Related Experiment Video
Updated: May 16, 2026
Identifying, Diagnosing, and Grading Malignant Peripheral Nerve Sheath Tumors in Genetically Engineered Mouse Models
Published on: May 17, 2024
Giant malignant peripheral nerve sheath tumor of the scalp
Teles F1, Ataíde A M M, De Lima B A
1Department of Internal Medicine, Santa Casa de Misericordia de Maceio Hospital, Alagoas, Brazil. flavioteles@hotmail.com
Purpose:
Malignant peripheral nerve sheath tumors (MPNST) constitute a group of soft tissue neoplasms with neurogenic origin. Association of MPNST with neurofibromatosis type 1 (NF1) has been frequently reported as a result of malignant transformation of a pre-existing plexiform neurofibroma. However, most of the cases are small and few have been described with giant dimensions. We describe a case of a giant MPNST in an unusual location (scalp), in a NF1 patient.
Case Report:
A fifty-six years old woman with a history of NF1 since childhood was admitted due to a rapid growing of a scalp tumor in occipital region. The mass had 15 cm in the largest dimension and a central ulceration with purulent secretion. Computed tomography revealed no bone invasion. The tumor was surgically removed and the histopathological examination revealed a neurofibrosarcoma. The patient had a good post operatory evolution and notwithstanding the tumor size, no metastasis was found and she was discharged to oncologic follow-up.
Conclusion:
Given the poor prognosis of MPNST, patients with NF1 should be aware that neurofibromas can undergo malignant transformation and report signs such as rapid growth of a pre-existing lesion.
