Elsevier

World Neurosurgery

Volume 92, August 2016, Pages 580.e1-580.e4
World Neurosurgery

Case Report
Brain Metastasis From Malignant Peripheral Nerve Sheath Tumors

https://doi.org/10.1016/j.wneu.2016.06.069Get rights and content

Background

Metastatic disease is a well-known sequela of malignant peripheral nerve sheath tumors (MPNSTs). Metastic spread to the brain is unusual.

Case Description

A 56-year-old man was found to have a high grade MPNST of the sciatic nerve. Despite en-bloc excision of the sciatic nerve mass and local radiation postoperatively, he developed pathologically confirmed systemic metastases. He was found to have lung nodules and received chemotherapy 25 months after the diagnosis, and 32 months after the initial diagnosis, he presented with left leg weakness and sensory changes and was found to have a lesion of the frontal lobe for which he received palliative radiation. He developed systemic metastases and died 35 months after initial presentation. We retrospectively reviewed the charts of 179 patients treated at our institution with MPNSTs since 1994. This was the only case of a pathology proven brain metastasis, resulting in an incidence of 0.5%. Literature review revealed 21 cases. The mean age was found to be 37.5 years, and mean survival after development of a brain metastasis was 9.9 months.

Conclusions

Brain metastases from MPNSTs are very rare and represent a poor prognosis, with survival after brain metastasis reported to be approximately 10 months. Early and effective initial diagnosis and treatment of MPNSTs likely represent the best opportunity for increased overall survival.

Introduction

Malignant peripheral nerve sheath tumors (MPNSTs) are rare soft tissue sarcomas that occur most commonly in patients with neurofibromatosis type 1, but also can occur spontaneously in otherwise healthy patients (∼0.001% incidence)1 and after radiation. MPNSTs are usually found arising from peripheral nerves, but from cranial nerves as well.2 These tumors often have a poor outcome despite wide local surgical resection followed by radiotherapy with or without chemotherapy.1, 3, 4 Despite these treatments, local recurrence and distant metastases are common. We present a patient who developed a metastatic lesion to the brain after initially being treated for an MPNST of the sciatic nerve. In addition, we searched our surgical records to identify any other cases of pathology-proven brain metastases after initial diagnosis and treatment of an MPNST.

Section snippets

Case Report

A 56-year-old man presented with symptoms of right thigh pain as well as weakness of the lower extremity that was progressive in nature, ultimately requiring him to use a cane for ambulation. He had no pertinent past medical history, only reporting a remote history of trauma to the right leg approximately 25 years prior (fall resulting in a femur and tibia/fibula fracture requiring fixation). On initial physical examination, he had an area of tenderness to percussion on the distal aspect of the

Review of Records at our Institution

Approval for this study was obtained from our Institutional Review Board. A search of our surgical records at our institution was performed using an institutionally created tool that searches all clinical notes for a particular search term or string. It identifies any patients whose clinical data contain the searched term. For our search, we used the term MPNST as well as malignant+peripheral+nerve+sheath+tumor. After patients had been identified, we excluded any patient who did not have a

Discussion

Although MPNSTs are a very rare soft tissue sarcoma, metastatic lesions to the brain are even less common, with at present only 21 cases reported in the literature.1, 5, 6, 7, 8, 9, 10, 11, 12, 13, 14, 15, 16, 17, 18, 19, 20, 21, 22, 23, 24, 25 Recent data from a large series of MPNSTs suggest that median time to distant recurrence was 1.4 years, with the most common location being the lungs, followed by the paraspinal region.26 With the addition of the case at our institution to the

Conclusion

Brain metastases from MPNSTs are very rare and represent a poor prognosis, with survival after brain metastasis reported to be approximately 10 months. Early and effective initial diagnosis and treatment of MPNSTs likely represent the best opportunity for increased overall survival.

References (26)

  • T. Flannery et al.

    Gamma knife radiosurgery as a therapeutic strategy for intracranial sarcomatous metastases

    Intern J Rad Oncol Biol Physics

    (2010)
  • S.K. Park et al.

    Metastasizing malignant peripheral nerve sheath tumor initially presenting as intracerebral hemorrhage. Case report and review of the literature

    Surg Neurol

    (2007)
  • Q. Xu et al.

    Primary malignant peripheral nerve sheath tumor of the cauda equina with metastasis to the brain in a child: case report and literature review

    Spine J

    (2012)
  • B.W. Scheithauer et al.

    Malignant peripheral nerve sheath tumors of cranial nerves and intracranial contents: a clinicopathologic study of 17 cases

    Am J Surg Pathol

    (2009)
  • B.S. Ducatman et al.

    Malignant peripheral nerve sheath tumors. A clinicopathologic study of 120 cases

    Cancer

    (1986)
  • F.K. Storm et al.

    Neurofibrosarcoma

    Cancer

    (1980)
  • P. Cras et al.

    Malignant pigmented spinal nerve root schwannoma metastasizing in the brain and viscera

    Clin Neuropathol

    (1990)
  • A.N. D'Agostino et al.

    Sarcomas of the peripheral nerves and somatic soft tissues associated with multiple neurofibromatosis (Von Recklinghausen's disease)

    Cancer

    (1963)
  • V. D'Angelo et al.

    Cerebral metastasis from an epithelioid malignant schwannoma: case report

    Neurosurgery

    (1991)
  • F. Fenzi et al.

    Brain metastases from post-radiation malignant peripheral nerve sheath tumour

    ItalJ NeurolSci

    (1995)
  • T. Haisa et al.

    Malignant schwannoma metastasizing to the dura mater—case report

    Neurol Med Chirurg

    (1996)
  • M. Hasegawa et al.

    Malignant schwannoma and follicular thyroid carcinoma associated with von Recklinghausen's disease

    J Laryngol Otol

    (1984)
  • T. Hirose et al.

    Malignant peripheral nerve sheath tumor (MPNST) showing perineurial cell differentiation

    Am J Surg Pathol

    (1989)
  • Cited by (0)

    Conflict of interest statement: The authors declare that the article content was composed in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

    View full text