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Adamantinomatous craniopharyngioma is a grade 1 tumor that arises in a sellar/suprasellar location. Despite being a grade 1 tumor, there is high recurrence and endocrinal insufficiency. Malignancy arising in craniopharyngioma is extremely rare, has a dismal prognosis, and is currently not included as a separate entity in the World Health Organization Classification of Central Nervous System 5th edition. Here we describe a case of adamantinomatous craniopharyngioma and its malignant counterpart. The malignant part had unique histomorphology and basaloid cells with pseudoglandular architecture and a myxoid background. It bore a striking resemblance to adenoid cystic carcinoma. Both the benign and malignant counterparts were beta-catenin and SOX-2 positive, providing proof of the malignant part arising from the benign part. Tumors like squamous cell carcinoma and odontogenic ghost cell carcinoma have been described in cranipharyngioma. This case study is the first to describe this unique morphology of adenoid cystic carcinoma-like features. The possibility of adenoid cystic carcinoma was excluded by immunohistochemistry., (© 2024 Japanese Society of Neuropathology.)

Background: Papillary tumors of pineal region (PTPR) comprise a very rare subset of pineal region tumors that have been recently described. Literature on the management and outcome of PTPR is scarce owing to the rarity of these tumors. To address this lacuna, we analyzed our experience in management of PTPR., Methods: We retrospectively analyzed the outcome of 11 patients with histopathologically proven PTPR who underwent surgical excision at our center., Results: Mean patient age was 33.3 years (range, 12-45 years), and male-to-female ratio was 1.75:1. Headache was the most common presentation followed by visual disturbances, altered sensorium, Perinaud syndrome, and seizures. Cerebrospinal fluid diversion was required in 6 patients. Krause approach was the most common approach used for tumor excision (9/11 cases). There was no perioperative mortality. Two patients were lost to follow-up. In the remaining 9 patients, the average follow-up period was 45 months (range, 12-79 months). On first postoperative magnetic resonance imaging, 8 patients showed no evidence of residual tumor (gross total resection), while 1 patient had small residual tumor (near-total resection) that remained stable during follow-up. Four patients underwent adjuvant chemoradiotherapy. None of the patients developed recurrence during follow-up., Conclusions: PTPR are a rare subgroup of pineal region tumors with distinct cells of origin but presentation similar to other pineal region tumors. Surgical resection constitutes the mainstay of management, and the extent of resection appears to be the most important determinant of prognosis. The role of adjuvant therapy still needs to be determined., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Introduction: Multiple synchronous spinal extradural arachnoid cysts are extremely rare in children and can lead to cord compression and disability. Multiple spinal extradural arachnoid cysts (SEDACs) in children with immature and growing spine need to be studied separately from multiple SEDACs in adults with mature and fully grown spine because of multiple surgical limitations in children (blood loss, surgery duration, long-term spinal stability after long segment exposure, etc.)., Material and Methods: We planned a non-systematic literature review of similar cases described in literature to analyse the pattern of presentation, management, and outcome of this surgically curable disease., Results: A total of 28 cases of paediatric multiple SEDACs in the age range of 5 months to 17 years and mean age of 11.54 years were analysed. Exposure was achieved by laminectomy in 9, laminoplastic laminotomy in 13, laminectomy in first surgery followed by laminoplasty in second surgery (re-exploration) in 1, hemilaminectomy in 2, and technique "not reported" in 3 children. Complete/near-complete recovery was seen in 27 (96.42%) children. Postoperative spine deformity was reported in 7 (25%) children and was progressive in 2 children during serial follow-up., Conclusions: Symptomatic multiple synchronous SEDACs in children is a rare surgically curable condition. Sincere attempt to find out a dural defect in all cysts is a very important step in multiple SEDAC surgery. Total cyst wall excision with closure of all dural defects is the gold standard treatment for symptomatic cases. Laminoplasty is preferred for excision of multiple SEDACs in children to prevent postoperative spine deformity during long-term follow-up. Long serial follow-up for postoperative spine deformity is necessary., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background: Aneurysmal bone cyst is composed of variable -sized cystic blood-filled spaces separated by connective tissue septae. First-line surgical resection of spinal aneurysmal bone cyst in a child with limited total blood volume can lead to massive intraoperative bleeding, thus limiting extent of resection. Our Centre's has good experience of using absolute alcohol as an effective immediate devascularizing agent during vertebral hemangioma surgery in children., Material and Methods: We report the first case of pediatric lumbar primary aneurysmal bone cyst in which completely blood-less piecemeal total resection of the lesion was performed after intraoperative absolute alcohol intralesional sclerotherapy., Results: Completely blood-less piecemeal total resection of the lumbar aneurysmal bone cyst was performed after intraoperative absolute alcohol intralesional sclerotherapy., Conclusion: Intraoperative absolute alcohol intralesional sclerotherapy is a very effective devascularizing adjunct for complete piecemeal resection of spinal aneurysmal bone cyst in children with limited blood volume., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: To study clinical and radiological outcomes of pediatric cervical kyphosis correction with a standalone posterior cervical approach. Cervical spine kyphotic deformity in pediatric age group is a distinct entity and the management is challenging. Pediatric cervical kyphosis is less often encountered, and literature is sparse with only few case series. Management algorithms are devised keeping the flexibility of the deformity at the core of decision making. Circumferential fusion is mostly recommended for non-flexible (rigid) kyphosis., Methods: Authors present a single center retrospective analysis of cases of pediatric cervical kyphosis managed by a standalone posterior approach. Pre- and post-operative clinical and radiological parameters were recorded and analyzed. Changes in neurological status, kyphosis correction and bony fusion were assessed. Surgical and implant related complications were noted., Results: Seven cases (6 male, 1 female) were included. Mean age was 13.9±2.9 years, ranging from 8-17 years. Etiology was traumatic in 2 cases, developmental in 2, and syndromic, Hirayama disease and post-laminectomy in 1 case each. Mean kyphosis correction was 36.80±19.30 (87%±21%) with a mean pre-operative kyphosis angle of 37.80±15.30 and mean immediate post-operative kyphosis angle of 3.70±8.70. Mean hospital stay duration was 10±6 days. Median follow-up duration was 36 months. Myelopathy improved in 5 cases at last follow-up. Six cases demonstrated bony fusion at a mean follow-up of 8.4±1.5 months., Conclusion: Significant immediate correction in pediatric cervical kyphosis may be achieved with a standalone posterior approach with proper planning and technique in selected cases. Inserting pedicle screws at strategic locations of implant construct offer better corrections and pull-out strength and maintain long-term stability resulting in higher arthrodesis rates. Larger studies with longer follow up are needed to further ascertain the role of standalone posterior cervical approaches in pediatric cervical kyphosis., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background: IgG4-related disease (IgG4RD) is a multisystemic progressive fibroinflammatory and lymphoproliferative autoimmune disorder of unknown etiopathogenesis; and its occurrence in intradural extramedullary (IDEM) location is extremely rare., Aim and Objective: The aim of this study was to review all IDEM IgG4RD cases described in English literature and to signify the role of surgery in its management at this rare location along with the surgical technique and intraoperative findings., Methods: An Internet-based search (PubMed) for the published reports describing spinal cases of IgG4RD with IDEM involvement was done using the keyword: spinal IgG4-related disease. An illustration of a case of cranial IgG4-related disease in a young man who developed metachronous cervical involvement in intradural extramedullary (IDEM) location is also presented., Results: Amongst 45 spinal IgG4RD cases reported in English Literature, only 3 cases were IDEM IgG4RD. In the illustrated case, portion of the cervical lesion causing cervical cord encasement was intradural but extraarachnoidal- located between arachnoid and dura, without any pial involvement. All these 4 cases improved following surgery., Conclusion: Timely meticulous resection of mass lesion in situations of medical treatment failure or progressive neurological decline can lead to reversibility of mass effect-associated neurological manifestations of IgG4RD., Competing Interests: None

Aim: To retrospectively evaluate the outcome of patients with intracranial meningeal hemangiopericytomas (MHPCs) and to analyze various factors for recurrence and survival in these patients., Materials and Methods: We retrospectively reviewed the clinical data of 39 patients undergoing microsurgical resection for MHPCs at our institute from 2009 to 2015., Results: Gross total excision (GTE) was achieved in 27 (69.2%) patients, whereas 12 (30.8%) underwent subtotal excision (STE). A total of 25 patients received radiotherapy (RT) (Conventional RT-15; GKT-10), 21 patients had a low grade tumor, while 18 had an anaplastic variant. Twenty patients (51.3%) developed recurrences and the average recurrence-free survival (RFS) was 56 months (range: 12-180 months). Eight patients (20.5%) died during the study period. The average overall survival (OS) was 77.2 months (range: 36-192 months). Two patients (5.1%) developed systemic metastases during follow-up. Patient age was not found to affect RFS or OS. GTE was associated with prolonged RFS and OS but the impact was not statistically significant (P-values = 0.160 and 0.414, respectively). Low tumor grade was associated with statistically significant longer RFS as well as OS (P-values = 0.049 and 0.013, respectively). Addition of adjuvant RT was associated with statistically significant prolongation of RFS (P value = 0.016); however, it was not associated with statistically significant OS benefits (P-value = 0.758)., Conclusions: Our study suggests that a greater extent of excision, lower tumor grade, and addition of adjuvant RT have a positive impact on both RFS and OS; however, low grade and adjuvant RT were the only factors associated with statistically significant prolongation of RFS and only tumor grade was associated with statistically significant OS benefits., Competing Interests: None

Background: Meningocele is defined as a protrusion of the meninges through an opening in the skull or spinal column, forming a bulge or sac filled with cerebrospinal fluid. A pseudomeningocele is defined as a cerebrospinal fluid (CSF) collection formed due to escape of CSF through a dural defect with trapping of CSF into the surrounding soft tissues. We herby report rare occurrence of a large (pseudo)meningocele in a young patient with congenital skull base defect presenting as upper lateral neck swelling., Case Presentation: We present the case of a 17-year-old boy who had painless progressive swelling right side of the upper neck without any history of meningitis or CSF leak. He had a history of undergoing cranioplasty using steel plates for nontraumatic boggy swelling right parieto-occipital region at the age of 5 years at another hospital. Clinical examination showed painless swelling right side of the upper neck, with positive cough impulse and transillumination. CT head with cisternography showed a large right skull base defect through which a large pseudomeningocele was herniating, thus producing upper neck swelling and compressing oral cavity. The neck swelling and intraoral bulge reduced in size after the coperitoneal shunt., Conclusion: Differential diagnosis of (pseudo)meningocele should be considered while evaluating a painless progressive upper neck swelling having cough impulse and transillumination in a young patient.

Objective: To describe management and outcome in a large cohort of patients with spinal tuberculosis (TB)., Methods: Of 212 patients with spinal TB treated between January 1999 and June 2011, 179 patients were included in the study (≥6 months follow-up; mean age, 34.8 years; age range, 10-75 years). The cohort was divided into two groups (n = 89 and n = 90); group I was treated from 1999-2003, and group II was treated from 2004-2011., Results: The study cohort comprised 93 male patients. Mean age was 34.8 years ± 7.2 (range, 10-75 years). Mean duration of symptoms was 2.4 months. Sensorimotor deficits were present in 167 patients (93.5%; 74 patients were paraplegic), pain was present in 156 patients (87%), bladder involvement was present in 127 patients (71.7%), and extraspinal TB was present in 36 patients (22.3%). Of patients, 92% were receiving prior chemotherapy; one fifth of these patients were on second-line chemotherapy. Thoracic spine involvement was most common (n = 86; 57%), followed by cervical spine (n = 50; 29%), craniovertebral junction (n = 22; 15%), and lumbosacral spine (n = 20; 10.5%). Surgery was performed in 146 patients (68% instrumented fusions and 16% circumferential fusions). Mean follow-up was 20.2 months (range, 6-60 months). Sensorimotor deficits improved in 89% of patients, pain improved in 71%, bladder symptoms improved in 88%, and paraplegia improved in 77%. Patients in group II had a higher incidence of cord compression (P < 0.01), severe vertebral body collapse (P < 0.001), and paraplegia (P < 0.001). Group II patients underwent more instrumented surgeries (P < 0.01), especially circumferential fusions (P < 0.001). The improvement in paraplegia was better after 2004 (group II). Bladder symptoms correlated with the timing of surgery (P < 0.1)., Conclusions: Medical treatment of spinal TB is the mainstay; however, radical, instrumented surgeries should be offered when indicated. The presence of paraplegia should not preclude surgery. A practical management paradigm is also suggested., (Copyright © 2013 Elsevier Inc. All rights reserved.)