Inoperable Renal Malignant Glomus Tumor, the answers for all the "W's"?

Glomus tumor, arising from glomus bodies (specialized neurovascular structures involved in thermoregulation), commonly occurs in extremities and rarely in viscera. The spectrum of glomus tumors range from benign tumors to tumors with uncertain malignant potential to tumors of the malignant subtype. A vast majority of visceral glomus tumors are benign. Most common visceral tumors arise in the gastrointestinal tract. Glomus tumors of the kidney are a rare entity of which malignant glomus tumors are exceedingly rare. The index patients in the existing case reports were middle-aged males. We report our experience with malignant glomus tumor of the left kidney in a 60-year-old female, with computed tomography (CT) showing involvement of renal vein and inferior vena cava (IVC). Percutaneous biopsy was performed as imaging did not conform to the appearance of a conventional renal tumor and was reported as malignant glomus tumor after immunohistochemistry. After informed decision, the patient and family elected to proceed with surgery. However, intraoperatively, the left renal mass was found to infiltrate the pancreas, duodenum, aorta, and root of the colonic mesentery due to which surgery was aborted. Biopsy obtained intraoperatively again confirmed diagnosis of left renal malignant glomus tumor. She had an uneventful postoperative recovery. Options of treatment were reviewed by a multidisciplinary board. In light of no proven benefit for systemic therapy, she was referred for supportive care. She was under follow-up and she expired after 7 months due to progressive disease. Our literature review focuses on the clinicopathologic features and the current standard of management of malignant renal glomus tumors.

Sarcomatoid Carcinoma Metastasis to the Colon from a Small Renal Mass: Case Report with Review of Literature.

A third of patients with renal cell carcinoma (RCC) present with metastatic disease. Metastasis in RCC from small renal mass (SRM) (≤4 cm) is rare. We report a case of stage cT1a clear-cell RCC with low-risk features on pathology presenting with disproportionately large synchronous solitary metastasis to the transverse colon. He underwent resection of the mass with the involved transverse colon and adjoining mesocolon. Intestinal continuity was restored, following which partial nephrectomy was performed for the right renal tumor. Final pathology of the right renal mass confirmed clear-cell RCC. The large mass after immunohistochemistry profile confirmed metastasis from the renal tumor.

Alcohol and the central nervous system.

Ethanol use is common to most cultures but with varying doses and to varying extents. While research has focused on the effects on the liver, alcohol exerts a range of actions on the function and structure of the nervous system. In the central nervous system (CNS) it can provoke or exacerbate neurological and psychiatric disease; its effects on the peripheral nervous system are not included in this review. Sustained alcohol intake can predispose to acute neurochemical changes which, with continued ingestion and incomplete treatment, can lead to chronic structural changes in the CNS: these include generalised cortical and cerebellar atrophy, amnesic syndromes such as Korsakoff's syndrome, and specific white matter disorders such as central pontine myelinolysis and Marchiafava-Bignami syndrome. Alcohol in pregnancy commonly and significantly affects fetal health, though this receives less medical and political attention than other causes of fetal harm. This review looks at the range of disorders that can follow acute or chronic alcohol use, and how these should be managed, and we provide a practical overview on how neurologists might diagnose and manage alcohol addiction.

Paratesticular Osteosarcoma-A Rare Tumor with Distinctive Imaging Findings.

Extraosseous osteosarcoma (EOO) is a rare mesenchymal malignancy representing 4% of all osteosarcomas and 1% of soft tissue sarcomas. The testes, its supporting structures, that is, paratestes, and the spermatic cord are among the rarest sites for EOO, with only 11 published English language reports to date. We report our experience with a 73-year-old male presenting with left hemiscrotal swelling, noted to have extensive amorphous intratumoral calcification on imaging. He underwent left high inguinal orchidectomy with en bloc hemiscrotectomy, with a final pathologic diagnosis of primary paratesticular osteosarcoma. Our literature review corroborates this distinctive, hitherto overlooked imaging feature.

Testicular malignancy in persistent Mullerian duct syndrome: Experience from an apex cancer center with review of literature.

OBJECTIVES: Persistent Mullerian duct syndrome (PMDS) is a rare disorder of sexual differentiation resulting from aberrations in the Mullerian inhibiting factor (MIF) pathway, with consequent failure of regression of fetal Mullerian duct. The concomitant association of undescended testis increases the likelihood of developing testicular tumors in these patients. Due to its rarity, clinic-pathologic and treatment outcome data on testicular cancer in PMDS is sparse. We present our institutional experience and review published literature on testicular cancer in PMDS. MATERIAL AND METHODS: We retrospectively queried our institutional testicular cancer database for all patients with a diagnosis of testicular cancer and PMDS, between January 1980 and January 2022. Additionally, a Medline/PubMed search was performed for English language articles published during the same time period. Data on pertinent clinical, radiologic, and pathologic disease characteristics were abstracted, in addition to treatment received and outcomes. RESULTS: Of 637 patients treated for testicular tumors during the specified time period in our institution, 4 patients had a concomitant diagnosis of PMDS. Testicular tumor was pathologically confirmed as seminoma in 3, 1 had mixed germ cell tumor. All patients in our series presented with stage 2B or higher disease and required chemotherapy, either in the neoadjuvant or adjuvant setting, in addition to surgery. With a mean follow up of 67 months, all patients were disease free. Medline/PubMed search retrieved 44 articles (49 patients) of testicular tumors associated with PMDS, with majority (59%) presenting with a large abdominal mass. Only 5 cases (10%) had a preceding history of appropriately managed cryptorchidism. CONCLUSIONS: Testicular cancer in PMDS usually presents in adults with advanced stage disease resulting from neglected or inadequate management of cryptorchidism. Appropriate management of cryptorchidism in childhood is likely to decrease malignant degeneration, if not, enable early-stage diagnosis.

Stability of renal parenchymal volume and function during active surveillance of renal oncocytoma patients.

INTRODUCTION AND OBJECTIVE: To evaluate whether significant loss in ipsilateral renal parenchymal volume (IRPV) and renal function occurs during active surveillance (AS) of renal oncocytoma (RO) patients. METHODS: Renal function (estimated glomerular filtration rate, eGFR) dynamics were retrospectively analyzed in 32 consecutive biopsy-diagnosed RO patients managed with AS at a National Comprehensive Cancer Network institute. Three-dimensional kidney and tumor reconstructions were generated and IRPV was calculated using volumetry software (Myrian®) for all patients with manually estimated RO growth >+10 cm3. GFR and IRPV were compared at AS initiation vs. the last follow-up using 2-sided paired t-tests. The correlation between change in IRPV and change in RO size or GFR was tested using a Spearman coefficient. RESULTS: With median follow-up of 37 months, there was no significant change between initial vs. last eGFR (median 71.0 vs. 70.5 ml/min/1.73 m2, P = 0.50; median change -3.0 ml/min/1.73 m2). Among patients (n = 17) with RO growth >+10 cm3 during AS (median growth +28.6 cm3, IQR +16.9- + 46.5 cm3), IRPV generally remained stable (median change +0.5%, IQR -1.2%- + 1.2%), with only 2 cases surpassing 5% loss. No IRPV loss was detected among any patient within the top tertile of RO growth magnitude. RO growth magnitude did not correlate with loss of either IRPV (ρ = -0.30, P = 0.24) or eGFR (ρ = -0.16, P = 0.40), including among patient subsets with lower initial eGFR. Study limitations include a lack of long-term follow-up. CONCLUSIONS: Volumetry is a promising novel tool to measure kidney and tumor tissue changes during AS. Our study using volumetry indicates that clinically significant loss of IRPV or eGFR is uncommon and unrelated to tumor growth among untreated RO patients with intermediate follow-up. These findings support that AS is in general functionally safe for RO patients, however longer study is needed to determine safety durability, particularly among uncommon ≥cT2 RO variants.

Perioperative anaemia and iron deficiency.

Anaemia affects a third of surgical patients and is associated with increased morbidity and mortality. Iron deficiency is the most common cause of anaemia and can be absolute or functional. Patients may require treatment with oral or intravenous iron.

Prevalence and severity of coronal and radicular caries among patients with type 2 diabetes mellitus: A cross sectional study.

Background: Studies among type 2 diabetes mellitus patients have reported total caries experience; however the severity and clinical consequences of untreated dental caries are often ignored. Methods: For this study, 150 well (I) and poorly controlled (II) diabetic participants were recruited. The spectrum of caries was evaluated using DMFT (Decayed, Missing and Filled Tooth) index, Dental Caries Severity Classification Scale, PUFA (Pulpal involvement, Ulceration, Fistula and Abscess) index, RCI (Root Caries Index) and the severity of radicular caries by Root Surface Caries Severity Index. Results: The prevalence of coronal and root caries was 90.7% and 23.3%, respectively. There was significant difference among caries experiences for D, M and DMFT. In group II, severity of coronal caries and mean rank of P, F, A and PUFA scores were higher, so were prevalence of root caries and severity of RD2, RD3 and RD4. HbA1c level had positive correlation with DMFT and PUFA scores (r = 0.458 and 0.522), so was the duration of diabetes with coronal caries, DMFT, PUFA score, root caries and RCI score (r = 0.235, 0.320, 0.273, 0.308 and 0.323). Conclusion: This is probably the first study to examine the severity of coronal caries, prevalence of untreated dental caries and severity of radicular caries in diabetic patients. Uncontrolled diabetes causes substantial increase in prevalence and severity of coronal and radicular caries.

Covid-19 ICU remote-learning course (CIRLC): Rapid ICU remote training for frontline health professionals during the COVID-19 pandemic in the UK.

Background: The unprecedented increase in critically ill patients due to the COVID-19 pandemic mandated rapid training in critical care for redeployed staff to work safely in intensive care units (ICU). Methods: The COVID-19 ICU Remote-Learning Course (CIRLC) is a remote delivery course developed in response to the pandemic. This was a one-day course focused on the fundamentals of Intensive Care. The course used blended learning with recorded lectures and interactive tutorials delivered by shielding and frontline ICU trained professionals. The course was developed within one week and piloted at three NHS Trusts. It was then made publicly available free of charge to redeployed healthcare professionals across the UK and Ireland. An iterative cycle of improvement was used to update the course content weekly. A course confidence questionnaire with quantitative and qualitative questions was used to evaluate effectiveness. Data is reported as n (%), means (SD) and thematic analysis was used for the open questions. Results: 1,269 candidates from 171 organisations completed the course, with 99 volunteer trainers. 96% of respondents rated the course as very or extremely useful. 86% rated the online platform as excellent. Overall confidence improved from 2.7/5 to 3.9/5. Qualitative data showed that the course was pitched at the appropriate level, accessible and built clinicians confidence to work in intensive care. Conclusion: This model of educational delivery with a rapid iteration cycle was a pragmatic, effective solution to knowledge-based training under social distancing measures. Whilst full course evaluation was not possible, we believe that this work demonstrates practical guidance on educational response in a pandemic as well as highlighting the altruistic nature of the critical care community.

Primary Renal Synovial Sarcoma Case Series: Clinical Profile and Management of a Rare Entity.

Synovial sarcomas occur predominantly in the extremities. Primary renal synovial sarcoma is a rare entity. Very few cases have been reported in the literature. Clinical and radiological features are similar to renal cell carcinoma with the diagnosis being established after surgery based on histopathology, immunohistochemistry, and chromosome studies. There are no established guidelines on the role of adjuvant treatment in the management of this disease. We herein present a series of 3 cases managed at 2 institutions. In the current series, all patients had venous thrombus, and surgery was the mainstay of treatment. One patient received neoadjuvant chemotherapy after a preoperative biopsy which was done as she did not respond to chemotherapy for a presumptive diagnosis of Wilm's tumor.

Acute Intraoperative Hyperkalemia During Robot-Assisted Radical Cystectomy: A Case Report.

A 50-year-old man with muscle-invasive bladder cancer was scheduled for a robotic radical cystectomy. Four hours into the surgery, his electrocardiogram showed rhythm disturbances. Arterial blood gas analysis showed a serum potassium concentration of 6.6 mEq/L. Hyperkalemia was managed immediately with intravenous 10% calcium gluconate, insulin, and glucose administrations, and levosalbutamol was administered through the tracheal tube. Subsequently, normal sinus rhythm returned. The procedure was completed after conversion to an open surgery. The postoperative serum potassium concentration was reduced to 4.6 mEq/L, and the patient was extubated. The remainder of his hospital stay was uneventful.

FRAX-based osteoporosis treatment guidelines for resource-poor settings in India.

Using the FRAX® model for India, thresholds for osteoporosis evaluation and treatment without bone mineral density measurement were derived and were validated in a cohort of 300 patients. We suggest the use of this newer age and ethnic-specific FRAX®-derived thresholds for management of osteoporosis in India. PURPOSE: Our study aimed to formulate population-specific intervention thresholds for treatment of osteoporosis in India which can be used even without dual X-ray absorptiometry (DXA). METHODS: Using the FRAX® model for India, thresholds for different age groups for men and women were calculated without bone mineral density (BMD) measurement. The lower assessment threshold (LAT) was based on the 10-year probability of a major osteoporosis fracture (MOF) or hip fracture (HF) equivalent to patients without clinical risk factors. The intervention threshold (IT) was based on the 10-year probability equivalent to patients with fracture. The upper assessment threshold (UAT) was set at 1.2 times the IT. Probability-based thresholds for no intervention (LAT), treatment initiation (UAT) and BMD assessment (between LAT and UAT) were derived. The thresholds were validated in a cohort of 300 patients who were referred for BMD testing. RESULTS: Graphs for age, gender, BMI and ethnic-specific LAT, IT and UAT for MOF and HF are derived. In the validation cohort, BMD testing to initiate/defer treatment was required in only 32.3% patients. The intervention thresholds derived without BMD testing were valid in 98.7% patients. Use of National Osteoporosis Foundation (NOF) guidelines would have resulted in overtreatment in 56/300 (18.6%) patients. CONCLUSION: We suggest the use of this newer age and ethnic-specific FRAX®-derived thresholds for management of osteoporosis. Adopting these cut-offs will ensure that those requiring osteoporosis treatment will not be denied of it just because of lack of a DXA machine and will also help avoid overtreatment.

Active Surveillance for Risk Stratification of All Small Renal Masses Lacking Predefined Clinical Criteria for Intervention.

PURPOSE: Despite general indolence of small renal masses and no known adversity from treatment delays, broad usage of active surveillance as a means to risk-stratify patients with small renal masses for more selective treatment has not been studied. We describe outcomes for a novel approach in which active surveillance was recommended to all patients with small renal masses lacking predefined progression criteria for intervention. MATERIALS AND METHODS: All nondialysis dependent patients with nonmetastatic small renal masses seen by 1 urologist at a comprehensive cancer center during January 2013-September 2017 were managed with active surveillance if standardized progression criteria for intervention were absent, with delayed intervention recommended only upon progression criteria for intervention development. Progression criteria for intervention were defined prospectively as small renal mass-related symptoms, unfavorable histology, cT3a stage or either of the following without benign neoplastic biopsy histology: longest tumor diameter >4 cm; growth rate >5 mm/year for longest tumor diameter ≤3 cm or >3 mm/year for longest tumor diameter >3 cm. RESULTS: In all, 96% (123/128) of patients with small renal masses lacked progression criteria for intervention at presentation and underwent active surveillance. With median/mean 31/34 months followup, none developed metastasis and 30% (37/123) developed progression criteria for intervention, 78% (29/37) of whom underwent delayed intervention. One (1%) patient crossed over to delayed intervention without progression criteria for intervention. Three-year progression criteria for intervention-free and delayed intervention-free rates were 72% and 75%, respectively. Delayed intervention resections were enriched (62%) for pT3 and/or nuclear grade 3-4 malignant pathology, with no benign resections. CONCLUSIONS: Active surveillance using predefined progression criteria for intervention in otherwise unselected patients with small renal masses allows intervention to be focused on at-risk small renal masses with common adverse pathology, avoiding treatment for most patients with small renal masses. Long-term delayed intervention and oncologic safety require study.

Monogenic diabetes: A single center experience from South India.

Monogenic forms of diabetes in children are frequently misclassified as either type 1 diabetes or young-onset type 2 diabetes. There is a paucity of literature regarding pediatric monogenic diabetes in the Indian population. A retrospective analysis of case records of 37 children with monogenic diabetes who were diagnosed between 2008 and 2019 in a South Indian tertiary care center was performed. The write-up describes the clinical, biochemical, and genetic characterization of these patients with the diagnoses of neonatal diabetes mellitus (15 patients), MODY (five patients), and various forms of syndromic diabetes (13 with Wolfram syndrome, two with H syndrome, one with mitochondrial diabetes, and one with thiamine responsive megaloblastic anemia).

Health related quality of life in pediatric onset Type 1 diabetes mellitus in Kerala, India.

BACKGROUND: To study the health-related quality of life (HRQoL) in pediatric onset Type 1 diabetes mellitus (TIDM) in the state of Kerala in India. METHODS: Diabetes specific HRQoL was determined in 107 subjects with onset of T1DM less than 21 years using the Pediatric Quality of Life Inventory (PedsQL) 3.2 questionnaire of which 69 also completed the PedsQL 4.0 generic questionnaire. The parents of these patients completed the proxy scoring questionnaire. The generic HRQoL of T1DM was compared with control population without diabetes. RESULTS: Mean age of onset of T1DM was 8.2 ± 4.6 years and mean duration was 8.2 ± 5.6 years. The median HbA1c was 8.7% (72 mmol/mol). Microvascular complications were present in 14.9% of the patients. The generic HRQoL was lower in patients with T1DM compared to peers without diabetes (77.43 vs. 87.45; p < 0.001). Parents reported lower scores in diabetes management, treatment barriers, physical functioning and generic scales compared to patients. Parents reported greater worry about the disease with lesser age of onset of diabetes and with presence of complications. Those using insulin vials and on splitmix regimen had lower HRQoL compared to those using insulin pens. The patients belonging to lower socioeconomic status (SES) reported lower diabetes scores (73.7 vs. 58.7; p = 0.015). CONCLUSIONS: The generic HRQoL was lower in children with T1DM compared to peers without diabetes. Parents and children had different perception about HRQoL in many aspects. The diabetes specific scores were lower in those belonging to lower SES.

Clinical Profile of Medullary Thyroid Carcinoma: Audit from a Tertiary Care Center in South India.

CONTEXT: Medullary thyroid carcinoma (MTC), being an aggressive disease, requires meticulous follow-up and multidisciplinary management. The clinical presentation, management, outcome of MTC varies among different populations. AIMS: An audit was conducted to evaluate the demography, clinical presentation, management, and outcome of MTC in a tertiary care center in South India. SETTINGS AND DESIGN: A retrospective analysis was conducted of data from hospital records of patients with MTC treated at our center from 2004 to 2019. STATISTICAL ANALYSIS: All analyses were performed with SPSS software (version 16). RESULTS: Among the 82 patients (M 42, F 40), mean age was 42.07 years (SD 14.5), 46 were operated at our center and 36, outside. Follow-up data were not available for all patients. Median duration of the disease was 36 months and median follow-up was 28 months. Lymphnode dissection was more common (37/46) in patients operated at our center than outside operated patients (17/36) (P < 0.01). At presentation, more than half of the patients had stage IV disease and 8 had distant metastases. Bone, lung, and liver were the common sites of metastases. Persistent hypercalcitoninemia >50 pg/mL was seen in 49.9%. Salvage surgeries of the neck were necessary in 29 patients (38.2%). Mean survival was 66 months and 10-year survival was 35%. Male gender (P = 0.008) and Stage IV disease at presentation (P = 0.038) were associated with poorer survival. CONCLUSION: MTC, in our population, presented at an advanced stage. Male gender and stage IV at presentation had poor survival. Early diagnosis, aggressive initial neck clearance, close follow-up with tumor markers, appropriate imaging, along with prompt surgical intervention will help to improve outcome.