What Is a Neurofibrosarcoma?

Neurofibrosarcoma is a rare tumor that accounts for 5% to 10% of malignant soft tissue tumors. The age of onset is more common in young and middle-aged people. The most common sites are head and neck, buttocks, extremities, and retroperitoneum. They can also occur in the abdominal wall and mediastinum of the back.

Neurofibrosarcoma is a rare tumor that accounts for 5% to 10% of malignant soft tissue tumors. The age of onset is more common in young and middle-aged people. The most common sites are head and neck, buttocks, extremities, and retroperitoneum. They can also occur in the abdominal wall and mediastinum of the back.

Chinese name

Neurofibrosarcoma

Foreign name

Neurofibrosarcoma

Department

Oncology

Neurofibrosarcoma concept

Generally, it is a painless tumor mass that does not develop quickly. In some cases, there is little pain in the affected limb, and then there are tumors and affected neurogenic dysfunction. Local tenderness, with neurofibromatosis in 40% of cases. Because the symptoms are mild, most tumors are more than 10 cm longer than most when they come to the clinic.

Neurofibrosarcoma pathological changes

1. Typical tumors have a large neural stem attached to the naked eye, but the neurofibrosarcoma belongs to an unknown nerve, so there is no nerve to be found, and the lesion is deep. Like other sarcomas, the cut surface is gray and may be accompanied by bleeding. Necrosis.

2. Malignant Schwann cells can be seen under the microscope. Tumor cells are spindle-shaped, the nucleus is deeply stained, the size is uneven, mitotic images can be seen, and epithelioid Schwann cells can be seen. The tumor cells are round and deformed. , The cytoplasm varies, the powder is granular, arranged into solid nests, and there are acinar or cord-like structures.

Clinical manifestations of neurofibrosarcoma

The age of onset is more common in young and middle-aged people. The most common sites are head and neck, buttocks, limbs, and retroperitoneum. They can also occur on the back, abdominal wall, and mediastinum. Generally, it is a painless tumor mass that does not develop quickly. In some cases, there is pain in the affected limb first, and then there are masses and neurological dysfunction of the affected body. Local tenderness, with neurofibromatosis in 40% of cases. Due to the mild symptoms, the tumors are larger when they come to the clinic, and most of them have a diameter of more than 10cm.

Differential diagnosis of neurofibrosarcoma

It is mainly distinguished from fibrosarcoma. Fibrosarcoma cells are spindle-shaped, intertwined, and have obvious atypia, but there is no nuclear grid arrangement, and there is no connection between tumor and neural stem.

Neurofibrosarcoma treatment measures

Extensive resection is the main treatment. Amputation is feasible for those who have difficulty resecting extensive resection, and those who cannot be extensively resected or incompletely resected in the pelvis and vertebra can be supplemented with radiotherapy, but the effect is not good. There are reports in the literature that chemotherapy is effective for metastatic tumors. Commonly used drugs are doxorubicin, vincristine, cyclophosphamide and so on. Bloody and lymphatic metastases can occur, and the 5-year survival rate is about 30%.