Wilms tumor with Mulibrey Nanism: A case report and review of literature.

BACKGROUND: Mulibrey-Nanism (Muscle-liver-brain-eye Nanism = dwarfism; MUL) is a rare genetic syndrome. The underlying TRIM37 mutation predisposes these children to develop tumors frequently. In the largest published series of MUL, 8% patients were reported to develop Wilms tumor (WT). The published literature lacks data regarding the best treatment protocol and outcome of this cohort of children with WT and MUL. We report here a 2-year-old boy with WT and MUL and present a review of literature on WT in MUL. CASE: Our patient had associated cardiac problems of atrial septal defect, atrial flutter and an episode of sudden cardiac arrest. We managed him successfully with chemotherapy, surgery and multi-speciality care. He is alive and in remission at follow-up of 6 months. CONCLUSION: A total of 14 cases (including present case) of WT have been reported in MUL and treatment details were available for six cases. They were managed primarily with surgery, chemotherapy with/without radiotherapy, and all achieved remission. The outcome data is available only for two cases, one has been followed up till 15 years post treatment for WT and other is our patient.

Renal arteriovenous malformation and venous thrombosis: a tumour-like presentation.

Renal arteriovenous malformation is a primarily congenital renal vascular abnormality. It is usually diagnosed incidentally on imaging, and the most common subtype is 'cirsoid', consisting of multiple, enlarged arterial feeders interconnecting with draining veins. We present a 74-year-old woman with an incidental finding of what was at first considered a hypervascularised kidney tumour but turned out to be a left intrarenal arteriovenous malformation associated with a left renal vein thrombosis. Selective endovascular embolisation was performed. The cause-consequence relationship between the arteriovenous malformation and the thrombosis is unique. To our knowledge, no such case has ever been reported.

Case Report: Adequate T and B Cell Responses in a SARS-CoV-2 Infected Patient After Immune Checkpoint Inhibition.

After the COVID-19 outbreak, non-evidence based guidelines were published to advise clinicians on the adjustment of oncological treatment during this pandemic. As immune checkpoint inhibitors directly affect the immune system, concerns have arisen about the safety of immunotherapy during this pandemic. However, data on the immune response in oncology patients treated with immunotherapy are still lacking. Here, we present the adaptive immune response in a SARS-CoV-2 infected patient who was treated with immune checkpoint inhibitors for advanced renal cell cancer. To evaluate the immune response in this patient, the number of T cells and their major subsets were measured according to expression of markers for co-signalling, maturation, and chemotaxis at baseline, during therapy, and during the SARS-CoV-2 infection. In addition, plasma samples were analyzed for IgM and IgG antibodies and the ability of these antibodies to neutralise SARS-CoV-2. Despite several risk factors for an impaired immune response to SARS-CoV-2, both T- and B-cell responses were observed. Moreover, after treatment with immune checkpoint inhibitors, a sufficient cellular and humoral immune response was achieved in this SARS-CoV-2 infected patient. These findings warrant renewed discussion on withholding of immune checkpoint inhibitors during an ongoing COVID-19 pandemic.

Diagnostic bias during the COVID-19 era: COVID-19 or renal abscess?

INTRODUCTION: The Coronavirus disease-2019 (COVID-19) has been declared as a pandemic in March 2020 by the World Health Organization (WHO). Since then, this pandemic has dramatically affected the entire world, even radically influencing the way patients are framed at triage. Symptoms and tests in most cases lead to a correct diagnosis; however, error may be around the corner. CASE REPORT: A 60 years old patient was referred with weight loss, fatigue and mild fever for 3 weeks as he was working in a COVID-19 ward. After a positive swab and chest CT scan, he was admitted in the hospital and treated as mild COVID-19 patient. A CT scan performed after the patient was discharged revealed a renal lesion misidentified as a tumor then clarified to be an abscess which retrospectively appears to be the main cause of his symptoms. CONCLUSION: Clinicians should consider other life-threatening disease in the differential diagnosis of patients presenting with similar symptoms to minimize mistakes and avoid further unnecessary investigations.

Challenging Cases of Renal Cell Cancers With or Without Tumor Thrombus During the Covid-19 Pandemic.

BACKGROUND/AIM: Large or bilateral multiple renal cell carcinoma (RCC) without/with tumor thrombus (TT) in the renal vein (RV) or inferior vena cava (IVC) poses a challenge to the surgeon due to the potential for massive hemorrhage, tumor thromboemboli and dialysis, and the situation is more critical due to Covid-19 pandemic. We report our experience and measures in dealing with challenging cases of large or multiple RCCs without/with TT during the ongoing Covid-19 pandemic. PATIENTS AND METHODS: Between 4/2020-10/2020, five patients underwent RCC resection with/without TT. Patients 1 and 2 had RCCs/TT in RV; Patient 3 had RCC/TT supradiaphragmatic below right atrium; Patient-4 had a 26 cm RCC; Patient-5 had multiple RCCS as part of Birt-Hogg-Dube syndrome. RESULTS: Patients were preoperatively tested negative for Covid-19. Operation times were 105, 85, 255, 200 and 247 minutes for Patients 1-5. Estimated blood loss was: 100, 50, 3,900,100 and 50 ml, respectively. Patient 3 underwent RCC resection en bloc with IVC/TT. Patients 1 and 2 underwent resections of RCC/TT in RV. Patient 4 underwent a 26 cm RCC resection. Patient 5 underwent laparoscopic bilateral radical nephrectomies. No immediate postoperative complications were reported. CONCLUSION: We successfully managed 5 challenging cases of RCCs despite the recommendations imposed by hospitals due to Covid-19 pandemic, with favorable outcomes.