CST, Manchester Royal Infirmary, Manchester, UK
Oral and Maxillofacial Surgery, Newcastle Upon Tyne Hospitals NHS Foundation Trust, Newcastle Upon Tyne, UK.
BMJ Case Rep. 2020 Nov 30;13(11):e238110. doi: 10.1136/bcr-2020-238110.
Malignant peripheral nerve sheath tumours (MPNSTs) are tumours that arise from or differentiate towards cells of the peripheral nerve sheath. They are extremely rare with an incidence of 0.001% in the general population. The most common association is with neurofibromatosis 1-an incidence of 5%-42% in this subset of the population is quoted in the literature. These tumours are aggressive with a high rate of local recurrence. Complete surgical excision remains the mainstay of treatment. A literature search found only three case reports of MPNSTs originating in the salivary glands-in all other cases the parotid gland. We present here the first documented case of a patient presented with an MPNST of the submandibular gland managed surgically by a specialty centre.
恶性外周神经鞘瘤（MPNST）是起源于或向周围神经鞘分化的肿瘤。它们非常罕见，在普通人群中的发病率为 0.001%。最常见的关联是神经纤维瘤病 1-在这部分人群中，文献报道的发病率为 5%-42%。这些肿瘤具有侵袭性，局部复发率很高。完全手术切除仍然是主要的治疗方法。文献检索仅发现三例起源于唾液腺的 MPNST 病例报告-在所有其他情况下，都是腮腺。我们在这里报告首例经专门中心手术治疗的下颌下腺 MPNST 患者的病例。
