Treatment of pediatric cerebral radiation necrosis using hyperbaric oxygenation.

Introduction: Cerebral radiation necrosis is rarely encountered in pediatric patients. This case report describes a child with cerebral radiation necrosis who was successfully treated using corticosteroids, bevacizumab, and hyperbaric oxygenation. Case report: A 3-year-old boy developed progressive extremity weakness six months after the completion of radiation therapy for the treatment of a neuroepithelial malignancy. Treatment with corticosteroids and bevacizumab was initiated, but his symptoms did not improve, and he was then referred for hyperbaric oxygen therapy. After completing 60 hyperbaric treatments, he experienced significant improvements in mobility, which remained stable over the next year. Discussion: Cerebral radiation necrosis typically presents in children with symptoms of ataxia or headache. Corticosteroids and bevacizumab are common treatments, but hyperbaric oxygen therapy has also been studied as a therapeutic modality for this condition. When considering the use of hyperbaric oxygenation in pediatric patients, careful attention to treatment planning and patient safety can reduce the risks of adverse events such as middle ear barotrauma and confinement anxiety. Conclusion: In addition to other available pharmacologic therapies, hyperbaric oxygenation should be considered for the treatment of pediatric patients with cerebral radiation necrosis.

[Recurrent High-grade Astroblastoma Treated with Stereotactic Radiotherapy:A Case Report].

INTRODUCTION: Astroblastoma is a rare, supratentorial glial tumor, occurring predominantly in children and young adults. However, treatment strategies have not yet been established for this rare disease. CASE PRESENTATION: A 6-year-old boy presented with headache and nausea. CT and MRI revealed a left frontal mass lesion with slight edema and macrocalcifications. Gross tumor resection was performed. Histological examination found neoplastic cells with astroblastic characteristics, and a striking perivascular array of pseudorosettes. The final diagnosis was high-grade astroblastoma. MRI 13 months after surgery suggested local recurrence, and an enlargement was found 3 months later. Stereotactic radiotherapy(SRT)was performed. MRI after SRT showed enhanced cyst formation around the tumor bed, suggesting tumor recurrence. However, 11C-methionine positron emission tomography(PET)revealed radiation necrosis. The last follow-up MRI 15 months after SRT showed no further recurrence. CONCLUSION: Astroblastoma is rare, therefore, no optimal management is known. SRT may be effective to treat recurrent astroblastomas. 11C-methionine PET/CT was useful to differentiate metastatic disease from radiation necrosis.

Posterior fossa astroblastoma in a child: a case report and a review of the literature.

A 4-year-old girl presented to the hospital with a progressive headache, difficulty walking, and persistent daily vomiting for 3 weeks. Papilledema was observed on fundoscopic examination. A large left cerebellovermian tumor with "bubbly" appearance was discovered. Total removal of the tumor mass was performed, and a diagnosis of low-grade astroblastoma was made. Adjuvant radiotherapy was performed due to the risk of recurrence. The patient is disease-free and has been kept on close follow-up for 6 months. The occurrence of posterior fossa astroblastoma has been rarely reported in the literature. Thus, when a "bubby" appearance enhancing cystic solid tumor is located on the cerebellar hemisphere in a child, an astroblastoma should also be included in the differential diagnosis.

Radiologic response to radiation therapy concurrent with temozolomide for progressive simple dysembryoplastic neuroepithelial tumor.

Dysembryoplastic neuroepithelial tumors (DNETs) are low-grade neuroglial tumors that are traditionally considered to be benign hamartoma-like mass lesions. Malignant transformation and disease progression have been reported in complex DNETs. We report a case of a simple DNET with disease progression following subtotal resection. A 34-year-old woman underwent craniotomy with subtotal resection of a large nonenhancing right temporal lobe and insular mass. Histopathological analysis revealed a simple DNET. Magnetic resonance imaging obtained 6 months after surgery demonstrated disease progression with no enhancement or change in signal characteristics. Following concurrent therapy with temozolomide and external beam radiation therapy, a significant radiologic response was observed. Progressive DNET with malignant transformation exhibits predominantly glial transformation and occurs predominantly in complex DNETs. The histological classification of DNETs into simple, complex, and nonspecific are reviewed. Contrast-enhancing regions are more frequently seen in complex tumors, with nonenhancing regions having fewer complex histologic features. Close clinical and radiographic follow-up is important in all cases of DNET. Following tumor progression, radiation therapy with concurrent and adjuvant temozolomide chemotherapy may be an effective treatment.

Patterns of presentation and failure in patients with gliomatosis cerebri treated with partial-brain radiation therapy.

BACKGROUND: The patterns of lobar involvement, optimal treatment, and disease course among patients with gliomatosis cerebri (GC) have not been fully characterized. The current study evaluates the clinical presentations and outcomes for patients with GC treated with radiation therapy (RT) at our institution. METHODS: A total of 26 patients (25 with follow-up) with GC were diagnosed and treated between January 2004 and June 2012. Inclusion criteria consisted of brain magnetic resonance imaging and neuroradiology confirmation of contiguous involvement of ≥ 3 lobes/lobar equivalents with preservation of neural architecture. Patients were treated with either partial-brain RT to involved tumor (25 patients) or whole-brain RT (1 patient). The median RT dose was 54.0 Gray. The median follow-up was 17.3 months. RESULTS: The median age of the patients at the time of diagnosis was 57 years. Twenty-one patients (81%) and 5 patients (19%) had 3 to 6 and ≥ 7 involved lobes/lobar equivalents, respectively. The median progression-free survival and overall survival were 7.4 months and 14.9 months, respectively. Fifteen patients experienced radiographic disease progression after partial-brain RT, 14 of whom (93%) developed infield disease recurrence. On univariate analysis, higher tumor grade and type II GC (with focal mass) were associated with a poorer progression-free survival. The extent of lobar involvement and chemotherapy were not associated with overall survival. CONCLUSIONS: Even with partial-brain RT, nearly all disease recurrences were infield and clinical outcomes were similar to previous GC series, thereby suggesting that whole-brain RT is not necessary for this patient population. A greater number of involved lobes did not correlate with inferior outcomes. Further studies are necessary to establish more uniform and optimal treatments for this rare disease.

Pediatric astroblastoma: a rare case with a review of the literature.

Astroblastoma is a rare glial tumor with uncertain histopathological origin and unpredictable clinical behavior. We present a case of an 11-year-old girl who presented with headache and blurring of vision for 2 months. A well-demarcated mass was found in the right frontoparietal lobe on a brain MRI. The patient was treated with total tumor resection followed by postoperative radiotherapy. Histologically, the features were suggestive of high-grade astroblastoma. The patient is alive and disease free 23 months after surgery. The characteristic radiological and histopathological features and treatment of this case are described with a literature review.

Rosette Forming Glioneural Tumor Treated with Conformal Radiation.

Rosette forming glioneural tumors (RGNT) are a rare type of low-grade brain tumor included in 2007 in WHO classification. Given the benign nature of the disease, a complete surgical excision has been considered optimum. However, a handful of cases have reported the locally aggressive nature of RGNT. In addition, radiation may also be considered for a tumor located in areas where surgical excision is difficult. We present a similar case, where surgical risk was weighed against resection and we treated the patient with conformal radiation.

Long survival of primary diffuse leptomeningeal gliomatosis following radiotherapy and temozolomide: case report and literature review.

OBJECTIVE: Primary diffuse leptomeningeal gliomatosis (PDLG) is a rare neoplasm with a short survival time of a few months. There is currently no standardized therapeutic approach for PDLG. - MATERIAL AND METHODS: We report on a 53-year-old male patient who presented with epileptic seizures, gait disturbance, paraparesis and sensory deficits in the dermatomes T8-10. - RESULTS: Magnetic resonance imaging (MRI) revealing numerous spinal and cranial gadolinium-enhancing nodules in the meninges and histopathology led us to diagnose primary diffuse leptomeningeal gliomatosis with WHO grade III astrocytic cells. Consecutively, the patient underwent craniospinal radiotherapy (30Gy) and 11 sequential cycles of temozolomide. This regimen led to partial tumor regression. Thirteen months later, spinal MRI revealed tumor progression. Second-line chemotherapy with 5 cycles of irinotecan and bevacizumab did not prevent further clinical deterioration. The patient died twenty-two months after diagnosis, being the longest survival time described thus far with respect to PDLG consisting of astrocytic tumor cells. - CONCLUSIONS: Radiochemotherapy including temozolomide, as established standard therapy for brain malignant astrocytomas, might be valid as a basic therapeutic strategy for this PDLG subtype.

Two cases of pediatric high-grade astroblastoma with different clinical behavior.

Astroblastoma is a rare glial tumor occurring in older children and defined by histological criteria as low or high-grade. We describe two children with high-grade astroblastoma. The first patient, with multiple recurrences of a frontoparietal tumor, died 10 years from diagnosis after progression of the disease despite surgery, radiotherapy and chemotherapy. The second patient underwent subtotal resection of a temporal mass; the residual tumor progressed five months after radiotherapy, but after a subsequent gross total resection the patient is now in complete remission 54 months from diagnosis. Although both patients had high-grade astroblastomas, there were histological differences between the two tumors, in particular regarding the proliferative index, which was 30% and 5-10%, respectively. High-grade astroblastoma is usually treated with surgery and radiotherapy, but may have an unpredictable behavior even when tumor excision is deemed complete. The two cases reported here illustrate the variable clinical course of this rare tumor. The proliferative index may be a useful tool to better define prognosis.

Gliomatosis cerebri.

Gliomatosis cerebri is a rare diffusely infiltrating glial tumor involving two or more lobes and is frequently is bilateral. Infiltrative extent of tumor is out of proportion to histological and clinical features. We present a case in which finally the diagnosis of gliomatosis cerebri was made. In this case, computed tomography showed that midline structures were insignificantly shifted to the left, there was a mild dilatation of lateral ventricles more expressed on the right, and no pathologic changes of brain tissue density were found. On magnetic resonance tomography, T2W/SE and T2W/FLAIR images revealed zones of hyperintense signal, spreading with time, through several lobes of the brain with no enhancement on T1W images. Diagnosis of gliomatosis cerebri was suspected, stereotaxic biopsy was performed, and pathological examination revealed changes typical of diffuse glial tumor. In this article, changes typical of gliomatosis cerebri seen in other radiological methods such as computed tomography, magnetic resonance spectroscopy, dynamic contrast-enhanced T2*-weighted magnetic resonance, and positron emission tomography also are discussed.

Primary diffuse leptomeningeal gliomatosis.

BACKGROUND: Primary diffuse leptomeningeal gliomatosis (PDLG) is a rare presentation of a primary central nervous system glial tumor. METHODS: Four case reports of PDLG in young males aged 14-24 years are presented. These reports are discussed in the context of the existing literature. RESULTS: The clinical presentation of 4 new cases of PDLG resembled chronic meningitis with and without polyradiculopathy. Spinal fluid studies are typically nondiagnostic, but characteristically show elevated opening pressure, an elevated protein level, and a relative paucity of cellular reaction. An accurate antemortem diagnosis required contrast-enhanced imaging and meningeal biopsy in all 4 of our cases. Treatment strategies including craniospinal radiation and chemotherapeutic approaches, alone or in combination, have not been proven to alter the course of the disease. Initial responses to temozolomide and radiation treatments in all 4 of our cases were promising, resulting in temporary stabilization of the disease and prolonging life expectancy over what was previously reported in the literature. CONCLUSION: Total neuroaxis contrast-enhanced MRI scanning is required for directing biopsy confirmation and detecting the extent of the disease. More effective therapeutic strategies are needed, but the combination of temozolomide and radiation therapy may slow disease progression.

Supratentorial high grade astroblastoma: report of two cases and review of the literature.

Astroblastoma is a rare glial tumor with uncertain histopathological origin and unpredictable clinical behavior. In this report, the authors present two cases of high grade intracerebral astroblastomas. Both tumors occurred in children as supratentorial, well-circumscribed, peripheral masses. The lesions differed radigraphically; one contained a huge cystic component and heterogeneously enhancing mural nodule while the other appeared as a prominently contrastenhancing solid mass lesion. Both patients were treated with surgery and postoperative radiotherapy. They were followed-up long-term and no recurrence of the tumor was detected in either case. We also discussed the radiological and histological characteristics with prognostic features.

Procarbazine and CCNU as initial treatment in gliomatosis cerebri.

BACKGROUND: Gliomatosis cerebri (GC) is a diffuse infiltrating glial tumor with involvement of at least 3 cerebral lobes. There are only few data on the efficacy of initial chemotherapy in patients with GC. PATIENTS AND METHODS: In 3 neurooncological centers, patients with newly diagnosed GC who had received procarbazine (60 mg/m(2), days 8-21/56) and CCNU (110 mg/m(2), day 1/56) chemotherapy (PC) as initial treatment were analyzed for progression-free survival, overall survival and toxicity. RESULTS: Twelve patients (median age 46 years, range 27-72) were analyzed. The median progression-free survival and the median overall survival were 16 and 37 months. Grade 3 or 4 hematotoxicity was observed in 3 of 12 patients (25%). CONCLUSIONS: These data support the efficacy of PC chemotherapy in newly diagnosed GC. Initial PC chemotherapy should be considered as a treatment option and evaluated in larger clinical trials.

Gliomatosis cerebri treatment in 11 elderly patients.

The Authors report their experience in the treatment of eleven patients over 70 years old (range from 70 to 83, average age 74.8, 7 males and 4 females), with histologically proven diagnosis of glioblastoma multiforme. The GC golden standard treatment is still debated, particularly in elderly patients. All the patients underwent a first line treament with chemotherapy (Temozolomide), followed by Whole Brain Radiotherapy (WBRT) and PCV schedule without Vincristine in case of progression of the disease. The median survival was 16.3 months, ranging from 13 to 22 months. According to our experience, elderly patients should undergo the same treatment of younger patients, provided they are in good health conditions.

[Gliomatosis cerebri. Report of two cases].

Gliomatosis cerebri is a rare tumor of the central nervous system, and here we report two cases of this tumor. Case 1: A 41-year-old female was admitted to our department for evaluation of activity loss and mental changes. MR imaging revealed a high intensity symmetrical lesion in the bilateral frontal and temporal regions, thalamus and midbrain on FLAIR images. The patient had no neurological deficits and no abnormal findings in clinical laboratory data, including data for the cerebral spinal fluid. A specimen obtained by open biopsy revealed widespread infiltration of neuronal structures by small astrocytic cells, although without destruction of the neuronal structures. The patient was diagnosed with gliomatosis cerebri, and surgery and whole brain radiation at 44Gy were performed. The MRI lesion showed some shrinkage 20 months after surgery, and the KPS score was 90, the same as that before admission. Case 2: A 56-year-old male was admitted for numbness of the face and extremities. MR imaging revealed a high intensity lesion in the bilateral thalamus and a ringed enhanced lesion in the right thalamus. A specimen obtained by open biopsy revealed anaplastic oligodendroglioma, which was diagnosed as gliomatosis cerebri. Radiation at 54Gy, chemotherapy (ACNU, vincristine) and gamma-knife surgery were performed, and two months later MR imaging showed that the tumor (including the ringed enhanced lesion) had shrunk markedly. His KPS was 90 at 13 months after onset. These cases suggest that radiation therapy is effective for gliomatosis cerebri.

Gliomatosis cerebri having a poor performance status without recurrence after radiotherapy: a single institutional experience.

OBJECTIVE: Radiotherapy (RT) has been advocated for gliomatosis cerebri (GC) to delay tumor progression. However, patients with GC may experience poor performance status without recurrence after RT. Herein, we reviewed the responses of patients with GC treated with RT. MATERIALS AND METHODS: Seventeen patients with diffuse radiologic infiltration of more than two cerebral lobes and diagnosed as having grade II (N=12) or III (N=5) glioma were treated with a mean radiation dose of 51.8 Gy (range, 42-59.4 Gy). The mean radiation volume to a mean marginal dose of 42.8 Gy (range, 36-45 Gy) was 693.4 cc (range, 316.5-1279.6 cc), which was 45.3% of the mean whole brain volume. We reviewed the clinical prognostic factors related to progression-free survival (PFS) and overall survival (OS) in all patients and described characteristics of patients with poor performance (Eastern Cooperative Oncology Group performance scale 3-4) without recurrence. RESULTS: In all patients, the median PFS was 12.3 months, and oligodendroglial pathology (p=0.002) and non-enhanced tumor (p=0.002) were associated with an improved PFS. The median OS was 39.3 months, and young age and adjuvant chemotherapy were associated with improved OS (p=0.022 and 0.002, respectively). Based on multivariate analysis, adjuvant chemotherapy was significantly associated with a longer OS (p=0.012; hazard ratio=0.099; 95% CI, 0.016-0.596). Five patients (29.4%) showed poor performance without recurrence. The median age of these 5 patients was 65 years (range, 60-75 years). The mean radiation volume to a mean marginal dose of 41 Gy (range, 36-45 Gy) was 539.9 cc (range, 255.5-983.1 cc). The pathologic diagnosis was grade II glioma in 2 patients and grade III in 3 patients. The median OS was 10.0 months (range, 8.2-45.9 months). CONCLUSIONS: Some GC patients, especially the elderly, might have a poor performance status without recurrence after RT of a larger radiation field.

Gliomatosis cerebri: improved outcome with radiotherapy.

PURPOSE: Gliomatosis cerebri is a rare, diffuse involvement of the central nervous system by a malignant glioma that permeates the brain extensively without destroying the neural architecture and involves more than two lobes. In this study, we sought to assess the role of radiotherapy (RT) and identify prognostic factors in gliomatosis cerebri. METHODS AND MATERIALS: Thirty patients who received RT at a single institution and had radiographic follow-up were retrospectively reviewed with respect to outcome, radiation parameters, extent of surgery, and chemotherapy. All cases were analyzed histologically and documented. All pathology slides and radiology images were reviewed. RESULTS: The median age at diagnosis was 38.6 years (range 16-68). The median follow-up was 12.8 months (range 3-110). The mean radiation dose was 54.9 Gy, given in a mean of 28 fractions. Radiographic improvement or disease stabilization was achieved in 87% of patients. Clinical improvement was observed in 70%. The median time to progression was 10 months, and it was significantly longer for patients <40 years old (p = 0.0007) and for patients having a tumor histologic subtype other than glioblastoma (p = 0.01). The median overall survival was 18 months and was also longer for patients <40 years (p = 0.0001) and for patients having nonglioblastoma histologic features (p = 0.007). Extensive surgery, administration of chemotherapy, or increased RT volume improved neither overall survival nor the time to disease progression. CONCLUSION: RT is effective against gliomatosis cerebri. Patients who are young and have a nonglioblastoma tumor histologic subtype perform more favorably. In this analysis, no role for chemotherapy, extensive surgery, or whole-brain RT was found.

Serial positron emission tomography (PET) in gliomatosis cerebri treated with radiotherapy: a case report.

Results of serial positron emission tomography (PET) in a biopsy-proven case of gliomatosis cerebri (GC) are reported. Computed tomography (CT) with and without contrast failed to detect focal abnormalities, but magnetic resonance (MR) revealed iso-intensity or low-intensity lesions in T1-weighted images and high-intensity lesions in T2-weighted images. Lesions were seen in the left thalamus, right temporal lobe and claustrum, and pons. Radiotherapy remarkably improved clinical and imaging findings. Both before and shortly after radiotherapy, 11C-methionine PET images showed hypermetabolism while 15O-water PET images showed a marked increase in cerebral blood flow in GC lesions. However, 6 months later PET images had remarkably improved, appearing nearly normal.

Auxologic and biochemical characterization of the three phases of growth failure in pediatric patients with brain tumors.

Pediatric patients with brain tumors can loose 1 SD of height prior to beginning growth hormone (GH) therapy. The objectives of this study were to characterize the early growth failure, identify contributing factors and propose interventions. Five children were followed quarterly for 2 years to monitor auxological parameters, nutritional indices, and endocrine measuremnts. GH stimulation tests were done every 6 months to determine the timing of the onset of GH deficiency. The nadir for height velocity (HV) occurred 6 months after diagnosis. Poor gains in height correlated with decreased calorie count (p <0.001), poor weight gain (p <0.001), decreased BMI (p <0.001) and lowered leptin levels (p <0.001). All patients were able to secrete GH normally during this nadir of growth. Children treated for brain tumors demonstrate an early triphasic pattern of growth. Growth failure due to cachexia occurs first, then a second transient phase of normal growth is observed followed by a third phase of growth failure due to GH deficiency. Phase 1 is characterized by decreased HV, BMI, leptin levels and calorie counts. With recognition of this profile, the early growth failure might be preventable with aggressive nutritional rehabilitation.

[Gliomatosis cerebri: clinico-pathological and neuroimaging characteristics and the results of treatment with radiotherapy]. / Gliomatosis cerebri: características clinicopatológicas, de neuroimagen y resultados del tratamiento con radioterapia.

INTRODUCTION: Gliomatosis cerebri is a rare form of malignant neoplastic glial transformation that involves large areas of the central nervous system. OBJECTIVE: To describe clinical manifestations, pathognomonic neuroimaging findings and results of radiotherapy of gliomatosis cerebri. PATIENTS AND METHODS: We review clinical records and neuroimaging studies of two patients with gliomatosis cerebri identified from the files of brain tumor registries of two university hospitals. One patient underwent radiotherapy after surgery. RESULTS: Clinical manifestations and evolution were totally different in both patients despite the fact that both tumors had the same extension on neuroimaging studies. Magnetic resonance imaging revealed the extent of the lesion in both cases, comprising both cerebral hemispheres. Histopathological study revealed G-I and G-II astrocytomas. The patient treated with whole brain irradiation experienced clinical improvement and involution of the brain tumour on neuroimaging studies, and survived 20 months after surgery. CONCLUSIONS: Clinical manifestations of gliomatosis cerebri are protean. Therefore, neuroimaging studies and histopathological analysis of brain tissue allow the correct diagnosis. Radiotherapy may improve neurological function in some patients. However, it is necessary to compare the long-term evolution of treated and non-treated patients to evaluate clinical efficacy of radiotherapy.