Soleman Jehuda, Roth Jonathan, Ram Zvi, Yalon Michal, Constantini Shlomi
Departments of Neurosurgery and Pediatric Neurosurgery, Tel Aviv Medical Center & Dana Children's Hospital, 6 Weizmann Street, Tel Aviv, Israel.
Tel-Aviv University, Tel Aviv, Israel.
Childs Nerv Syst. 2017 Dec;33(12):2169-2175. doi: 10.1007/s00381-017-3566-z. Epub 2017 Aug 14.
Incidental findings on neuroimaging in the pediatric population are an emerging treatment challenge. Treatment options for these incidental childhood brain mass lesions, which radiologically may be assumed to be low-grade gliomas (LGG), vary, ranging from careful conservative "wait and scan" treatment to surgical biopsy, gross total resection, and upfront radiation and/or chemotherapy. As malignant transformation of LGG in children is extremely rare, some series advocate careful conservative management of these lesions; however, universal treatment protocols are not totally agreed upon.
We present the case of a 10-year-old boy with a fronto-basal incidental cerebral mass lesion, suspected to be a low-grade glial neoplasm. Initially, magnetic resonance imaging (MRI) was done to rule out a pathology causing his growth to be delayed. A treatment with growth hormone was initiated. After close clinical and radiological follow-up of this asymptomatic lesion for 6 years, a minimal growth of the lesion was seen, which we decided to continue following. After 7 years, a clear growth with new contrast enhancement was seen on routine MRI. At this point, the lesion was surgically resected. The diagnosis was, surprisingly, glioblastoma multiforme (WHO grade IV, BRAF V-600E mutation).
Malignant transformation of LGGs in children is a very rare phenomenon. This is to our knowledge the first well-documented case describing malignant transformation of a suspected benign pediatric cerebral mass lesion, which did not undergo radiation, in a patient without a cancer predisposition syndrome (e.g., neurofibromatosis), with the transformation occurring after such a long follow-up period. The management of these lesions is still controversial. Unfortunately, radiological risk factors for malignant transformation of such lesions in the pediatric age group are lacking.
Conservative treatment of incidental cerebral mass lesions in children seems a valid option. These lesions should probably be followed indefinitely, while carefully watching for changes in imaging characteristics.
儿科人群神经影像学检查中的偶然发现是一个新出现的治疗挑战。这些偶然发现的儿童脑内肿块病变，放射学上可能被认为是低级别胶质瘤（LGG），其治疗选择各不相同，从谨慎的保守“观察与扫描”治疗到手术活检、全切除以及早期放疗和/或化疗。由于儿童LGG的恶性转化极为罕见，一些系列研究主张对这些病变进行谨慎的保守处理；然而，尚未完全达成普遍适用的治疗方案。
我们报告一例10岁男孩，其额底部偶然发现脑内肿块病变，怀疑为低级别胶质肿瘤。最初，进行磁共振成像（MRI）以排除导致其生长发育迟缓的病理情况，并开始使用生长激素治疗。对这个无症状病变进行密切的临床和影像学随访6年后，发现病变仅有微小生长，我们决定继续观察。7年后，常规MRI显示病变明显生长并出现新的强化。此时，对病变进行了手术切除。令人惊讶的是，诊断为多形性胶质母细胞瘤（世界卫生组织IV级，BRAF V - 600E突变）。
儿童LGG的恶性转化是一种非常罕见的现象。据我们所知，这是首例有充分记录的病例，描述了在无癌症易患综合征（如神经纤维瘤病）的患者中，一个疑似良性的儿童脑内肿块病变在未接受放疗的情况下发生恶性转化，且这种转化发生在如此长的随访期之后。这些病变的处理仍存在争议。不幸的是，缺乏关于儿科年龄组此类病变恶性转化的放射学危险因素。
对儿童偶然发现的脑内肿块病变进行保守治疗似乎是一种有效的选择。这些病变可能需要长期随访，同时密切观察影像学特征的变化。
