Background: Extraskeletal mesenchymal chondrosarcoma (MCS) of the central nervous system (CNS) is extremely rare. Herein, we present the clinicopathological features of five CNS extraskeletal MCS. Material and Methods: Over the past 10 years, five cases of CNS MCS have been retrieved from in the archives of histopathology department. All biopsies were stained with vimentin, S-100, CD99, desmin, GFAP, INI1, WT1, STAT6, and EMA. Results: There were four males and one female patient in the age group of 1.5–35 years. The clinical and radiological impression was meningioma in three cases, glomus jugulare and primitive neuroectodermal tumor in one case each. All showed classic biphasic morphology, areas of undifferentiated small blue round cells sharply demarcated from the island of cartilage. Three patients experienced multiple recurrences and died subsequently. Conclusion: Extraskeletal MCS of CNS is rare and favors children and young adults. They show aggressive behavior and tend to recur despite surgery and radiotherapy.
Background: Surgical excision of giant (>4 cm size) vestibular schwannomas (VS) with preservation of facial nerve (FN) function remains a challenge. Objective: Our surgical technique using an extra-arachnoid plane of dissection and limited meatal drilling is described with the goal of improving FN preservation. Methods: Surgical results with respect to FN preservation were analyzed for two groups of giant VS patients: Group A–operated between 2002 and 2009 using “standard” surgical technique, group B—operated between 2009 and 2016 using extra-arachnoidal dissection and limited meatal drilling. All patients had a minimum follow-up of 1 year. Results: Group A: Of the 115 patients, total excision was possible in 103 (89.5%), near-total in 7 (6%), and subtotal in 5 (4.3%) patients. At a >6-month follow-up, 68 (59.1%) patients had good FN function (House-Brackmann [H&B] grades 1–2), while 21 (18.3%) patients had poor function (H&B grade 3–5). Grade 6 involvement was seen in 26 (22.6%). Five patients had lower cranial nerve impairment necessitating tracheostomy. Group B: Of the 98 patients, total excision was achieved in 70 (71.4%) patients, near-total in 9 (9.2%), and subtotal in 19 (19.4%). Four patients had repeat surgery; 14 underwent gamma-knife radiosurgery. At >6-month follow-up, 78 (79.5%) patients had good FN function (H&B grades 1–2), while 20 (20.4%) had poor function (H&B grade 3–5). Conclusions: With our 'modified' surgical technique of extra-arachnoidal dissection of VS throughout surgery and limited meatal drilling, an improved rate of functional FN preservation was observed.
Background: Management strategies for petroclival menigiomas remain controversial Objectives: We share our experience in management of large and giant true petroclival meniongiomas with special emphasis on patient reported quality of life parameters. Methods: This is a single center study of 47 patients between 2008 and 2018. All patients were checked for tumor specific parameters, clinical parameters, extent of surgical excision, and outcome, as assessed by Karnofsky performance score (KPS), Glasgow outcome score, clinical status, and by SF-36 questionnaire. Results: 32/47 patients' data were assessed. Symptoms included headache (62.5%), involvement of 5th nerve (47%), facial nerve (40.6%), lower cranial nerves (37.5%), cerebellar signs (84%), and long tract signs in (50%) of patients. The mean preoperative KPS was 83.75+/−6.59. Surgical approaches included retromastoid suboccipital craniotomy (50%), Kawase's approach (31.25%), and others in 18.25% patients. 40.625% (n = 13) had a gross total excision, near total resection (NTR) was achieved in 53.125% (n = 17), and 6.25% (n = 2) had a subtotal excision (STE). In 13 patients who had gross total resection (GTR), there were 12 (70.5%) new neurological deficits, while among the 19 patients with NTR, only 5 (29.5%) new neurological deficits were seen. No new onset neurological deficit was seen in patients with STE of tumor. Patient assessed QoL parameters were worse in patients with GTR and best in patients with NTR/STE + GKRS. Conclusion: In patients of large/giant petroclival meningiomas, NTE/STE with adjuvant GKRS provided better preservation of quality of life.
Background and Introduction Interrupted aortic arch (IAA) is a very rare congenital anomaly carrying high neonatal mortality rate if left untreated. Rarer still, is its presentation in teenage or adulthood. This condition has been found to be complicated with cerebral aneurysms, which is a consequence of hemodynamic stress and hypertension secondary to arch interruption. Cerebral aneurysms can further complicate the clinical course and lead to poor clinical outcomes, especially if ruptured. Clinical Presentation A 17-year-old female presented with ruptured basilar top aneurysm and was considered for endovascular coiling. Transfemoral access was chosen but the catheter could not be negotiated beyond proximal thoracic aorta. A computed tomographic angiography (CTA) of thorax and abdomen was performed, which showed isolated interruption of aortic arch. Subsequently, transradial route was used for coiling of the aneurysm. Conclusion To the best of our knowledge, the index case is one of the only seven cases of IAA with cerebral aneurysm that have been reported till date in medical literature. It also holds the unique distinction of being the first case of IAA with cerebral aneurysm treated by endovascular approach. Our case highlights the importance of transradial access in such pathological conditions.
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