Rare malignant peripheral nerve sheath tumor of vagus nerve: A case report

Aleksandra Borovika; Renārs Deksnis; Jānis Zariņš; Sergejs Isajevs

doi:10.1016/j.ijscr.2023.107940

Rare malignant peripheral nerve sheath tumor of vagus nerve: A case report

Int J Surg Case Rep. 2023 Mar:104:107940. doi: 10.1016/j.ijscr.2023.107940. Epub 2023 Feb 18.

Authors

Aleksandra Borovika¹, Renārs Deksnis², Jānis Zariņš³, Sergejs Isajevs⁴

Affiliations

¹ Riga Stradiņš University, Faculty of Residency, Riga, Latvia. Electronic address: 013430@rsu.edu.lv.
² Riga East University Hospital Latvian Centre of Oncology, Department of Head and Neck Surgery, Riga, Latvia; Riga Stradiņš University, Department of Otolaryngology, Riga, Latvia.
³ Department of Hand and Plastic Surgery, Microsurgery Centre of Latvia, Riga, Latvia; Baltic Biomaterials Centre of Excellence, Headquarters at Riga Technical University, Riga, Latvia.
⁴ Department of Pathology, Faculty of Medicine, University of Latvia, Riga, Latvia; Riga East University Hospital, Centre of Pathology, Riga, Latvia; Hospital of Traumatology and Orthopaedics, Department of Microbiology and Pathology, Riga, Latvia.

Abstract

Introduction: MPNST is a rare type of malignancy classified as malignant soft tissue sarcoma. One-fourth to one-half of MPNST arise in patients with neurofibromatosis type 1 (NF1) and generally involves major nerve trunks of proximal extremities and body, rarely head and neck region. Aggressive nature of the disease shows poor overall prognosis, where treatment modalities are also limited.

Presentation of case: 62-year-old otherwise healthy female underwent radical surgical treatment due to the mass of the right side of the neck. Preoperative MRI studies showed well defined partly cystic and visually malignant neoplasm of the carotid sheath in upper third of the neck. Well-defined tumor of the right vagus nerve was detected during the surgery and was excised with safe and radical margins. Further histological study confirmed MPNST diagnosis. Defect of the vagus nerve was reconstructed with a nerve grafts to maintain and improve patients quality of the life. Adjuvant radiotherapy was appointed. At one year follow-up period no evidence of disease recurrence was found. Nevertheless, patient reported significant improvement of functionality and less vagus nerve impairment symptoms.

Discussion: In this article we discuss main epidemiological data of MPNST as well as distinction of our clinical case peculiarities from data mentioned in literature.

Conclusion: MPNST are described as aggressive neoplasms with unfavorable short and long-term prognosis. Early diagnosis and radical surgical intervention not only improve patient prognosis but also allow to use additional treatment options to improve patients survival and quality of the life even in case of MPNST.

Keywords: Case report; MPNST; Nerve graft; Nerve reconstruction; Vagal nerve.

Publication types

Case Reports