Neurosarcoma (schwannoma malignum) belongs to the group of sarcomas, which are one of the most malignant neoplasms. Neurosarcoma originates in nerve cells and can develop anywhere on the body and may not always be symptomatic. What are the causes of neurosarcomas and how is the treatment performed? What's the prognosis?

Neurosarcoma( schwannoma malignum ) is the old name of a neoplastic lesion, which is now called a malignant neoplasm of the peripheral nerve sheaths (English malignant peripheral nerve sheath tumor , abbreviatedMPNST ). Neurosarcomas arise from cells associated with peripheral nerves, such as Schwann cells and fibroblasts. It is a high-grade neoplastic lesion - the World He alth Organization classifies neurosarcoma in the fourth highest grade. The typical age of onset for neurosarcoma is around 20-50 years. About half of all these changes occur in patients with type I neurofibromatosis (also known as von Recklinghausen's disease). Neurosarcomas account for up to 10% of all human soft tissue sarcomas.

Neurosarcoma (Schwannoma malignum): causes

The exact causes of neurosarcoma are unknown. It is suspected that genetic disorders primarily contribute to the development of this disease. This thesis could be confirmed by the fact that mutations in the p53 gene are found in patients with neurosarcoma. Under normal conditions, the protein product of this gene inhibits the growth of neoplasms by, inter alia, blocking cell division. In a situation where the p53 protein does not function properly, the tendency to develop cancer significantly increases - one of the diseases that may occur in patients with p53 mutations is neurosarcoma. Another explanation for the hypothesis that neurosarcoma is associated with genetic mutations may be the fact that 50% of patients with this cancer also suffer from neurofibromatosis type I (NF1). However, this disease is caused by mutations of genes located on chromosome 17. The risk of a patient with NF1 developing neurosarcoma is relatively high, it may even be up to 13% over the course of a lifetime.

Neurosarcoma (Schwannoma malignum): symptoms

It happens that schwannoma malignum develops completely asymptomatically in a patient. In other patients - even because the lesion may be located in different places on the body - the clinical picture presented by them may be very variable. Possible symptoms of neurosarcoma are:

  • the appearance of a nodular bulge within the soft tissues of e.g. limbs (upper or lower) or the trunk,
  • paresthesia,
  • feeling irritable around the developing tumor,
  • disturbances in the mobility of various parts of the body (especially when neurosarcoma develops in the area of ​​the shoulder or lumbar plexus),
  • unusual sensations in the area of ​​the body affected by the change, such as stinging, burning or general discomfort.

Neurosarcoma (Schwannoma malignum): diagnosis

Imaging studies are of fundamental importance in the diagnosis of neurosarcomas. Among them, magnetic resonance imaging is considered standard in this cancer. In the diagnosis of neurosarcomas, other imaging diagnostic techniques, such as positron emission tomography (PET), computed tomography (CT) and ultrasound examinations, are also used. In this case, imaging tests are used not only to assess the primary lesion, but also to detect its possible metastases to other organs. The tests described above allow for suspecting that the examined patient suffers from neurosarcoma. Final confirmation of the nature of the neoplastic disease is possible by histopathological examination of the tissue previously collected from the patient (the material can be obtained before the treatment of neurosarcoma by biopsy, sometimes histopathological assessments are performed intraoperatively).

Neurosarcoma (Schwannoma malignum): treatment

Surgical management is essential in the treatment of neurosarcoma. Different types of interventions may be necessary in different patients. It may happen that a simple tumor resection will be sufficient to cure the tumor. In other patients, in whom the neurosarcoma developing, e.g. in the area of ​​the extremities, has an exceptionally large size and when it is very vascularized, it may unfortunately even be necessary to perform an amputation of the affected part of the body. Other treatments for the treatment of neurosarcoma include radiotherapy and chemotherapy. Radiotherapy can be prescribed to patients both in the pre- and postoperative period. Illumination before surgery is usually aimed at minimizing the size of the tumor and making itresection as little as possible for the patient (in order to prevent recurrence of the disease, the neurosarcoma must be resected with a margin of he althy tissue).

Schwannoma malignum: prognosis

Neurosarcomas are neoplasms with a poor prognosis for patients. Five-year survival is observed in 15-50% of people suffering from this disease. Longer survival times are observed in patients whose treated lesion was less than 5 cm in size, as well as in those patients whose entire mass of neurosarcoma was removed during surgery. The presence of distant metastases may worsen the prognosis of patients with schwannoma malignum. . Unfortunately, the tumor has a great ability to form metastases - their appearance occurs in up to 40% of patients with neurosarcoma, the lungs are the most common location of secondary neoplasms.

Category:

Oncology