Metastatic Squamous Cell Carcinoma of Unknown Primary to the Right Ventriclea Rare Cause of Acute Heart Failure

Background Large autopsy studies have shown intracardiac metastases in 15% of patients with disseminated cancer. We present a rare case of metastatic squamous cell carcinoma (SCC) of the heart with unknown primary. Case 55-year-old female with DVT, COVID-19 infection and stage IV metastatic SCC of uncertain origin presented with progressive fatigue and dyspnea. She had occlusion of left interlobar pulmonary artery and new large right ventricle (RV) mass consistent with tumor and thrombus invasion. Systemic anticoagulation was initiated. Decision-making Echocardiography showed a large mass in RV measuring 5 cm x 2.5 cm and occupying most of the RV cavity. Though her RV systolic function was reduced, she was unlikely to benefit from surgical resection due to disseminated disease. Piecemeal removal of RV mass alleviated the obstructed RV outflow tract. Histopathology featured squamous cell carcinoma. She had symptom resolution within a week following procedure. The patient was discharged home with oral anticoagulant. On follow up, she had not experienced any worsening of symptoms and changes in RV mass size despite compliance with chemotherapy (carboplatin and paclitaxel) and anticoagulation. Conclusion Metastatic SCC with unknown primary is a rare cause of acute heart failure that highlights the interplay between clinical findings and multimodal cardiac diagnostic imaging. An individualized approach is required for patients, balancing both risks of surgical and percutaneous intervention. [Formula presented]Copyright © 2023 American College of Cardiology Foundation

Jugular Paraganglioma Presenting with a Tumor Thrombus Extending from Posterior Fossa to Right Atrium and Multiple Skeletal Metastases.

INTRODUCTION: Paragangliomas are tumors of neuroendocrine origin, may appear in different localizations, and are related to the autonomic nervous system. Paragangliomas are generally asymptomatic and may rarely appear with adrenergic symptoms, and clinical findings depend on the catecholamines they secrete. Extra-adrenal paragangliomas are mostly benign, like all paragangliomas. Malignancy criteria consist of local recurrence, metastasis after total resection, and presence of distant metastasis during primary diagnosis. CASE PRESENTATION: This report presents the case of a 31-year-old man with jugular paraganglioma, multiple skeletal metastases, and a long-segment tumor thrombus. Imaging procedures showed a continuous tumor thrombus extending from the posterior fossa to the right atrium and metastases in C2, T1, T6, T8, L5, and right humerus. Histopathological assessment of the metastasis in C2 identified malignant paraganglioma. Curative surgery was not an option for this patient, hence combined chemotherapy was given. CONCLUSION: In cases of malignant paraganglioma with multiple distant metastases, chemotherapy and radiotherapy are feasible treatment methods.

Rarely Described Renal Malignancies Associated With Venous Tumor Thrombus.

BACKGROUND/AIM: Collecting duct carcinoma, epithelioid angiosarcoma and neuroendocrine/carcinoid tumor are uncommon renal malignancies, and their association with tumor thrombus extending into the inferior vena cava is extremely rare. Owing to the rarity of the above-mentioned malignancies and short follow-up of the cases published in the literature, the prognosis and clinical behavior of these tumors remains unclear. Up to date, the culprit of treatment is surgical management with radical nephrectomy, lymph node dissection, thrombectomy and vascular reconstruction if necessary. PATIENTS AND METHODS: We herein describe in detail the first cases published of the above-mentioned renal malignancies associated with extensive inferior vena cava (IVC) thrombus, in which complex vascular reconstruction was performed. RESULTS: Three male patients were identified as having collecting duct carcinoma, epithelioid angiosarcoma and neuroendocrine/carcinoid tumor with IVC involvement. Tumor thrombus levels were II, I and IIIc respectively. Patient ages were 42, 60 and 47 years and tumor sizes were 9.2, 10.9 and 3.7 cm correspondingly. Patient 2 underwent cavectomy, IVC replacement using polytetrafluoroethylene (Gore-Tex®) vascular graft and IVC filter deployment inside the graft. None of the patients developed any pulmonary emboli postoperatively. At the last follow-up, IVC graft for patient 2 remained patent. CONCLUSION: Owing to the rarity of the aforementioned malignancies and short follow-up of cases published in the literature, the prognosis and clinical behavior of these tumors remains unclear. Up to date, the culprit of treatment is surgical management with radical nephrectomy, lymph node dissection, thrombectomy and vascular reconstruction if necessary. Polytetrafluoroethylene (Gore-Tex) vascular grafts are an excellent and safe option for complex vascular reconstructions in patients with evidence of IVC invasion.

OUTCOMES OF NEPHRECTOMY FOR RENAL CELL CARCINOMA WITH VENOUS THROMBUS FOLLOWING IMMUNE CHECKPOINT INHIBITOR THERAPY: A MULTICENTER COLLABORATIVE STUDY

INTRODUCTION AND OBJECTIVE: Nephrectomy and venous thrombectomy is a challenging procedure with potential morbidity and mortality. Despite the increasing use of immune checkpoint inhibitors (ICI) in the management of advanced renal cell carcinoma (RCC), data regarding the outcomes of venous thrombectomy following ICI is limited. We evaluated the feasibility and perioperative outcomes of nephrectomy and venous thrombectomy following ICIs. METHODS: Patients with locally advanced or metastatic RCC with venous thrombus undergoing nephrectomy following ICI therapy were evaluated in four high-volume US academic centers between June 2017 and June 2021. Clinical data, perioperative outcomes, and 90-day complications were recorded. RESULTS: Out of 79 patients who received post-ICI nephrectomy, 27 had venous thrombus. Median (IQR) age was 64 (55-71) years. ICI regimens were Nivolumab ± Ipilimumab (n=19), and Pembrolizumab± Axitinib (n=8). Nephrectomy was indicated following either a good clinical response to ICI (n=24) or as a palliative surgery (n=3). Venous thrombi levels are shown in Table-1. Among all patients, 26 (96%) underwent radical and 1 (4%) partial nephrectomy;12 (44.5%) open, 12 (44.5%) robotic and 3 (11%) laparoscopic. One robotic case converted electively to open. Vascular procedures included renal vein thrombectomy (n=6), IVC thrombectomy and primary repair (n=19), IVC patch repair (n=1), and suprarenal cavectomy (n=1). No intraoperative complications were reported. Nine patients showed no viable tumor in the thrombus, of whom 2 had complete response in the primary tumor as well (ypT0N0). 90-day complication rate was 33% (n=9), with 8 patients (30%) requiring readmission (Table-2). One death was reported within 90 days due to COVID-19 infection. CONCLUSIONS: Nephrectomy and venous thrombectomy following systemic immune checkpoint inhibitor therapy is feasible. One third of patients show no viable tumor in the thrombus. Larger studies are needed to predict pathological response.

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.