Paediatric Rhabdoid Meningioma: Clinical and Therapeutic Features Findings - Case Series of 3 Patients.

INTRODUCTION: Paediatric rhabdoid meningioma (RM) is the rarest but most aggressive subtype of meningioma, related to a severe prognosis. They account for 1-3% of all intracranial meningiomas. CASE PRESENTATIONS: We report an institutional experience of 3 cases through which we discuss clinical, histological, and therapeutic features of this tumour. Two of our patients were female-gendered (3 years old and 1 year and 6 months old), and one was male-gendered (16 years old). Revealing symptoms were related to intracranial hypertension, cerebellar syndrome, cranial nerve palsy, and skull tumefaction. Imaging showed extra-axial tumour located in the right ponto-cerebellar angle in the first case, in the left occipital region in the second case, left parietal tumour in the third case. All patients underwent a surgical intervention with a gross total resection. Histological evaluation supported by immunohistochemistry confirmed the diagnosis of RM. Tumour recurrence was observed at 45 days in the first case with a fatal outcome. Despite adjuvant radiotherapy, both second and third cases had local recurrence after a mean follow-up of 1 month following the radiotherapy. CONCLUSIONS: RM is very aggressive tumours. Standardized therapeutic guidelines are still under debate as actual approaches are still inefficient to prevent quick recurrence and fatal outcome.

Supra-sellar granular cell tumor: Report of a case with literature review.

INTRODUCTION AND IMPORTANCE: Granular cell tumor (GCT) originating from the sellar and suprasellar regions, specifically from the neurohypophysis, is a rare neoplasm. Distinguishing GCT from other pituitary tumors, including pituitary adenoma, pituicytoma, and spindle cell oncocytoma, poses significant challenges. Here, we present a rare case of GCT originating from the posterior pituitary in the supra-sellar region. CASE PRESENTATION: A 41-year-old woman, with no past medical history, presented to neurology department with decreased visual acuity and peripheral facial paralysis since 3 months. The MRI showed a well-defined supra-sellar, retrochiasmatic, oblong, hypothalamic expansive process. It was isointense T1-weighted, discretely hypotensive T2-weighted, measuring 19x17x16 mm, suggesting pituicytoma or craniopharyngioma. An endoscopic transsphenoidal surgical resection was performed. Microscopic examination showed a proliferation of diffuse architecture made up of rounded polyhedral cells with granular eosinophilic cytoplasm. On immunohistochemistry, tumor cells expressed diffusely TTF1, S-100 protein and SOX-10 confirming the diagnosis of supra-sellar GCT. DISCUSSION AND CONCLUSION: GCTs are rare neoplasms that predominantly exhibit benign behavior, while the malignancy rate remains at 2 %. Histopathology serves as the definitive diagnostic approach for GCTs. These tumors are resistant to radiotherapy and chemotherapy, necessitating surgical resection as the primary treatment modality. Due to the potential absence of distinct tumor masses and local tissue infiltration by tumor cells, complete excision is crucial, with resection extent extending beyond areas of infiltration.

Epiduritis related to IgG4 disease: A very rare cause for spinal cord compression.

Background: Inflammatory pseudotumors are rare, and those attributed to immunoglobulin G4 (IgG4) diseases are even less frequently encountered. Here, we reviewed 41 cases from the literature of spinal inflammatory pseudotumors due to IgG4 and have added our single new case. Case Presentation: A 25-year-old male presented with progressive back pain, bilateral paraparesis, and sphincter dysfunction. His deficit was attributed to MR-documented posterolateral lesion between the T5 and T10 levels for which he levels underwent a T1-T1010 laminectomy. The pathology revealed an immunoglobulin G4-related inflammatory pseudotumor. Postoperatively, the patient additionally required systemic and epidural administration of glucocorticoids. Conclusion: IgG4-related disease is an emerging clinical condition that rarely involves the central nervous system. Spinal inflammatory pseudotumors, including IgG4 disease, should be more commonly considered among the potential differential diagnoses of lesions compressing the spinal cord.

Exploring Rare Traumatic Injuries: A Miniseries of 4 Cases Discussing Epidural Hematomas Bridging the Infratentorial and Supratentorial Regions.

Supratentorial-infratentorial epidural hematomas (SIEH) are a rare occurrence following traumatic head injuries, representing only 2% of traumatic epidural hematomas. Given the unique anatomical characteristics of the infratentorial region, mainly its small size, surgical intervention is commonly undertaken to alleviate the pressure on the posterior fossa components. Consequently, there is ongoing debate surrounding the optimal surgical approaches.In this report, we present four cases of SIEH that were treated surgically. Furthermore, we conduct a comprehensive review of existing literature, encompassing clinical, radiological, and therapeutic aspects associated with this condition.SIEH are uncommon post-traumatic lesions that require urgent and individualized management on a case-by-case basis, as guided by multiplanar cerebral computed tomography scan findings. Preoperative planning is essential; however, intraoperative exploration and identification of transverse sinus and torcula lesions are crucial for optimal patient care. The surgical approach may be modified intraoperatively based on the nature and extent of these lesions. In all cases, prompt hematoma evacuation and meticulous hemostasis are the two primary objectives of this surgery.

Intramedullary spinal cord tumors: A retrospective multicentric study.

CONTEXT: Intramedullary tumors are neoformations taking part on the spinal cord, and they are a rare pathology. Due to the rarity of such lesions, clinical studies take years to ensure a decent feedback with a significant number of cases. DESIGN: Our study is retrospective and descriptive. PARTICIPANTS: We share a Tunisian multicentric experience of 27 years through a retrospective study of 120 cases of spinal cord tumors that have been operated in six different centers. OUTCOME MEASURES: The clinical, radiological, and histological findings have been analyzed along with postoperative results and tumoral progression so that we could conclude to some factors of prognosis concerning the management of these tumors. RESULTS: The mean age of our patients is 33.84 years. We had 57 males and 63 females. The most frequent revealing symptom was motor trouble presented as frequent as 77.5% of the patients. Glial tumors were represented in 81 of the cases (67.5%) and nonglial by 39 cases (32.5%). Glial tumors we found were essentially 39 ependymomas and 35 astrocytomas. Surgical resection is key in the management of these lesions; the quality of tumoral resection was a significant factor of disease progression as subtotal resection is correlated to more important progression than total one. CONCLUSION: We conclude this work with some statements. In terms of functional results, age is not a significant factor. Presurgical functional state, the histological type, and the extent of surgical resection are the important factors.

Plagiocephaly due to Frontosphenoidal Suture Synostosis: Report of 2 Cases and Literature Review.

BACKGROUND/AIMS: The most common cause of anterior plagiocephaly is the fusion of the unilateral coronal suture. In some rare cases, however, the fusion of the frontosphenoidal suture may lead to a resembling pattern with specific clinical and radiological features. The aim of the present study is to further enlighten this entity as it is misdiagnosed most of the time. METHODS: A report of 2 cases of plagiocephaly due to frontosphenoidal synostosis with a review of the literature. RESULTS: One female and one male baby were identified. The mean age at presentation was 11.5 months (range: 1-22). Head circumference was normal in each patient. The two fusions were on the left side. Unilateral frontal flattening and recession of the supraorbital rim were the most important physical findings. Computed tomography confirmed the patency of the frontoparietal suture and the closure of the frontosphenoidal suture. Fronto-orbital advancement was performed in both cases with good cosmetic outcome. CONCLUSION: Frontosphenoidal synostosis should be suspected and carefully searched when dealing with plagiocephaly with patent coronal suture. Good outcome requires a prompt diagnosis and early correction.