Borovika Aleksandra, Deksnis Renārs, Zariņš Jānis, Isajevs Sergejs
Riga Stradiņš University, Faculty of Residency, Riga, Latvia.
Riga East University Hospital Latvian Centre of Oncology, Department of Head and Neck Surgery, Riga, Latvia; Riga Stradiņš University, Department of Otolaryngology, Riga, Latvia.
Int J Surg Case Rep. 2023 Mar;104:107940. doi: 10.1016/j.ijscr.2023.107940. Epub 2023 Feb 18.
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.
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.
In this article we discuss main epidemiological data of MPNST as well as distinction of our clinical case peculiarities from data mentioned in literature.
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.
恶性周围神经鞘膜瘤（MPNST）是一种罕见的恶性肿瘤，归类为恶性软组织肉瘤。四分之一至二分之一的MPNST发生在1型神经纤维瘤病（NF1）患者中，通常累及近端肢体和身体的主要神经干，很少累及头颈部区域。该疾病的侵袭性导致总体预后较差，治疗方式也有限。
一名62岁的健康女性因右侧颈部肿块接受了根治性手术治疗。术前MRI研究显示，颈部上三分之一的颈动脉鞘有边界清晰的部分囊性且外观呈恶性的肿瘤。手术中发现右侧迷走神经有边界清晰的肿瘤，并以安全的根治性切缘将其切除。进一步的组织学研究证实了MPNST的诊断。用神经移植物重建迷走神经缺损，以维持和改善患者的生活质量。给予辅助放疗。在一年的随访期内，未发现疾病复发的迹象。尽管如此，患者报告功能有显著改善，迷走神经损伤症状减轻。
在本文中，我们讨论了MPNST的主要流行病学数据，以及我们临床病例特点与文献中提及的数据的差异。
MPNST被描述为具有不良短期和长期预后的侵袭性肿瘤。早期诊断和根治性手术干预不仅能改善患者预后，而且即使在MPNST病例中，也能使用额外的治疗选择来提高患者的生存率和生活质量。
