Adult medulloblastoma: a single institution experience.

UNLABELLED: Medulloblastoma is an aggressive posterior fossa tumor which is rare in adult. We aim to study the clinical features and outcome of adult medulloblastoma patients, and to identify poor outcome predictors. PATIENTS AND METHODS: From January 1995 to December 2006, 35 adult patients (>15 years of age at the time of diagnosis) underwent surgery for medulloblastoma followed by full dose of radiotherapy (54 Gy), with or without systemic chemotherapy. RESULTS: The mean age was 24 years (maximum 52). There were 19 male and 16 female patients. CSF spread was documented in 23 patients. Eight patients developed relapse. Four patients developed isolated CNS relapse, two patients developed isolated lung metastasis and 2 patients developed both lung metastasis and CNS relapse. The craniospinal radiation dose ranged from 34 to 36 Gy and the total posterior fossa dose ranged from 54 to 56 Gy. The 10 year disease free survival rate reached 65% +/- 10. The relapse free survival was significantly different according to the M stage (M0, M1, M2 & 3), as none of the patients in the M0 group had relapsed while 4 patients in the M1 group and 4 patients in the M2&3 group relapsed (p=0.0026). CONCLUSION: The distinction between adult and pediatric medulloblastoma is not clear. Overall, M stage is an important prognostic factor. Although chemotherapy is a part of standard practice in pediatric treatment protocols, its role in older patients should be further evaluated in clinical studies.

Successful therapy of cerebral phaeohyphomycosis due to Ramichloridium mackenziei with the new triazole posaconazole.

Cerebral phaeohyphomycosis caused by Ramichloridium mackenziei is universally fatal. All reported cases with long-term follow-up have indicated 100% mortality despite antifungal therapy and surgical intervention. We describe the case of a 62-year-old patient who underwent renal transplantation and had a cerebral abscess caused by R. mackenziei. The infection progressed despite surgical evacuation and therapy with liposomal amphotericin B, itraconazole, and 5-flucytosine. The patient was subsequently treated with the investigational triazole posaconazole oral suspension, 800 mg/day, in divided doses. Treatment with posaconazole resulted in progressive clinical and radiologic improvement. The patient is alive four years after diagnosis and maintained on posaconazole therapy. This case supports the potential role of this extended-spectrum azole in the treatment of this serious fungal infection of the central nervous system.

Cerebellar mutism caused by arteriovenous malformation of the vermis.

Transient mutism following posterior fossa tumour resection in children is well known in the literature. To our Knowledge, this phenomenon has never been reported without surgical intervention. We report a case of cerebellar mutism secondary haemorrhage from a vermian arteriovenous malformation (AVM), which resolved to ataxic dysarthria after 6 weeks. Embolization of the AVM was performed and the patient's clinical status continued to improve gradually till she became normal 6 months from the insult. The mutism was due to rupture of the AVM which might correlate the cerebellar mutism with the transient vasospasm of the blood vessels supplying the cerebellum. It is possible for a spontaneus posterior fossa bleed to result in transient mutism similar to post surgical mutism.

Intraparenchymal pericatheter cyst. A rare complication of ventriculoperitoneal shunt for hydrocephalus.

Intraparenchymal pericatheter cyst is rarely reported. Obstruction in the ventriculoperitoneal shunt leads to recurrence of hydrocephalus, signs of raised intracranial pressure and possibly secondary complications. Blockage of the distal catheter can result, unusually, in cerebrospinal fluid oedema and/or intraparenchymal cyst around the ventricular catheter which may produce focal neurological deficit. We report two cases of distal catheter obstruction with formation of cysts causing local mass effect and neurological deficit. Both patients had their shunt system replaced, which led to resolution of the cyst and clinical improvement. One patient had endoscopic exploration of the cyst which confirmed the diagnosis made on imaging studies. Magnetic resonance imaging was more helpful than computed tomography in differentiating between oedema and collection of cystic fluid. Early recognition and treatment of pericatheter cyst in the presence of distal shunt obstruction can lead to complete resolution of symptoms and signs.

Prognostic factors for medulloblastoma.

PURPOSE: To evaluate prognostic factors for medulloblastoma. METHODS AND MATERIALS: One hundred and seventy-three consecutive patients with medulloblastoma, treated at King Faisal Specialist Hospital (KFSH) from 1988-1997, were reviewed. Eighty-four percent were children less than 15 years old. From 1988-1994, treatment was at the discretion of the investigator. From 1994-1998, patients entered a single-arm best practice protocol in which, in staged patients, the surgical intent was total resection, standard radiation treatment was defined, and adjuvant chemotherapy was given to a "high-risk" subset. RESULTS: For 150 patients who completed surgical and radiation treatment, the 5-year survival rate was 58%, compared with 0% for 16 patients who were unable to start or complete radiation treatment. For staged patients, the 5-year survival was M0 + M1, 78% and M2 + M3, 21% (p < 0.0001). Other favorable significant prognostic factors were age >14 years and gross cystic/necrotic features in the primary tumor. The size of the primary tumor, the degree of hydrocephalus at diagnosis, the presence of residual tumor in the post-operative CT/MRI, and the functional status of the patient prior to radiation treatment were not significant factors. CONCLUSIONS: Stage M0 + M1 was the most powerful favorable prognostic factor. In Saudi Arabia more patients present with advanced disseminated disease, 41% M2 + M3, than in the West, and this impacts adversely on overall survival. Total resection and standard radiation treatment were not sensitive prognostic factors in a treatment environment in which 78% of patients underwent at least 90% tumor resection and 60% received standard radiation treatment. In order to improve the proportion of patients able to complete radiation treatment, consideration should be given to limiting resection when the attainment of total resection is likely to be morbid, and to delaying rather than omitting radiation treatment in the patient severely compromised postoperatively.

Oligodendroglioma: an analysis of prognostic factors and treatment results.

This study, an analysis of variable prognostic factors affecting the treatment outcome for patients with oligodendroglioma, included a retrospective analysis of the medical charts of patients diagnosed with oligodendroglioma treated at our institution between 1975 and 1997. The endpoints analyzed were the progression-free survival (PFS), as well as the overall survival. The factors analyzed included extent of surgery, postoperative radiotherapy, pathologic grade, performance status, age, and sex. Of a total of 37 cases, 19 were male and 18 were female. The median age at diagnosis was 30 years. The most common presenting symptoms were headache (78%), seizures (43%), motor symptoms (38%), and to a lesser extent behavioral changes (16%). The median duration of symptoms was 9 months. The most common location on computed tomography or magnetic resonance imaging scans was the frontal region (43%). Low grade tumors (grades I and II) were found in 60% of patients, and the remaining 40% had high grade tumors (grades III and IV). Eight patients had complete surgical excision, whereas 27 patients had partial excision, and two patients had biopsy only. The operative mortality rate was 14%. There were 24 patients who received postoperative radiotherapy, and only 3 patients received adjuvant chemotherapy. The median postoperative radiation dose was 5,580 cGy. With a median follow-up of 7 years, the 5-year PFS and overall survival for the whole group were 58% and 67%, respectively. The pathologic grade of the tumor was the only prognostic factor significantly affecting both PFS and overall survival. The 5-year PFS for patients with low grade tumors was 79% in comparison to 32% for patients with high grade tumors (p < 0.01). Patients with good performance status at initial presentation (performance status of 1 and 2) had a higher 5-year PFS in comparison to those with poor performance status (62% vs. 38%, respectively); however, this difference did not reach statistical significance. Similarly, patients who were subjected to complete surgical excision had a marginally higher PFS in comparison to those who had biopsy or partial excision (75% vs. 53%). There was no difference in the 5-year PFS between patients who received postoperative irradiation versus those who did not (51% vs. 47%, respectively). Patients with high grade oligodendrogliomas have a relatively poor prognosis. The pathologic grade of the tumor was the single most important prognostic factor significantly affecting both the PFS and overall survival. A prospective randomized clinical trial is needed to address the impact of postoperative irradiation on PFS of those tumors. In view of the poor outcome for patients with high grade oligodendroglioma, the use of adjuvant systemic chemotherapy should be studied in future multicenter randomized trials.

Childhood intracranial arteriovenous malformation in Saudi Arabia.

Twenty-three patients in whom intracranial arteriovenous malformation (ICAVM) occurred while they were in the pediatric age group (16 years or less) were identified. The patients had presented to three major centers in Saudi Arabia (King Fahad Hospital of the University, King Faisal Specialist Hospital and Research Center, and Riyadh Military Hospital). The incidence of ICAVM in Saudi Arabia is estimated. ICAVM in the pediatric age group accounts for 7.72% of all ICAVM cases. Most of the pediatric patients presented with intracranial bleeding, and seizure was the only other mode of presentation. Spontaneous changes involving ICAVM occurred in 4 children, in the form of either increase or decrease in size or new vascularization. Most children had higher grade ICAVM and the morbidity rate was higher than in adults. The cure rate was 50% with different therapeutic modalities. This report represents the first study to provide the hospital-based frequency of pediatric ICAVM in Saudi Arabia.

Collaborative management of intracranial arteriovenous malformation.

Full text is available as a scanned copy of the original print version.

Craniopharyngioma in children.

PURPOSE: The treatment regimens at presentation and recurrence, the related morbidity, and survival rates were studied for children with craniopharyngioma who were treated at King Faisal Specialist Hospital & Research Center (KFSH&RC). METHODS & MATERIALS: From 1975 to 1996, a total of 56 children younger than 18-years-old at diagnosis underwent treatment with surgery and/or radiotherapy for craniopharyngiomas at KFSH&RC. There was evidence that these patients had advanced disease at diagnosis; 36% of 51 patients whose visual status pretreatment was known were either blind or had major bilateral visual defects prior to treatment, and 36% of the 56 patients had diabetes insipidus. This report concentrates on 44 patients who had first definitive resection at KFSH&RC. Treatment policy was total resection if possible (17 patients), if not, subtotal resection (17 patients) or lesser procedures (10 patients). Five patients received postoperative irradiation after first definitive resection. RESULTS: Ten-year-survival, and progression-free and event-free survival rates were 65%, 39%, and 29%, respectively. There were 9 postoperative deaths; 7 following the first procedure and 2 following resection for relapse, and 3 early deaths at home prior to relapse, following the first resection. None of the 22 patients who underwent less than total resection without postoperative radiation treatment were progression-free at 5 years, whereas the 5 patients who were irradiated remain progression-free. CONCLUSION: A more selective approach to total resection, and the routine use of postoperative irradiation following lesser surgical procedures requires evaluation.

Nocardial brain abscess in a renal transplant recipient successfully treated with triple antimicrobials.

Nocardia is a serious opportunistic infection in renal transplant recipients and nocardial brain abscess in these patients has a high mortality. In addition to antimicrobial therapy, treatment usually involves craniotomy and excision of the abscess. We describe a renal transplant recipient maintained on cyclosporine and prednisone developing Nocardia Asteroides brain abscess. After stereotactic aspiration of the abscess, successful treatment was achieved by triple therapy with trimethoprim sulfamethoxazole (TMP/SMX), ceftriaxone and amikacin. The allograft function remained stable. Long-term prophylaxis with TMP/SMX is necessary to prevent the relapse of nocardia.

Cerebellar astrocytoma: report of 13 cases aged over 20 years and review of the literature.

Cerebellar astrocytoma in adulthood is a distinct neoplasm with a variable prognosis. We reviewed retrospectively 13 cases in patients aged 21 years or over, seen between 1976 and 1992. These accounted for 19% of all cases of cerebellar astrocytoma seen during that period. The tumour was located in the midline in six patients and laterally in seven; seven patients were females and six were males. The low grade tumours had an overall favourable course, similar to that seen in cerebellar astrocytoma in childhood, with follow-up ranging from 4 to 10 years. The poorly differentiated form, seen in four of the cases, showed progression or recurrence within 4-24 months, regardless of the extent of removal or post-operative radiation therapy.

Treatment results for 149 medulloblastoma patients from one institution.

PURPOSE: Retrospective analysis of patients with medulloblastoma to determine the effectiveness of previous treatments for medulloblastoma and plan for future management strategies. METHODS AND MATERIALS: During the period March 1976 to December 1991, 172 patients with cerebellar medulloblastoma were referred to King Faisal Specialist Hospital and Research Center. One hundred and forty-nine patients were treated with curative intent. There were six postoperative deaths, and 10 patients planned for radiotherapy treatment failed to complete the prescribed course. One hundred and thirty-three patients completed a course of radiotherapy after surgery. Adjuvant chemotherapy was not used routinely (six patients only). Tumors were staged retrospectively according to the Chang staging system. There were no T1 patients, 32 patients had T2 tumors, 76 had T3 tumors, and 29 had T4 tumors. The T stage could not be allocated in 12 patients. Ninety-nine patients required a shunting procedure either pre- or postoperatively. Forty-six patients had complete resection of tumor, 91 had incomplete resection, and 6 patients had biopsy only. The extent of resection could not he determined in six patients. The median radiation dose for the whole brain was 34 Gy, spine 32.5 Gy, and posterior fossa 52.8 Gy. Fraction sizes ranged from 1.7-1.8 Gy for craniospinal fields and 2 Gy for the posterior fossa boost. Seventy percent completed the prescribed course within 7 weeks. RESULTS: Actuarial survival for the whole group of 149 patients was 53% at 5 years and 38% at 10 years. On univariate analysis, patients with T2 tumors did significantly better as compared to patients with T3 and T4 tumors. Survival of patients who had clinical and radiological complete resection of tumor at surgery was significantly better than patients with incomplete tumor removal. The presence of a ventriculoperitoneal (VP) shunt had a significant negative impact on survival. Treatment failure by site was analyzed with respect to the radiation dose. Doses greater than 50 Gy for the posterior fossa, and greater than 30 Gy for craniospinal axis, resulted in significantly better survival. On multivariate analysis, the only significant prognostic factor was the presence of a VP shunt in patients with T2 tumors. CONCLUSION: T stage, VP shunt, radiation doses and extent of surgery were important prognostic factors. In this study, radiation doses of more than 50 Gy to the posterior fossa and 30 Gy to the craniospinal axis resulted in improved survival.

Hindbrain herniation: an unusual occurrence after shunting an intracranial arachnoid cyst. Case report.

The case is presented of a prepontine arachnoid cyst in a 28-year-old woman. She was treated by craniotomy, stereotactic aspiration, internal shunting, and finally by insertion of a cystoperitoneal shunt. Eight months later she developed an asymptomatic Arnold-Chiari type I malformation. The pathogenesis of this unusual hindbrain herniation is explained on the basis of the differential pressure between two compartments and cephalocranial disproportion.

Microsurgery for giant craniopharyngiomas in children.

The authors report 20 cases of giant craniopharyngioma in children operated on during the 6-year period from 1978 through 1984. The tumor size (maximal diameter) ranged from 5 to 11 cm. These patients presented with increased intracranial pressure, severe visual loss, and neurological and endocrinological deficits. Tumor growth had occurred in several directions, causing displacement of the circle of Willis, the optic apparatus, and the hypothalamus, as well as encroachment into the interpeduncular and prepontine cisterns and into the frontal, temporal, and posterior fossae. The surgical technique for total removal of these giant tumors in the last 10 cases is described. Emphasis is placed on the use of microsurgical technique during the first operation, close endocrinological follow-up, and early reevaluation by computed tomographic scanning.

Primary cranial and intracranial chondrosarcoma. A survey.

Fifty cases of intracranial and cranial chondrosarcoma were reviewed in the world literature including two of our own. These were analyzed relevant to their histological subgroup, site of origin, age and sex incidence, calcification and vascularity, recurrence, and metastases. The analysis was done in order to clarify points in the diagnosis, management, and prognosis of this rare tumour. The mesenchymal subtype is a more malignant form with a higher tendency for recurrence, metastasis, and increased vascularity.