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Retroperitoneal germ cell tumor with tumor thrombosis of the inferior vena cava (IVC) represents a rare phenomenon. Its extension to the hepatic veins (HVs) has not been reported yet. In the present case, a 30-month-old girl had a recurrent retroperitoneal yolk sac tumor with liver metastasis. In addition, there were tumor thrombi in the IVC, right atrium (RA), and all three HVs. The child was operated after a satisfactory response to chemotherapy. Excision of the retroperitoneal tumor with right hepatectomy, retrohepatic caval resection, HV, and RA thrombectomy was performed under a cardiac bypass. HV cloaca was patched with pericardium while the IVC resection was tolerated without reconstruction. Her postoperative recovery was normal. The child currently remains disease-free and symptom-free at 12 months. This case demonstrates the technique of hepatic venous thrombectomy and the feasibility of radical surgery even for a metastatic disease when tumor biology is favorable. Despite wide-spread tumor thrombosis that would have been deemed unresectable, complete resections are possible in referral centers.

To evaluate the efficacy and toxicity of dose-escalated image guided-intensity modulated radiation therapy (IG-IMRT) in osteosarcoma (OGS), chondrosarcoma (CS) and chordoma (CH) of head and neck (HN) and pelvis.In this prospective non-randomized study, 65 patients of HN or pelvic OGS (24), CS (7) and CH (34) mandating definitive or post-operative radiotherapy from May 2013 to December 2018 were included. Radiotherapy doses in definitive setting were 72.0 Gy for CH and 70.2 Gy for OGS and CS; while in post-operative setting it was 66.6 Gy and 64.8 Gy respectively (at 1.8 Gy per fraction).Planned doses of radiotherapy could be completed in 61 (93.8%) patients; with grade III or higher acute and late toxicities of 3% and 0% respectively. With a median follow-up of 52 (range 6-92) months, the five-year actuarial local control (LC) rates were 66% in OGS, 38.1% in CS and 75.9% in CH; while cause-specific survival (CSS) rates were 54.7%, 64.3% and 92.2% respectively. There was no statistically significant difference in outcomes for patients receiving definitive and post-operative radiotherapy. Locally controlled disease at first follow-up after radiotherapy was associated with improved CSS and OS in CS (p = 0.014) and CH (p 0.001). Radiotherapy resulted in significant and sustained improvement in Musculoskeletal tumour society (MSTS) score and reduction in pain score. Salvage re-irradiation was feasible in local progression after radiotherapy, with good outcomes and tolerability.Dose-escalated IG-IMRT results in good LCfunctional improvement with minimal toxicity in OGS, CS and CH.

Hereditary hemorrhagic telangiectasia (HHT) is a rare genetic disease with prevalence of approximately 1 in 5000–10,000. We evaluated the prevalence and association of cerebrovascular and cardiovascular comorbidities in HHT patients using national database. Retrospective observational study was performed using National Inpatient Sampling (NIS) database for the year 2014. HHT patients and comorbidities were identified using ICD-9 codes. Univariate and multivariate analyses were performed using SAS. Prevalence of HHT was 0.0119% with predominance in White population. Mean age of HHT patients was 59 years. Increased proportion of HHT patients had hypertension (46.8% vs 42%), anemia (28.9% vs 15.1%), chronic pulmonary disease (24.8% vs 16.4%), congestive heart failure (15.7% vs 7.5%), liver disease (7.9% vs 2.8%), migraine (4.5% vs 1.5%), and cerebrovascular malformations (0.8% vs 0.03%), whereas chronic kidney disease (12.7% vs 12.2%), headaches (1.3% vs 1.1%), seizures (0.7% vs 0.9%), transient ischemic attacks (1.06% vs 1.03%), ischemic (1.2% vs 1.0%), and hemorrhagic (0.5% vs 0.3%) strokes were similar to those without HHT. Multivariable model shows increase in cerebrovascular malformations (OR 11.04, CI 2.49–22.26, p < 0.0001), migraine (OR 3.23, CI 2.30–4.52, p < 0.0001), chronic blood loss anemia (OR 6.83, CI 5.36–8.71, p < 0.0001), congestive heart failure (OR 1.55, CI 1.26–1.91, p < 0.0001), chronic pulmonary disease (OR 1.30, CI 1.09–1.56, p = 0.0038), and hepatic disease (OR 2.63, CI 2.01–3.45, p < 0.0001) in HHT patients as compared to non-HHT patients. There is a need for a large prospective registry of HHT patients that can corroborate these associations and burden of cerebrovascular and cardiovascular diseases.

Cemento-ossifying fibromas are benign fibro-osseous lesions characterized by deposition of cementum and fibrous tissue. Familial gigantiform cementoma (FGC) is an exceeding rare and distinct subtype of cemento-osseous-fibrous lesion. We hereby present a case of FGC in a young boy who was left to die due to the social stigma caused by the massive bony growth in the upper and lower jaw. The patient was somehow rescued by a non-governmental organization and underwent his surgical management at our hospital. On family screening, the mother also had similar smaller asymptomatic lesions in the jaw, but she refused further investigations and treatment. FGC is frequently associated with “calcium-steal phenomenon,” which was also seen in our patient. Family screening is therefore necessary to identify asymptomatic patients in the family and follow them up with radiology and whole body dual-energy absorptiometry scans.

Background Despite being mandated by cooperative groups, omission of nodal sampling is the most frequent protocol deviation in surgery for Wilms tumor. The stations as well as the number of nodes that should be sampled are not clearly defined resulting in a marked variation in practices among surgeons. We propose a systematic method for nodal sampling intending to reduce interoperator variation. In this study, we have assessed the feasibility and yield of systematic lymph node sampling and also evaluated the factors influencing nodal metastasis. Methods Prospective evaluation of 113 Wilms tumor patients operated at a single tertiary cancer center between 2015 and 2019. All these patients underwent a systematic 5-station nodal sampling. Results Median lymph node yield was 8 and 13.2% (15/113) patients harbored a histologically positive nodal disease. Of the patients with positive nodal disease, interaortocaval nodes had metastasis in 46.7% (n = 7). They represented isolated sites of nodal disease (skip metastases) in 28.6% (n = 4) of patients. Right-sided tumors had more frequent involvement of interaortocaval nodes and skip disease. Tumors with high-risk histology had 12.5 times more odds of harboring nodal disease as compared to low and intermediate-risk histology Wilms tumor. Conclusions The proposed method of systematic station wise sampling provides a template to guide surgeons in performing lymph node harvesting. Interaortocaval nodes sampling should be performed routinely as the incidence of disease at this station is sufficiently high and metastasis may skip hilar nodes. Study of diagnostic test Level III evidence.

Background: Overburdened systems and concerns of adverse outcomes have resulted in deferred cancer surgeries with devastating consequences. In this COVID pandemic, the decision to continue elective cancer surgeries, and their subsequent outcomes, are sparsely reported from hotspots. Methods: A prospective database of the Department of Surgical Oncology was analysed from March 23rd to April 30th, 2020. Findings: Four hundred ninety-four elective surgeries were performed (377 untested and 117 tested for Covid 19 before surgery). Median age was 48 years with 13% (n = 64) above the age of 60 years. Sixty-eight percent patients were American Society of Anaesthesiology (ASA) grade I. As per surgical complexity grading, 71 (14·4%) cases were lower grade (I-III) and 423 (85.6%) were higher grade complex surgeries (IV – VI). Clavien-Dindo ≥ grade III complications were 5.6% (n = 28) and there were no postoperative deaths. Patients >60 years documented 9.3% major complications compared to 5.2% in

Lately, NTRK-positive mesenchymal tumors are being increasingly identified, mostly in pediatric patients, in view of associated treatment implications, especially in recurrent and unresectable tumors. A 1-year-old male child presented with a rapidly growing tumor mass in his cervical region of 2 months duration. Radiologic imaging disclosed a tumor measuring 11 cm in size, almost filing his right neck spaces. Review of biopsy sections revealed a cellular tumor comprising spindle cells arranged in sheets and fascicles with interspersed collagenous strands and areas of adipocytic, myxoid, and hyaline degeneration. Immunohistochemically, tumor cells were diffusely positive for CD34 and S100 protein. Subsequently, on testing the tumor for a solid tumor gene panel by next-generation sequencing, it was found to be positive for inv(1)(q23q31) TPR-NTRK1 fusion. Furthermore, tumor cells displayed NTRK1 gene rearrangement by fluorescence in situ hybridization technique. The patient was offered chemotherapy; however, he had a rapid local progression, leading to respiratory obstruction; he then succumbed to the disease. The present case underpins the value of next-generation sequencing as a useful technique for uncovering NTRK-fusion–positive mesenchymal tumors. Review of similar cases, diagnostic challenge, and treatment implications in such cases are discussed.

Unlike other pediatric extra cranial solid neoplasms, neuroblastomas have very low incidence of pulmonary metastases. Neuroblastomas also tend to encase rather than infiltrate the major vessels with very low incidence of tumor thrombus. Even in cases with inferior vena cava tumor thrombosis, pulmonary metastases are a rare occurrence. These patients usually show widespread disseminated disease at presentation. We present a rare case of adrenal neuroblastoma with tumor thrombus and bilateral pulmonary metastases at presentation. This presentation can be confused with adrenocortical carcinoma and differentiating between the two is essential for further management.

Background and hypothesis Bone and lung are the common sites of metastasis in pediatric round cell tumors and its presence indicates poor outcomes. Staging workup for these malignancies thus includes bone marrow biopsy (BMB) along with evaluation of thorax, and tissue analysis for N MYCN status in neuroblastoma. BMB is an invasive procedure requiring general anesthesia with known disadvantages.With an aim of avoiding an invasive BMB a study was taken up to evaluate efficacy of FDG PET CT in detecting marrow involvement in neuroblastoma and rhabdomyosarcoma at staging. Materials and method Prospective observational study evaluated 83 newly diagnosed treatment naive patients of neuroblastoma (n = 43) and rhabdomyosarcoma (n = 42) who underwent conventional imaging of PETCT with CECT of local region along with a CT thorax and BMB (both iliac crest) done within 1 week. Findings of FDG PETCT were compared with bone marrow histology and accuracy parameters were calculated. Result The overall sensitivity, specificity, accuracy, positive-predictive value (PPV), negative-predictive value (NPV) of FDG PETCT for detection of marrow disease was 100%, 86.1%, 89.4%. 68.9% and 100%, respectively. Subset analysis showed sensitivity, specificity, PPV and NPV of 100%, 66%, 71.4% and 100%, respectively, for neuroblastoma, with rhabdomyosarcoma patients having few events NPV of 100% accuracy of 97.6%. Conclusion FDG PETCT with sensitivity and NPV of 100% can be considered as a first stop imaging and biopsy can be avoided in patients with a negative scan.

Background: Immunomodulation and cell signaling involve several cytokines, proteins, and other mediators released in response to the trauma, inflammation, or other insults to the central nervous system. This pilot study is part of the registry designed to evaluate the temporal trends among these molecules after an acute ischemic stroke (AIS) in patients. Methods: Twelve AIS patients were enrolled within 24 hours of the symptoms onset. Two sets of plasma samples were collected: First at admission and second at 24 hours after admission. Cytokines/chemokines and other inflammatory molecules were measured using multiplex assay kit. Results: An increased trend in IL-6 (22 vs. 34 pg/ml), IL-8/CXCL8 (87 vs. 98 pg/ml), MMP-9 (16225 vs. 18450 pg/ml), and GMF-β (999 vs. 3739 pg/ml) levels was observed overtime after an AIS. Patients ≤60 years had lower levels of plasma MCP-1/CCL2 (50–647 vs. 150–1159 pg/ml), IL-6 (9–25 vs. 20–68 pg/ml), and IL-8 (30– 143 vs. 72–630 pg/ml), when compared with patients >60 years old. Conclusion: Cytokines/chemokines and other inflammatory mediators play an important role in the pathogenesis of stroke in addition to mediating poststroke inflammation. Further research is needed to evaluate and characterize the cumulative trends of these mediators for the clinical prognosis or as surrogate biomarkers.

Pediatric peritoneal sarcomatosis is an exceedingly rare entity with unknown incidence. Within these tumors, primary peritoneal rhabdomyosarcoma constitutes a small fraction. Majority of them are probably treated inadequately and have dismal outcomes. A favorable subset exists where aggressive treatment in the form of cytoreductive surgery supplemented by hyperthermic intraperitoneal chemotherapy in the multimodal setting can be attempted. We present a case of primary peritoneal embryonal rhabdomyosarcoma in a 2-year-old child who was treated with systemic chemotherapy, cytoreductive surgery, and hyperthermic intraperitoneal chemotherapy while avoiding radiation, with its evidence and rationale.

Wilms tumour is the commonest renal tumour in children with peak age group of 2–5 years [ 1 , 2 ]. It commonly presents as an incidentally detected abdominal mass; however fever, hematuria and hypertension may also be seen [ 1 ]. Extra-renal Wilms tumour is extremely rare, with an incidence of 0.5%–1% [ 3 , 4 ]. Wilms tumour, most commonly metastasizes to the lungs, followed by lymph nodes and liver, however, metastasis to the bones and spinal cord at diagnosis is extremely rare [ 1 , 2 , [5] , [6] , [7] , [8] , [9] , [10] ]. We hereby, report an extremely rare presentation of suprarenal Wilms tumour with intraspinal metastases. Our case highlights the high degree of vigilance as well as timely intervention with multimodality therapy required to improve the outcomes in the setting of advanced metastatic disease; notwithstanding the potentially irreversible neurological weakness in the absence of optimal intervention.

Objectives To evaluate the diagnostic accuracy of multidetector computed tomography (MDCT)–based staging of Wilms’ tumor (WT) in comparison with surgical-pathological staging (reference standard) and to assess the interobserver agreement in predicting CT stage for WT. Method Retrospective audit of 50 consecutive cases of WT meeting our eligibility criteria was performed, and an electronic database of CT scan findings, intraoperative findings, and final histopathology staging were created. Two radiologists blinded to surgical and histologic findings reviewed the CT scans, using multiplanar reformations to assess various parameters pertaining to tumor extent and assign a possible stage, which was then compared with the final surgical-pathological stage. Interobserver agreement was assessed using κ-coefficient. Results CT scan correctly staged 31 (62%) of 50 WT cases (both observers’ consensus data, all stages combined). CT accuracy was 37.5% in stage I disease, 66% in stage II disease, and 75% in stage III. There was substantial interobserver agreement (in 80% of the cases) in assigning the stage. Subset analysis showed a 100% positive predictive value (PPV) in detecting renal vein thrombosis. The negative predictive value (NPV) for ureteric involvement was 91 to 93%, and that for retroperitoneal adenopathy, it was 93 to 97%. Conclusion The accuracy of CT in staging WT is dependent on the disease stage. While it overestimates the extent of the disease in early stage, it predicts the advanced stage with very good accuracy and can be used to avoid upfront surgeries in locally advanced WT.

Skull base osteomyelitis (SBO) is a complex and fatal clinical entity that is often misdiagnosed for malignancy. SBO is commonly a direct complication of otogenic, sinogenic, odontogenic, and rhinogenic infections and can present as central, atypical, or pediatric clival SBO. This review describes the clinical profi le, investigational approach, and management techniques for these variants. A comprehensive literature review was performed in PubMed, MEDLINE, Research Gate, EMBASE, Wiley Online Library, and various Neurosurgical and Neurology journals with the keywords including: SBO, central or atypical SBO, fungal osteomyelitis, malignant otitis externa, temporal bone osteomyelitis, and clival osteomyelitis. Each manuscript's reference list was reviewed for potentially relevant articles. The search yielded a total of 153 articles. It was found that with early and aggressive culture guided long-term intravenous broad-spectrum antibiotic therapy decreases post-infection complications. In cases of widespread soft tissue involvement, an early aggressive surgical removal of infectious sequestra with preferentially Hyperbaric Oxygen (HBO) therapy is associated with better prognosis of disease, less neurologic sequelae and mortality rate. Complete resolution of the SBO cases may take several months. Since early treatment can improve mortality rates, it is paramount that the reporting radiologists and treating clinicians are aware of the cardinal diagnostic signs to improve clinical outcomes of the disease. It will decrease delayed diagnosis and under treatment of the condition. However, due to rarity of the condition, complete prognostic factors have not fully been analyzed and discussed in the literature.

The present study was aimed at evaluating clinicopathologic and immunohistochemical (IHC) features of 300 rhabdomyosarcomas (RMSs), including differential IHC expression and prognostic value of myogenin and MyoD1 across various subtypes of RMSs. IHC expression of myogenin and MyoD1 was graded on the basis of percentage of tumor cells displaying positive intranuclear immunostaining i.e. grade 1 (1-25%); grade 2 (26-50%); grade 3 (51-76%) and grade 4 (76-100%).Clinical follow-up was available in 238 (79.3%) patients. Various clinicopathologic parameters were correlated with 3-year disease free survival (DFS) and overall survival (OS). There were 140 cases (46.7%) of alveolar RMS (ARMS), 90 of embryonal RMS (ERMS) (30%), 61 (20.3%) of spindle cell/sclerosing RMS and 9 cases (3%) of pleomorphic RMS. Most cases, barring pleomorphic RMSs, occurred in the first two decades (228 cases) (76%), frequently in males, in the head and neck region (126) (42%). By immunohistochemistry, desmin was positive in 292/299 (97.6%) tumors; myogenin in 238/267 (89.1%) and MyoD1 in 192/266 (72.2%) tumors. High myogenin expression (in ≥51% positive tumor cells) was significantly associated with ARMSs (95/121, 78.5%), as compared to other subtypes (48/117, 41%) (p value 0.001). High MyoD1 expression (≥51% tumor cells) was seen in more cases of pure sclerosing, combined with spindle cell/sclerosing RMSs (10/10, 100%), as compared to the other subtypes (91/141, 67.4%) (p = 0.032). There was no significant difference between high myogenin expression and clinical outcomes. Patients without metastasis and harbouring tumors, measuring ≤5 cm showed a significant increase in OS, with p values = 0.01 and0.001, respectively. ARMS was the most frequent subtype. There was a significant association between high myogenin expression and ARMSs and high MyoD1 expression and spindle cell/sclerosing RMSs. High myogenin expression did not correlate with clinical outcomes. Patients with smaller sized tumors and without metastasis had significantly better clinical outcomes.

Lymph node metastasis is a considerable variable influencing postoperative American Thyroid Association (ATA) risk stratification in pediatric differentiated thyroid cancer (DTC). The primary aim of this study was to ascertain the factors predicting nodal metastasis and describe the outcomes in relation to the ATA risk. Patients 18 years or younger operated between December 2005 and December 2019 were analyzed. Demographic, clinicopathological, treatment, and outcome data were recorded. Factors associated with nodal metastasis were assessed by univariate and multivariate regression analysis. Patients were stratified into low-, intermediate-, and high-risk as per the pediatric ATA guidelines. A total of 86 patients (43% male; median [IQR] age, 12 (10–14) years) underwent surgery during the study period. Lymph node metastases were present in 70 (82.4%) patients involving the lateral (8%) and central compartment (4.7%) alone and both (88.6%) compartments. Extrathyroid extension (ETE) was present in 65%; 35%, minimal; and 30%, extensive. On univariate analysis, nodal metastasis was more frequent in male patients, multifocal tumor, lymphovascular invasion, and ETE. On multivariate analysis, only ETE was predictive of nodal disease with an odds ratio of 8. Minimal and extensive ETEs were both significantly associated with lymph node metastases when compared to the absence of ETE. The 5-year disease-free survival was 100%, 95.7%, and 66% in the low-, intermediate-, and high-risk groups respectively (p

Inflammation is an important pathophysiological process after an acute stroke (AS). Pro- and anti-inflammatory molecules (cytokines and interleukins) are the key players during this mechanism. Emerging evidence indicate that these molecules can serve as biomarkers of stroke progression and outcome and as novel therapeutics agents. The aim of this study is to explore the temporal changes in these molecules and validate them as biomarker of AS progression and neurological outcome. The “Cytokine Registry In Stroke Patients (CRISP)” is a prospective cohort study of 600 AS patients presenting to the tertiary hospital with-in 24 h of the onset of symptoms. Plasma cytokines and interleukins will be collected at admission and 24 h after and will be measured using enzyme-linked immunosorbent assay (ELISA) to evaluate the difference in their variation among different gender, race and ethnicity and their association with various neurological outcomes. The primary exposures are biological sex (male, female) and race/ethnicity. Confounding variables include age, vascular risk factors, infarct size, stroke onset to presentation time, and identified stroke etiologies. Matched controls will be used for the comparison and evaluation of the difference among gender and race/ethnicities. CRISP is a prospective observational study that investigates the role and relationship of molecular biomarkers identifying specific and relevant targets pertinent for monitoring the progression and outcome in AS patients. Trial Registration: The study is registered on ClinicalTrial.gov: https://clinicaltrials.gov/ (NCT03297827).

Introduction: Tumors located at the hepatocaval junction with the involvement of hepatic veins conventionally warrant major liver resections. Appreciation of sizeable inferior right hepatic vein can allow preservation of uninvolved inferior segments of the liver. Operative Technique: Preoperative and intraoperative identification of sizeable inferior right hepatic and other accessory or communicating veins in combination of intra-operative ultrasound guidance for resection to preserve inferior segments of the liver with adequate venous drainage. Application of upper transversal resections for pediatric liver tumors at the hepatocaval confluence was illustrated with description of operative technique and patient selection. Conclusions: Parenchymal preserving surgeries are possible for pediatric liver tumors located at the hepatocaval confluence by identification of accessory draining veins to the inferior segments. This along with meticulous parenchymal dissection with ultrasound guidance to preserve all uninvolved veins is key to safe upper transversal resections.

Introduction Treatment guidelines for hepatoblastoma discourage nonanatomic liver resections. However, the evidence for this is inadequate and comes from a study performed almost two decades ago which additionally contained inherent limitations. This study aimed to assess the feasibility and oncologic outcomes of nonanatomic resections (NAR) performed in diligently selected patients and compare the results with anatomic resections (AR). Materials and Methods A total of 120 patients who underwent liver resections for hepatoblastoma between January 2008 and July 2019 were reviewed. Feasibility of NAR was based on postchemotherapy relations to vessels, site of the lesion, and possibility of achieving negative resection margins. Results AR was performed in 95 patients and 25 had NAR. The NAR cohort had similar International Childhood Liver Tumors Strategy Group (SIOPEL) risk group distribution. Blood loss and operative times were lower in patients undergoing NAR. No differences were noted between the two groups concerning postoperative morbidity and hospitalization. There were no pathologic positive margins or local recurrences in the NAR patients. Relapse free (RFS) and overall survival (OS) was similar in the two groups (p = 0.54 and 0.96, respectively). Subgroup analysis of only posttreatment extent of tumor (POSTTEXT) I and II patients also showed no difference in RFS or OS for the two groups with a persistent significant difference in operative times and blood loss. Conclusion NAR is feasible with clear margins in carefully selected patients. It is not associated with more complications and outcomes are not inferior to AR. NAR is associated with lesser blood loss and operative time.

Injury to the intracranial venous sinuses can be life threatening. Therefore, a thorough understanding of this anatomy is important to those clinicians and surgeons caring for such patients. This chapter outlines the common injuries that can occur to the intramural venous sinuses and reviews the surgical nuances.

Surgery continues to remain an integral component of treatment, especially for nonrhabdomyosarcoma soft tissue sarcoma as compared to rhabdomyosarcoma owing to their general insensitivity to chemotherapy. A key determinant of outcomes, particularly for extremity tumors includes complete tumor resection with negative margins; however, a significant limitation for limb salvage surgery is the adherence of sarcomas to vital vascular structures. Hitherto, vascular involvement constituted an adequate reason for amputation. However, modern reconstructive techniques and availability of prosthetic grafts in addition to autologous venous grafts have rendered limb salvage surgery possible in a substantial majority of patients. Vascular resection and reconstruction for extremity soft tissue sarcoma in children have not been used routinely for reasons like the small-caliber of native vessels, limited options for conduits and rapid somatic growth. The situation is inconceivable in infants owing to the contemporaneous diminutive caliber of the vessels. We report two infants with lower extremity nonrhabdomyosarcoma soft tissue sarcoma who underwent limb salvage surgery with resection of femoral vessels following which vascular reconstruction was successfully performed using the great saphenous vein allograft harvested from the father.

The current multidisciplinary approach in the treatment of Ewing sarcoma has improved cure rates, with contemporary dose-dense chemotherapy attaining 5-year event-free survival (EFS) of 73% in localized cases. Dose-intense and dose-dense chemotherapy is difficult in the majority of resource-limited settings with limited access to optimal supportive care. We report on patients with Ewing sarcoma treated on EFT-2001, a nondose-dense chemotherapy protocol.A retrospective analysis was conducted of patients (15 years) with Ewing sarcoma treated with curative intent during January 2013-June 2017 with an institutional ethics committee-approved nondose-dense protocol (EFT-2001). Local therapy was planned after 9-12 weeks of chemotherapy with metastatic sites addressed with radiotherapy. The study assessed outcomes and prognostic factors.We analysed 200 patients with M:F ratio of 1.27:1 and metastases in 41 patients (20.5%). At a median follow up of 41.5 months (range 4.5-81.8 months), respective 3-year EFS and overall survival (OS) of the whole cohort is 65.3% (95% confidence interval [CI]: 58.1-71.7%) and 79.3% (95% CI: 72.8-84.5%); for localized and metastatic cohort, 70.9% (95% CI: 62.9-77.5%) and 82.8% (95% CI: 75.7-89.0%); and for metastatic cohort, 42.8% (95% CI: 28.0-58.6%) and 65.3% (95% CI: 47.7-78.3%). Presence of residual disease (morphologic/metabolic) on positron emission tomography-computed tomography scan done 3 months post definitive radiotherapy (hazard ratio [HR] 7.92 [95% CI: 3.46-18.14]) and delay in any form of local control4 months (HR 3.42 [95% CI: 1.32-8.89]) affected outcomes. Nonrelapse mortality during treatment was 6.5%, mainly due to cardiomyopathy (3.0%) and bacterial sepsis (1.5%). Cardiotoxicity was seen in 11.5% of patients.Nondose-dense chemotherapy provides good outcomes with manageable toxicities in a multidisciplinary treatment approach, while reducing cumulative drug exposures in the developing world where dose-intense or dose-dense chemotherapy could potentially increase toxicity, and hence seems a feasible approach in resource-limited settings. Presence of any residual disease post definitive radiotherapy or delay in local control portends poor outcome.

Spinal cord injury (SCI) is a life-shattering neurological condition that affects between 250,000 and 500,000 individuals each year with an estimated two to three million people worldwide living with an SCI-related disability. The incidence in the USA and Canada is more than that in other countries with motor vehicle accidents being the most common cause, while violence being most common in the developing nations. Its incidence is two- to fivefold higher in males, with a peak in younger adults. Apart from the economic burden associated with medical care costs, SCI predominantly affects a younger adult population. Therefore, the psychological impact of adaptation of an average healthy individual as a paraplegic or quadriplegic with bladder, bowel, or sexual dysfunction in their early life can be devastating. People with SCI are two to five times more likely to die prematurely, with worse survival rates in low- and middle-income countries. This devastating disorder has a complex and multifaceted mechanism. Recently, a lot of research has been published on the restoration of locomotor activity and the therapeutic strategies. Therefore, it is imperative for the treating physicians to understand the complex underlying pathophysiological mechanisms of SCI.

Background Cranial osteomyelitis is a rare but potentially life-threatening condition that requires early diagnosis with prompt and appropriate management by neurosurgeons to prevent further central nervous system complications. Methods The literature in the Medline database was comprehensively reviewed with the keywords “cranial osteomyelitis,” “skull base osteomyelitis (SBO),” “central skull base osteomyelitis,” and “temporal bone osteomyelitis.” Items in the reference list of each article relevant to the objective of this study were reviewed. Results This review produced 183 articles: 13 book chapters, 24 case reports, 17 case series, 98 original articles, 30 review articles, and 1 meta-analysis. We classified cranial osteomyelitis as sinorhino-otogenic, including anterior, middle, and posterior skull base osteomyelitis; and non-sinorhino-otogenic, including iatrogenic, posttraumatic, hematologic, and osteomyelitis with other causes. Conclusions New diagnostic modalities, the introduction of broad-spectrum antibiotics, and recent advances in neurosurgical procedures have led to a decrease in the rate of treatment failure in cranial osteomyelitis. Early recognition of initial nonspecific symptoms is key to diagnosing and managing this treatable but life-threatening condition. Early identification of the causative pathogen, appropriate broad-spectrum antibiotic therapy over a period of 8–20 weeks, and aggressive surgical debridement are essential for managing cranial osteomyelitis. On the other hand, inadequate treatment is responsible for refractory cases and poses a great diagnostic challenge. A new classification dividing cranial osteomyelitis into sinorhino-otogenic versus nonsinorhino-otogenic groups could prove valuable for clinical communication and treatment.

Introduction Brain is suspended in cerebrospinal fluid (CSF)-filled subarachnoid space by subarachnoid trabeculae (SAT), which are collagen-reinforced columns stretching between the arachnoid and pia maters. Much neuroanatomic research has been focused on the subarachnoid cisterns and arachnoid matter but reported data on the SAT are limited. This study provides a comprehensive review of subarachnoid trabeculae, including their embryology, histology, morphologic variations, and surgical significance. Methods A literature search was conducted with no date restrictions in PubMed, Medline, EMBASE, Wiley Online Library, Cochrane, and Research Gate. Terms for the search included but were not limited to subarachnoid trabeculae, subarachnoid trabecular membrane, arachnoid mater, subarachnoid trabeculae embryology, subarachnoid trabeculae histology, and morphology. Articles with a high likelihood of bias, any study published in nonpopular journals (not indexed in PubMed or MEDLINE), and studies with conflicting data were excluded. Results A total of 1113 articles were retrieved. Of these, 110 articles including 19 book chapters, 58 original articles, 31 review articles, and 2 case reports met our inclusion criteria. Conclusions SAT provide mechanical support to neurovascular structures through cell-to-cell interconnections and specific junctions between the pia and arachnoid maters. They vary widely in appearance and configuration among different parts of the brain. The complex network of SAT is inhomogeneous and mainly located in the vicinity of blood vessels. Microsurgical procedures should be performed with great care, and sharp rather than blunt trabecular dissection is recommended because of the close relationship to neurovascular structures. The significance of SAT for cerebrospinal fluid flow and hydrocephalus is to be determined.

Background Subdural empyema can present as a spinal subdural empyema (SSE) or a cranial subdural empyema (CSE). Although they differ somewhat in epidemiology, etiology, pathophysiology, and symptomatology and occur separately, they rarely manifest together. The aim of this article is to review the literature concerning the clinical presentation, clinical course, and treatment options for managing concurrently occurring SSE and CSE. Methods The literature in the Medline database was reviewed with key words including but not limited to subdural empyema, retroclival empyema, and Streptococcus mitis . No similar reports were found in the database involving infection with this type of microorganism in this anatomical region. Results Only 3 cases with concurrent CSE and SSE were found in the literature caused by various etiologic agents. Two of the patients recovered with no neurologic deficit, whereas one fatality was reported. One new illustrative case caused by Streptococcus mitis is also presented. Conclusions CSE and SSE are neurosurgical emergencies, often requiring prompt surgical evacuation. Although very rare, Streptococcus mitis can cause spinal subdural empyema or retroclival abscesses. Natural history of this disease is grave without treatment. Delays in diagnosis and treatment are directly related to mortality and severe morbidity in patients with intracranial and spinal subdural empyema. Prompt recognition and treatment are essential to preclude severe neurologic disabilities or in rare cases a fatal outcome. A treatment paradigm for cranio-spinal empyema is proposed.

To describe the outcomes of elective cancer surgeries and adverse consequences on the patients and medical staff due to the surgical interventions in children during the Coronavirus Disease 2019 (COVID-19) pandemic.The study included children younger than 15 years who underwent elective cancer surgeries from March 4, 2020 and December 3, 2020.A total of 121 patients (62% male; median age, 3 years) underwent surgery. The surgical procedures included nephrectomies (n = 18), neuroblastoma (n = 26) and soft tissue tumor resections (n = 24) and complex surgical procedures like extended liver resections (n = 2), intra-atrial thrombectomy under cardiopulmonary bypass (n = 2), pancreatoduodenectomy (n = 1), and free microvascular flaps (n = 7). Clavien-Dindo Grade III complications were 5% (n = 6), and there were no postoperative deaths. Preoperative COVID-19 testing was performed in 82% of children, and only 2% showed severe acute respiratory syndrome coronavirus 2 positivity. Postoperatively, 26 children were tested because of specific symptoms and, 6 tested positive for severe acute respiratory syndrome coronavirus 2. Except for a median delay of 23 days in treatment, none of the patients with COVID-19 required critical hospital management. None of the surgical residents or faculty acquired COVID-19, while 4 each medical and support staff were tested positive in the study period.COVID-19 was not a deterrent for continued cancer care, and surgeries could be safely performed adopting universal preventive measures without any added morbidity from COVID-19. Caregivers and centers dealing with childhood cancers can be encouraged to sustain or seek early healthcare.

Objective The systematic review aimed to determine demographic characteristics, clinical features, lab evaluation, management and complications of the studies focusing on Guillain-Barre syndrome (GBS) as a sequele of novel coronavirus (COVID-19) infection. Methods After protocol registration, PubMed, Web of Science and Cumulative Index to Nursing & Allied Health Literature (CINHAL) databases were searched for relevant articles using MeSH key-words and imported into referencing/review softwares. The data, regarding demographic and clinical characteristics, diagnostic workup and management, was analyzed in International Business Machines (IBM) Statistics SPSS 21. Many statistical tests, such as t-test and the Mann–Whitney U test, were used. P Results We identified 64 relevant articles. The mean age of the patients was 56 ± 16 years; the majority were males (64.9%). Among the neurological findings, paresthesia was the most typical symptom (48.9%). Most of the patients had been diagnosed by reverse transcriptase-polymerase chain reaction (RT-PCR) (69.2%). Two-third of the patients received immunoglobulins (IVIg) (77.7%). Although functions recovered in most patients, there were four patients with facial diplegia during follow-up (4.26%). Acute inflammatory demyelinating polyneuropathy (AIDP) was more likely to be associated with paresis of the lower extremity (p Conclusions GBS is being recognized as one of the many presentations of the COVID-19 infection. Although the common form is AIDP that might lead to complications, other variants are possible as well, and more studies are needed to focus on those subvariants.

Purpose The excision of retroperitoneal germ cell tumor (GCT) is a surgical challenge compounded by the absence of definite surgical guidelines. The aim of this study is to present the surgical difficulties and morbidity associated with resection of these tumors. Methods Fifteen patients (7 males and 8 females) undergoing excision of retroperitoneal GCT between February 2008 and February 2016 were evaluated. Results Except for an adolescent, the majority of patients were infants (median age: 4months). The surgical excision entailed extensive vascular dissection in all patients with associated significant blood loss in two, adjacent organ removal in five, and vessel repair in one patient. The resection was complete in all except two patients. Both the patients with incomplete resections had immature teratoma and received postoperative chemotherapy. At a median follow-up of 53months, 13 patients are alive and disease free, one patient is alive with stable disease, and one patient had died owing to respiratory complications. There were no local recurrences in the patients with complete excision. Conclusion Although the outcomes are excellent after surgery, resection of retroperitoneal GCT is a distinct surgical challenge. The surgical difficulties emanate from the need for extensive vascular dissection and risk to adjacent structures. Level IV evidence Therapeutic study.

Background It is not exceptional to come by children with initial unplanned surgical intervention for nonrhabdomyosarcoma soft tissue sarcomas (NRSTS). The aim of this study was to evaluate the presence of residual disease in these patients after re-excision and compare the treatment outcomes with patients who had planned upfront excision. Methods The data of patients with primary nonmetastatic NRSTS with initially unplanned excision who underwent re-excision between March 2006 and December 2014 were analyzed and the results compared with patients having planned upfront excision in the similar period. Results Of the 84 patients, 40 (48%) had an unplanned excision elsewhere; 35 of these patients had a re-excision. Twenty-one of the remaining 44 patients underwent upfront planned excision. A residual tumor was present in 16 (45.7%) patients. There was no significant difference in the local recurrence, distant metastases, or deaths in patients with re-excision or planned excision. The 5-year overall, disease-free survival and local control rates were, respectively, 93.5%, 90.2% and 96.6% for the re-excision group and 84.9%, 65.2% and 88.5% in the planned excision group ( p =NS). Conclusions The probability of residual disease following unplanned excision of NRSTS is high. The outcomes following re-excision are similar to that with planned excision. Prognosis study Level II evidence.

Background Nuclear protein of the testis (NUT) carcinoma is a rare and aggressive malignancy associated with rearrangements of the nuclear protein of the testis (NUT) gene on chromosome 15q14. Because of its rarity, this tumor is often underdiagnosed and underreported, and there is limited literature regarding its biology and optimal management. Methods and Results We report our experience of five patients with pediatric NUT carcinoma, all of whom presented with midline head and neck mass. In spite of aggressive multimodality treatment, only one patient survives. Conclusion NUT carcinoma has a dismal prognosis in spite of aggressive multimodality management (surgery and adjuvant chemotherapy and/or radiation). Novel strategies are required to improve outcomes of patients with this tumor.

Hepatic resection is the mainstay of treatment for hepatoblastoma. However, the presence of adequate future liver remnant (FLR) is essential to prevent postoperative liver failure. Portal vein embolization (PVE) is commonly utilized in adults for promoting hypertrophy of FLR, however, it is sparingly used in children. Secondly, bile leak after liver resections is a well-defined complication. Apart from conservative treatment such as drainage and antibiotic, several management strategies including endoscopic, percutaneous, and surgical approaches have been described for its management. We present an 18-month old child with hepatoblastoma for whom PVE was performed to enhance the FLR so that an extended right hepatectomy could be accomplished. The same patient endured delayed postoperative biliary leak wherein the conservative, and non-operative interventional procedure failed, however, surgery combined with intraoperative interventional radiology procedure was utilized with a favorable outcome.

Patient selection is of prime significance when considering intra-arterial thrombectomy (IAT) for patients with basilar artery occlusion (BAO). We present a case of BAO with the clinical locked-in syndrome and “smoggy” appearance diffusion restriction on diffusion-weighted imaging (DWI) in the pons on a hyper-acute magnetic resonance imaging (MRI) scan. A 62-year-old man was admitted with a National Institutes of Health Stroke Scale (NIHSS) of 21. An admission computed tomography angiogram (CTA) showed a mid-distal BAO. As the alteplase was being infused, the patient started declining with an examination suggesting locked-in syndrome. An emergent hyper-acute MRI showed true restricted diffusion on DWI at the entire right anterior inferior cerebellar artery territory, multiple punctate areas in the bilateral posterior inferior cerebellar artery territory, right > left hippocampus, and thalamic region mesial aspect, compatible with an acute posterior circulation infarct. He also had patchy or hazy pontine involvement with slight DWI signal changes but an impressive apparent diffusion coefficient (ADC) darkening in the bulk of pons. The patient was taken to the operating room (OR) for IAT within five hours of the witnessed stroke onset, successfully revascularized, and then discharged on the eighth day with NIHSS of 3. MRI at discharge showed a pronounced DWI restriction in the same areas involved in the hyperacute MRI done at admission. However, now the ADC hyper-intensity was less noticeable, with continued hazy and smoggy pontine signal changes on DWI. On the two-month follow-up, the patient had zero NIHSS and modified Rankin scale (mRS) scores, respectively. DWI-MRI changes in acute ischemic stroke behave differently in tract areas with a density of nerve axons, manifesting as a hazy or smoggy appearance: the “smog sign” on DWI. In hyper-acute MRI, "hazy" or "smoggy" diffusion restriction on DWI in different axonal tract areas like the pons can correlate with good functional outcomes if successful reperfusion therapy is offered.

Introduction Acute basilar artery occlusion (BAO) accounts for 1% of all ischemic strokes and is associated with high mortality and morbidity. The purpose of study was to analyze functional outcome of patients with BAO in relation to the location of occlusion, posterior circulation branches and time interval from last known well (LKW) to groin puncture. Methods This is a descriptive, retrospective study from a single center database. We reviewed patients presenting with BAO or with symptomatic high grade stenosis in basilar artery at UNMH between 2007 and 2017. Data was collected on secured Redcap™ database. The imaging data was reviewed by two independent physicians separately. Patients with no available imaging or follow up data, and no identifiable BAO or high grade stenosis on vascular imaging, were excluded. The outcome was defined as 1–3 months modified Rankin Scale (mRS). All patients were divided into three group based on their follow up mRS. Patients with mRS 0–2, patients with mRS 3–5 and patients with mRS 6. Results A total of 66 patients with stenosis or occlusion of posterior circulation presenting with acute ischemic stroke were identified. Out of 66, complete imaging review of 52 patients was completed. Out of 52, no BAO occlusion or high grade stenosis was identified in 11 patients on vascular imaging. In 8 patients, there was no follow up mRS score or baseline vascular imaging available. Thirty two patients were included in the final analysis, out of which, 10 patients were mRS 0–2 group, 6 patients were in mRS 3–5 group and 16 patient were in mRS 6 group (see Table 1). Mean age in both groups was 64.7, 51.6 and 69.2 years respectively with male predominance. Hypertension was the most common baseline comorbidity in all groups. In mRS 6 group, 37.5% had occlusion in vertebrobasilar junction. Thrombectomy was performed in 40%, 33.3% and 37.6% patients repectively. The last known well (LKW) to groin puncture time was increased in patients with mRS 6. Conclusion This retrospective analysis of BAO database depicts that older age, presence of HTN, history of prior stroke, location of clot at vertebrobasilar junction, and absence of bilateral posterior communicating arteries is associated with higher mortality. Moreover, delayed time to groin puncture was also observed in patients with higher mortality. Large scale studies are needed to validate these findings. Disclosures A. Zafar: None. S. Suriya: None. M. Farooqui: None. A. Ikram: None. S. Ortega-Guiterrez: None. M. Torbey: None.

Objectives The primary objective is to evaluate difference in outcomes after endovascular treatment (EVT) between patients directly admitted through Emergency Department (ED) and patients transferred from outside institutions. Background Over last few years, EVT has been established as the most-effective treatment for acute ischemic stroke (AIS) patients with large vessel occlusion (LVO). In New Mexico state, University of New Mexico Hospital (UNMH) serves as the only institution providing 24/7 services for EVT. In addition to transfers, patients are also admitted directly to UNMH through ED to undergo EVT. Considering the dynamics of New Mexico sate, representing various other similar states, we are conducting this study to evaluate any significant difference in the outcome of transferred patients in comparison to directly admitted patients. Methods This is an observational retrospective cohort study. Patients who underwent EVT for LVO from January 01, 2018 to December 31, 2018 at UNMH were included. Data was collected on secured Redcap™ database. Patients were categorized into direct admission (DA) vs transfer groups (TG). Patients who were not candidates for EVT were excluded. Results A total of 53 patients underwent EVT. Seventeen patients were directly admitted through ED while 36 were transferred from outside hospitals. Age, gender, ethnicity and risk factor profile are reported in table 1. The two groups had similar NIHSS and GCS on presentation. The median time of arrival of patients to UNM, from last known well, was 211 minutes in DA and 321 minutes in TG (p=0.03). Median UNM arrival to groin puncture time was 121 minutes in DA and 66 minutes in TG (p=0.04). Median procedural time for recanalization was 41 minutes for DA and 28 minutes for TG. One patient had unsuccessful thrombectomy (TICI score of 0). Although no statistical significance was noted, mean mRS at 3 months was 3 in each group. The 3-month mortality was similar in the two groups (DA=23.5% vs TG=25%). Conclusion To the best of our knowledge, this is the first study reporting EVT data reflecting experiences from an academic program of an underserved state after the expansion of EVT time-window. This study showed no statistical difference in the outcomes of patients admitted directly through ED versus patients who were transferred from outside hospitals for EVT. Larger scale studies are needed to corroborate this data. Disclosures A. Zafar: None. S. Suriya: None. M. Farooqui: None. O. Owens: None. A. Alvarado Arias: None. A. Ikram: None. D. Sorte: None. A. Carlson: None. M. Torbey: None.

Objectives The primary objective of this study is to evaluate the difference in the functional and neurological outcomes after mechanical thrombectomy (MT) for an acute ischemic stroke (AIS), between Native Americans (NA) patients versus other ethnicities. Background There is scarcity of data regarding outcome of MT in NA population. Considering the dynamics of the state of New Mexico and a diverse patient population, we evaluated the difference in outcome between NA patients with comparison group representing other ethnicities. Methods This is a preliminary, observational retrospective case-control study. All NA patients who underwent MT for LVO from January 01, 2016 to December 31, 2018 at UNMH were included. Data was extracted from Cerner database at UNMH and was collected on secured Redcap™ database. Patients were categorized into NA vs comparison group (other ethnicities) into two groups, with the ratio of 1:4 respectively. Results A total of 35 patients who underwent MT at UNMH for an AIS were included in analysis: seven NA and 28 controls. Nine patients (NA=7) were directly admitted through ED while 26 were transferred from outside hospitals (NA=6). Prevalence of vascular risk factors, including Hypertension, hyperlipidemia, Coronary artery disease, Diabetes Mellitus was noticeably higher in NA than the comparison group. There was no statistical difference in the two groups with regards to median time interval from last known well (LKW) to groin puncture, and from LKW to reperfusion. The mortality was 14% in NA group and 4% in the comparison group. The median NIHSS on discharge between NA and comparison group was 13 and 15.5, respectively. The median mRS at 6 months follow up was 1 in NA and 2 in comparison group, however this was not statistically significant. Conclusion This is the first ever descriptive study evaluating outcomes in NA patients undergoing MT. Prevalence of vascular risk factors in NA was noticeably higher compared to the comparison group. Our retrospective study showed that Native American patients similar functional and neurological outcomes for thrombectomy. Disclosures A. Ikram: None. S. Suriya: None. M. Farooqui: None. O. Owens: None. A. Alvarado Arias: None. D. Sorte: None. A. Carlson: None. S. Ortega-Guiterrez: None. M. Torbey: None. A. Zafar: None.

Background The availability of robust, equivalent data regarding outcomes for upfront or delayed surgery for renal tumors in children leads to a dilemma in selecting the initial treatment. Imaging criteria associated with the probability of rupture or incomplete resection may provide a more objective assessment for customization for the timing of surgery. Procedure Eighty-three children with unilateral, nonmetastatic renal tumors were enrolled between January 2012 and April 2018. Upfront nephrectomy was performed in the absence or delayed surgery (after a biopsy and chemotherapy) in the presence of one or more imaging-based high-risk features, including perinephric spread or adjacent organ infiltration, tumors crossing the midline, intravascular thrombus, and extensive adenopathy. Post hoc analysis for interobserver concordance for high-risk imaging features was also performed. Results The upfront surgery group (19) had predominantly stage I or II diseases (89%) and the histological types were Wilms (13), non-Wilms (5) renal tumor, and an inflammatory lesion. The delayed surgery group had 60% with stage I or II diseases and the histological types were Wilms (60) and non-Wilms (4) tumor. In addition, high-risk pathology was identified in nine patients. Overall, 27 patients with Wilms tumors required radiotherapy and anthracycline because of stage III disease, including one in the immediate surgery group. The event-free and overall survival (OS) at a median follow-up of 39 months for Wilms tumor are 88% (95% confidence interval [CI]: 78.5-94.9%) and 89% (95% CI: 81.4-96.6%), 85.1% (95% CI: 73.8-93.4%) and 86.5% (95% CI: 77.4-95.8%) for the delayed, and 100% event-free survival as well as OS (P = .1) in the upfront surgery group. Conclusion A customized approach pivoted on image-based high-risk features facilitates identification of patients with early-stage renal tumor when the timing of surgery is tailored. Moreover, non-Wilms tumor and high-risk pathology are also identified.

Introduction: Hereditary Hemorrhagic Telangiectasia (HHT) is a genetic disorder with vascular anomalies of various organ systems including the skin, brain, heart, lungs, and liver. While the most commonly recognized disease burden of HHT are epistaxis and mucocutaneous telangiectasia, the development of vascular malformations can result in more severe symptoms such as cerebral hemorrhage, liver cirrhosis, and stroke. Various case reports/series have reported high output cardiac failure, secondary to presence of a single large AVM or multi organ AVMs, as a major cause of morbidity and mortality in HHT population. To the best of our knowledge, this is the first study analyzing the cardiovascular disease burden in HHT patients from a large national database. Methodology: All HHT patients were identified using ICD-9 code 448.0 using the 2014 National Inpatient Sample (NIS) database. Comorbidities - intracerebral hemorrhage (431), cerebrovascular anomaly (747.81), congestive heart failure (428.0), anemia (285.9) were also identified using the ICD-9 coding system. Descriptive and inferential statistical analysis were performed using SAS 2015. Results: A total of 842 HHT patients were identified with relatively older patients having increased prevalence of anemia (29% vs 15%), congestive heart failure (16% vs 8%), intra-cerebral hemorrhage (0.5% vs 0.3%), hypertension (47% vs 42%) compared to general population, respectively. We report HHT to be linked with having higher odds (p < 0.05) of congestive heart failure (1.6 times), chronic pulmonary lung disease (1.7 times), liver disease (3 times), anemia (1.6 times), cerebrovascular anomaly including cerebral AVMs (26 times), and other neurological disorders than patients without HHT, after adjusting for confounders. Conclusion: This is the first database study confirming higher prevalence of CHF in HHT. We infer that chronic anemia and AVM/AVF can cumulatively cause high output congestive heart failure causing increased morbidity and mortality in HHT population especially in advanced ages.

This chapter discusses neurological aspects of spinal cord tumors, including clinical symptoms and syndromes, examination techniques, and diagnostic modalities. An emphasis is placed on earlier disease diagnosis as spinal cord tumors are known for delayed diagnoses. Usual causes for this delay consist of slow progression, an incomplete physical examination or misinterpretation of exam findings, late pursuit of imaging, imaging at the incorrect level, or nonspecific symptoms—such as back pain—that is misconstrued as the more prevalent degenerative spine disease. Every physician should maintain an elevated level of suspicion of spinal cord tumors and take the appropriate steps toward diagnosis, until neoplastic disease is definitively ruled out.

Summary Background Studies describing intravascular involvement in Wilms tumor have focused on illustrating individual institutional experience and the elements of surgical management. Thrombus characteristics like extent, patterns of regression, and correlation with the surgical findings, intraluminal adhesion, and viable tumor in the thrombus, and patency of the inferior vena cava (IVC) have not been systematically described. Objectives The aim of this study is to evaluate these thrombus characteristics and explore their impact on the overall outcomes. Methods All patients with histologically confirmed Wilms tumors with intravascular thrombus diagnosed in the pediatric oncology unit of Tata Memorial Hospital registered from 2006 to 2019 were included. Data regarding clinical, radiological, and surgical particulars were retrieved from the prospectively maintained institutional database. Specific data for the thrombus included: distal extent before and after neoadjuvant chemotherapy, correlation of extent with the surgical findings, completeness of thrombectomy, the presence of a viable tumor in the thrombus, and the patency of the IVC. Survival analysis was performed utilizing the Kaplan–Meier method on SPSS software version 25. Results The study included 43 (9.9%) of the 432 patients with Wilms tumor having intravascular extension. Retrohepatic IVC (33.3%) followed by atrioventricular (26%) formed the frequent levels of thrombus with maximum regression occurring after chemotherapy in the latter (Summary figure). The overall concordance rate between computed tomography (CT) scan and surgical findings for the presence of thrombus was 86% and 4 patients had the thrombus limited to a lower level than the preoperative scan. At a median follow-up of 5-years, the 5-year event-free and overall survival was 81% and 82.2% respectively. Atrioventricular thrombus (p = 0.003) and postoperative patency of IVC (p = 0.02) were significantly associated with inferior survival, while the extent of regression, thrombus fracture, and viability was not significant. Discussion The findings of this study bring forth the characteristics of intravascular tumor thrombus affecting the outcomes which can be validated in future prospective studies. Although the ideal method for radiological assessment of the intravascular thrombus is elusive, CT scan provided adequate information for the presence and level of the intravascular thrombus with reasonable accuracy in this study. Study limitations include small sample size, the limited number of events, and lack of multivariate analysis to rule out confounding factors that could influence the observed findings. Conclusion Atrioventricular thrombus and occlusion of IVC represent adverse prognostic factors. The extent of regression, fracture, and viability of thrombus did not affect survival in this study. Download : Download high-res image (541KB) Download : Download full-size image Summary fig. . A collage of axial, coronal and sagittal computed tomogram images showing retrohepatic [A and A1], atrial [B and B1], ventricular [C and C1] thrombus.

Objective To study the central nervous system (CNS) complications in patients with COVID-19 infection especially among Native American population in the current pandemic of severe acute respiratory syndrome virus (COVID-19). Methods Patients with confirmed COVID-19 infection at University of New Mexico hospital (UNMH) were screened for development of neurological complications during Feb 01 to April 29, 2020 via retrospective chart review. Results Total of 90 hospitalized patients were screened. Out of seven patients, majority were Native Americans females, and developed neurological complications including subarachnoid hemorrhage (SAH), Intraparenchymal hemorrhage (IPH), Ischemic stroke (IS) and seizure. All 7 patients required Intensive care unit (ICU) level of care. Patients who developed CNS complications other than seizure were females in the younger age group (4 patients, 38-58 years) with poor outcome. Out of 7, three developed subarachnoid hemorrhage, two developed ischemic infarction, and four developed seizure. Two patients with hemorrhagic complication expired during the course of hospitalization. All three patients with seizure were discharged to home. Conclusion Patients with serious CNS complications secondary to COVID-19 infection were observed to be Native Americans. Patients who developed hemorrhagic or ischemic events were observed to have poor outcomes as compared to patients who developed seizures.

Summary Introduction Non-Wilms renal tumors represent a compelling subset of childhood renal tumors. However, their relative rarity renders accurate diagnosis, and therapy challenging which in some instance is inferred from their adult counterparts. Objective To describe the incidence and analyze the diagnostic challenges, therapies and, outcomes of non-Wilms renal tumors at the largest tertiary cancer centre in India. Methods All patients with histologically confirmed non-Wilms renal tumours diagnosed in the paediatric oncology unit of Tata Memorial Hospital between 2006 and 2019 were included. Data regarding clinical and radiological features and treatment outcomes were retrieved from the prospectively maintained institutional database. At the outset, histological types were categorised into a high and low-risk group depending on anticipated survival. Survival analysis was performed utilising the Kaplan–Meier method on SPSS software version 24.0. Results Of the 569 patients with renal tumors, 109 (19%) patients with primary (n = 97) or recurrent (n = 12) non-Wilms renal tumors were included. Histological high-risk group included clear cell sarcoma (CCSK) (39.4%), renal cell carcinoma (RCC) (19.3%), malignant rhabdoid tumor (MRTK) (12.8%), Ewing's sarcoma (rES) (15.6%), synovial sarcoma (2%), and undifferentiated sarcoma (2%). The low-risk group comprised of congenital mesoblastic nephroma (CMN) (4.6%), cystic partially differentiated nephroblastoma (2%), and other rare tumors (3%). Diagnostic error occurred in 2 patients in the high-risk group. All low-risk tumours were treated with surgery alone and most (97%) high-risk tumors were operated either upfront (61.5%) or after preoperative chemotherapy (38.4%). Adjuvant therapy based on histology was offered to 70%. The recurrent tumors received various salvage treatments including chemotherapy; radiotherapy; surgery and immunotherapy, however, only 2 patients could be salvaged. The 3-year overall survival for the entire cohort with primary tumors was 59%, and the survival rates were 76.7%, 77.9%, 0.0%, and 52% for CCSK, RCC, MRTK, and rES (summary figure). Low-risk tumors had 100% survival while the recurrent tumors had a median survival of 10.5 months. Conclusions Non-Wilms renal tumors constitute a heterogeneous group of tumors, accounting for less than 20% of all renal tumors. Low-risk tumors are associated with excellent outcomes following surgery alone while the high-risk tumours have a variable outcome. MRTK and recurrent non-Wilms tumour have the worst survival. Favourable outcomes for CCSK and RCC and worst outcomes for MRTK were observed in this study. Renal ES have higher incidence of treatment failure and unsatisfactory outcomes. Recurrent non-Wilms tumours have an extremely poor outcome and more alternative or innovative approaches are needed for their treatment.

Extramedullary hematopoiesis is production of blood cells outside bone marrow which occurs in a variety of disorders including myelofibrosis. We present a 15 month old child with stage 4 neuroblastoma with secondary myelofibrosis which resulted in intracranial extramedullary hematopoiesis, posing a diagnostic and therapeutic dilemma. This was resolved with a sulfur colloid isotope scan which confirmed extramedullary hematopoiesis and not a progressing metastasis. This helped us continue therapy with curative intent. To the best of our knowledge we did not come across any report of neuroblastoma with myelofibrosis and intracranial extramedullary hematopoiesis mimicking progression of metastasis.

OBJECTIVESeveral retrospective studies have supported the use of conscious sedation (CS) over general anesthesia (GA) as the preferred methods of sedation for stroke thrombectomy, but a recent randomized controlled trial showed no difference in outcomes after CS or GA. The purpose of the Ideal Sedation for Stroke Thrombectomy (ISST) study was to evaluate the difference in time and outcomes in the reperfusion of anterior circulation in ischemic stroke using GA and monitored anesthesia care (MAC).METHODSThe ISST study was a prospective, open-label registry. A total of 40 patients who underwent mechanical thrombectomy for anterior circulation ischemic stroke were enrolled. Informed consent was obtained from each patient before enrollment. The primary endpoint included the interval between the patient’s arrival to the interventional radiology room and reperfusion time. Secondary endpoints were evaluated to estimate the effects on the outcome of patients between the 2 sedation methods.RESULTSOf the 40 patients, 32 received thrombectomy under MAC and 8 patients under GA. The male-to-female ratio was 18:14 in the MAC group and 4:4 in the GA group. The mean time from interventional radiology room arrival to reperfusion in the GA group was 2 times higher than that in the MAC group. Complete reperfusion (TICI grade 3) was achieved in more than 50% of patients in both groups. The mean modified Rankin Scale score at 3 months was < 2 in the MAC group and > 3 in the GA group (p = 0.021).CONCLUSIONSThe findings from the pilot study showed a significantly shorter time interval between IR arrival and reperfusion and better outcomes in patients undergoing reperfusion for ischemic stroke in the anterior circulation using MAC compared with GA.Clinical trial registration no.: NCT03036631 (clinicaltrials.gov)

Background: The outcome of endovascular treatment for emergent large vessel occlusion (ELVO) is dependent on timely recanalization. To identify ELVO in the field, we present a simplified score, which has been applied and validated in the field by emergency medical services (EMS). Methods and Analysis: Ventura ELVO Scale (VES) comprise of 4 components: Eye Deviation, Aphasia, Neglect, and Obtundation with score range 0-4. The score of greater than or equal to 1 will be considered as ELVO positive. A positive VES along with positive Cincinnati scale prompts ELVO activation. EMS then notify to neurointervention protocol at the receiving stroke center. The performance of VES was evaluated retrospectively. For statistical analysis, SAS version 9.4 was used and Fisher's modelling was used for the comparative analysis. Results: Total 184 patients were included in the final analysis, 62 (33.7%) patients were called VES positive from the field. Out of 62, 36 (58%) patients had ELVO. The mean NIHSS on arrival was 16 in VES positive and 5 in VES negative patients. VES was 94.7% sensitive and 82.4% specific while the PPV and NPV of VES were 58.1% and 98.4%, respectively. It showed 84.9% accuracy. Conclusions: VES is an effective and simplified prehospital screening tool for detection of ELVO in the field. Its implementation can beat the target door to groin time to improve outcomes and in future it can be used for rerouting of ELVO patients to comprehensive stroke center.

INTRODUCTION: The Pediatric Hematolymphoid Disease Management Group (PHL-DMG) at a tertiary cancer care hospital developed extensive patient support programs to improve retention and outcomes while focusing on protocols adapted to meet patient needs. An audit of measures and outcomes was done for a 7-year period from January 2010 to December 2016. MATERIALS AND METHODS: DMG protocols and patient support activities over the study period were documented and audited. Data was retrieved from internal databases and records. Measures taken and their impact were assessed by descriptive analytical tools. Survival outcomes were calculated using Kaplan–Meier method on SPSS v. 24™ software. RESULTS: Holistic patient support measures were undertaken through a charitable foundation entirely under pediatric oncology. Activities included infrastructure growth, socioeconomic support, provision of accommodation, nutrition, education, and multiple blood component donation drives. Patient registrations increased from 502 in 2009 to 874 in 2016, with the steepest rise in acute lymphoblastic leukemia (ALL) – 330 (2009) to 547 (2016). Treatment refusal and abandonment rates decreased from 32% to 3.4% over the same period, and male to female ratio decreased from 2.56 to 2.28:1. Early mortality in acute myeloid leukemia (AML) fell within 2 years from 26.7% in 2009 to 7%. Five-year overall survival (OS) was 69.5% for all patients registered in 2010, whereas disease-specific 5-year OS was ALL 67.1%, AML 49.3%, chronic myeloid leukemia 100%, Hodgkin lymphoma 90.4%, and non-Hodgkin lymphoma 74.2%. CONCLUSIONS: Holistic patient support-specific activities and adapted protocols made a measurable impact on patient outcomes. High survival outcomes of patients have been achieved despite relatively few receiving salvage therapies or stem cell transplant.

Introduction/purpose Mechanical thrombectomy was shown superior to intravenous tissue-plasminogen activator (IV t-PA) in recently published clinical trials. The anecdotal data supports the use of conscious sedation over general anesthesia, as the preferred mode of sedation but no randomized trial exist to show the best practice. Therefore, the procedure is performed with wide spectrum of sedation practices with general anesthesia being the most common practice. The ideal sedation for stroke thrombectomy (ISST) registry will evaluate the feasibility of two sedation methods and delay in recanalization using general anesthesia. Methods ISST is a prospective open-label consecutive enrollment registry. It was approved by Western Institutional Review Board (WIRB). The study compares the feasibility and time to recanalize using the two techniques of sedation. Being a pilot study, total 40 patients who received mechanical thrombectomy for anterior circulation ischemic stroke are enrolled. Total 30 patients have completed the study and therefore their results are being presented. Informed consent was obtained from each patient before enrollment. Primary endpoint includes time from arrival to interventional radiology (IR) room to recanalization time (RRT). Secondary endpoints are evaluated to estimate the effects on the outcome of patients between the two groups. Results Total of 23 patients received thrombectomy under monitored anesthesia care (MAC)/conscious sedation arm referred as group 1 and 7 patients under general anesthesia arm referred as group 2. Male to female ratio was 14:9 (mean age: 73) in group 1 while 4:3 (mean age: 69) in group 2. The median duration RRT was 59 min in group 1 while it was 112 in group 2 (p-value 0.03). Good reperfusion (TICI ≥2 b) was achieved in all patients (100%) in group 1 and group 2 with TICI score of grade 2b in 10 patients (43.5%) and grade 3 in 13 patients (56.5%). While in group 2, grade 2b was achieved in 4 patients (57.1%) and grade 3 in 3 patients (42.9%). Median mRS score at 3 months was 1 in group 1 and 4 in group 2 (p-value 0.01). Two patients from each group developed asymptomatic SAH or ICH with Odds ratio 3.2 (95% CI: 0.38–27.78). 1 patient in each group developed aspiration pneumonia with Odds ratio 3.2 (95% CI: 0.18–59.60). Total 3 patients died in group 1 and 1 patient died of respiratory failure in group 2. Conclusion Early findings from our pilot study showed statistically significant delay in time to recanalization with general anesthesia and better outcome in patients who underwent mechanical thrombectomy under MAC/conscious sedation. Disclosure A financial research grant was provided by Stryker Neurovascular to support this study. Disclosures M. Taqi: 1; C; Stryker Neurovascular. 2; C; Stryker Neurovascular. A. Sodhi: None. S. Suriya: 1; C; Stryker Neurovascular. F. Adl: None. M. Farooqi: None. S. Quadri: None. M. Mortazavi: 3; C; Haag-Streit Surgical, DePuy Synthes, American Surgical Company.