Schick B, Brors D, Draf W
Klinik für HNO-Krankheiten, Kopf-, Hals- und Plastische Gesichtschirurgie, Kommunikationsstörungen, Städtisches Klinikum Fulda.
Laryngorhinootologie. 1998 May;77(5):256-63. doi: 10.1055/s-2007-996971.
Haemangiopericytomas are rare vascular neoplasms which show either slow local tumour growth or aggressive progression in size with a high tendency of recurrence and metastasis. Manifestations of haemangiopericytomas in the area of the nose and sinuses are supposed to have a relatively benign course. To date clinical and histological parameters for prognosis are uncertain. Therefore in a quite large number of cases only the clinical course allows to evaluate the dignity of a haemangiopericytoma.
We present our experience with five haemanglopericytomas in contact with the skull base out of a total number of 457 skull base tumours. The site of origin of these five haemangiopericytomas were: nasal septum, pterygopalatine fossa, oronasopharynx, temporal bone, and parotid gland. All neoplasms showed primary or recurrent tumor in contact with the skull base and an intracranial extension was observed twice. One patient died postoperatively of a cerebral infarction. Three patients showed one or more recurrences which were treated surgically. In one case 5 recurrences occurred. Despite of 4 surgical procedures within a period of 15 months, the tumour could be controlled only for a short period of time, and the patient died 16 months after the first operation.
One has to consider that haemangiopericytomas of the head and neck show a potential malignant course. The vascular tumor may recur decades later. Adequate therapy and life-long follow-up are therefore mandatory in haemangiopericytomas. Complete surgical resection of the tumour is usually the treatment of choice, in spite of the high degree of surgical skill required, especially in advanced tumour and in case of recurrence.
血管外皮细胞瘤是罕见的血管性肿瘤,其局部肿瘤生长缓慢或大小呈侵袭性进展,具有较高的复发和转移倾向。鼻和鼻窦区域的血管外皮细胞瘤表现被认为病程相对良性。迄今为止,预后的临床和组织学参数尚不确定。因此,在相当多的病例中,只有临床病程能够评估血管外皮细胞瘤的严重程度。
我们报告了在457例颅底肿瘤中5例与颅底相关的血管外皮细胞瘤的治疗经验。这5例血管外皮细胞瘤的起源部位分别为:鼻中隔、翼腭窝、口咽、颞骨和腮腺。所有肿瘤均显示原发或复发性肿瘤与颅底相关,有2例观察到颅内扩展。1例患者术后死于脑梗死。3例患者出现1次或多次复发,均接受了手术治疗。其中1例出现了5次复发。尽管在15个月内进行了4次手术,但肿瘤仅得到短期控制,患者在首次手术后16个月死亡。
必须认识到头颈部血管外皮细胞瘤可能具有恶性病程。这种血管性肿瘤可能在数十年后复发。因此,对于血管外皮细胞瘤,必须进行充分治疗并进行终身随访。尽管手术难度较大,尤其是对于晚期肿瘤和复发情况,但完整切除肿瘤通常仍是首选治疗方法。