Excision of pediatric craniopharyngioma: pattern of recurrence in 35 patients at a tertiary care hospital in Saudi Arabia.

BACKGROUND AND PURPOSE: Craniopharyngiomas are benign tumors of central nervous system which are known to affect both adults and children. Despite their benign origin, the recurrence is still one of the main postoperative challenges. The aim of this study was to investigate in retrospect factors related to recurrence of craniopharyngioma in a tertiary center in Riyadh, Saudi Arabia. PATIENTS AND METHODS: We conducted a review of charts of all craniopharyngioma patients operated in neurosurgery department at King Faisal Specialist Hospital & Research Center in Riyadh (KFSH-RC). Age at surgery, gender, body mass index, symptoms at presentation, hormonal data, tumor characteristics and location, presence of hydrocephalus, previous treatments, neuroimaging features, surgical results, and recurrence were abstracted from the medical charts of the patients retrospectively. RESULTS: In all, 70.6% of patients had gross total resection (GTR). The recurrence after GTR in our series was 25% which considered low when compared to most surgical series. From all above studied variables, VP shunt insertion at presentation was constantly significant in both uni- and multi-variable analysis. CONCLUSION: In this study, we analyzed several factors to determine if they had any significant correlation with recurrence. Only VP shunt insertion was found significant. Further researches are needed to verify these factors and to discover others.

Spinal Pilomyxoid Astrocytoma.

Pilomyxoid astrocytoma (PMA) is a rare brain tumour generally located in the chiasmatic-hypothalamic region. In comparison to pilocytic astrocytoma, PMA has distinct histopathological features, aggressive clinical behaviour, a high recurrence rate, and early cerebrospinal fluid dissemination. Only 14 cases of PMA have been reported in the spinal cord since its pathological description in 1999. Here, we report the 15th case in a 3-year-old girl who was treated with chemoradiotherapy and followed up for 5 years. In this report, we also present a review of spinal PMA including treatment options and prognosis.

Aspergillus flavus-Induced Brain Abscess in an Immunocompetent Child: Case report.

Intracranial aspergillosis is an extremely rare manifestation of invasive aspergillosis in immunocompetent children and is associated with high morbidity and mortality. We report a 12-year-old immunocompetent male child who was referred to the King Faisal Specialist Hospital & Research Centre, Riyadh, Saudi Arabia, in May 2010 after a sudden-onset headache and loss of consciousness. Brain imaging revealed a large right space-occupying occipital lesion and the patient underwent a craniotomy and resection. Histopathology of the lesion revealed necrotising granulomatous fungal encephalitis with many hyphae engulfed by multinucleated giant histiocytes. Two days later, a computed tomography scan showed debulking of the fungal mass and the patient was discharged on oral voriconazole. However, imaging at a six-week follow-up showed progression of the abnormality. A residual or persistent fungal brain lesion was suspected. Further neurosurgical resection of the lesion was performed and cultures showed growth of Aspergillus flavus. The patient was treated successfully with antifungal therapy over the following two years.

Ventriculoperitoneal shunt peritoneal catheter knot formation.

The ventriculoperitoneal (VP) shunt is a common procedure in pediatric neurosurgery that carries a risk of complications at cranial and abdominal sites. We report on the case of a child with shunt infection and malfunction. The peritoneal catheter was tethered within the abdominal cavity, precluding its removal. Subsequently, laparoscopic exploration identified a knot at the distal end of the peritoneal catheter around the omentum. A new VP shunt was inserted after the infection was healed. This type of complication occurs rarely, so there are a limited number of case reports in the literature. This report is complemented by a literature review.

Turcot syndrome: a synchronous clinical presentation of glioblastoma multiforme and adenocarcinoma of the colon.

Turcot syndrome (TS) is a rare hereditary disorder clinically characterized by the occurrence of primary tumors of the colon and the central nervous system (CNS). Here we present the case of an 11-year-old boy with a synchronous clinical presentation of both glioblastoma multiforme (GBM) and colonic adenocarcinoma. A molecular genetic study revealed microsatellite instability in the DNA mismatch repair (MMR) gene. This patient ultimately survived for 13 months after clinical presentation. Based on this case study, the synchronous presentation of glioblastoma multiforme and adenocarcinoma of the colon might suggest a shorter survival rate for patients with Turcot syndrome. A literature review complements this paper.

Cauda equina syndrome as the initial presenting clinical feature of medulloblastoma: a case report.

INTRODUCTION: Medulloblastoma is one of the most common pediatric brain malignancies. The usual presenting clinical features are related to posterior fossa syndrome or/and hydrocephalus. Cauda equina syndrome is a very rare presentation for this disease. CASE PRESENTATION: We describe the case of a three-year-old boy with cauda equina syndrome as the initial presenting clinical feature for medulloblastoma. He was initially diagnosed as having a spinal tumor by magnetic resonance imaging scan. Subsequently, a cranial magnetic resonance imaging scan revealed a posterior fossa tumor with features of dissemination. He had substantial improvement after treatment. This case report is complemented by a literature review related to this unusual presentation. CONCLUSIONS: Medulloblastoma primarily presenting with cauda equina syndrome is very rare. However, spinal drop metastasis should be considered in the pediatric age group to avoid suboptimal management.

Response of medulloblastoma cells to vincristine and lomustine: role of TRKC, CTNNB1 and STK15.

BACKGROUND: Vincristine and lomustine are two important chemotherapeutic drugs used for the treatment of different types of neoplasms, including medulloblastomas. MATERIALS AND METHODS: We investigated the effects of vincristine and lomustine on 12 primary medulloblastoma cell cultures and the DAOY cell line using the annexinV-flow cytometry and immunoblotting techniques, following treatment of cells for different periods of time. RESULTS: Both drugs triggered apoptosis and cell cycle delay at the G(2)/M phase and also up-regulated p16. Furthermore, the expression of 8 different cancer-related genes were assessed and their mRNA and protein levels were found to be highly heterogeneous and did not correlate in several medulloblastoma cultures. Importantly, there was significant correlation between the level of cadherin-associated protein beta 1 (CTNNB1) and Aurora kinase A (STK15) proteins and neurotrophic tyrosine kinase receptor type 3 (TRKC) mRNA and the proportion of apoptosis induced by vincristine, the combination of both drugs, and lomustine, respectively. CONCLUSION: These genes could be of great importance as therapeutic biomarkers during the treatment of medulloblastoma patients with vincristine and lomustine.

Curcumin inhibits the Sonic Hedgehog signaling pathway and triggers apoptosis in medulloblastoma cells.

Medulloblastoma is an aggressive primary brain tumor that arises in the cerebellum of children and young adults. The Sonic Hedgehog (Shh) signaling pathway that plays important roles in the pathology of this aggressive disease is a promising therapeutic target. In the present report we have shown that curcumin has cytotoxic effects on medulloblastoma cells. Curcumin suppressed also cell proliferation and triggered cell-cycle arrest at G(2)/M phase. Moreover, curcumin inhibited the Shh-Gli1 signaling pathway by downregulating the Shh protein and its most important downstream targets GLI1 and PTCH1. Furthermore, curcumin reduced the levels of beta-catenin, the activate/phosphorylated form of Akt and NF-kappaB, which led to downregulating the three common key effectors, namely C-myc, N-myc, and Cyclin D1. Consequently, apoptosis was triggered by curcumin through the mitochondrial pathway via downregulation of Bcl-2, a downstream anti-apoptotic effector of the Shh signaling. Importantly, the resistant cells that exhibited no decrease in the levels of Shh and Bcl-2, were sensitized to curcumin by the addition of the Shh antagonist, cyclopamine. Furthermore, we have shown that curcumin enhances the killing efficiency of nontoxic doses of cisplatin and gamma-rays. In addition, we present clear evidence that piperine, an enhancer of curcumin bioavailability in humans, potentiates the apoptotic effect of curcumin against medulloblastoma cells. This effect was mediated through strong downregulation of Bcl-2. These results indicate that curcumin, a natural nontoxic compound, represents great promise as Shh-targeted therapy for medulloblastomas.

Localized acalvaria with craniosynostosis.

The authors report here a 3-year-old boy with localized absence of calvarial bones (partial acalvaria/hypocalvaria), associated with unique sagittal calcified bone and craniosynostosis. This major skull deformation led to a prominent protrusion of the cerebral hemisphere and other significant parenchymal changes that were well demonstrated by neuro-imaging. No digital abnormalities and only minor facial dysmorphism were observed; however, his development is grossly delayed. History and detailed investigations failed to explain the cause of his condition. Mutation testing of fibroblast growth factor receptor (FGFR) 1, 2 and 3 gave normal results. The combination of craniosynostosis and acalvaria in this surviving boy could be a rare presentation of a more lethal condition.

Congenital supratentorial meningeal arteriovenous malformation with hemangioma and massive arachnoid cell hyperplasia.

CASE REPORT: We describe the clinical, radiological and pathological findings of concurrent, congenital leptomeningeal arteriovenous malformation with hemangioma diagnosed in a newborn by prenatal and immediately postnatal magnetic resonance imaging. Vascular abnormalities were accompanied by massive arachnoidal cell hyperplasia reminiscent of meningioma. CONCLUSION: To the best of our knowledge, this is the first case of such a lesion reported in the literature.

Retained orbital wooden foreign body: a surgical technique and rationale.

OBJECTIVE: To emphasize the potential complications of a retained orbital wooden foreign body (WFB) and the rationale of a surgical technique. DESIGN: Two interventional case reports. PARTICIPANTS: Two patients sustained an orbital WFB injury. Both patients had ocular complications despite repeated attempts to remove the suspected residues. INTERVENTION: Computed tomography and magnetic resonance imaging of both patients at different intervals revealed evidence of orbital foreign body migration toward the cranium. A surgical technique combing transcranial and orbital approaches was used to remove the residues. MAIN OUTCOME MEASURES: Preoperative and postoperative vision, proptosis, ocular motility, and various ocular symptoms and signs. RESULTS: In both patients, no postoperative complications were seen, and all preoperative symptoms and signs were resolved at 9- and 19-month follow-ups, respectively. CONCLUSIONS: A retained orbital WFB can cause early or late complications and is known to have the potential to migrate intracranially. In selected patients, a team approach may be the best technique to ensure complete removal.