Intracranial solitary fibrous tumors: Clinical, radiological, and histopathological insights along with review of literature.

BACKGROUND: Intracranial solitary fibrous tumors (SFTs) are rare mesenchymal neoplasms, often challenging to diagnose due to their resemblance to meningiomas and other central nervous system tumors. While advancements in molecular genetics have aided in classification, diagnostic nuances and optimal management strategies remain areas of interest. MATERIALS AND METHODS: This retrospective study analyzed 11 cases of intracranial SFTs treated at a neurosurgical centre in India between February 2020 and January 2024. Clinical data, radiological findings, histopathological features, and follow-up details were reviewed. Immunohistochemistry, particularly STAT6, facilitated diagnosis confirmation. RESULTS: The median age of presentation was 32 years, with a male predominance. Headache was the most common presenting symptom, often leading to misdiagnosis as meningiomas on radiological imaging. Histologically, SFTs exhibited spindle to ovoid cells with staghorn vessels and collagenized stroma, posing challenges in differential diagnosis. WHO grading predominantly revealed grade 1 tumors, though recurrence occurred, emphasizing the importance of long-term follow-up. Immunohistochemistry, particularly STAT6, played a pivotal role in distinguishing SFTs from other entities. CONCLUSION: Intracranial SFTs present diagnostic challenges due to overlapping features with other tumors, warranting a comprehensive approach integrating clinical, radiological, and histopathological findings. Immunohistochemistry, particularly STAT6, emerges as a valuable diagnostic tool. Long-term follow-up is essential for monitoring recurrence and potential malignant transformation. Further research is needed to delineate optimal treatment strategies, including the role of radiotherapy in SFT management.

Enigmatic echoes: Exploring brainstem neurenteric cysts - A case series and literature review.

INTRODUCTION AND IMPORTANCE: Neurenteric cysts (NCs) are rare congenital anomalies within the central nervous system, originating from the endodermal layer due to inappropriate embryonic notochordal plate and endoderm separation. Despite constituting a minute fraction of CNS tumors, their diverse clinical presentations and brainstem occurrence pose diagnostic challenges. CASE PRESENTATION: This study presents a series of three brainstem NCs, detailing demographics, clinical presentations, radiological features, and operative findings. Patients aged 2-40 years exhibited varied symptoms including headaches, vomiting, cranial nerve deficits, and motor/sensory deficits. Literature review of 27 cases showed a mean age of 26.8 years, predominantly in females, with diverse cystic characteristics on MRI. Surgical intervention achieved mostly complete resection, with varying outcomes including recurrence and malignant transformation. CLINICAL DISCUSSION: This study sheds light on the rare entity of brainstem NCs, emphasizing their diagnostic complexity. NCs, though constituting a minute fraction of CNS tumors, present a diagnostic challenge due to their diverse clinical presentations and rarity in the brainstem. CONCLUSION: This study provides insights into brainstem NCs, emphasizing diagnostic complexity and the importance of MRI in accurate diagnosis and surgical intervention. Enhanced collaboration is needed to refine management strategies for these intriguing lesions.

Epidermoid cyst of central nervous system: A case series and review of literature.

INTRODUCTION: Epidermoid cysts, originating from ectodermal cell remnants during embryogenesis, are rare, slow-growing tumours commonly found in the cerebellopontine angle, fourth ventricle, and sellar/parasellar regions. This retrospective study explores clinicopathological features, demographics, and radiological details of fifteen central nervous system epidermoid cyst cases over three years. MATERIALS AND METHODS: Histologically confirmed cases undergoing surgical excision from July 2020 to July 2023 were retrospectively evaluated. Patient age, clinical presentations, radiological features, and histopathological findings were analyzed using descriptive statistics and aligned with the PROCESS criteria. RESULTS: Fifteen cases, spanning various age groups and clinical presentations, were included. There were four cases each in the posterior fossa and cerebellopontine angle, three in temporal region, two in intraspinal region, and one each in the retromastoid intra-diploic and third intra-ventricular region. Lesions exhibited diverse distribution, gross findings, and microscopic features. Radiological imaging exhibited well-defined hypo-attenuated masses on computed tomography and contributed to the preoperative diagnosis in eight cases. Despite challenges in complete excision due to adherence, 13 cases underwent successful resection. Chemical meningitis, a common postoperative complication, underscores the importance of meticulous surgery. CONCLUSION: This study highlights clinicopathological characteristics, challenges in surgical management, and postoperative complications of central nervous system epidermoid cysts. Comparative analysis with existing literature emphasizes the uniqueness of epidermoid cysts, differentiating them from craniopharyngiomas and dermoid cysts. Despite the retrospective design, valuable insights are provided, emphasizing the need for prospective studies. Effective management involves complete excision, ensuring long-term remission and emphasizing the distinctive nature of these intriguing intracranial lesions.

A Rare Tumor of Clivus Masquerading as Pituitary Adenoma.

Giant cell tumors (GCT) are generally benign, commonly affecting young adults, with a slight preponderance in females. They are locally aggressive with a high rate of local recurrence. Most of them are found in the epiphysis of long bones, making the base of the skull a rare site. We report the case of a 35-year-old female, who presented with neurological symptoms of headache and diplopia. On magnetic resonance imaging, a space-occupying lesion was discovered in the clivus. Histopathology was diagnostic of a GCT. GCT arising from the clivus is extremely uncommon, with about 15 cases published in the literature. The present case highlights the rarity of this tumor and contributes to the existing literature with analysis and evaluation of the management strategies and prognosis.

Ruptured Distal Middle Cerebral Artery Mycotic Aneurysm: A Rare, First Presentation of Infective Endocarditis.

Mycotic cerebral aneurysms are rare inflammatory aneurysms associated with high mortality and morbidity reaching up to 80% after rupture. We report a case of incidentally diagnosed infective endocarditis presenting with rupture of distal middle cerebral artery mycotic aneurysm and intracerebral hematoma. Aneurysmectomy with clip ligation of the terminal cortical branch and hematoma evacuation was done with good surgical outcome.

Primary Giant Sphenotemporal Intradiploic Meningioma.

Intradiploic meningioma is a rare subset of meningioma accounting for 1% of all cases. Authors report a rare case of giant sphenotemporal intradiploic meningioma with orbital extension in a 27-year-old female. It was managed successfully with complete surgical excision and bony reconstruction using autologous split thickness bone graft.

A rare case of white pearls in brainstem.

Intrinsic brainstem epidermoid is extremely rare, and only 14 cases have been reported. Authors report a classic case of brainstem epidermoid in a 14-year-old male child presenting with symptoms of brainstem involvement. The child underwent a successful surgical excision. The lesion was intrinsic and caused diagnostic dilemma based on conventional radiological images. Based on our experience in this case and a thorough review of literature, we are of the opinion that diffusion-weighted images are very important in establishing the diagnosis. Such lesions are challenging and attempt to remove adherent tumor capsule may produce additional neurological deficits.

Dwarf with dual spinal kyphotic deformity at the cervical and dorsal spine unassociated with odontoid hypoplasia: Surgical management.

Morquio's syndrome is associated with systemic skeletal hypoplasia leading to generalized skeletal deformation. The hypoplasia of odontoid process is frequent association, which is responsible for atlantoaxial dislocation causing compressive myelopathy. However, development of sub-axial cervical kyphotic deformity unassociated with odontoid hypoplasia is extremely rare, and coexistence of dorsal kyphotic deformity is not reported in the western literature till date and represents first case. Current case is 16-year-old boy, who presented with severe kyphotic deformity of cervical spine with spastic quadriparesis. Interestingly, he also had additional asymptomatic kyphotic deformity of dorsal spine; however, odontoid proves hypoplasia was not observed. He was only symptomatic for cervical compression, accordingly surgery was planned. The patient was planned for correction of cervical kyphotic deformity under general anesthesia, underwent fourth cervical corpectomy with resection of posterior longitudinal ligament and fusion with autologous bone graft derived from right fibula, which was refashioned approximating to the width of the corpectomy size after harvesting and fixed between C3 and C5 vertebral bodies and further secured with anterior cervical plating. He tolerated surgical procedure well with improvement in power with significant reduction in spasticity. Postoperative X-ray, cervical spine revealed complete correction of kyphotic deformity cervical spine. At follow-up 6 months following surgery, he is doing well. Successful surgical correction of symptomatic cervical kyphotic deformity can be achieved utilizing anterior cervical corpectomy, autologous fibular bone graft, and anterior cervical plating.

A case of primary spinal atypical teratoid/rhabdoid tumor in a 5-year-old child.

Atypical teratoid/rhabdoid tumor (AT/RT) is a rare central nervous system neoplasm affecting children, and isolated primary spinal involvement is extremely rare. Authors describe a case of spinal AT/RT in a 5-year-old male child presenting with rapidly progressing quadriparesis diagnosed and managed surgically and medically. Biopsy revealed large, rhabdoid cells with prominent nucleoli in nest and immunohistochemistry further showed loss of integrase integrator 1 expression considered to be gold standard for diagnosis. AT/RT has extremely poor prognosis with median survival being 6 months.

Prediction of facial nerve position in large vestibular schwannomas using diffusion tensor imaging tractography and its intraoperative correlation.

OBJECTIVE: Resection of large Vestibular Schwannomas (VSs) can be associated with postoperative facial nerve injury. Diffusion-based tractography has emerged as a powerful tool for three-dimensional imaging and reconstruction of white matter fibers; however, tractography of the cranial nerves has not been well studied. In this prospective study, we aim to predict the position of facial nerve in large VSs (>3 cm) using Diffusion Tensor Imaging (DTI) tractography and correlate it with the intraoperative finding of the position of facial nerve. MATERIALS AND METHODS: Twenty patients with a large VS (>3 cm) undergoing surgery were subjected to preoperative DTI to predict the position of the facial nerve in relation to the tumor. The surgeon was blinded to the results of the preoperative DTI tractography. A comparative analysis was then made during operation. The location of the facial nerve in relation to the tumor was recorded during surgery using facial nerve stimulator. RESULTS: Of the 20 patients who underwent DTI tractography, it was not possible to preoperatively identify facial nerve in one patient. In another patient, although DTI tractography predicted the position of facial nerve, it was not identified intraoperatively. In the remaining 18 patients, DTI tractography accurately predicted the facial nerve position. The predicted position was in synchronization with the intraoperative facial nerve position in 16 patients (89% concordance). It was discordant in two patients (11%), but this was not found to be statistically significant (P = -0.3679). CONCLUSION: This study validates the reliability of facial nerve DTI-based fiber tracking for prediction of the facial nerve position in patients with large VSs. The reliable preoperative visualization of facial nerve location in relation to the VS will allow surgeons to plan tumor removal accordingly and may increase the safety of surgery.

Pediatric interhemispheric arachnoid cyst: An institutional experience.

BACKGROUND: Interhemispheric arachnoid cysts (IHACs) are a rare type of congenital arachnoid cyst accounting for <5% of all cases. The optimum surgical management of symptomatic IHAC is still controversial, and there are no clear guidelines. MATERIALS AND METHODS: Retrospective analysis of six pediatric patients of IHAC operated from 2012 to 2015 at our institute. There was definitive sex predisposition with all patients being males. Endoscopic cystoventriculostomy, cystocisternostomy, and cystoperitoneal shunt surgeries were performed in three patients each. RESULTS: The median age at presentation was 13 months. The most common clinical presentations were macrocrania with rapidly increasing head size, seizures, infantile spasms, and developmental delay. The mean duration of follow-up was 24.16 months (range: 3-36 months). Cyst size decreased in all the patients on follow-up imaging. Head size stabilized in all the patients. None of the patients required a second surgical procedure till the last follow-up. CONCLUSIONS: Both endoscopic cyst fenestration and shunt surgery are safe and effective in management of IHAC.

A rare case of giant occipital meningocele with Dandy Walker Syndrome: Can it grow bigger than this?

Association of Dandy-Walker syndrome with occipital meningocele (OMC) is extremely rare and about thirty cases are reported till date in the Western literature. However, OMC is classified by Talamonti et al. into small, large, and giant categories with respective diameters were upto 5 cm in small, large with 5-9 cm, and giant with >9 cm. Usually the size of OMC progressively increases as raised intracranial pressure leads to compensatory cerebrospinal fluid escape into sac with the growth of children. Authors report an interesting case of an 18-month-old female child with extra-gigantic OMC, whose size was almost same since birth, representing the first case of its kind, who underwent successful surgical repair. Clinical presentation, radiological features, and surgical management options in literature are reviewed briefly for this rare disease association.

A rare case of thoracic myelocystocele associated with type 1 split cord malformation with low lying tethered cord, dorsal syrinx and sacral agenesis: Pentad finding.

Myelocystocele is a rare form of spinal dysraphism. Thoracic myelocystocele is still rarer. The occurrence of thoracic myelocystocele associated with type-1 split cord malformation, low lying tethered cord, dorsal syrinx and spina bifida is extremely rare. Clinical presentation of such a rare case and an early surgical management is discussed briefly.