Objective: To describe the clinical features and surgical outcome of a combined anomaly, i.e. split cord malformation (SCM) with meningomyelocele (MMC), and to propose an addition to Pang’s classification of SCM to accommodate a combined form of anomaly. Methods: We retrospectively analyzed 16 cases of such a combination, out of a total of 106 cases of spinal dysraphism treated and studied prospectively for outcome at our center. The clinical profile and outcome of these cases are described. Results: All cases had SCM and MMC. Nine patients were males, and the mean age of presentation was 3.9 years. Twelve patients had Pang’s type I SCM and the other 4 were of type II. The MMC sac was lumbar in 11 cases. In all the patients, SCM was present either at the same level as the MMC or one to two segments above it. Nine patients had motor weakness, 6 had hypoesthesia, 4 had urinary incontinence and 3 had trophic ulcers. Nine patients had neuroorthopedic syndrome. All cases, except 3 (who were operated on at birth, at which time ‘superficial surgery’ was performed without relevant imaging), underwent repair of the MMC and excision of the spur/septum at the same sitting. The 3 cases who had undergone superficial surgery, however, were operated on for SCM following investigation at our center, obviously at a second sitting. After an average follow-up of 7.2 months, 4 patients showed improvement in motor weakness, 5 in hypoesthesia and 3 in urinary symptoms, whereas trophic ulcers had healed in all cases. Conclusion: With respect to the occurrence of SCM at or above the level of an MMC, we feel it is apt to screen the entire spinal/neuraxis by MRI in children with MMC. We labeled this combined pathology ‘complex spina bifida’, and feel it is necessary to make a minor modification to Pang’s classification to accommodate the pure/combined anomalies together.
Human gliomas are the most common primary central nervous system neoplasm, and they are a complex, heterogeneous, and difficult disease to treat. In the past two decades, advances in molecular biology have revolutionized our understanding of the mechanism by which these neoplasms are initiated and progress. While surgery, radiation therapy, and chemotherapy have roles to play in the treatment of patients with gliomas; these therapies are self-limited because of the intrinsic resistance of glioma cells to therapy, and the diffusely infiltrating nature of the lesions. It is now known that malignant gliomas arise from a number of well-characterized genetic alterations and activations of oncogenes and inactivation of tumor suppressor genes. These genetic alterations disrupt critical cell cycle, growth factor activation, apoptotic, cell motility, and invasion pathways that lead to phenotypic changes and neoplastic transformation. Research in each of these fields has uncovered potential therapeutic targets that look promising for disease control. Gliomas can now be modeled with fidelity and reproducibility using several transgenic and knockout strategies. Transgenic mouse models are facilitating the testing of various therapeutic strategies in vivo. Finally, the recognition of the putative brain tumor stem cell, the tumor initiating cell in brain cancer, provides an enticing target through which we could eliminate the source of the brain tumor with increased efficacy and less toxicity to normal tissues. In this review, we provide an up-to-date discussion of the many of key technologies and tools that are being used in molecular biology to advance our understanding of the biological behavior of human malignant gliomas.
After familiarizing readers with some promising new techniques in the field of oncogenomics, this review will present the current state of knowledge as it pertains to the molecular biology of pediatric brain neoplasms. Along the way, we hope to highlight specific instances where the detailed mechanistic knowledge acquired thus far may be exploited for therapeutic advantage.
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