What Is Gliosarcoma?

Meningeal sarcoma is a general term for a class of malignant tumors that originate in the meningeal interstitial tissue. It belongs to interstitial non-meningeal epithelial tumors and accounts for 0.7% to 3% of central nervous system tumors. Meningeal sarcoma occurs frequently in infants and young children. The tumor is soft and brittle, infiltrating and growing, and has a high degree of malignancy. It can directly invade the skull, sinuses, brain parenchyma, or distant metastasis through the meninges. Meningeal sarcoma is not an independent type of tumor, but a general term for a class of malignant tumors originating from the meningeal interstitial tissue, mainly including the following tumors: fibrosarcoma, leiomyosarcoma, rhabdomyosarcoma, chondrosarcoma, osteosarcoma, malignant fibrous histiocytic cells Tumor, liposarcoma, angiosarcoma, and primary meningeal sarcoma (referring to sarcomatoid disease that diffusely invades the meninges with malignant spindle cells and interstitial tumor cells and lacks local mass).

Basic Information

English name

meningeal sarcoma

Visiting department

Neurosurgery, Oncology

Multiple groups

Baby, toddler

Common locations

Meninges

Common symptoms

Headaches, epilepsy, mental symptoms, neurological dysfunction, and hydrocephalus, etc .; persistent severe headaches, frequent vomiting, and significant neck strength are characteristics

Causes of meningeal sarcoma

Meningeal sarcoma is a general term for a class of malignant tumors that originate in the meningeal stromal tissue. It is divided into primary meningiosarcoma and secondary meningiosarcoma. The former originates from the malignant transformation of meningeal mesenchymal cells; the latter is caused by benign meningiomas. Come.

Meningeal sarcoma classification

Primary meningeal sarcoma

Malignant transformation of meningeal mesenchymal cells.

2. Secondary meningeal sarcoma

It comes from benign meningiomas malignancy, and hemangioblastoma malignancy is more common.

Clinical manifestations of meningeal sarcoma

Patient characteristics

Meningeal sarcoma can occur at any age, but it is more common in children before the age of 10, with no significant gender differences, especially sarcomas with muscular components. Malignant fibrous histiocytoma and fibrosarcoma are more common in adults.

2. Special medical history

Some patients have a history of trauma, a history of craniotomy, a history of meningiomas without radical surgery, a history of radiotherapy, chemotherapy or AIDS, and a related history of neurofibromatosis, Fi-Fraumeni syndrome, and soft tissue sarcoma.

3. Progress of disease course

With the exception of a few disease course extensions, the disease course is generally short, rarely exceeding one year, and may even present with acute or subacute encephalitis.

4. Clinical manifestations

Common symptoms such as headache, epilepsy, mental symptoms, neurological dysfunction, and hydrocephalus, persistent and severe headache, frequent vomiting, and significant neck strength are the clinical characteristics of this disease.

5. Physical examination

In addition to the signs of localization of the nervous system related to the lesion site, papilledema and neck resistance are common, painless masses can be found due to tumor invasion of the skull and scalp, and anemia, systemic exhaustion, and fever of unknown origin can be found throughout the body which performed.

Meningeal sarcoma imaging

Meningiosarcoma lacks specific imaging features.

1. Head CT (Computed Tomography)

Presented as a single or multiple lesions, with uneven enhancement, cystic changes and bone invasion and destruction can be seen, tumor calcification and skull hyperplasia are rare.

2. Head MRI (magnetic resonance imaging)

Except for liposarcoma, T ₁ and T ₂ weighted images are high signal, most meningeal sarcomas are T ₁ weighted image with low signal, T ₂ weighted image is high signal, enhanced scanning unevenness, moderate enhancement with cystic changes and severe Peritumoral edema.

Meningeal sarcoma diagnosis

Children under 10 years of age or patients with the above-mentioned related medical history, short course, obvious symptoms of cranial hypertension, persistent and severe headache, frequent vomiting and significant neck strength similar to the symptoms of acute or subacute encephalitis, especially physical examination Patients with systemic manifestations of papillary edema and subscalp masses accompanied by malignant tumors such as anemia, systemic exhaustion, and fever of unknown origin need to consider the possibility of this disease.

Such as CT and MRI found intracranial off-axis lesions with uneven enhancement and cystic changes, accompanied by severe peritumoral edema and skull destruction, the possibility of this disease needs to be considered.

The final diagnosis depends on postoperative pathological diagnosis, especially PAS staining, immunohistochemistry and even electron microscopy to confirm and distinguish various types of meningeal sarcoma.

Differential diagnosis of meningeal sarcoma

Benign meningiomas

The course is long and the progress is slow. Most of them are chronic headache, adult epilepsy, mental changes, localized mass of the skull, edema of the fundus optic papilla. Skull x-rays are mostly osteoproliferative changes. CT and MRI are mostly round masses with smooth edges and regular morphology. The enhanced scans are uniform and intensive without obvious cystic changes and necrotic areas. Calcification is common. Peritumular edema is mild to severe unless it affects venous return. .

2. Malignant meningioma

According to the clinical manifestations and imaging examinations of patients, it is difficult to distinguish them from malignant meningiomas before surgery. They often need to be diagnosed by postoperative pathology, especially PAS staining, immunohistochemistry, and even electron microscopy to distinguish them, and are used to distinguish various types of meninges. sarcoma.

3. Bacterial meningitis

Due to the short course and rapid progress of meningeal sarcoma, persistent and severe headaches, frequent vomiting, and significant neck strength similar to the manifestations of acute or subacute encephalitis can be easily confused with bacterial meningitis in clinical practice. Cerebrospinal fluid examination after careful assessment of lumbar puncture risk can help distinguish the two: Centrifugal examination of cerebrospinal fluid in patients with meningeal sarcoma can detect tumor cells; changes in white blood cell count, high protein content, and reduced sugar and chloride in patients with bacterial meningitis , Bacterial culture can identify pathogenic bacteria.

4. Primary hydrocephalus

Neuroimaging tumor space occupying effects and other changes, symptoms such as vomiting and neck strength can disappear after lumbar puncture and drainage of cerebrospinal fluid (and the above symptoms are difficult to improve or disappear due to infiltration of the meninges and nerve roots), and the cerebrospinal fluid can not be found by centrifugal examination Tumor cells.

5. Other malignant tumors

For example, meningeal implantation of hemangiopericytoma, malignant neuroectodermal tumor, and gliosarcoma, etc., must be diagnosed with the patient's primary tumor history and postoperative pathology.

Meningeal sarcoma treatment

1. Radical tumor resection under the microscope is the preferred treatment method, and strives to achieve Simpson I resection, which includes the affected dura and skull resection, and if possible, can expand the resection of peripheral dura.

2. At present, it is often advocated that postoperative radiotherapy is used to delay recurrence, and chemotherapy is not effective. In recent years, people who advocate chemotherapy with lomustine (CCNU) and teniposide (VM-26) must consider intrathecal chemotherapy drugs, such as methotrexate (MTX) and cytarabine, if necessary. It is supplemented with dexamethasone to reduce chemical meningitis.

3. As long as the patient's systemic conditions allow for recurring tumors, the second operation should be performed as soon as possible. It is recommended to remove all the dura mater and the affected bone within 4cm of the tumor, and to perform radiotherapy after surgery.

4. Patients with severe hydrocephalus can try lateral ventricle abdominal shunt. Although there are risks such as tumor implantation in the abdominal cavity and clogging of the shunt tube, sometimes it is still a preferable method to relieve cranial hypertension and prolong the life of patients.

Meningeal sarcoma prognosis

At present, there is no very effective treatment, it is difficult to completely remove the operation, it is easy to relapse after surgery, and extracranial metastasis can occur. The overall prognosis is poor, the patient has a short survival time, the average survival time does not exceed 2 years, and the 5-year survival rate is low.