Management of intrinsic gliomas of the tectal plate in children. A ten-year review

The natural history, management, and long-term outcome for patients with benign, intrinsic tectal plate gliomas remain controversial in spite of their propensity to cause late-onset hydrocephalus. A 10-year retrospective review has identified 11 consecutive children with tectal plate lesions. Headache, vomiting, a decline in school performance, tremor, and complex partial seizures were common presenting symptoms. All patients presented with signs and symptoms of hydrocephalus. Magnetic resonance (MR) imaging delineated an intra-axial mass lesion of the midbrain primarily localized to the tectal plate which uniformly was hyperintense on T2-weighted imaging and had a more variable appearance on T1-weighted imaging and rare enhancement with gadolinium. No patient underwent surgical resection, chemotherapy, or radiotherapy. Three of 11 patients (27%) showed evidence of progression in size or a new focus of enhancement on MR imaging, which was clinically asymptomatic. In this series, no patient with a tectal plate lesion less than 1.5 cm in maximal diameter and without gadolinium enhancement showed any evidence of clinical or radiological progression. Although intrinsic tectal lesions in children are clinically indolent and the initial management consists of CSF diversion, these lesions may eventually progress and still warrant long-term follow-up with serial MR imaging.     

Tectal gliomas in children: the implications for natural history and management strategy

Tumors involving the tectal region constitute a distinct subgroup of brain stem gliomas with an indolent clinical course. Here, we present the clinical and neuroradiologic features of 9 children with tectal tumors. All patients presented with signs and symptoms of hydrocephalus and were managed with ventriculoperitoneal shunt insertion. MRI studies revealed focal hyperintense lesions on T2-weighted images without any contrast enhancement, and no evidence of progression was demonstrated in any patient. We also reviewed the published series of tectal gliomas in the literature to compare with our results. Based on these and other published series, it was concluded that intrinsic tectal gliomas of childhood with sizes less than 2 cm in diameter and without any tumor extension or contrast enhancement constitute a specific subgroup of tectal masses which rarely display invasive clinical behavior and should be managed conservatively. CSF diversion procedures and long-term yearly follow-up examinations with MRI scans are sufficient in these patients.     

Symptomatic hydrocephalus: initial findings in brainstem gliomas not detected on computed tomographic scans

In a retrospective review of 85 patients younger than 18 years of age with a diagnosis of brainstem glioma treated between 1974 and 1987, seven (8.3%) initially had hydrocephalus and no evidence of tumor on CT scans. Intrinsic brain tumors, six in the pons and one in the diencephalon, were discovered later, either on follow-up CT scans or on magnetic resonance images obtained despite persistently normal CT scan findings. The initial radiologic study of choice for children and adolescents with hydrocephalus should be magnetic resonance imaging, including axial and sagittal T1- and T2-weighted images. If a CT scan is obtained first and hydrocephalus but not tumor is found, magnetic resonance image should be obtained to rule out the possible presence of an intrinsic brainstem tumor.     

Intracranial metastasis of Wilms' tumor involving the tectal plate without pulmonary involvement. Case report.

Intracranial metastasis without pulmonary involvement of Wilms' tumor is very rare, and most previously reported metastatic sites have been in the cerebral parenchyma. We experienced a rare case of metastasis of Wilms' tumor in the tectal plate without pulmonary involvement. A 3-month-old boy was admitted to our hospital due to hydrocephalus. After insertion of a ventriculoperitoneal shunt, there were no neurological deficits. Five months after the operation, a mass 5 cm in diameter extending from the tectal plate into the third ventricle was found. A mass 10 cm in diameter within the retroperitoneal space, which occupied the right kidney, was also found. Both tumors were resected and diagnosed histologically as Wilms' tumor. Despite every effort including chemotherapy and radiotherapy, the patient died at 14 months old. The majority of children with hydrocephalus showing thickening of the tectal plate would most likely have tectal glioma; however, this unusual case report reminds us that metastasis of Wilms' tumor can occur in the tectal plate causing hydrocephalus.     

神经节细胞瘤20例

目的提高对小儿神经节细胞瘤的术前诊断率,改良手术方式,为改善预后提供依据。方法回顾性分析20例神经节细胞瘤患儿的临床资料。男9例,女11例;对其临床、病理特点、生物学特性及治疗原则进行分析。结果无临床症状患儿15例;B超检查16例,均可发现实质性低回声包块;CT平扫20例,为低密度到中等密度病灶,增强后显著强化;MRI检查14例,T1W1呈低信号,T2W1为不均匀高信号。患儿均行手术切除肿瘤,14例完整切除,4例部分切除,2例仅取活检。随访15例,复发1例。结论小儿神经节细胞瘤多为发生于胸腹交感链,生长缓慢的良性肿块。结合本文所总结的B超、CT、MRI影像学特点,术前可获提示性诊断。手术切除肿瘤时应注意切口的选择并避免损伤周围重要血管,病检证实含神经母细胞瘤成分患儿建议采用预防性化疗,远期随访效果良好。     

儿童幕上原始神经外胚叶肿瘤的临床病理诊断与显微外科手术治疗

目的分析儿童颅内幕上原始神经外胚层肿瘤的临床、影像学、病理及手术治疗。方法回顾性分析了8例经手术和病理证实的儿童颅内幕上原始神经外胚层肿瘤。结果本组病例肿瘤均位于颅内幕上，影像学上表现有肿瘤呈圆形或类圆形，边界清楚，病灶周围无水肿或水肿较轻；MRIT1WI显示肿瘤实质为等低信号，T2WI为等信号，增强时肿瘤实质明显强化；肿瘤可呈囊性变，部分肿瘤内可见出血。在病理上原始神经外胚层肿瘤由小圆细胞构成，可观察到菊花团形成，免疫组化染色显示肿瘤有多向分化的倾向。本组病例全部进行了显微外科手术治疗，其中4例进行了术后的放射治疗。随访的病例生存期2～16个月，平均8个月。结论需结合临床、影像学和病理特点对原始神经外胚层肿瘤进行诊断，手术后需进行放射治疗。     

Successful use of intracavitary bleomycin for low-grade astrocytoma tumor cyst

We report successful use of bleomycin in a low-grade astrocytoma tumor cyst of the tectal plate. A 6-year-old male underwent subtotal resection of a low-grade astrocytoma of the tectal plate followed by chemotherapy and proton beam radiation at age 2 and a half. Despite resolution of the solid portion of the tumor, serial MRI showed enlargement of a bilobar tumor cyst 3 years after the original diagnosis. The patient developed progressive ataxia, short-term memory loss and dysconjugate gaze. Following stereotactic placement of an Ommaya reservoir into the cyst, Isovue contrast and CT scan were used to confirm the integrity of the cyst. Five consecutive daily doses of 3.0 mg of bleomycin were instilled into the cyst after removal of cyst fluid. The therapy was well tolerated in the outpatient setting, and the clinical findings resolved. Subsequent CT and MRI at 4 months and 2 years after bleomycin confirmed no recurrence of the tumor or cyst.     

Neuroradiological differential diagnosis in medulloblastomas and ependymomas: results of the HIT'91-study

BACKGROUND: Imaging criteria of medulloblastomas and ependymomas were defined retrospectively to allow a preoperative differential diagnosis of these malignant tumors of the posterior fossa. PATIENTS AND METHODS: CT and/or MR-studies of 103 patients with medulloblastomas and 19 ependymomas treated in the HIT'91-trial were centrally reviewed. Evaluation concerned the origin, CT-density or MR-signal intensity, enhancement, internal structural homogeneity and results of staging of the tumors. RESULTS: Medulloblastomas and Ependymomas showed different results concerning all imaging criteria. CONCLUSION: On the basis of differences in localisation, enhancement and tumor growth in most cases a preoperative differential diagnosis between medulloblastomas and ependymomas is possible. Increased density on CT allows the differentiation of the malignant tumors from pilocytic astrocytomas of the fourth ventricle.     

儿童实体瘤合并侵袭性真菌感染3例临床分析

目的总结实体瘤患儿合并侵袭性真菌感染的诊断与治疗经验。方法回顾性分析3例实体瘤患儿于化疗过程中合并侵袭性真菌感染的临床特点及诊治经过。结果 3例实体瘤患儿均于多疗程化疗后发生肺部真菌感染,其中1例合并其他部位感染。3例患儿均有发热、中性粒细胞缺乏,曾使用广谱抗生素、激素治疗。3例患儿血真菌培养均为阳性,其中近平滑假丝酵母菌2例,白色念珠菌1例,1-3-β-D葡聚糖检测结果明显升高。3例患儿肺部CT均表现为密度增高、渗出炎症阴影。2例患儿治疗后好转存活,1例经治疗好转后,因肿瘤多发转移而死亡。结论肺部真菌感染为儿童实体瘤合并侵袭性真菌感染的常见表现形式,多发生于多次化疗后骨髓抑制期;临床表现缺乏特异性,需结合病史、实验室及影像学检查作出诊断;可疑侵袭性真菌感染时应及时进行经验性治疗。[临床儿科杂志,2012,30(5):425-427]     

Role of MRI in the management of children with diffuse pontine tumors: a study of 15 patients and review of the literature

Background: Pontine tumors carry the worst prognosis of all brain tumors. In most cases, the diagnosis is based solely on MR imaging, without biopsy. Objective: To describe the MR findings of pontine tumors at diagnosis and during follow-up and correlate those with prognosis and to assess the value of MR imaging in patient management compared to clinical evaluation. Materials and methods: Ninety-one MR scans of 15 children with diffuse pontine tumors were reviewed at diagnosis and during follow-up. The parameters analyzed were as follows: tumor extent, area, and volume; encasement of the basilar artery; presence of exophytic component; necrosis; cysts; hydrocephalus; and intensity and enhancement. Findings were correlated to length of progression-free and overall survival. Trends of amelioration or worsening on imaging were compared with the clinical findings. Results: Median length of progression-free survival was 10 months, and median survival was 20 months. Only hydrocephalus at presentation was associated with shorter progression-free survival (P=0.02). On the last examination of each patient, the craniocaudal diameter was significantly greater than at diagnosis (P=0.03). The concordance between the imaging and the clinical findings was good.Conclusion: MR is the mainstay for the diagnosis and management of pontine tumors. Cranial growth seems to be an ominous sign. However, the prognostic value of MR is limited. MR findings correlate well with the clinical examination.     

Definitive neuroradiological diagnostic features of tuberculous meningitis in children

Background: Although CT scanning is used widely for making the diagnosis and detecting the complications of tuberculous meningitis (TBM) in children, the radiological features are considered non-specific. CT is particularly suggestive of the diagnosis when there is a combination of basal enhancement, hydrocephalus and infarction, and even then the diagnosis may be in doubt. In this paper we introduce a new CT feature for making the diagnosis of TBM, namely, hyperdensity in the basal cisterns on non-contrast scans, and we assess which of the recognized CT features is most sensitive and specific. Objective: To determine the sensitivity and specificity of the presence of high-density exudates in the basal cisterns (on non-contrast CT) and basal enhancement (on contrast-enhanced CT) for the diagnosis of TBM in children, and to correlate these with the complications of infarction and hydrocephalus. Materials and methods: Retrospective review of CT scans with readers blinded to the diagnosis, which was based on a definitive culture of cerebrospinal fluid (CSF) for TBM or other bacteria. Computer-aided conversion of hard-copy film density to Hounsfield units was employed as well as a density threshold technique for determining abnormally high densities. Results: The most specific feature for TBM is hyperdensity in the basal cisterns prior to IV contrast medium administration (100%). The most sensitive feature of TBM is basal enhancement (89%). A combination of features (hydrocephalus, infarction and basal enhancement) is as specific as pre-contrast hyperdensity, but has a lower sensitivity (41%). There were statistically significant differences in the presence of hydrocephalus (p=0.0016), infarcts (P=0.0014), basal enhancement (P<0.0001) and pre-contrast density (P<0.0001) between the negative and positive TBM patient groups. The presence of granulomas was not statistically significant between the two groups (P=0.44). Conclusions: The presence of high density within the basal cisterns on non-contrast CT scans is a very specific sign for TBM in children. This will enhance diagnostic confidence, allow early institution of therapy and could reduce expenditure on contrast medium, scan time and radiation exposure. With the use of threshold techniques we believe that the pre-contrast hyperdensity may be detectable by a computer program that will facilitate diagnosis, and may also be modified to detect abnormal enhancement. Basal enhancement is a sensitive sign for the diagnosis of TBM and should be sought after contrast medium administration when no hyperdensity is seen in the basal cisterns or when this finding needs to be confirmed. The CT scan feature of hyperdense exudates on pre-contrast scans should be added to the inclusion criteria for the diagnosis of TBM in children.     

儿童毛细胞黏液样星形细胞瘤的影像与病理特征研究

目的　探讨儿童毛细胞黏液样星形细胞瘤（PMA）的影像特征及其与病理学改变的关系。方法　回顾性分析2010年10月至2018年6月首都医科大学附属北京天坛医院收治的20例儿童颅内PMA患者的临床资料、CT和磁共振成像（MRI）影像表现以及病理和免疫组织化学结果。结果　20例儿童PMA患者中，男15例，女5例；肿瘤位于幕下小脑半球10例，幕上大脑半球5例，视交叉-下丘脑4例，三脑室内1例。在CT图像上，PMA表现为低密度10例；在MRI图像上，肿瘤呈混杂信号11例，T1WI低信号7例，T2WI高信号7例，DWI未见弥散受限12例，MRI增强扫描表现为不均匀强化17例；肿瘤边界清楚18例，发生囊变16例，瘤周水肿11例。组织病理学检查可见双极性梭形细胞，以血管为中心生长，间质内含有大量黏液，缺乏Rosenthal纤维。结论　儿童PMA具有较为特征性的影像表现和低度恶性的肿瘤特征，肿瘤不均质性、边界清楚、边缘囊变和不均匀强化是PMA的特征性表现，CT和MRI可为儿童PMA的术前诊断、临床治疗和预后评估提供参考。     

CT,magnetic resonance imaging and clinicopathological characteristics of perivascular epithelioid cell tumor in children北大核心CSCD

目的评价儿童血管周上皮样细胞瘤(PEComa)的CT、磁共振成像(MRI)和临床病理特征,以提高对该肿瘤的诊断水平。方法回顾性分析2019年3月至2022年4月首都医科大学附属北京儿童医院经病理确诊的6例儿童PEComa的CT、MRI和临床病理特征。结果患儿年龄1.7~11.9岁(平均7.6岁)。男女比例为1∶2。6例单发肿瘤发病部位分别见于肾脏(2例)、腹腔(2例)、盆腔(1例)和腹股沟(1例)。肿瘤边界清晰,呈类椭圆形(5例)或多结节样(1例),边缘见花边样或结节样改变(3例)。肿瘤长径4.8~15.9 cm(平均9.3 cm)。5例肿瘤表现出周围侵犯,2例存在淋巴结转移。2例患儿合并结节性硬化症。1例肿瘤为术后复发。CT平扫(6例)示肿瘤呈等/低密度,增强后呈不均匀渐进性强化。多数(5例)肿瘤见瘤内迂曲增粗血管影,少数(2例)见沙砾样钙化。肿瘤中心在MRI(3例)T1WI及T2WI上呈斑片样或结节样高信号,周边呈等肌肉信号,弥散加权成像(DWI)(b=800 s/mm2)上病灶周边部分弥散受限且增强后强化明显,中心部分呈等信号,增强后不强化或仅轻微强化。1例组织学分类为良性PEComa,5例为恶性PEComa。Melan-A和SMA的表达率分别为100.0%、83.3%。结论儿童PEComa的CT、MRI和病理表现存在一些特异性,常表现为腹盆腔内边界清楚的类椭圆形肿块,伴结节样边缘,可见瘤内迂曲增粗血管,增强后以周边强化为主,可有局部侵犯和远处转移,病理上以恶性病变居多,免疫组织化学上呈黑色素-肌肉双源性表达。     

Cervicomedullary astrocytomas of childhood: clinical and imaging follow-up

Background. Cervicomedullary astrocytomas are a unique subset of brainstem tumors in children because they have a good prognosis when compared to the pontine subset of brainstem gliomas. Objective. To review the clinical and imaging findings in a series of children with cervicomedullary astrocytomas as to diagnosis and management. Materials and methods. A retrospective review of eleven children (six females, five males, age range: 10 days-18 years; mean = 7 years) with cervicomedullary tumors was done including the clinical presentation, imaging studies (MR: eleven, CT and MR: four), surgical findings, pathological results, and follow-up clinical and imaging findings (range: 0.2–11 years; mean = 5.2 years). Results. Symptoms and signs were delayed and protracted, often occurring over months to years (mean = 2.3 years, range 0.5–7 years). The tumors expanded the dorsal medulla and involved the upper cervical spinal cord (mean maximum tumor diameter = 4.4 cm). Only three patients had hydrocephalus. In three of four cases the tumor was not seen on CT. On MR, the majority of the tumors were T1 hypointense and T2 hyperintense. Treatment consisted of surgery only in six patients, surgery and radiation therapy in four, and surgery, chemotherapy, and radiation in one. There was recurrent local disease in four patients and on follow-up metastatic disease in the brain in one. On follow-up the majority of the patients are alive and stable (mean = 5.2 years, range 0.2–11 years). There has been one death. The majority of tumors were pilocytic astrocytomas. Conclusion. Cervicomedullary tumors are a unique subset of brainstem gliomas in childhood that present with a long duration of symptoms and a greater long-term survival than pontine gliomas. Received: 14 January 1999 Accepted: 22 March 1999     

Management and algorithm for focal nodular hyperplasia of the liver in children.

PURPOSE: The aim of this study was to determine an appropriate management plan for childhood and adolescent FNH, in particular to establish an algorithm for preoperative diagnosis and treatment. PATIENTS AND METHODS: Between 1985 and 2003, 4 children with FNH were diagnosed. Of these 4 patients, 3 (Group A) underwent tumor resection, and 1 (Group B) was treated by conservative management. Clinical data, pathological findings and follow-up were evaluated retrospectively. RESULTS: The 3 patients in Group A were symptomatic, while the 1 patient in Group B was asymptomatic. In 3 of 4 patients, a homogeneous tumor with a central stellate area was noted on abdominal ultrasonography, CT scan and MR imaging. In case 2, SPIO-enhanced MR imaging was useful for differentiating FNH from hepatocellular carcinoma. Though percutaneous needle biopsy was performed in case 3, a pathologically definitive diagnosis was impossible. An open biopsy was performed in case 4 and FNH was diagnosed. In case 4 treated by conservative management, the tumor size did not change during the 7 years after the diagnosis of FNH. CONCLUSION: FNH is usually treated conservatively because of the good evolutionary outcome of the lesion. Surgery is indicated in cases of complications, compressed adjacent organs, lesion progression, or for symptomatic patients. We advocate the use of less invasive SPIO-enhanced MR imaging instead of open biopsy when the diagnosis of focal liver lesions is not clear after contrast-enhanced CT scan and non-enhanced MR imaging.     

儿童及青少年卵巢肿瘤187例

目的 探讨儿童及青少年卵巢肿瘤的发病特点、病理类型与发病年龄的关系,并评价其诊治手段及预后因素.方法 选取1980年1月-2005年12月在中山大学肿瘤防治中心治疗并确诊的儿童及青少年卵巢肿瘤患儿187例,并对其临床及随访资料进行回顾性分析.采用SPSS 13.0统计软件,定性变量比较采用χ2检验,生存率计算用寿命表法.采用Kaplan-Meier法进行生存分析.结果 187例卵巢肿瘤中良性56例,恶性119例,交界性12例.不同病理类型的卵巢肿瘤与患儿发病年龄有一定关系(χ2=12.396,P=0.015).187例卵巢肿瘤中,56例良性肿瘤5 a总生存率为98%,119例恶性肿瘤5 a总生存率为76%,12例交界性肿瘤5 a生存率为83%.单因素生存分析显示,影响总生存时间的危险因素为肿瘤分期、是否行规范化疗和化疗方案.结论 超声及CT是卵巢肿瘤诊断的主要方法.不同病理类型的儿童卵巢肿瘤与发病年龄有一定关系.肿瘤分期、术后辅助治疗方式均影响患儿总生存时间.     

A single-pass tunneling technique for CSF shunting procedures

Implantation of ventriculoperitoneal shunts in the precoronal position is generally accomplished using a retroauricular incision for subcutaneous tunneling. Retroauricular incisions can be associated with complications, including cerebrospinal fluid leak and shunt infection. We describe a technique for 'single-pass' shunt tunneling from frontal to abdominal incisions and our initial results in a consecutive, prospective series of 15 children (age 2 days to 5 years). Eleven patients presented with congenital hydrocephalus (including 5 with myelomeningocele and 3 with posthemorrhagic hydrocephalus) and 4 with hydrocephalus secondary to central nervous system (CNS) tumors. The average length of clinical follow-up was 6 months (range 1-13 months). There were no perioperative or long-term complications of the single-pass technique. Nine of the 11 patients with congenital hydrocephalus are currently well without any further medical or surgical intervention. Two underwent shunt revision for proximal obstruction, with an intact distal system. Three of the 4 patients with hydrocephalus secondary to CNS tumor suffered secondary shunt complications during periods of severe neutropenia resulting from chemotherapy (6 weeks to 6 months after shunt insertion). For primary ventriculoperitoneal shunt insertion in infants and young children, the single-pass tunneling technique is safe and avoids one source of complications.     

VISUAL PATHWAY TUMORS AND HYDROCEPHALUS

The authors evaluated the impact of hydrocephalus on the clinical picture of children with visua pathway tumor (VPT) with or without neurofibromatosis (NF).Charts of children with VPT treated in the authors' center since 1985 were retrospectively reviewed, and those with hydrocephalus were selected and summarized. Thirty-five children with VPT were found, of whom 20 had NF.Hydrocephalus was found in 4 children with NF (20% ) and in 5 without NF (33.3% ). In 6 ofthechildren, ventricular dilatation with signs of acute increased intracranial pressure already existed at the time of diagnosis and the hydrocephalus was shunted at this time. In the other 3 children, all with NF,the hydrocephalus resulted from slowly developing aqueductal stenosis, leading in 2 to severe visual acuity deterioration. The results suggest that in children with VPT and NF, hydrocephalus, and especially hydrocephalus resulting from aqueductal stenosis, is more frequent than in the general population of NF patients, and less frequent than in VPT patients without NF. The possibility of the indolent development of hydrocephalus should be borne in mind while following children with NF. The optic nerve, when already involved with a glioma, is more vulnerable to increased pressure. Thus, in children with VPT and NF, any ventricular dilatation should lead to a consideration of early shunting.     

Congenital medulloblastoma with atypical MRI appearance

A 13-day-old female infant was admitted with hydrocephalus that had been diagnosed on prenatal ultrasound at 33 weeks' gestation. She was delivered by Caesarean section at 34 weeks with an Apgar score of 10. On admission, she weighed 2,103 g. The head circumference was 32.3 cm, and the fontanelle was tense. T(1)- and T(2)-weighted MR images revealed an isointense mass occupying the fourth ventricle with multiple cysts in the vermis. The mass was not enhanced after gadolinium administration. CT showed no definite calcification in the lesion. Preoperatively, vermian tumors, including medulloblastoma, ependymoma, astrocytoma, and hamartomas, were considered in the differential diagnosis. Hamartoma was strongly suspected due to the lack of enhancement on MRI. After a suboccipital midline craniotomy, subtotal resection of a soft grayish tumor with areas of hematoma was carried out. The pathological diagnosis was medulloblastoma. Despite chemotherapy, CSF dissemination resulted in death at 11 months. We report this case of congenital medulloblastoma with atypical MRI findings and discuss the clinical characteristics of this lesion.     

Tectal gliomas: natural history of an indolent lesion in pediatric patients

The mesencephalic tectal glioma is a distinctive form of brain stem glioma with an unusually benign clinical course. Periaqueductal location, lack of contrast enhancement, and long periods of stability are classic features. The clinical management of these lesions, especially at the time of radiographic enlargement varies widely in the published literature. It is unclear whether these progressive lesions need to be treated. Accordingly, clinical and radiologic features of 7 patients were reviewed, with attention to the clinical course of the disease after radiologic enlargement. The age at diagnosis ranged from 3.3 to 16.6 years. Six of 7 had MRI tumor enlargement beginning 0.3-5.7 years after initial diagnosis. One of these 6 patients had radiographic progression coupled with a new clinical symptom which was treated with stereotactic radiation therapy. The remaining 5 patients with MRI progression and normal neurological exams were not treated and remain free of new neurologic deficits 1.8-6.9 years after the first radiographic tumor enlargement. The results suggest that pediatric tectal gliomas are a very low-grade lesion. Conservative management in the absence of new clinical symptoms could be argued, reserving radiotherapy or chemotherapy for clinical progression.