Rückert R I, Fleige B, Rogalla P, Woodruff J M
Department of Surgery, Humboldt University Medical School, Campus Charité Mitte, Berlin, Germany.
Cancer. 2000 Oct 1;89(7):1577-85.
Schwannoma with angiosarcomatous change is a rare tumor, the clinical characteristics of which have not been analyzed.
A patient with schwannoma with angiosarcoma arising in the midneck and clinically mimicking a carotid body paraganglioma is described with a literature review of all previously reported cases and a comparison of their clinical features with those of schwannoma with conventional malignant transformation and cases of neurofibroma and malignant peripheral nerve sheath tumor (MPNST) with angiosarcoma.
There are four reported cases, including the present case. Schwannoma with angiosarcoma affects older adults, mainly men. Three tumors arose from the vagus nerve in the neck. Three of the four angiosarcomas were epithelioid in type. Treatment in all cases was surgical resection followed by radiation and chemotherapy in one case and by radiation alone in another. One patient died with residual local angiosarcoma 5 months after the diagnosis. The remaining three patients were alive and disease free at 27 months, 43 months, and 90 months, with distant metastasis (after 15 months) reported only in the patient described in this case report.
Schwannoma with angiosarcoma should be included in the differential diagnosis of presumed carotid body paragangliomas. Like angiosarcoma alone and schwannoma with conventional malignant transformation, but unlike cases of neurofibroma and MPNST with angiosarcoma, the patients are older adults, and there is a male prevalence. Schwannoma with angiosarcoma is capable of local spread with a fatal outcome and of distant metastasis, but follow-up strongly suggests that these patients have a better prognosis than patients with neurofibroma or MPNST with angiosarcoma. Recommended treatment is attempted complete surgical resection followed by radiation therapy and chemotherapy, if it can be tolerated by the patient.
伴有血管肉瘤样改变的神经鞘瘤是一种罕见肿瘤，其临床特征尚未得到分析。
描述了一名颈部中段发生伴有血管肉瘤的神经鞘瘤且临床上酷似颈动脉体副神经节瘤的患者，并对所有先前报道的病例进行文献回顾，将其临床特征与伴有传统恶性转化的神经鞘瘤以及伴有血管肉瘤的神经纤维瘤和恶性外周神经鞘膜瘤（MPNST）的临床特征进行比较。
包括本病例在内，共报道了4例。伴有血管肉瘤的神经鞘瘤多见于老年人，以男性为主。4例中有3例肿瘤起源于颈部迷走神经。4例血管肉瘤中有3例为上皮样型。所有病例均采用手术切除治疗，1例术后接受放疗和化疗，另1例仅接受放疗。1例患者在诊断后5个月因局部血管肉瘤残留死亡。其余3例患者分别在27个月、43个月和90个月时存活且无疾病，仅本病例报告中的患者出现远处转移（15个月后）。
伴有血管肉瘤的神经鞘瘤应纳入疑似颈动脉体副神经节瘤的鉴别诊断。与单纯血管肉瘤和伴有传统恶性转化的神经鞘瘤一样，但与伴有血管肉瘤的神经纤维瘤和MPNST病例不同，患者多为老年人，且男性居多。伴有血管肉瘤的神经鞘瘤能够局部扩散并导致致命后果，也可发生远处转移，但随访结果强烈提示，这些患者的预后优于伴有血管肉瘤的神经纤维瘤或MPNST患者。推荐的治疗方法是尝试完整手术切除，若患者能够耐受，术后进行放疗和化疗。
