Correlation between large rearrangements and patient phenotypes in NF1 deletion syndrome: an update and review.

About 5-10% of neurofibromatosis type 1 (NF1) patients exhibit large genomic germline deletions that remove the NF1 gene and its flanking regions. The most frequent NF1 large deletion is 1.4 Mb, resulting from homologous recombination between two low copy repeats. This "type-1" deletion is associated with a severe clinical phenotype in NF1 patients, with several phenotypic manifestations including learning disability, a much earlier development of cutaneous neurofibromas, an increased tumour risk, and cardiovascular malformations. NF1 adjacent co-deleted genes could act as modifier loci for the specific clinical manifestations observed in deleted NF1 patients. Furthermore, other genetic modifiers (such as CNVs) not located at the NF1 locus could also modulate the phenotype observed in patients with large deletions. In this study, we analysed 22 NF1 deletion patients by genome-wide array-CGH with the aim (1) to correlate deletion length to observed phenotypic features and their severity in NF1 deletion syndrome, and (2) to identify whether the deletion phenotype could also be modulated by copy number variations elsewhere in the genome. We then review the role of co-deleted genes in the 1.4 Mb interval of type-1 deletions, and their possible implication in the main clinical features observed in this high-risk group of NF1 patients.

National UK guidelines for the management of paediatric craniopharyngioma.

Although rare, craniopharyngiomas constitute up to 80% of tumours in the hypothalamic-pituitary region in childhood. Despite being benign, the close proximity of these tumours to the visual pathways, hypothalamus, and pituitary gland means that both treatment of the tumour and the tumour itself can cause pronounced long-term neuroendocrine morbidity against a background of high overall survival. To date, the optimal management strategy for these tumours remains undefined, with practice varying between centres. In light of these discrepancies, as part of a national endeavour to create evidence-based and consensus-based guidance for the management of rare paediatric endocrine tumours in the UK, we aimed to develop guidelines, which are presented in this Review. These guidelines were developed under the auspices of the UK Children's Cancer and Leukaemia Group and the British Society for Paediatric Endocrinology and Diabetes, with the oversight and endorsement of the Royal College of Paediatrics and Child Health using Appraisal of Guidelines for Research & Evaluation II methodology to standardise care for children and young people with craniopharyngiomas.

Fat in the Fossa and the Sphenoid Sinus: A Simple and Effective Solution to CSF Leaks in Transsphenoidal Surgery. Cohort Study and Systematic Review.

Objectives Cerebrospinal fluid (CSF) leak following endoscopic transsphenoidal surgery (TSS) remains a challenge and is associated with high morbidity. We perform a primary repair with f at in the pituitary f ossa and further fat in the s phenoid sinus (FFS). We compare the efficacy of this FFS technique with other repair methods and perform a systematic review. Design, Patients, and Methods This is a retrospective analysis of patients undergoing standard TSS from 2009 to 2020, comparing the incidence of significant postoperative CSF rhinorrhea (requiring intervention) using the FFS technique compared with other intraoperative repair strategies. Systematic review of current repair methods described in the literature was performed following the preferred reporting items for systematic reviews and meta-analyses (PRISMA) guidelines. Results In all, there were 439 patients, with 276 patients undergoing multilayer repair, 68 patients FFS repair, and 95 patients no repair. No significant differences were observed in baseline demographics between the groups. Postoperative CSF leak requiring intervention was significantly lower in the FFS repair group (4.4%) compared with the multilayer (20.3%) and no repair groups (12.6%, p < 0.01). This translated to fewer reoperations (2.9% FFS vs. 13.4% multilayer vs. 8.4% no repair, p < 0.05), fewer lumbar drains (2.9% FFS vs. 15.6% multilayer vs. 5.3% no repair, p < 0.01), and shorter hospital stay (median days: 4 [3-7] FFS vs. 6 (5-10) multilayer vs. 5 (3-7) no repair, p < 0.01). Risk factors for postoperative leak included female gender, perioperative lumbar drain, and intraoperative leak. Conclusion Autologous fat on fat graft for standard endoscopic transsphenoidal approach effectively reduces the risk of significant postoperative CSF leak with reduced reoperation and shorter hospital stay.

Spontaneous rupture of malignant papillary craniopharyngioma with CSF seeding and metastatic deposits: A case report and literature review.

Introduction: Craniopharyngiomas are benign tumours mainly confined to the cranial cavity in the suprasellar region. Research Question and Case Description: We present a rare case of an aggressive papillary craniopharyngioma with disseminated spinal intradural disease. A 67-year-old woman presented with a 4-month history of headache, visual disturbance, acute confusion and radicular leg pain. Previous history of breast carcinoma (ER â€‹+ â€‹PR â€‹+ â€‹HER2-) was noted. The importance of histological diagnosis prior to treatment of sellar or suprasellar lesions with atypical or aggressive features is explored. Materials and methods: MRI demonstrated a partly solid and partly cystic pituitary mass lesion in the sellar and suprasellar region with chiasmal compression and hypothalamic involvement. The sella was mildly enlarged and there were no calcifications. Whole neuraxis MRI revealed intradural deposits involving the ventricular system, spinal cord and conus. Within a month, the lesion rapidly increased in size. The patient underwent a craniotomy and transventricular resection of the sellar and suprasellar mass. Cranial lesion histology favoured papillary craniopharyngioma, confirmed by BRAF V600 mutation. Lumbar puncture CSF cytology confirmed craniopharyngioma with BRAF mutation and no evidence of metastatic breast cancer. Results: The patient remained confused postoperatively without focal neurological deficit and underwent palliative whole brain radiotherapy. She died 4 months later. A review of the literature identified 29 reports of ruptured craniopharyngioma. Discussion and Conclusion: Ruptured craniopharyngioma presents with a suprasellar mass and drop lesions in the spinal canal, characteristics radiologically indistinguishable from metastatic disease. The importance of histological diagnoses in directing the management of these cases is highlighted.

Prognostic factors for surgically managed intramedullary spinal cord tumours: a single-centre case series.

PURPOSE: Intramedullary spinal cord tumours (IMSCTs) are comparatively rare neoplasms. We present a single-centre clinical case series of adult patients with surgically managed IMSCTs. METHODS: We performed a retrospective analysis of electronic patient records in the time period spanning July 2010 to July 2021. All adult patients that had undergone surgical management for IMSCTs were eligible for inclusion. Baseline and post-operative clinical and radiological characteristics, along with follow-up data, were assessed. We also performed a literature review with a focus on surgical outcomes for IMSCTs. RESULTS: Sixty-six patients matched our selection criteria, with a median age of 42 years (range 23-85). Thirty-four ependymomas, 17 haemangioblastomas, 12 astrocytomas, 2 lymphomas and 1 teratoma were included. Statistical analysis yielded several significant findings: IMSCTs spanning a greater number of vertebral levels are significantly associated with poor McCormick outcomes (p = 0.03), presence of gait disturbance before surgery is significantly associated with poor outcome for both post-operative McCormick and Nurick scores (p = 0.007), and radicular pain present pre-operatively is significantly associated with a good post-operative McCormick score (p = 0.045). Haemangioblastomas are significantly more likely to have a clear intra-operative dissection plane compared to ependymomas and astrocytomas (p = 0.009). However, astrocytomas have a significantly higher prevalence of good McCormick outcomes compared to ependymomas and haemangioblastomas (p = 0.03). CONCLUSION: Histological diagnosis, cranio-caudal extent of the tumour and the presence or absence of baseline deficits-such as gait impairment and radicular pain-are significant in determining neurological outcomes after surgery for IMSCTs.

Do All Notochordal Lesions Require Proton Beam Radiotherapy? A Proposed Reclassification of Ecchordosis Physaliphora as Benign Notochord Cell Tumor.

Objectives Ecchordosis physaliphora (EP) is a benign notochord lesion of the clivus arising from the same cell line as chordoma, its malignant counterpart. Although usually asymptomatic, it can cause spontaneous cerebrospinal fluid (CSF) rhinorrhea. Benign notochordal cell tumor (BNCT) is considered another indolent, benign variant of chordoma. Although aggressive forms of chordoma require maximal safe resection followed by proton beam radiotherapy, BNCT and EP can be managed with close imaging surveillance without resection or radiotherapy. However, while BNCT and EP can be distinguished from more aggressive forms of chordoma, differentiating the two is challenging as they are radiologically and histopathologically identical. This case series aims to characterize the clinicopathological features of EP and to propose classifying EP and BNCT together for the purposes of clinical management. Design Case series. Setting Tertiary referral center, United Kingdom. Participants Patients with suspected EP from 2015 to 2019. Main Outcome Measures Diagnosis of EP. Results Seven patients with radiological suspicion of EP were identified. Five presented with CSF rhinorrhea and two were asymptomatic. Magnetic resonance imaging features consistently showed T1-hypointense, T2-hyperintense nonenhancing lesions. Diagnosis was made on biopsy for patients requiring repair and radiologically where no surgery was indicated. The histological features of EP included physaliphorous cells of notochordal origin (positive epithelial membrane antigen, S100, CD10, and/or MNF116) without mitotic activity. Conclusion EP is indistinguishable from BNCT. Both demonstrate markers of notochord cell lines without malignant features. Their management is also identical. We therefore propose grouping EP with BNCT. Close imaging surveillance is required for both as progression to chordoma remains an unquantified risk.

Systematic Review Comparing Open versus Endoscopic Surgery in Clival Chordomas and a 10-Year Single-Center Experience.

Objectives Chordomas are rare, slow-growing, and osteo-destructive tumors of the primitive notochord. There is still contention in the literature as to the optimal management of chordoma. We conducted a systematic review of the surgical management of chordoma along with our 10-year institutional experience. Design A systematic search of the literature was performed in October 2020 by using MEDLINE and EMBASE for articles relating to the surgical management of clival chordomas. We also searched for all adult patients surgically treated for primary clival chordomas at our institute between 2009 and 2019. Participants Only articles describing chordomas arising from the clivus were included in the analysis. For our institution experience, only adult primary clival chordoma cases were included. Main Outcome Measures Patients were divided into endoscopic or open surgery. Rate of gross total resection (GTR), recurrence, and complications were measured. Results Our literature search yielded 24 articles to include in the study. Mean GTR rate among endoscopic cases was 51.9% versus 41.7% for open surgery. Among the eight cases in our institutional experience, we found similar GTR rates between endoscopic and open surgery. Conclusion Although there is clear evidence in the literature that endoscopic approaches provide better rates of GTR with fewer overall complications compared to open surgery. However, there are still situations where endoscopy is not viable, and thus, open surgery should still be considered if required.

Pituitary apoplexy in an adolescent male with macroprolactinoma presenting as middle cerebral artery territory infarction.

Pituitary apoplexy typically presents with acute headache, vomiting, visual disturbance, and confusion. Herein, we report a rare presentation of ischemic stroke due to pituitary apoplexy. A 16.5-year-old male presented with reduced Glasgow Coma Scale (GCS) score, slurred speech, right-sided hemiparesis, and bitemporal hemianopia. Magnetic resonance imaging of the brain showed a large hemorrhagic sellar/suprasellar mass and an area of cortical T2/FLAIR hyperintensity with corresponding diffusion restriction in the middle cerebral artery territory. Computed tomography (CT) intracranial angiogram showed luminal occlusion of the clinoid and ophthalmic segments of both internal carotid arteries (ICAs, left>right) due to mass pressure effect. Biochemical investigations confirmed hyperprolactinemia and multiple pituitary hormone deficiencies. Stress-dose hydrocortisone was commenced with cabergoline, followed by urgent endoscopic transsphenoidal debulking of the tumor (subsequent histology showing prolactinoma). Postoperative CT angiogram showed improved caliber of ICAs. Intensive neurorehabilitation was implemented and resulted in complete recovery of motor and cognitive deficits. At the last assessment (18.8 years), the patient remained on complete anterior pituitary hormone replacement without cabergoline. Pituitary apoplexy is a medical emergency requiring prompt recognition and treatment and should be suspected in patients presenting with sudden, severe headache; nausea; or visual disturbance and meningism. Ischemic stroke is a rare manifestation of pituitary apoplexy in the pediatric population.

ERN GENTURIS clinical practice guidelines for the diagnosis, treatment, management and surveillance of people with schwannomatosis.

A Guideline Group (GG) was convened from multiple specialties and patients to develop the first comprehensive schwannomatosis guideline. The GG undertook thorough literature review and wrote recommendations for treatment and surveillance. A modified Delphi process was used to gain approval for recommendations which were further altered for maximal consensus. Schwannomatosis is a tumour predisposition syndrome leading to development of multiple benign nerve-sheath non-intra-cutaneous schwannomas that infrequently affect the vestibulocochlear nerves. Two definitive genes (SMARCB1/LZTR1) have been identified on chromosome 22q centromeric to NF2 that cause schwannoma development by a 3-event, 4-hit mechanism leading to complete inactivation of each gene plus NF2. These genes together account for 70-85% of familial schwannomatosis and 30-40% of isolated cases in which there is considerable overlap with mosaic NF2. Craniospinal MRI is generally recommended from symptomatic diagnosis or from age 12-14 if molecularly confirmed in asymptomatic individuals whose relative has schwannomas. Whole-body MRI may also be deployed and can alternate with craniospinal MRI. Ultrasound scans are useful in limbs where typical pain is not associated with palpable lumps. Malignant-Peripheral-Nerve-Sheath-Tumour-MPNST should be suspected in anyone with rapidly growing tumours and/or functional loss especially with SMARCB1-related schwannomatosis. Pain (often intractable to medication) is the most frequent symptom. Surgical removal, the most effective treatment, must be balanced against potential loss of function of adjacent nerves. Assessment of patients' psychosocial needs should be assessed annually as well as review of pain/pain medication. Genetic diagnosis and counselling should be guided ideally by both blood and tumour molecular testing.

Pituitary Adenoma Surgery Survey: Neurosurgical Centers and Pituitary Adenomas.

Objective: Pituitary adenoma surgery has evolved rapidly in recent decades. This study aims to determine current practice across a wide range of European neurosurgical centers. Methods: A list of eligible departments performing pituitary adenoma surgery was created. The survey consisted of 58 questions. For analysis, the departments were divided into four subgroups: academic/nonacademic, high-volume/low-volume, "mainly endoscopic/mainly microscopic practice," and geographical regions. Results: Data from 254 departments from 34 countries were obtained. In 108 centers (42.5%), <30 pituitary adenomas were operated per year. Twenty (7.9%) centers performed >100 adenoma surgeries per year. Number of neurosurgeons performing endonasal surgeries are as follows: 1 in 24.9% of centers and 2 in 49.8% of centers. All residents assisted endonasal surgeries in 126 centers (49.8%). In 28 centers (21.1%), all residents performed endonasal surgery under supervision during residency. In 141 centers (56.8%), the endoscopic approach was used in >90% of the surgeries. Regular pituitary board (either weekly or once a month) meetings were held in 147 centers (56.3%). Nonfunctioning adenomas represent >70% of pituitary caseload in 149 centers (58.7%). Conclusions: In our survey, most centers perform less than 100 surgeries for pituitary adenomas. In most centers, pituitary surgeries are performed by one or two neurosurgeons. Residents have a limited exposure to this type of surgery, and the formal pituitary board is not a standard. Nonfunctioning adenomas make up most of surgically treated adenomas. This study can serve as a benchmark for further analyses of pituitary adenoma centers in Europe.

Endocrinological aspects of pituitary adenoma surgery in Europe.

Hormone-secreting adenomas are treated in many neurosurgical centers within Europe. The goal of the survey is to understand variance in practice management of pituitary tumors amongst neurosurgical centers. A list of departments performing pituitary surgery was created. The survey consisted of 58 questions. This study focuses on neurosurgical care of hormone-secreting adenomas. For analysis, the departments were divided into four subgroups: academic/non-academic, high-volume/low-volume, "mainly endoscopic/mainly microscopic practice" and geographical regions. Data from 254 departments from 34 countries were obtained. Most centers surgically treat 1-5 hormone-secreting adenomas per year. In prolactinomas this is the case in 194 centers, (76.4%), in GH-secreting adenomas: 133 centers, (52.4%), ACTH-secreting adenomas: 172 centers, (69.8%). Surgery as a primary treatment of prolactinomas is considered in 64 centers (25.2%). In 47 centers (18.8%), GH-secreting microadenomas are often treated pharmacologically first. Debulking surgery for an invasive GH-secreting adenoma in which hormonal remission is not a realistic goal of the surgery and the patient has no visual deficit surgery is always or mostly indicated in 156 centers (62.9%). Routine postoperative hydrocortisone replacement therapy is administered in 147 centers (58.6%). Our survey shows that in most centers, few hormone-secreting adenomas are treated per year. In about 25% of the centers, prolactinoma surgery may be regarded as first-line treatment; in about 20% of the centers, medical treatment is the first-line treatment for GH-secreting adenomas. Pretreatment for ACTH-secreting adenomas is routinely used in 21% of centers. This survey may serve as plea for neurosurgical care centralization of hormone-secreting adenomas.

What is the current clinical practice in pituitary adenoma surgery in Europe? European Pituitary Adenoma Surgery Survey (EU-PASS) results-technical part.

Pituitary adenoma surgery has evolved rapidly in recent decades, changing clinical practice markedly and raising new challenges. There is no current consensus or guidelines for perioperative care that includes possible complication management. This study aims to determine current practice across European neurosurgical centres. We created a list of eligible departments performing pituitary adenoma surgery based on cooperation with EANS, national neurosurgical societies, and personal communication with local neurosurgeons. We contacted the chairpersons from each department and asked them (or another responsible neurosurgeon) to fill out the survey. The survey consisted of 58 questions. For further analysis, departments were divided into subgroups: "academic"/ "non-academic centre", "high-volume"/"low-volume", "mainly endoscopic"/ "mainly microscopic"/ "mixed practise", and by geographical regions. Data from 254 departments from 34 countries were obtained. The average time to complete the survey was 18 min. Notably, the endoscopic approach is the predominant surgical approach in Europe, used in 56.8% of the centres. In routine cases without intraoperative cerebrospinal fluid leak, high-volume centres are less pedantic with sellar closure when compared with low-volume centres (p < 0.001). On the other hand, when a postoperative cerebrospinal fluid leak occurs, high-volume centres are more active and indicate early reoperation (p = 0.013). Less than 15% of the participating centres perform early postoperative MRI scans. Marked variation was noted among different groups of respondents and some contentious issues are discussed. Such information can encourage useful debate about the reasons for the variations seen and perhaps help develop standardised protocols to improve patient outcomes. A future research focus is to compare European results with other regions.

Neurosurgical Approaches to the Skull Base: A Guide for the Radiologist.

Skull base surgery relies on the assessment of detailed neuroimaging studies to assist with surgical planning. We review typical neuroimaging features associated with common neurosurgical skull base approaches, highlighting relevant imaging anatomy and pertinent postoperative imaging appearances.

Surgical Management of Spinal Chordoma: A Systematic Review and Single-Center Experience.

OBJECTIVE: Chordoma is a rare neoplasm of the neuraxis derived from remnants of the primitive notochord. The importance of wide margins and use of adjuvant therapy in spinal chordomas are still contentious points in the management of spinal chordomas. We conducted a systematic review of the surgical management of spinal chordomas alongside our 10-year institutional experience. METHODS: A systematic search of the literature was performed in November 2020 using Embase and MEDLINE for articles regarding the surgical management of chordomas arising from the mobile spine and sacrum. We also searched for all adult patients who were surgically managed for spinal chordomas at our institute between 2010 and 2020. In both the systematic review and our institutional case series, data on adequacy of resection, use of adjuvant therapy, complications, recurrence (local or metastatic), and survival outcomes were collected. RESULTS: We identified and analyzed 42 articles, yielding 1531 patients, from which the overall gross total or wide resection rate was 54.9%. Among the 8 cases in our institutional experience (4 sacral, 3 cervical, and 1 lumbar), we achieved gross total resection in 50% of initial operations. The recurrence rate was 25% in our gross total resection group and 50% where initial resection was subtotal. Of patients, 75% had no evidence of recurrence at most recent follow-up. CONCLUSIONS: Albeit difficult at times because of the proximity to neurovascular tissue, achieving a wide resection followed by adjuvant therapy for spinal chordomas is of great importance. Multidisciplinary discussion is valuable to ensure the best outcome for the patient.

Prolactinoma in childhood and adolescence-Tumour size at presentation predicts management strategy: Single centre series and a systematic review and meta-analysis.

OBJECTIVE: To report the clinical presentation, management and outcomes of young patients with prolactinomas (<20 years) and conduct a systematic review and meta-analysis. PATIENTS AND DESIGN: Clinical, biochemical and radiological data (1996-2018) were collected from our centre. A systematic review and meta-analysis of published literature (1994-2019) on prolactinoma (age <20 years) were conducted. Both random and fixed effects meta-analysis were used to pool outcomes across studies. RESULTS 1 CASE SERIES: Twenty-two patients (14 females) were identified; median age at diagnosis 15.7 years (range 13-19); 12 patients (6 females) had a macroprolactinoma. Seven patients (macroprolactinoma-6) had associated pituitary hormone deficiencies at presentation. Five patients (4 males) underwent surgical resection due to poor response to cabergoline or apoplexy. Patients undergoing surgery had larger tumours (p < .02) and higher serum prolactin concentration (p < .005). All patients with macroprolactinoma >20 mm required surgical intervention. RESULTS 2 SYSTEMATIC REVIEW AND META-ANALYSIS: We selected 11 studies according to strict inclusion criteria describing 275 patients. Macroprolactinoma was more common in girls (78.7% [95% CI 70.5-85.9]) than boys and was more frequent than microprolactinoma (56.6% [95% CI 48.4-64.5]). In males, only 6/57 (10.5%) of tumours were microprolactinoma as compared to 102/198 (51.5%) microprolactinoma in females (risk difference -0.460; [95% CI -0.563 to -0.357]; p < .001). Surgery was first-line therapy in 18.9% patients, with another 15.4% requiring it as a second line (overall 31.3%). CONCLUSIONS: Macroprolactinoma, particularly if >20 mm, usually requires multimodal therapy including surgical intervention. While overall prolactinomas in <20 years age group are more common in females, the proportion of macroprolactinoma vs microprolactinoma is greater in males, particularly for large invasive tumours. Microprolactinoma is a rare diagnosis in adolescent males.

Lack of Correlation Between Immunohistochemical Expression of SPARC and Invasion in Different Grades of Meningiomas.

BACKGROUND: Grade I meningiomas are generally benign and non-invasive whereas Grade II (atypical) and Grade III (malignant) meningiomas tend to be invasive with a high risk of recurrence. SPARC, secreted protein, acidic and rich in cysteine, is a multifunctional glycoprotein which has been proposed to be a potential diagnostic marker of invasive meningiomas. There has been increased reporting of atypical meningiomas since the current World Health Organization (WHO) included brain invasion as a grading criterion for classification of these particular meningiomas. MATERIALS AND METHODS: The aim of this study was to re-evaluate any correlation between immunohistochemical expression of SPARC in 34 meningiomas of various grades using the current classification (2016). We had previously classified these cases using the 2002 WHO criteria. RESULTS: There is no correlation between expression of SPARC and invasion in different grades of meningioma. CONCLUSION: SPARC does not appear to be a good predictor of invasion in meningiomas.

Endoscopic skull base neurosurgical practice in the United Kingdom.

Background: Endoscopic skull base surgery (ESBS) is a new subspecialty area that has become more popular over the past 20 years. It is fast evolving and the indications are getting increasingly diverse. The skill-sets also continue to significantly improve, hence, we, the authors aim to assess the current status of this unique sub-specialty within the United Kingdom. Objective: Our objectives included identifying the proportion of neurosurgeons within the United Kingdom who utilize endoscopic skull base approaches, the background training involved and how much of the overall neurosurgical workload is constituted by endoscopic skull base neurosurgery. There was also a focus on the technical nuances involved with ESBS. Materials and methods: 45 neurosurgeons were included in this study, and a 47% response rate (n = 21) was obtained. Results: 7 (33%) had training in ESBS during residency and 8 (38%) had some fellowship exposure to ESBS. Each respondent did an average of 1.9 endoscopic skull base courses prior to commencing their practice. The length of practice ranged from 3 to 15 years (mean - 7.2 years). Although most of the surgeons had mixed sub-specialty interests, the most commonly associated sub-specialty with ESBS was lateral skull base surgery (38%). Conclusion: In the United Kingdom, ESBS appears to be commonly combined with a lateral/open skull base neurosurgical practice. Most of the surgeons had their ESBS training after formal residency. Collaboration with ENT occurs more commonly for extended transphenoidal procedures.

Giant thoracic discs: treatment, outcome, and follow-up of 33 patients in a single centre.

PURPOSE: To help guide treatment strategies and create insight into functional outcomes in patients with Giant herniated thoracic discs (GHTD), which are defined as occupying more than 40% of spinal canal. METHODS: Authors did a retrospective analysis of prospective cohort of 33 cases of GHTD, using clinical letters, notes, and telephonic questionnaires to determine their pre and postoperative functional status, surgical details, and complication rates. 16 males and 17 females operated between 2006 and 2014 were included in the study. A total of 23 patients underwent thoracotomy, 9 costotransversectomy, and 2 transpedicular approaches for excision of thoracic discs. Neuromonitoring was used in seven patients. RESULTS: Frankel grade improved by 1 in 13 patients and by 2 in 1 patient. One patient of T11/12 GHTD with neurogenic claudication recovered completely, taking the overall improvement rate to 45.5%. It remained static in 15 patients (45.5%) and deteriorated by 1 in 3 patients (9%). By mJOA scoring too, the outcome was favorable in majority (84.4%) of patients. There were three intraoperative complications (9%), which included two incidental durotomies and one massive blood loss. Late postoperative complications were seen in 12(39%) patients. They included intercostal neuralgia, mechanical pain around costotransversectomy, progressive thoracic paraplegia due to spinal cord herniation and residual disc fragments, reactive pleural effusion, CSF fistula induced pleural effusion, and infective discitis. CONCLUSIONS: Surgery for giant herniated thoracic disc has favorable outcome in majority (91%) of patients. However, significant approach-related complications are to be anticipated in patients undergoing thoracotomies, most of them being manageable. Author recommends costotransversectomy, only in paracentral discs with smaller percentage canal stenosis.