Komotar Ricardo J, Carson Benjamin S, Rao Chandrankant, Chaffee Sara, Goldthwaite Patricia T, Tihan Tarik
Department of Neurosurgery, Columbia University, New York, New York, USA.
Neurosurgery. 2005;56(1):191. doi: 10.1227/01.NEU.0000146212.95421.B3.
Pilomyxoid astrocytoma (PMA) is a recently described, rare, circumscribed glioma similar to pilocytic astrocytoma. Despite its circumscribed nature, PMA implies a worse overall outcome than typical pilocytic astrocytoma. All PMAs reported to date involved the hypothalamic/chiasmatic region. Since these original reports, we have encountered three tumors in the spinal cord with histological features identical to those of PMA.
We report three male pediatric patients. The first patient presented at age 6 with back pain and constipation. The second patient presented at age 8 with back pain and a bout of urinary incontinence. The third patient, a neonate, presented with respiratory failure and hypotonia.
The first patient's cervical and thoracic neoplasm was treated with subtotal resection and multiple laminectomies and spinal fusion for recurrence. The second patient underwent gross total resection of his thoracic neoplasm. The neonate underwent laminectomy and biopsy of his holocord tumor.
PMAs typically occur in the hypothalamic region and within the first 4 years of life. Tumors with identical histological features are being recognized outside this typical clinical setting. The cases presented in this report suggest that PMA can occur at a later age and can involve the spinal cord. We believe that increased recognition of these circumscribed gliomas will help to elucidate their nature and lead to better management decisions.
毛黏液样星形细胞瘤（PMA）是一种最近描述的、罕见的、边界清楚的胶质瘤，类似于毛细胞型星形细胞瘤。尽管其边界清楚，但PMA的总体预后比典型的毛细胞型星形细胞瘤更差。迄今为止报道的所有PMA均累及下丘脑/视交叉区域。自这些最初的报道以来，我们遇到了3例脊髓肿瘤，其组织学特征与PMA相同。
我们报告3例男性儿科患者。首例患者6岁时出现背痛和便秘。第二例患者8岁时出现背痛和一阵尿失禁。第三例患者为新生儿，表现为呼吸衰竭和肌张力减退。
首例患者的颈段和胸段肿瘤接受了次全切除，并因复发进行了多次椎板切除术和脊柱融合术。第二例患者接受了胸段肿瘤的全切除。新生儿接受了椎板切除术和全脊髓肿瘤活检。
PMA通常发生在下丘脑区域且在生命的前4年内。在这种典型临床情况之外，具有相同组织学特征的肿瘤也被认识到。本报告中呈现的病例表明，PMA可发生在较晚年龄且可累及脊髓。我们认为，对这些边界清楚的胶质瘤的更多认识将有助于阐明其性质并导致更好的治疗决策。
