Primary Oropharyngeal SMARCA4-Deficient Carcinoma: Expanding the Diagnostic Spectrum in Head and Neck Cancer.

With the advent of molecular immunohistochemistry and next generation sequencing, Switch/sucrose non-fermentable (SWI/SNF) chromatin remodeling complex altered tumors have gained recognition recently. SWI/SNF related, matrix associated, actin dependent regulator of chromatin subfamily B member 1 (SMARCB1) and SMARCA4 are the primary SWI/SNF components altered in several recently described undifferentiated malignancies in head and neck region with predilection for paranasal sinuses in SMARCB1-deficient tumors and nasal cavity in SMARCA4-deficient tumors. However, to the best of our knowledge, SMARCA4-deficient tumors of the oropharynx have not been described. We present an unusual case of SMARCA4-deficient carcinoma of the oropharynx (palatine tonsil) which is the first case in the literature, expanding the topographic distribution of SMARCA4-deficient tumors in the head and neck region and emphasizing the importance of BRG1 as an essential immunohistochemical marker for the diagnosis of this distinct entity.

Posterior keratometry changes after steep axis phacoemulsification: a prospective study.

Purpose: To measure changes in posterior corneal curvature after steep axis phacoemulsification and investigate the possibility of its effect on SIA. Methods: This was a prospective longitudinal study on 60 consecutive eyes of age-related cataract with regular astigmatism and absence of co-morbidities undergoing uneventful cataract surgeries with main incision at steep meridian. Preoperative and 4 weeks postoperative measurements of anterior and posterior corneal curvatures using Scheimpflug based corneal topographer were performed. Posterior corneal curvature was assessed at 3, 5- and 7-mm diameters. Results: The study found a statistically significant change in posterior corneal K1, K2 and mean astigmatism in all zones (3, 5 and 7 mm) at 4 weeks postoperative, when compared to preoperative readings. Conclusion: Posterior keratometry is likely to be an important determinant of Surgically Induced Astigmatism (SIA) and should be factored in for refractive cataract surgery.

Domino liver transplantation for maple syrup urine disease in children: A single-center case series.

BACKGROUND: Domino liver transplant (DLT) represents another type of liver donor to expand the donor pool. Recent reports of successful DLT in children with maple syrup urine disease (MSUD) show promising long-term outcomes. METHODS: It was a retrospective study. All children with MSUD were paired with either recipients with end-stage liver disease (ESLD) or non-MSUD metabolic disease. Each pair underwent simultaneous liver transplant (LT), where the MSUD recipient received the graft from a living-related donor and the liver explanted from the MSUD donor was transplanted to the respective paired domino recipient. We report our experience regarding the techniques and outcomes of DLT at our center. RESULTS: Eleven children with MSUD and 12 respective DLT recipients were enrolled, one of which was domino split-liver transplantation. DLT recipients included seven ESLD, two propionic acidemia (PA), one glycogen storage disease(GSD) type-1, one GSD type-3, and one Citrullinemia. Post-LT ICU and hospital stays were comparable (p > .05). Patient and graft survival was 100% and 66.6% in the MSUD group and DLT recipients at a mean follow-up of 13.5 and 15 months. There was no death in the MSUD group as compared to four in the DLT group. The amino acid levels rapidly normalized after the LT in the children with MSUD and they tolerated the normal unrestricted diet. No vascular, biliary, or graft-related complications were seen in the post-transplant period. No occurrence of MSUD was noted in DLT recipients. CONCLUSION: DLTs have excellent post-surgical outcomes. DLT should be strongly considered and adopted by transplant programs worldwide to circumvent organ shortage.

Effectiveness of Head-and-Neck Molecular Imaging Reporting and Data System Criterion in Head-and-Neck Squamous Cell Carcinoma PostConcurrent Chemoradiotherapy.

Purpose: Postconcurrent chemoradiotherapy (CRT) response assessment has been challenging in locally advanced head-and-neck squamous cell carcinoma (LA-HNSCC) due to prevailing postradiation changes. Molecular response methods have been encouraging, although further clarifications and validations were needed. We tested the effectiveness of a proposed semi-quantitative molecular response criterion in these patients. Materials and Methods: Two subspecialty-trained physicians evaluated 18F-fluorodeoxyglucose positron emission tomography/computed tomography of LA-HNSCC patients (n = 83) post 3 months CRT using a five points Head and Neck Molecular Imaging-Reporting and Data System (HAN-MI-RADS) criterion. Where available, histopathology examination with clinical and imaging interpretation was taken as a reference for the disease. A diagnostic accuracy comparison was done with the existing Hopkins score. Further effectiveness was analyzed with disease-free survival (DFI) and overall survival (OS). Results: Metastasis was developed in 11/83 patients at 3 months of evaluation. Of 72 patients, 39, 2, and 31 patients had a complete response, equivocal response, and partial response as per HAN-MI-RADS. Per patient sensitivity, specificity, positive predictive value, negative predictive value, and accuracy for predicting loco-regional disease up to 1 and 2 years was 93.3%, 92.5%, 90.3%, 94.9%, 92.9%, and 84.9%, 91.9%, 90.3%, 87.2%, and 88.6% respectively. One year and two years DFI for each HAN-MI-RADS score showed a statistically significant difference while it was not for OS. The receiver operating characteristic curve analysis showed significantly better outcome predictability of HAN-MI-RADS (area under the curve [AUC] 0.884) than Hopkins (AUC 0.699). Conclusions: A five points HAN-MI-RADS criterion was found promising for response assessment with less equivocal results and statistically significant higher AUC than Hopkins for loco-regional recurrence prediction.

Safety and feasibility of immediate tracheal extubation of small pediatric patients after living donor liver transplantation.

BACKGROUND: Immediate extubation is integral constituent of enhance recovery protocols. Purpose of this study was to examine success rates and safety of protocolized immediate extubation in pediatric living donor liver transplant recipients and to find out factors associated with non-immediate extubation in operation room. METHODS: We performed retrospective analysis for data of small (≤20 kg) pediatric patients transplanted between 2017 and 2019 (protocolized duration) and compared with data of transplants done between 2014 and 2016 (non-protocolized duration). Further, we compared data during each time duration between immediate extubation and non-immediate extubation group to find risk factors in that particular duration. RESULTS: Immediate extubation rates were significantly higher during protocolized duration compared with non-protocolized duration (85.52% vs. 48.29%, p < .001). Reintubation rates decreased during protocolized duration (10.9% vs. 4.6%). Hospital stays (20.47 ± 7.06 vs. 27.8 ± 6.2 days, p < .001) and mortality (13.2% vs. 28%, p = .04) were significantly decreased in protocolized duration. Higher age (OR: 2.85, 95% CI 1.22-6.67, p = .02), weight > 10 (OR: 4.37, 95% CI 1.16-16.46, p = .029) and high vasopressor support (OR: 32, 95% CI 6.4-160.13, p < .001) found as significant predictors of non-immediate extubation however only high vasopressor support found to be independent predictor during protocolized duration. CONCLUSIONS: Outcomes in pediatric transplants can be optimized by immediate extubation in majority of cases when protocolized as part of policy.

Neurofibromatosis 1: A family case series.

Neurofibromatosis type 1 (NF1) or Von Recklinghausen disease comes under a group of multisystem hereditary syndromes called phakomatoses. It presents with skin, ophthalmic, bony, and systemic manifestations. We present a photographically well-documented case series of NF in a family (n = 3). Skin manifestations were present in all the patients. The ophthalmic manifestations were Lisch nodules (100% of eyes), subcutaneous neurofibroma of eyelids (33% of eyes), mechanical ptosis (33% of eyes), and mechanical ectropion (16.5% of eyes). We report the rare occurrence of multiple solitary neurofibromas causing mechanical ptosis and mechanical ectropion.

Molecular Response Assessment with Immune Adaptive PERCIST in Lung Cancer Patients Treated with Nivolumab: Is It Better Than iRECIST?

Aims We compared the immune response evaluation criteria in solid tumors (iRECIST) with immune adaptive positron emission tomography response criteria in solid tumors (imPERCIST) in lung cancer patients treated with nivolumab. Materials and Methods Twenty lung cancer patients underwent fluorodeoxyglucose positron emission tomography/computed tomography (FDG PET/CT) scan at baseline (PET-0), after four cycles (PET-1) and six to eight cycles (PET-2) of nivolumab were included. Kappa coefficient ( k ) was derived to see the level of agreement in two response criteria. Progression-free survival (PFS) curves were computed by the Kaplan-Meier method and compared with the Log Rank test. Univariate and multivariate regression for the percentage change in the sum of diameters (SoD), standard uptake value maximum (SUVmax), sum of metabolic tumor volume (SoMTV), and sum of total lesion glycolysis (SoTLG) was computed. A p -value less than 0.05 was considered significant. Results Kappa coefficient showed a substantial level of agreement (k 0.769) in two response criteria. Mean PFS in partial response, stable disease, and progressive disease (PD) patients in iRECIST and imPERCIST was 27.3, 17.7, 4.2, and 23.3, 18.8, 3.8 months, respectively. The Kaplan-Meier method with the log rank test showed a significant difference in PFS on intracomparison within both criteria; however, it was not significant on intercomparison. On univariate analysis, the percentage change in SoD, SoMTV, SoTLG was significant. However, on multivariate analysis, only percentage change in SoD was a significant predictor. Conclusions We concluded that imPERCIST was equally effective as currently recommended criteria iRECIST for response evaluation of nivolumab in lung cancer patients.

Adamantinoma-like Ewing Sarcoma of the Nasal Cavity: A Rare Clinical Presentation with Deceptive Histopathologic Features.

Ewing sarcoma (ES) is a malignant round cell tumour (MRCT) that usually involves bone and soft tissue of young and paediatric populations. ES of the head and neck region is uncommon. Adamantinoma-like Ewing sarcoma (ALES) is a rare variant of ES that shows complex epithelial differentiation on histopathology and immunohistochemistry (IHC). It demonstrates a t(11;22) translocation and EWSR1- FLI1 fusion. Most documented cases of ALES of the head and neck region were initially misdiagnosed as epithelial tumours. We present a rare case of ALES of the nasal cavity in a young female. The patient subsequently underwent chemotherapy and showed an excellent response. Awareness of this entity is important for pathologists and oncologists due to its distinct therapeutic and prognostic implications. We propose performing upfront NKX2.2 and CD99 IHC studies, as well as other lineage specific IHC markers, in any poorly differentiated MRCT of head and neck region.

Rare Case of Spontaneous Pneumorrhachis in a Young Male with COVID-19.

Here we present a rare case of spontaneous pneumomediastinum complicated with pneumorrhachis (PR) in a young man who is a known case of carcinoma rectosigmoid region. Our young male was diagnosed with novel coronavirus disease 2019 (COVID-19) and remained asymptomatic for any respiratory complaints. Though an association of pneumomediastinum has been reported in COVID 19 patients, to the best of our knowledge, this is the first report of PR in a COVID-19 oncological case. The role of a radiologist is to identify this condition as cases reported earlier may have serious neurological consequences. Pursuing the cause can be a diagnostic challenge but it reaps the clinical benefit in the appropriate management.

Multicentric real world evidence with palbociclib in hormone positive HER2 negative metastatic breast cancer in Indian population.

The combination of cyclin dependent kinase 4/6 inhibitors with endocrine therapy is the standard therapy in hormone receptor positive HER-2 negative metastatic breast cancer (HR+/HER2- MBC). Several randomized trials have shown the benefits of this combination, however, real world evidence in the Indian patients is warranted. The present study reports the largest real world multicentric data from Indian population on the use of Palbociclib in HR+/HER2- MBC. A multicentric study on the HR+/HER2- MBC patients who received palbociclib with hormonal agent (Aromatase inhibitors/Fulvestrant) between February 2017 and May 2020 was conducted. Clinical and demographic information and survival data was retrieved from the Hospital medical records. Among a total of 188 patients, 57% patients were premenopausal and 17% patients had bone only disease. Altogether, 115 (61%) patients received palbociclib with Aromatase inhibitors in the first line whereas 73 (39%) patients received it in the second line with Fulvestrant. The median follow up period with advanced disease was 13 months. The median progression free survival in the first line and second line was 20.2 months and 12 months, respectively (p-value < 0.0001). The objective response rate was 80% and 47.9% in first and second lines, respectively. Dose interruptions/ discontinuation were done in 14.9% and 2.7% patients in the first and second lines, respectively. In terms of toxicity, 10% patients had grade 3-4 adverse events. The present real world data of the use of palbociclib in Indian population suggests similar effectiveness to previously published real world evidences and has been adapted as the standard of care in the first and second line treatment of HR+/HER2- MBC.

Should fluorodeoxyglucose positron emission tomography/computed tomography be the first-line imaging investigation for restaging the laryngeal carcinoma patients?

Posttreatment detection of residual/recurrence disease in the head and neck cancers is not an easy task. Treatment induces changes create difficulties in diagnosis on conventional imaging (computed tomography [CT], magnetic resonance imaging) as well as macroscopic inspection (direct laryngoscopy). Hence, we evaluate the diagnostic performance of contract-enhanced F-18 fluorodeoxyglucose positron emission tomography (FDG PET)/CT in restaging of laryngeal carcinoma Postchemotherapy-surgery and/or radiation therapy. We retrospectively analyzed patients of carcinoma larynx (n = 100) who has completed treatment and were referred for FDG PET/CT. Two reviewers performed image analysis to determine recurrence at primary site and/lymph nodes and distant metastases. Receiver operating characteristic (ROC) was used to determine the maximum standardized uptake value (SUVmax) cut off for disease detection. Histopathological examination and clinical or imaging follow-up were taken as gold standard for recurrence. One hundred laryngeal carcinoma patients with mean age of 57.2 years (range of 40-76) were included in the present study. Among the 100 patients, 96 were male and remaining 4 were female. The average interval between completion of treatment and FDG PET/CT scan was 8.5 months (minimum 6 months). Of the 100 patients, FDG PET/CT detected FDG avid lesions in 66 patients. Sensitivity, specificity, positive predictive value, negative predictive value, and diagnostic accuracy of FDG PET/CT for residual/recurrence disease detection was 90.3%, 73.7%, 84.8%, 82.3%, and 84.0%, respectively (P < 0.05). In addition, in 10 patients, metachronous primaries were detected (lung-4, thyroid-2, tongue, colon, esophagus, and lymphoma-one each). On ROC curve analysis, SUVmax >6.1 had sensitivity and specificity of 80.6% and 94.7% respectively for detection of recurrent/metastatic disease. FDG PET/CT demonstrates high diagnostic accuracy for detection of residual/recurrent disease in treated laryngeal cancer patients and our findings suggest that this imaging modality should be the first-line diagnostic investigation in this cohort of patients.

Unilateral hypopyon associated acute syphilitic posterior placoid chorioretinitis: Unusual presentation leading to HIV diagnosis.

Syphilis has a wide variety of ocular presentations such as anterior or posterior uveitis, chorioretinitis, retinal vasculitis, retinitis, perineuritis, papillitis, retrobulbar neuritis, optic atrophy and optic nerve gumma. Therefore, it is recommended to test every patient with ocular inflammation for syphilis. It is, however, a relatively rare cause of uveitis in HIV disease. A few studies suggested that HIV contributes to the ocular manifestations of syphilis and HIV co-infection in approximately 30%. Acute syphilitic posterior placoid chorioretinopathy is a rare ocular manifestation in immune-competent patients characterised by the development of a deposit in the outer retina. We describe an unusual such presentation with hypopyon.

Living Donor Liver Transplant in patients with Hepatorenal Syndrome without the use of Intraoperative Renal Replacement Therapy, a single-center experience.

BACKGROUND: Continuous Renal Replacement Therapy (CRRT) is often used to support the intraoperative course during liver transplantation (LT) for patients with HRS. However, the use of intraoperative CRRT (IOCRRT) is not without its problems. Living donor liver transplantation (LDLT) is a planned operation and is possible without IOCRRT as the recipient can be optimized. AIM: To study the peritransplant outcomes of patients with CLD and HRS undergoing LT without IOCRRT. METHODS: Analysis of LT program database for perioperative outcomes in patients with HRS from Feb 2017 to Dec 2018. RESULTS: 87/363 (23.9%) adult LDLT patients had HRS, of whom 31 (35.6%) did not respond (NR) to standard medical therapy (SMT) prior to LT. Modified perioperative protocol enabled the NR patients (who were sicker and in persistent renal failure) to undergo LT without IOCRRT. Postoperative renal dysfunction was similar (2 in NR and 2 in R) at 1 year. Post-LT survival was also not different at one month (83.87% in NR and 87.5% in R [p = .640]) and at 1 year (77% in NR vs 80.4% in non-responders [p = .709]). CONCLUSION: IOCRRT can be avoided in HRS patients undergoing LDLT without compromising their outcomes (post-LT survival and RD), even in patients who have not responded to SMT, preoperatively.

Is F-18 FDG PET/computed tomography a useful tool to restage the patients with tongue carcinoma?

OBJECTIVE: The aim of this study was to evaluate the diagnostic performance of contrast-enhanced F-18 fluoro-2-deoxyglucose (F-18 FDG) PET/computed tomography (CT) in restaging of tongue carcinoma. MATERIAL AND METHODS: Retrospectively analysis of carcinoma tongue patients (n = 110) who were treated and referred for FDG PET/CT. Histopathological examination and clinical or imaging follow-up were taken as gold standard. RESULTS: Of 110 patients, FDG PET/CT detected FDG avid lesions in 69 (62.7%) patients. Sensitivity, specificity and diagnostic accuracy of FDG PET/CT were 92.9%, 90.0% and 91.8%, respectively, for residual/recurrence disease detection (P < 0.05). Additionally, in six patients, metachronous primaries were also detected [lung (4), esophagus and lymphoma (1) each]. The mean maximum standardized uptake value (SUVmax) with 95% confidence interval for primary site recurrence, regional lymph nodes and metastatic lesions was 11.51 (9.53-13.48), 69 (62.7%) (7.88-11.48) and 8.94 (3.11-14.76), respectively. CONCLUSION: FDG PET/CT demonstrates high diagnostic accuracy for detection of residual/recurrent disease in treated tongue cancer patients and PET/CT should be considered as first-line diagnostic investigation in these patients.

Estimating technical efficiencies of Indian IT companies for setting improvement targets for inefficient companies: An empirical analysis with workers' effort as key input.

BACKGROUND: The Information Technology (IT) industry of India has proved its capabilities in delivering both on- and off-shore services to clients globally over the years. However, the technological advances and innovations taking place at the global level not only present a whole new range of growth prospects, but also challenges for this highly competitive industry. Moreover, the IT sector of India also witnessed the economic recession in 2008, which had an adverse impact on the prospects of this industry. In this scenario, it is imperative for Indian IT companies not only to maintain their focus on increasing their technical efficiencies, but also to deal with the increased competition emanating from the Asia Pacific region. OBJECTIVE: This study aimed to estimate the relative efficiency of the top 18 selected Indian IT software service companies in order to determine benchmarks, output slacks and target settings. METHODS: Data envelopment analysis has been used for achieving the stated objective. RESULTS: The paper found mixed trends in efficiency. The top five IT companies exhibited higher efficiency as compared to the rest of the selected IT companies. Tata Consultancy Services, HCL Technologies Ltd. and Tech Mahindra Ltd. are more efficient while Infosys Ltd. and Mphasis have lower efficiency. CONCLUSION: The inefficient companies have to increase their workers' productivity to become more efficient, and have to catch-up and follow the best practices of the benchmark company HCL.

Correlation between thromboelastography and rotational thromboelastometry values in adult liver transplant recipients.

BACKGROUND AND AIMS: Viscoelastic haemostatic assays (VHA) namely Thromboelastogram (TEG) and Rotational thromboelastometry (ROTEM) are used for global assessment of coagulopathy and guiding transfusion during living donor liver transplant (LDLT).We conducted a study to compare the interchangeability of the values obtained from these devices in patients with End stage liver disease (ESLD) undergoing LDLT. METHODS: In 76 patients undergoing LDLT, ROTEM and TEG were performed and assessed for interchangeability using Spearman Correlation. The direction and strength of correlation between equivalent parameters was calculated using Inter Class Correlation (ICC) and Bland Altman analysis. RESULTS: The correlation ρ between CT (clotting time) of ROTEM and R of TEG was 0.16 (P = 0.19).The ICC was 0.15, with 95% confidence interval (CI) of -0.38-0.48 (P = 0.25).The ρ of CFT (ROTEM) with K (TEG) was 0.425 (P=<0.001). The ICC was0.49 with 95% CI of 0.17-0.69, P = 0.003.Alpha of ROTEM correlated with Angle of TEG with ρ of 0.475 (P=<0.001). The ICC was 0.61, with 95% CI of 0.36-0.76, P=<0.001.Maximum Clot firmness (MCF) correlated with maximum amplitude (MA) with ρ=0.76 (P=<0.001).The ICC was 0.86, with 95% CI of 0.77-0.92, P=<0.001. Lysis index (L30) of ROTEM correlated clot lysis (CL30) of TEG with ρ of 0.16 (P = 0.18).However, the ICC was 0.45, with 95% CI of 0.11-0.66, P = 0.08. The correlation between CT of ROTEM and R of TEG as well as L30 of ROTEM and CL30 of TEG was not significant.The strongest correlation was found between MCF and MA (P < 0.001). However the MCF/MA showed an agreement of only 86% (ICC = 0.86). CONCLUSION: Values from ROTEM and TEG were not found to be interchangeable.