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Surgical Treatment of Tumors Involving Kidneys With Fusion Anomalies: A Contemporary Series.

ABSTRACT: To report a contemporary series of surgically treated patients with tumors involving kidneys with fusion anomalies.We retrospectively reviewed the medical records of all 10 patients treated at a single tertiary care institution for tumors involving kidneys with fusion anomalies between the years 2000 and 2015. One patient, diagnosed with lymphoma, did not undergo surgical treatment and was therefore excluded. Data regarding patient, tumor, and treatment characteristics were collected and described.The study cohort included 7 male and 2 female patients, at a median age of 52 years. Seven patients underwent open partial nephrectomy. Nephroureterectomy was performed on 2 patients; 1 open and 1 laparoscopic. All patients had localized disease at diagnosis. Tumor histologies were renal cell carcinoma in 5 patients, renal oncocytoma in 1 patient, urothelial carcinoma in 2 patients, and a well-differentiated liposarcoma involving the kidney in 1 patient. Accessory blood vessels were identified in 8 of 9 patients. Median estimated blood loss was 300?mL (interquartile range: 150-1000). Four patients had postoperative complications, including 3 major (Clavien grade???3) and 3 minor (Clavien grade???2) complications. During a median follow-up of 19.2 months (interquartile range: 3-34.8), 1 patient with urothelial carcinoma developed a bladder recurrence. None of the patients developed new-onset chronic kidney disease during the early postoperative period.Localized renal cortical tumors in kidneys with fusion anomalies may be treated with partial nephrectomy; however, complication rates are relatively high. Preoperative imaging of the blood vessels is necessary, as most patients have an accessory blood supply.

SUBMITTER: Mano R

PROVIDER: S-EPMC5125232 | biostudies-literature | 2016 Dec

REPOSITORIES: biostudies-literature

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Surgical Treatment of Tumors Involving Kidneys With Fusion Anomalies: A Contemporary Series.

Mano Roy R Hakimi A Ari AA Sankin Alexander I AI Sternberg Itay A IA Chevinsky Michael S MS Russo Paul P

Urology 20160803

<h4>Objective</h4>To report a contemporary series of surgically treated patients with tumors involving kidneys with fusion anomalies.<h4>Materials and methods</h4>We retrospectively reviewed the medical records of all 10 patients treated at a single tertiary care institution for tumors involving kidneys with fusion anomalies between the years 2000 and 2015. One patient, diagnosed with lymphoma, did not undergo surgical treatment and was therefore excluded. Data regarding patient, tumor, and trea ...[more]

PMID: 27498249

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Project description:ObjectiveThe aim of the study was to investigate surgical modalities and outcomes in patients with type A aortic dissection involving arch anomalies.MethodPatients with type A aortic dissection who underwent surgical treatment at our center between January 2017 and 31 December 2020 were selected for this retrospective analysis. Data including computed tomography (CT), surgical records, and cardiopulmonary bypass records were analyzed. Perioperatively survived patients were followed up, and long-term mortality and aortic re-interventions were recorded.ResultA total of 81 patients with arch anomalies were included, 35 with "bovine" anomalies, 23 with an aberrant right subclavian artery, 22 with an isolated left vertebral artery, and one with a right-sided arch + aberrant left subclavian artery. The strategies of arch management and cannulation differed according to the anatomic variation of the aortic arch. In total, seven patients (9%) died after surgery. Patients with "bovine" anomalies had a higher perioperative mortality rate (14%) and incidence of neurological complications (16%). Overall, four patients died during the follow-up period, with a 6-year survival rate of 94.6% (70/74). A total of four patients underwent aortic re-intervention during the follow-up period; before the re-intervention, three received the en bloc technique (13.6% 3/22) and one received hybrid therapy (11.1% 1/9).ConclusionWith complete preservation and reconstruction of the supra-arch vessels, patients with type A aortic dissection combining arch anomalies can achieve a favorable perioperative prognostic outcome. Patients who received the en bloc technique are more likely to require aortic re-intervention than patients who underwent total arch replacement with a four-branched graft vessel. Cannulation strategies should be tailored according to the variation of anatomy, but routine cannulation with the right axillary artery can still be performed in most patients with arch anomalies, even for patients with an aberrant right subclavian artery.

Project description:BackgroundNeuregulin 1 (NRG1) fusions, which activate ErbB signaling, are rare oncogenic drivers in multiple tumor types. Afatinib is a pan-ErbB family inhibitor that may be an effective treatment for NRG1 fusion-driven tumors.Patients and methodsThis report summarizes pertinent details, including best tumor response to treatment, for six patients with metastatic NRG1 fusion-positive tumors treated with afatinib.ResultsThe six cases include four female and two male patients who ranged in age from 34 to 69 years. Five of the cases are patients with lung cancer, including two patients with invasive mucinous adenocarcinoma and three patients with nonmucinous adenocarcinoma. The sixth case is a patient with colorectal cancer. NRG1 fusion partners for the patients with lung cancer were either CD74 or SDC4. The patient with colorectal cancer harbored a novel POMK-NRG1 fusion and a KRAS mutation. Two patients received afatinib as first- or second-line therapy, three patients received the drug as third- to fifth-line therapy, and one patient received afatinib as fifteenth-line therapy. Best response with afatinib was stable disease in two patients (duration up to 16 months when combined with local therapies) and partial response (PR) of >18 months in three patients, including one with ongoing PR after 27 months. The remaining patient had a PR of 5 months with afatinib 40 mg/day, then another 6 months after an increase to 50 mg/day.ConclusionThis report reviews previously published metastatic NRG1 fusion-positive tumors treated with afatinib and summarizes six previously unpublished cases. The latter include several with a prolonged response to treatment (>18 months), as well as the first report of efficacy in NRG1 fusion-positive colorectal cancer. This adds to the growing body of evidence suggesting that afatinib can be effective in patients with NRG1 fusion-positive tumors.Key pointsNRG1 fusions activate ErbB signaling and have been identified as oncogenic drivers in multiple solid tumor types. Afatinib is a pan-ErbB family inhibitor authorized for the treatment of advanced non-small cell lung cancer that may be effective in NRG1 fusion-driven tumors. This report summarizes six previously unpublished cases of NRG1 fusion-driven cancers treated with afatinib, including five with metastatic lung cancer and one with metastatic colorectal cancer. Several patients showed a prolonged response of >18 months with afatinib treatment. This case series adds to the evidence suggesting a potential role for afatinib in this area of unmet medical need.

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Surgical Treatment of Tumors Involving Kidneys With Fusion Anomalies: A Contemporary Series.

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Surgical Treatment of Tumors Involving Kidneys With Fusion Anomalies: A Contemporary Series.

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