Microsurgical management of giant malignant peripheral nerve sheath tumor of the scalp: two case reports and a literature review

Abstract

Malignant peripheral nerve sheath tumors of the scalp are rare lesions of the nervous system. Only 14 cases have been reported to date. The field of neurosurgery has struggled with diagnosing and treating these tumors. In this report, we present two cases of giant malignant peripheral nerve sheath tumors of the scalp and retrospectively analyze the clinical features, imaging findings, pathological features, and prognoses of these two patients. Each underwent microsurgery and radiotherapy. In addition, based on a literature review, we discuss the diagnostic and therapeutic strategies used to treat these unusual lesions.

Malignant Peripheral Nerve Sheath Tumors of the Scalp: Case Report and Review of Literature

“…MPNST is located mainly in the trunk and extremities, such as the buttocks, thighs, brachial plexus, sciatic nerve, and paraspinal region. Primary scalp MPNST is extremely rare, with only 16 cases reported to date in English literature [1].…”

“…A malignant peripheral nerve sheath tumor (also known as "Malignant schwannoma", "Neurofibrosarcoma" and "Neurosarcoma) is a form of cancer of the connective tissue surrounding nerves. As defined by the World Health Organization, however, the universal or standard terminology should be a malignant peripheral nerve sheath tumor [1]- [5]. Given its origin and behavior it is classified as a sarcoma [1]- [4].…”

“…As defined by the World Health Organization, however, the universal or standard terminology should be a malignant peripheral nerve sheath tumor [1]- [5]. Given its origin and behavior it is classified as a sarcoma [1]- [4]. Although the incidence of a malignant peripheral nerve sheath tumor of the scalp is very low, it is highly malignant and is associated with poor prognosis [1]- [3].…”

“…Given its origin and behavior it is classified as a sarcoma [1]- [4]. Although the incidence of a malignant peripheral nerve sheath tumor of the scalp is very low, it is highly malignant and is associated with poor prognosis [1]- [3].…”

Cutaneous Superficial Malignant Peripheral Nerve Sheath Tumor

“…MPNST is rare soft tissue sarcoma with an incidence of 0.001% in the general population and it represents 5-10% of all such tumors with slight male predominance (2)(3)(4)(5). One half to two thirds occur from neurofibromas or in the setting of neurofibromatosis type-1.…”