Introduction:
Malignant peripheral nerve sheath tumors (MPNST) are highly aggressive soft tissue sarcomas in which complete surgical resection is the mainstay of therapy.
Clinical presentation:
We report a case of MPNST, a 24 year old patient with right neck painful mass of 13 cm of greater dimension, skin spots, lentigos, cutaneous neurofibromas. The neck CT scan revealed a mass with liquid and soft tissues densities. Surgery was performed through right cervical Paul André incision and allowed exeresis of a hemorrhagic mass expensing on the cranial nerves X, XI, XII and the cervical sympathetic chain. Pathology analysis revealed a neurofibrosarcoma, the patient received six doses of chemotherapy. A second surgery was performed one year later after the appearance of a rapidly growing neck mass on the same site. The patient deceased in the intensive care unit one day post-op.
Discussion:
Neurofibrosarcoma is a rare clinical entity most often reported as case report. This tumor takes its origin from nerve structures such as the brachial plexus, the cervical sympathetic chain and the cranial nerves and their branches. The appearance of pain, size modification or signs of nerve root compression should warn us to search for a malignant degeneration. Surgery is the base of treatment, associated to radiotherapy and chemotherapy. Vinblastin and doxorubicin were used by other authors in association to radiotherapy with complete local control of inoperable tumors. The 5-year survival is 50% in patients with a neurofibrosarcoma de novo.
Conclusion:
The prognosis of neurofibrosarcoma is poor, prompting aggressive local (surgery) and systemic treatment (radiotherapy and/or chemotherapy).