treatment.

Although focal brain stem tumors can be treated with surgery to obtain a good prognosis, diffuse brain stem gliomas are still the most common tumor with the worst prognosis. For patients with diffuse brainstem glioma, radiotherapy or chemotherapy is the main treatment method today [21]. Due to the blood-brain barrier, systemic chemotherapy drugs rarely reach these tumors. These tumors have a short-term effect on radiotherapy, and the remission period is short. Significant clinical progress can be seen on average 9 months after diagnosis. In addition, there are delayed radiation-related toxicities. The latter include: radiation-induced necrosis of the brainstem, radiation-induced occipital lobe damage, and hypothalamic-pituitary axis damage.