Lazaridou Maria, Bourlidou Eleni, Vafeiadou Margarita, Papadiochos Yiannis, Barbetakis Nikolaos, Kiziridou Anastasia, Mangoudi Doxa
, Thessaloniki, Greece.
, Athens, Greece.
Oral Maxillofac Surg. 2019 Mar;23(1):109-112. doi: 10.1007/s10006-018-0740-8. Epub 2019 Jan 7.
An extremely rare case of divergent differentiation of a cutaneous angiosarcoma to a malignant peripheral nerve sheath tumor (MPNST) is presented.
A 62-year-old male patient presented with an angiosarcoma of the left buccal region, which was excised with wide surgical margins. Four months after the operation, the tumor recurred locally as a MPNST. The recurrent tumor was treated with surgical excision followed by postoperative radiotherapy. Four years later, the patient presented with solitary lung metastasis. The patient underwent upper right lobectomy.
The patient is alive and disease-free 2 years after the lobectomy and 6 years after initial presentation.
Divergent differentiation of an angiosarcoma to a neurosarcoma supports the theory that cells of the neural crest capable of differentiating both to neuroectoderm and to mesenchyme give origin to these tumors.
报告一例极其罕见的皮肤血管肉瘤向恶性周围神经鞘瘤（MPNST）分化的病例。
一名62岁男性患者，左侧颊部出现血管肉瘤，行广泛手术切除，切缘阴性。术后4个月，肿瘤局部复发为MPNST。复发性肿瘤行手术切除，术后进行放疗。4年后，患者出现孤立性肺转移，接受了右上叶切除术。
患者在肺叶切除术后2年、初次就诊后6年仍存活且无疾病复发。
血管肉瘤向神经肉瘤的分化支持了这样一种理论，即能够分化为神经外胚层和间充质的神经嵴细胞是这些肿瘤的起源。
