Intracranial Low-Grade Fibromyxoid Sarcoma with Atypical Radiology.

BACKGROUND: Low-grade fibromyxoid sarcoma (LGFMS), an uncommon soft tissue tumor, generally occurs in extremities and is characterized by an aggressive clinical course with frequent recurrences. Primary intracranial occurrence of LGFMS is rare. The few existing descriptions present the clinicopathologic aspects of LGFMS and lack information from a radiologic perspective. In these reports, the lesions appeared solid on imaging. CASE DESCRIPTION: In a 45-year-old woman, a falx-based lesion with solid-cystic radiologic appearance that turned out to be LGFMS on histopathology. The patient's clinical follow-up (2 years) is presented along with a literature review of other primary central nervous system cases. CONCLUSIONS: This report emphasizes the radiologic aspects of LGFMS and describes an atypical solid-cystic appearance of this uncommon central nervous system tumor. This adds another facet to the imaging of LGFMS. Although rare, LGFMS can form a differential diagnosis of dural-based lesions.

Improvise, adapt and overcome-challenges in management of pediatric congenital atlantoaxial dislocation.

OBJECTIVE: Congenital atlanto-axial dislocation (CAAD) often manifests in younger age group. Softer bones, deformed joints and the concern of fusing multiple segments in growing age set apart this subset of patients from adults. Objectives of this manuscript are to discuss the challenges faced and subsequent solutions during multiplanar realignment and fusion (short segment) of C1-2 joints through a direct posterior approach in pediatric CAAD. PATIENTS & METHODS: 56 pediatric patients with CAAD were operated through a direct posterior approach in the last 4 years. The joints were drilled and manipulated to achieve multiplanar realignment. C1-2 joints were fused (short segment). The pre and post operative clinical and radiological data was compared. RESULTS: Atlanto-axial dislocation was irreducible in 35 and reducible in 21 patients. Forty-nine (87.5%) patients were partially or totally dependent. The joints in the irreducible groups were oblique and deformed. Nine patients had lateral angular dislocation, 3 had C1-2 spondyloptosis and 5 had significant vertical dislocation. Drilling and manipulation was feasible in all cases. The bones were soft and partly cartilaginous in the pediatric population. Techniques were modified to achieve optimal bony purchase subsequent to drilling the relatively small bones and prevent screw pull-outs during intraoperative manipulation for the problems we had faced in our initial cases. Despite the challenges in initial cases, realignment could be achieved in all. There was a significant improvement in follow up modified JOA score and 28 patients were independent at 4-month follow up. Two patients had partial redislocation at 4-month follow up. CONCLUSIONS: Pediatric patients have deformed and oblique joints, thereby making complete spondyloptosis, severe vertical dislocation and lateral tilt common in this age group. Though pediatric bones are soft and small, it is possible to achieve multiplanar realignment by drilling and manipulation of C1-2 joints. The realignment and short segment C1-2 fusion in these patients has a good radiological and clinical outcome.

'Congenital anomalies of craniovertebral junction presenting after 50 years of age': An oxymoron or An unusual variation?

OBJECTIVES: Manifestation of congenital anomalies of cranio-vertebral junction (CVJ) in the later half of life is unusual and intriguing. Coexisting cervical spondylotic changes with multilevel compression, poorer bone quality as well as less smooth post-surgical recuperation make management of elderly Congenital Atlantoaxial Dislocation/ Basilar Invagination (CAAD/BI) challenging. The clinico-radiological presentation, pathogenesis and outcome are analysed here. PATIENTS AND METHODS: Clinico-radiological data of 20 patients of CAAD/BI (with markers of congenital anomalies) presenting after 50 years of age, the challenges faced and outcomes after C1-C2 fusion have been analysed. RESULTS: Three distinct groups were identified. Seven patients with Os-odontoideum had reducible AAD (Type I). Seven patients had assimilated C1, C2-3 fusion and deformed C1-2 joints with irreducible AAD/BI (Type II). In type III, 4 patients had similar segmentation defects but with compression at both cervico-medullary junction and subaxial spine, although clinical localisation pointed to the CVJ. Spastic quadriparesis was the commonest presentation. All underwent C1-2 fusion alone. There was significant improvement in 18, including those with compression at additional level. Bony fusion was documented in all patients followed up beyond one year. CONCLUSION: Congenital CVJ anomalies may present in later half of life, though attempts at reasoning remain speculative. These patients improve after multiplanar realignment and C1-2 fusion. Careful clinico-radiological evaluation is required in those with additional subaxial compression. Bone quality in elderly is not a deterrent for instrumentation. Fusion eventually occurs in most.