Department of Neurosurgery, Tohoku University Graduate School of Medicine.
Department of Pediatrics, Tohoku University Graduate School of Medicine.
Neurol Med Chir (Tokyo). 2020 Jun 15;60(6):277-285. doi: 10.2176/nmc.oa.2019-0208. Epub 2020 May 14.
Most cases of optic hypothalamic pilocytic astrocytoma (OHPA) develop during childhood, so few cases of histologically verified OHPA have been described in adolescents and young adults (AYA). To elucidate the clinical features of OHPA with histological verification in AYA, we reviewed the clinical and radiological finding of OHPA treated at our institute from January 1997 and July 2017. AYA are aged between 15 and 39 years. The clinical courses of 11 AYA patients with optic hypothalamic glioma (OHG) without neurofibromatosis type 1 were retrospectively reviewed. About six patients were diagnosed in childhood and followed up after 15 years of age, and five patients developed OHPA during AYA. Histological diagnosis, verified at initial presentation or recurrence, was pilocytic astrocytoma in 10 and pilomyxoid astrocytoma in one. After initial treatment including debulking surgery and/or chemotherapy, tumor progression occurred 16 times in seven patients as cyst formation, tumor growth, and intratumoral hemorrhage. Five of 10 patients suffered deterioration of visual function during AYA. One of 10 cases had endocrinopathies requiring hormone replacement at last follow-up examination. In conclusion, histological diagnoses of OHG before and in AYA were pilocytic astrocytoma or pilomyxoid astrocytoma. Both pediatric and AYA-onset OHPA demonstrate high incidences of tumor progression and visual dysfunctions in AYA, so that long-term follow up is essential after the completion of treatment for pediatric and AYA-onset OHPA. The optimal timing of debulking surgery and radiation therapy should be established to achieve the long-term tumor control and to preserve the visual function.
大多数视神经下丘脑毛细胞星形细胞瘤（OHPA）病例发生在儿童期，因此，在青少年和年轻成人（AYA）中，经组织学证实的 OHPA 病例很少见。为了阐明组织学证实的 AYA 中 OHPA 的临床特征，我们回顾了 1997 年 1 月至 2017 年 7 月在我院治疗的 OHPA 的临床和放射学发现。AYA 的年龄在 15 至 39 岁之间。回顾性分析了 11 例无 1 型神经纤维瘤病的 AYA 患者的视神经下丘脑胶质瘤（OHG）的临床经过。约 6 例患儿在儿童期诊断并在 15 岁后随访，5 例在 AYA 期间发生 OHPA。在初次就诊或复发时，组织学诊断为毛细胞星形细胞瘤 10 例，毛黏液样星形细胞瘤 1 例。初次治疗包括部分切除术和/或化疗后，7 例患者中有 16 次出现肿瘤进展，表现为囊肿形成、肿瘤生长和肿瘤内出血。10 例患者中有 5 例在 AYA 期间视力功能恶化。10 例中有 1 例在最后一次随访时出现内分泌疾病，需要激素替代治疗。总之，在儿童期和 AYA 期的 OHG 的组织学诊断为毛细胞星形细胞瘤或毛黏液样星形细胞瘤。儿科和 AYA 发病的 OHPA 均显示出肿瘤进展和 AYA 视力障碍的发生率较高，因此在完成儿科和 AYA 发病 OHPA 的治疗后，需要进行长期随访。应确定部分切除术和放疗的最佳时机，以实现长期肿瘤控制并保留视力功能。
