Department of Neurosurgery, Mossakowski Medical Research Centre, Medical University of Warsaw, Polish Academy of Sciences, Bielanski Hospital, Cegłowska 80, 01-809 Warsaw, Poland.
Folia Neuropathol. 2009;47(4):362-70.
Pilocytic astrocytoma (PA) usually occurs in younger patients. It is a benign, generally well-delineated, WHO grade I tumour with favorable prognosis, which makes it different from diffuse astrocytomas, classified as higher grades of malignancy. A case study of PA was presented in a young female patient, observed and treated at the Neurosurgical Department for the period of 10 years, during which time she had frequent surgical procedures due to recurrence and dissemination of the tumour. The initial symptom of the disease was epileptic seizure at the age of 16. Neuroradiological study revealed cerebral tumour in the right temporal lobe, then the first temporal lobe surgery followed by re-operation and radiotherapy was performed. The patient developed hydrocephalus, treated with the ventriculo-peritoneal shunt. After 5 years local recurrence of the tumour appeared in the right temporal region. The patient was operated and the tumour was totally removed. Initially, the histopathological diagnosis of ganglioglioma was suggested for primary tumour, finally the diagnosis of pilocytic astrocytoma for both recurrent and primary tumour was established. During the next years of observation increasing neurological symptoms in lower limbs developed. Subsequently, the patient reported pain syndrome in lumbosacral and perineal area. Consecutive MRI studies revealed a spinal canal tumours localized at the thoracic level and next at sacral level. The spinal tumour was surgically treated in both locations; the last operation was done 10 years after surgery of the primary temporal lobe tumour. Histopathological examinations of the excised foci from spinal canal revealed neoplasm consistent with WHO grade I pilocytic astrocytoma. The presented case indicates that despite the spread of the neoplastic process, a histopathologically benign tumour (WHO I grade) allows for long-term survival and observation period. Unfortunately, multifocal tumour involving midline structures causes major neurological symptoms and deficits. In the presented case we dealt now with the ascending spread process and the occurrence of the new foci in both subtentorial and parameningeal spaces inside the cranial cavity. It is a rare clinical manifestation of a disease ever described in the literature.
毛细胞型星形细胞瘤(PA)通常发生在年轻患者中。它是一种良性、界限清楚的、WHO 分级 I 肿瘤,预后良好,与弥漫性星形细胞瘤不同,后者属于更高恶性程度的肿瘤。本文报道了 1 例 10 年前在神经外科观察和治疗的年轻女性患者的 PA 病例。在这段时间里,由于肿瘤的复发和播散,她多次接受手术治疗。疾病的初始症状是 16 岁时的癫痫发作。神经放射学研究显示右侧颞叶脑肿瘤,随后进行了第一次颞叶手术,随后进行了再次手术和放疗。患者出现脑积水,行脑室-腹腔分流术治疗。5 年后,右侧颞叶区域出现肿瘤局部复发。患者接受手术治疗,肿瘤完全切除。最初,原发性肿瘤的组织病理学诊断提示为神经节细胞瘤,最终对复发性和原发性肿瘤均诊断为毛细胞型星形细胞瘤。在接下来的几年观察中,下肢逐渐出现神经功能障碍症状。随后,患者报告腰骶部和会阴部疼痛综合征。连续的 MRI 研究显示胸段和骶段椎管内肿瘤。两处椎管内肿瘤均进行手术治疗,最后一次手术是在原发性颞叶肿瘤手术后 10 年进行的。椎管内切除的病灶组织病理学检查显示为符合 WHO 分级 I 级毛细胞型星形细胞瘤的肿瘤。本例表明,尽管肿瘤扩散,但组织病理学良性肿瘤(WHO I 级)允许长期生存和观察期。不幸的是,中线结构多灶性肿瘤引起严重的神经症状和缺陷。在本例中,我们现在处理的是上升性扩散过程,以及颅腔内幕下和脑膜旁间隙新病灶的发生。这是一种罕见的临床表现,在文献中曾有过描述。