Bhargava D, Sinha P, Chumas P, Al-Tamimi Y, Shivane A, Chakrabarty A, Surash S, Novegno F, Crimmins D, Tyagi A K
Department of Neurosurgery, Leeds General Infirmary , Leeds , UK.
Br J Neurosurg. 2013 Aug;27(4):413-8. doi: 10.3109/02688697.2012.752430. Epub 2013 Jan 3.
To know the occurrence and distribution of Pilomyxoid Astrocytomas amongst tumours previously diagnosed histologically as Pilocytic Astrocytoma and to assess the clinical impact of this new entity.
Retrospective Diagnostic review of all cases histologically diagnosed as WHO Grade I Astrocytoma at a single Neurosurgical unit between 1990 and 2003.
Of a total of 91 cases identified, 9 were found to have Pilomyxoid histology. Of these, 8 were children (mean age 3.33 years) and 1 adult. 6 tumours were hypothalamochiasmatic in location. The clinical course of Pilomyxoid tumours was aggressive marked by maturation, multiple recurrences and disease control was rarely achieved with single treatment modality as opposed to typical pilocytics. The overall survival of the pilomyxoid group was not statistically different from the pilocytic tumours.
Encompassing all age-groups and locations, Pilomyxoid Astrocytomas constitute about 10% of all tumours previously diagnosed as Pilocytic Astrocytoma. Nearly two-thirds are hypothalamo-chiasmatic in location. Knowledge of this entity is essential for appropriate aggressive treatment and follow-up.
了解在先前经组织学诊断为毛细胞型星形细胞瘤的肿瘤中毛黏液样星形细胞瘤的发生情况和分布，并评估这一新实体的临床影响。
对1990年至2003年间在单一神经外科单位经组织学诊断为世界卫生组织I级星形细胞瘤的所有病例进行回顾性诊断复查。
在总共确定的91例病例中，发现9例具有毛黏液样组织学特征。其中，8例为儿童（平均年龄3.33岁），1例为成人。6例肿瘤位于下丘脑-视交叉区域。与典型的毛细胞型肿瘤相反，毛黏液样肿瘤的临床病程具有侵袭性，表现为成熟、多次复发，单一治疗方式很少能实现疾病控制。毛黏液样组的总生存率与毛细胞型肿瘤无统计学差异。
涵盖所有年龄组和部位，毛黏液样星形细胞瘤约占先前诊断为毛细胞型星形细胞瘤的所有肿瘤的10%。近三分之二位于下丘脑-视交叉区域。了解这一实体对于进行适当的积极治疗和随访至关重要。
