P37 Do all notochordal lesions require proton beam radiotherapy? A case series of ecchordosis physaliphora

2019 ◽  
Vol 90 (3) ◽  
pp. e34.3-e33
Author(s):  
A Sooltangos ◽  
A Giamouriadis ◽  
S Barazi ◽  
K Barkas ◽  
N Thomas ◽  
...  
Keyword(s):  
Proton Beam ◽  
Clinical Course ◽  
Case Series ◽  
Proliferation Index ◽  
Csf Leak ◽  
Endoscopic Endonasal ◽  
Proton Beam Radiotherapy ◽  
Csf Fistula ◽  
Ecchordosis Physaliphora

ObjectivesEcchordosis physaliphora (EP) is a benign, usually asymptomatic, notochord tumour but may also present with a spontaneous CSF fistula. Differentiating EP from its more aggressive counterpart, chordoma is challenging but important as the clinical course and management differ significantly, with the latter requiring proton beam radiotherapy. This case series aims to further inform this discussion.DesignCase series.SubjectsPatients with suspected EP from 2015–2018.MethodsRetrospective analysis.ResultsSix patients were identified. Four presented with suspected CSF leak. MRI revealed a T1-hypointense and T2-hyperintense clival lesion without enhancement. CT showed pneumocephalus, a clival defect and no mass lesion. All underwent endoscopic endonasal repair of CSF fistula with biopsy. Histology revealed physaliphorous cells with no mitoses; immunohistochemistry confirmed notochord origin (EMA, S100, CD10 and/or MNF116) and low proliferation index. In 2 patients, EP was found incidentally on imaging and these lesions have remained static on follow-up.ConclusionsIn the absence of a clival mass, notochord remnant lesions with benign histopathology can be treated as EP and patients can avoid radiotherapy. The diagnosis of EP, however, remains challenging as no distinctive histopathological marker exists to differentiate it from chordoma and until a genetic or immunohistochemical marker is identified, the diagnosis rests solely on the presence or absence of a mass on imaging.

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

2021 ◽  
Author(s):  
Aïsha Sooltangos ◽  
Istvan Bodi ◽  
Prajwal Ghimire ◽  
Konstantinos Barkas ◽  
Sinan Al-Barazi ◽  
...  
Keyword(s):  
Proton Beam ◽  
Case Series ◽  
Cell Tumor ◽  
Imaging Features ◽  
Csf Rhinorrhea ◽  
Proton Beam Radiotherapy ◽  
Tertiary Referral Center ◽  
Notochord Cell ◽  
Ecchordosis Physaliphora ◽  
Imaging Surveillance

Abstract 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.

Endoscopic Endonasal Minimal Access Approach to the Clivus: Case Series and Technical Nuances

2010 ◽  
Vol 67 (3) ◽  
pp. ons150-ons158 ◽  
Author(s):  
Justin F. Fraser ◽  
Gurston G. Nyquist ◽  
Nicholas Moore ◽  
Vijay K. Anand ◽  
Theodore H. Schwartz
Keyword(s):  
Skull Base ◽  
Case Series ◽  
Extent Of Resection ◽  
Csf Leak ◽  
Minimal Access ◽  
Endoscopic Endonasal ◽  
Vein Thrombosis ◽  
Lumbar Drain ◽  
Deep Vein

Abstract BACKGROUND: The endoscopic endonasal transclival approach is a novel minimal-access method of managing clival pathology. Limited cases have been published. OBJECTIVE: To summarize our clinical experience with this approach and discuss technical nuances. METHODS: We retrospectively reviewed a prospective database of 250 endoscopic, endonasal skull base surgeries. Patients in whom a transclival approach was performed were identified. Extent of resection, complications, and clinical outcome were analyzed. RESULTS: Seventeen patients underwent 21 procedures. Pathology included chordoma, meningioma, hemangiopericytoma, enterogenous cyst, epidermoid, and metastasis. Lumbar drain was placed intraoperatively in 9 cases and maintained for goal. The risk of postoperative CSF leak was 4.8% for all procedures, 9.1% for procedures with large skull base defect, and 0% if a gasket-seal closure was achieved. A nasoseptal flap was used in 2 patients. There was one perioperative infarct, one case of deep vein thrombosis, and one postoperative pulmonary embolus. Mean follow-up was 8.5 months. All but one patient with preoperative cranial nerve deficits improved at last follow-up. All patients were free of disease progression at last follow-up. CONCLUSIONS: The endonasal endoscopic transclival approach provides a minimal-access approach to the ventral midline posterior fossa skull base. The risk of CSF leak is low if appropriate closure techniques are applied.

Long-term outcomes of lumbar microdiscectomy in the pediatric population: a large single-institution case series

2019 ◽  
Vol 24 (5) ◽  
pp. 549-557
Author(s):  
Malia McAvoy ◽  
Heather J. McCrea ◽  
Vamsidhar Chavakula ◽  
Hoon Choi ◽  
Wenya Linda Bi ◽  
...  
Keyword(s):  
Conservative Management ◽  
Functional Outcomes ◽  
Pediatric Patients ◽  
Clinical Presentation ◽  
Case Series ◽  
Csf Leak ◽  
Single Institution ◽  
The Mean

OBJECTIVEFew studies describe long-term functional outcomes of pediatric patients who have undergone lumbar microdiscectomy (LMD) because of the rarity of pediatric disc herniation and the short follow-up periods. The authors analyzed risk factors, clinical presentation, complications, and functional outcomes of a single-institution series of LMD patients over a 19-year period.METHODSA retrospective case series was conducted of pediatric LMD patients at a large pediatric academic hospital from 1998 to 2017. The authors examined premorbid risk factors, clinical presentation, physical examination findings, type and duration of conservative management, indications for surgical intervention, complications, and postoperative outcomes.RESULTSOver the 19-year study period, 199 patients underwent LMD at the authors’ institution. The mean age at presentation was 16.0 years (range 12–18 years), and 55.8% were female. Of these patients, 70.9% participated in competitive sports, and among those who did not play sports, 65.0% had a body mass index greater than 25 kg/m2. Prior to surgery, conservative management had failed in 98.0% of the patients. Only 3 patients (1.5%) presented with cauda equina syndrome requiring emergent microdiscectomy. Complications included 4 cases of postoperative CSF leak (2.0%), 1 case of a noted intraoperative CSF leak, and 3 cases of wound infection (1.5%). At the first postoperative follow-up appointment, minimal or no pain was reported by 93.3% of patients. The mean time to return to sports was 9.8 weeks. During a mean follow-up duration of 8.2 years, 72.9% of patients did not present again after routine postoperative appointments. The total risk of reoperation was a rate of 7.5% (3.5% of patients underwent reoperation for the same level; 4.5% underwent adjacent-level decompression, and one patient [0.5%] ultimately underwent a fusion).CONCLUSIONSMicrodiscectomy is a safe and effective treatment for long-term relief of pain and return to daily activities among pediatric patients with symptomatic lumbar disc disease in whom conservative management has failed.

Malignant thyroid teratoma: an integrated analysis of case series and case reports

2021 ◽  
Author(s):  
Huy Gia Vuong ◽  
Truong P.x. Nguyen ◽  
Hanh T.t. Ngo ◽  
Lewis Hassell ◽  
Kennichi Kakudo
Keyword(s):  
Clinical Course ◽  
Case Reports ◽  
Case Series ◽  
Seer Program ◽  
Integrated Analysis ◽  
Continuous Variables ◽  
Chi Square ◽  
Large Tumor ◽  
Exact Test

Malignant thyroid teratoma (MTT) is a very rare thyroid malignancy. These neoplasms have been reported only in case reports and small-sized case series so far. In this study, we searched for MTTs in the Surveillance, Epidemiology, and End Result (SEER) program during 1975-2016. Subsequently, we incorporated the SEER data with published MTT cases in the literature to analyze the characteristics and prognostic factors of MTTs. Integrated data were analyzed using Chi-square or Fisher’s exact test for categorical covariates, and t-test or Mann-Whitney test for continuous variables. We included 28 studies with 36 MTT cases and found additional 8 cases from the SEER program for final analyses. Our results showed that MTT is typically seen in adult females. These neoplasms were associated with an aggressive clinical course with high rates of extrathyroidal extension (80%) and nodal involvement (62%). During follow-up, the development of recurrence and metastases were common (42% and 46%, respectively), and one-third of patients died at the last follow-up. Large tumor size (p = 0.022) and the presence of metastases during follow-up (p = 0.008) were associated with a higher mortality rate. In conclusion, our study demonstrated the characteristic features of MTT patients and outlined some parameters associated with a negative outcome which could help clinicians better predict the clinical course of these neoplasms.

WP1-21 Maximum safe resection of large medial temporal intrinsic tumours: does the outcome justify the risk?

2019 ◽  
Vol 90 (3) ◽  
pp. e7.1-e7
Author(s):  
A Kumaria ◽  
A Paterson ◽  
M Sitaraman ◽  
S Basu
Keyword(s):  
Case Series ◽  
Csf Leak ◽  
Visual Field Defects ◽  
Long Term Outcomes ◽  
Karnofsky Score ◽  
Female Age ◽  
Grade 3 ◽  
Age Range

ObjectivesTo analyse on the long-term outcomes in patients undergoing maximum safe resection (MSR) for large intrinsic temporal tumours.DesignCase seriesSubjectsAll patients undergoing MSR of large medial temporal intrinsic tumours between May 2006 and February 2012 at a tertiary neurosurgical centre with a minimum follow up of 6 years.MethodsRetrospective review of hospital records.ResultsFifty-one patients underwent MSR (28 male, 23 female); age range 20–80 years (mean age 55.3). There was no difference in laterality, although dysphasia was a feature in 32% of left-sided lesions. Presenting features in general included seizures (46%), headaches (27%), hemiparesis (12%) and visual field defects (6%). Surgery was generally well tolerated (median post-operative Karnofsky score 92.5). No patients developed new dysphasia or weakness, but there was transient worsening of existing hemiparesis (n=4) and dysphasia (n=2). Other complications included CSF leak/pseudomeningocoele (n=2), oculomotor palsy (n=1) and wound infection (n=1). Histopathological casemix was GBM (50%), WHO 3 gliomas (14%), WHO 2 gliomas (10%) and metastases (4%). In total, 57% of patients received radiotherapy and 35% received chemotherapy. Survival correlated with pathology; in glioblastoma patients it ranged from 2–19 months (mean 10.4 months). Survival in grade 3 tumours ranged from 10–38 months (mean 24.4 months). 60% of patients with Grade 2 tumours are surviving symptom free with no histological upscale at 8–10 years follow-up. No patient required a second debulking procedure.ConclusionsMSR did not result in survival benefit in glioblastoma. MSR is justified in lesions with pre-operative radiological features of Grade 2 glioma.

scholarly journals Synovial Sarcoma of the Nerve—Clinical and Pathological Features: Case Series and Systematic Review

Neurosurgery ◽  
2019 ◽  
Vol 85 (6) ◽  
pp. E975-E991 ◽  
Author(s):  
Stephen Shelby Burks ◽  
Ross C Puffer ◽  
Iahn Cajigas ◽  
David Valdivia ◽  
Andrew E Rosenberg ◽  
...  
Keyword(s):  
Systematic Review ◽  
Statistical Analysis ◽  
Synovial Sarcoma ◽  
Tumor Size ◽  
Clinical Course ◽  
Case Reports ◽  
Case Series ◽  
Pathological Features ◽  
Eligibility Criteria

Abstract BACKGROUND Synovial sarcoma of the nerve is a rare entity with several cases and case series reported in the literature. Despite an improved understanding of the biology, the clinical course is difficult to predict. OBJECTIVE To compile a series of patients with synovial sarcoma of the peripheral nerve (SSPN) and assess clinical and pathological factors and their contribution to survival and recurrence. METHODS Cases from 2 institutions collected in patients undergoing surgical intervention for SSPN. Systematic review including PubMed and Scopus databases were searched for related articles published from 1970 to December 2018. Eligibility criteria: (1) case reports or case series reporting on SSPN, (2) clinical course and/or pathological features of the tumor reported, and (3) articles published in English. RESULTS From patients treated at our institutions (13) the average follow-up period was 3.2 yr. Tumor recurrence was seen in 4 cases and death in 3. Systematic review of the literature yielded 44 additional cases with an average follow-up period of 3.6 yr. From pooled data, there were 10 recurrences and 7 deaths (20% and 14%, respectively). Adjuvant treatment used in 62.5% of cases. Immunohistochemical markers used in diagnosis varied widely; the most common are the following: Epithelial membrane antigen (EMA), cytokeratin, vimentin, cluster of differentiation (CD34), and transducin-like enhancer of split 1 (TLE1). Statistical analysis illustrated tumor size and use of chemotherapy to be negative predictors of survival. No other factors, clinically or from pathologist review, were correlated with recurrence or survival. CONCLUSION By combining cases from our institution with historical data and performing statistical analysis we show correlation between tumor size and death.

Complications Are Rare From Middle Turbinate Resection: A Prospective Case Series

2019 ◽  
Vol 33 (6) ◽  
pp. 657-664 ◽  
Author(s):  
Steven Pinther ◽  
Robert Deeb ◽  
Edward L. Peterson ◽  
Robert T. Standring ◽  
John R. Craig
Keyword(s):  
Middle Turbinate ◽  
Case Series ◽  
Operative Intervention ◽  
Sinus Surgery ◽  
Csf Leak ◽  
Complication Rates ◽  
Prospective Case Series ◽  
Polyp Recurrence ◽  
Snot 22

Background When chronic rhinosinusitis with nasal polyps (CRSwNP) fails to respond to medical therapy, endoscopic sinus surgery (ESS) plays an integral role in management. Some studies have shown that middle turbinate resection (MTR) during ESS leads to decreased polyp recurrence and revision ESS rates. Other studies suggest MTR can lead to complications. Objective The purpose of this study was to assess the safety of MTR during ESS for CRSwNP by determining the incidences of intraoperative cerebrospinal fluid (CSF) leak, postoperative epistaxis requiring operative intervention, and postoperative complete frontal stenosis. Methods A multiinstitutional, prospective case series of 91 adult CRSwNP patients was conducted. Patients with medically refractory CRSwNP underwent primary or revision ESS plus MTR by 3 surgeons. Two of the surgeons performed partial MTRs, and one of the surgeons performed complete MTRs. Patients were evaluated for the following complications: intraoperative CSF leak during MTR, postoperative epistaxis requiring operative intervention, and postoperative complete frontal ostial stenosis. Secondary outcomes included changes from preoperative to postoperative 22-item Sinonasal Outcome Test (SNOT-22) scores and revision ESS rates. Results Unilateral or bilateral complete ESSs with MTRs were performed on 91 CRSwNP patients. In total, 173 MTRs were performed. Two surgeons performed 97 partial MTRs on 49 patients, and the third surgeon performed 76 complete MTRs on 42 patients. One CSF leak occurred during partial MTR (1/173, 0.57%). No patients suffered postoperative epistaxis requiring operative intervention, and no patients developed complete frontal stenosis. From preoperatively to postoperatively, mean SNOT-22 scores decreased from 53.7 to 13.1 ( P = .001). No revision ESS was needed during the follow-up period. Mean follow-up time was 7.5 ± 5.4 months. Conclusions Partial and complete MTR during ESS for CRSwNP in this cohort resulted in very low, acceptable intraoperative and short-term postoperative complication rates and no detriment to SNOT-22 scores.

scholarly journals Long-Term Visual Outcomes for Small Uveal Melanoma Staged T1 Treated by Proton Beam Radiotherapy

Cancers ◽  
2019 ◽  
Vol 11 (8) ◽  
pp. 1047 ◽  
Author(s):  
Adélaïde Toutée ◽  
Martina Angi ◽  
Sylvain Dureau ◽  
Christine Lévy-Gabriel ◽  
Livia Lumbroso-Le Rouic ◽  
...  
Keyword(s):  
Proton Beam ◽  
Optic Disc ◽  
Uveal Melanoma ◽  
Early Stage ◽  
Tumor Control ◽  
Proton Beam Radiotherapy ◽  
Visual Outcomes ◽  
The Mean

There is increasing evidence of the survival benefit of treating uveal melanoma in an early stage, however it is important to discuss with the patient the associated risk of visual loss. We investigated visual outcomes for uveal melanomas staged T1 (T1UM) treated by proton beam radiotherapy (PBR) as a function of their distance to fovea-optic disc. This retrospective study included a cohort of 424 patients with T1UM treated with PBR between 1991 and 2010 with at least a 5-year follow-up. Visual acuity (VA) was analyzed for patients with posterior edge of tumor located at ≥3 mm (GSup3) or <3 mm (GInf3) from fovea-optic disc. The mean follow-up duration was 122 months, no tumor recurrence was observed. The mean baseline and final VA were 20/25 and 20/32 for GSup3 (n = 75), and 20/40 and 20/80 for GInf3 (n = 317) respectively. The frequency of a 20/200 or greater visual conservation was 93.2%(CI95%:87.7–99.1) and 60.1%(CI95%:54.9–65.9) for GSup3 and GInf3 respectively. This difference between groups was statistically significant (p < 0.001). The risk factors for significant VA loss (less than 20/200) were GInf3 location (p < 0.001), tumor touching optic disc (p = 0.04), initial VA inferior to 20/40 (p < 0.001), documented growth (p = 0.002), and age greater than 60 years (p < 0.001). In summary, PBR for T1UM yields excellent tumor control and good long-term visual outcomes for tumors located ≥3 mm from fovea-optic disc.

Surgical Outcomes of Endoscopic Endonasal Surgery in 29 Patients with Craniopharyngioma

2020 ◽  
Author(s):  
Arad Iranmehr ◽  
Mostafa Esmaeilnia ◽  
Khashayar Afshari ◽  
Seyed Mousa Sadrehosseini ◽  
Azin Tabari ◽  
...  
Keyword(s):  
Tumor Resection ◽  
Visual Outcome ◽  
Extent Of Resection ◽  
Csf Leak ◽  
Endoscopic Endonasal Surgery ◽  
Endonasal Surgery ◽  
Endoscopic Endonasal ◽  
Pituitary Dysfunction ◽  
Before And After

Abstract Background Recently the endoscopic endonasal surgery (EES) has been introduced as a modality for the treatment of patients with craniopharyngiomas. In this study, we describe our initial experience in treatment of 29 patients with craniopharyngiomas using this approach. Methods Twenty-nine consecutive patients with craniopharyngiomas who had undergone EES in a 5-year period were studied retrospectively. Patients underwent preoperative and postoperative endocrinologic and ophthalmologic evaluations. Radiologic characteristics of tumors and extent of resection were determined. The recurrence and complications were evaluated. Results Pituitary and visual dysfunction were observed preoperatively in 89.7 and 86% of patients, respectively. After EES, visual outcome either showed an improvement or else remained unchanged in 92.3% of the cases; however, pituitary function remained unchanged and even got worsened in 34.6% of the cases. Prevalence of diabetes insipidus before and after surgery was 58.6 and 69.2%. The rate of gross total resection was 62%. Moreover, 86.2% of the tumors were almost totally resected (more than 95% of the tumor size resected). After surgery, cerebrospinal fluid (CSF) leak and meningitis occurred in four (13.8%) and two (6.9%) patients, respectively. Perioperative mortality was seen in two of the cases (6.9%). The mean follow-up was 25 months and tumor recurrence was discovered in four patients (15.3%). Conclusion The EES with the goal of maximal and safe tumor resection could be used for the treatment of most craniopharyngiomas. Although the rates of visual improvement and gross tumor resection are high, CSF leak, pituitary dysfunction, and meningitis are serious concerns.

scholarly journals Ultra-wide-field imaging of choroidal melanoma before and after proton beam radiation therapy

2019 ◽  
Vol 30 (6) ◽  
pp. 1397-1402
Author(s):  
Angeliki Psomiadi ◽  
Gertrud Haas ◽  
Michael Edlinger ◽  
Nikolaos E Bechrakis ◽  
Georgios Blatsios
Keyword(s):  
Proton Beam ◽  
Choroidal Melanoma ◽  
Subretinal Fluid ◽  
Scanning Laser ◽  
Wide Field ◽  
Scanning Laser Ophthalmoscopy ◽  
Proton Beam Radiotherapy ◽  
Imaging Characteristics ◽  
Before And After

Objective: To evaluate the imaging characteristics of choroidal melanoma before and after proton beam radiotherapy via Optos® ultra-wide-field scanning laser ophthalmoscopy. Methods: Retrospective, descriptive study of choroidal melanoma patients treated with proton beam radiotherapy. All patients underwent full clinical evaluation, including best-corrected visual acuity, ultrasound examination and ultra-wide-field scanning laser ophthalmoscopy imaging in the pseudo-colour (red and green channel) as well as auto-fluorescence mode. Tumours were classified and evaluated according to their location, size, presence of subretinal fluid, drusen, orange pigment and reflectance intensity in ultra-wide-field scanning laser ophthalmoscopy. Tumour sonographic (basal diameter, height) and ultra-wide-field scanning laser ophthalmoscopy imaging dimensions (maximal diameter) were documented. Results: A total of 39 eyes (38 patients) were followed for 24 months (range 6–48 months). Mean best-corrected visual acuity dropped from 20/40 to 20/63 after proton beam radiotherapy. There was no change in the imaging tumour characteristics during follow-up. Subretinal fluid changes were better detected in the autofluorescence compared to pseudo-colour mode. Mean tumour diameter did not significantly change in the ultra-wide-field scanning laser ophthalmoscopy although it did so in the ultrasound. No patient showed local tumour recurrence. Conclusion: The ultra-wide-field scanning laser ophthalmoscopy imaging characteristics of choroidal melanoma in the Optos® system do not significantly change after proton beam radiotherapy after a mean follow-up of 2 years.

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