Multimodal management of locally advanced rhabdoid tumour of the kidney in an adult

2021 ◽  
Vol 14 (4) ◽  
pp. e236830
Author(s):  
Ritesh Goel ◽  
Sridhar Panaiyadiyan ◽  
Brusabhanu Nayak ◽  
Prabhjot Singh

Malignant rhabdoid tumours of the kidney (MRTK) are rare paediatric tumours known for their aggressive nature and early metastasis. However, MRTK in adults are even more rare with only a few cases reported in the literature. Herein, we report a case of 65-year-old woman with rapidly progressive left renal mass requiring en-bloc radical nephrectomy, splenectomy and distal pancreatectomy. Histopathology revealed a malignant rhabdoid tumour with characteristic histological and immunohistochemical findings with negative margins. To the best of our knowledge, this is the first reported case of aggressive surgical management of locally advanced MRTK. Despite surgery with curative intent, the patient developed early recurrence and started on tyrosine kinase inhibitor. Unfortunately, the patient expired after 8 months of surgery due to disease progression.

2011 ◽  
Vol 29 (7_suppl) ◽  
pp. 387-387
Author(s):  
A. A. Sheikh ◽  
A. Gharajeh ◽  
S. J. Hotte ◽  
J. H. Pinthus ◽  
A. Kapoor

387 Background: The current first-line treatment for advanced renal cell carcinoma (RCC) includes targeted therapy with or without cytoreductive radical nephrectomy. There is a paucity of data as to the effectiveness of adjuvant and neoadjuvant treatment before radical nephrectomy for localized high-risk or advanced disease. We initiated a trial of neoadjuvant Temsirolimus before radical nephrectomy for locally advanced and metastatic RCC examining tumor response and survival. Methods: Patients who presented with advanced RCC were offered enrolment into a prospective, single-centre, ethics approved trial with 12 weeks of temsirolimus before radical nephrectomy. Biopsy tissue was obtained at enrollment and at time of cytoreductive nephrectomy for diagnosis. Patients were administered 25 mg in temsirolimus on a weekly basis for 12 weeks, and then underwent radical nephrectomy. Computed tomography scans and biomarkers were obtained on enrolment, 6 weeks and 12 weeks (before nephrectomy). Ongoing outcome and survival data were analyzed. Results: Eight patients were enrolled into the trial. Patient #1 (10-cm renal mass with bulky adenopathy T2N2M0) had no evidence of disease (NED) at 6 months post-nephrectomy. Patient #2 (9-cm renal mass, bulky adenopathy, pulmonary metastases T2N2M1) also had NED at 6 months postnephrectomy. Patients #3 and #4 experienced regression of the primary mass and have recently undergone uneventful surgery with follow-up pending. Patients #5 and #6 expired prior to the full course of therapy, but had diagnoses other than RCC. Patient #7 experienced disease progression, however, this patient's nephrectomy was delayed by 3 months due to an unrelated myocardial infarct. Patient #8 experienced adverse events. Conclusions: Our findings suggest that neoadjuvant temsirolimus before radical nephrectomy for advanced RCC may improve disease regression post-surgery, and may lead to disease resolution in patients with low-volume disease. Randomized studies with longer term follow-up is necessary to assess overall progression-free survival and overall survival. [Table: see text]


2021 ◽  
pp. 1-7
Author(s):  
Patrick Schöffski ◽  
Sara Vander Borght ◽  
Isabelle Vanden Bempt ◽  
Sander Jentjens ◽  
Vincent Vandecaveye ◽  
...  

<b><i>Introduction:</i></b> We report the case of a young female patient with a technically resectable, nonmetastatic, rectoanal, anaplastic lymphoma kinase gene (<i>ALK</i>)-translocated inflammatory myofibroblastic tumor (IMFT). <b><i>Case Presentation:</i></b> The patient was successfully treated preoperatively with the tyrosine kinase inhibitor (TKI) crizotinib, to downsize the primary tumor, followed by sphincter-sparing surgery, and adjuvant radiotherapy and crizotinib. She is now in follow-up with good sphincter function and with no evidence of active disease. <b><i>Conclusion:</i></b> Pre- and postoperative treatment administration of crizotinib can be given with curative intent to patients with locally advanced, nonmetastatic IMFTs to avoid mutilating surgery.


2015 ◽  
Vol 53 (200) ◽  
pp. 301-303 ◽  
Author(s):  
Ramesh Singh Bhandari ◽  
Paleswan Joshi Lakhey ◽  
Parsu Ram Mishra

A 57 year old male presented to our outpatient clinic with history of on and off melena, weight loss and decreasing appetite for 10 months duration and a noticeable mass on the right upper quadrant. Abdominal examination revealed an intra-abdominal lump in right upper quadrant which was subsequently evaluated by colonoscopy, which revealed an ulcero-proliferative growth in the hepatic flexure and the biopsy from it confirmed well-differentiated adenocarcinoma. Contrast enhanced computed tomography demonstrated hepatic flexure mass with possible invasion into adjacent duodenum without features of advanced disease. After completion of necessary preoperative assessment and investigations, patient was explored with curative intent and underwent extended right hemicolectomy with en bloc pancreaticodudenectomy. Patient was discharged on 10th postoperative day and at 14 months follows up; he was doing well without any evidence of recurrence. Keywords: colectomy; locally advanced; pancreaticoduodenectomy.


Medicina ◽  
2020 ◽  
Vol 56 (1) ◽  
pp. 33
Author(s):  
Nicolae Bacalbasa ◽  
Irina Balescu ◽  
Mihaela Vilcu ◽  
Simona Dima ◽  
Camelia Diaconu ◽  
...  

Introduction: Extended pelvic resection might be the option of choice in patients presenting locally advanced cervical cancer. However, the possibility of a co-existence of an ectopic, pelvic kidney that is invaded by such a tumor is extremely rare. Case Presentation: A 54-year-old female patient, diagnosed with locally advanced cervical cancer in the presence of a pelvic kidney, was submitted to surgery with curative intent. A large, abscessed cervical tumor invading the urinary bladder and the rectum was found, so a total exenteration was planned. Intraoperatively, tumor invasion of the left kidney, which was found in an ectopic, pelvic position was also encountered; therefore, total pelvic exenteration in association with a left nephrectomy was successfully performed. Conclusions: The presence of an ectopic, pelvic disposition of the kidney makes it susceptible to be invaded by locally advanced pelvic tumors; in such cases, a nephrectomy might also be needed.


Medicina ◽  
2020 ◽  
Vol 56 (4) ◽  
pp. 185 ◽  
Author(s):  
Cornel Savu ◽  
Alexandru Melinte ◽  
Radu Posea ◽  
Niculae Galie ◽  
Irina Balescu ◽  
...  

Introduction: The purpose of this paper is to study the type, the clinical presentation, and the best diagnostic methods for pleural solitary fibrous tumors (PSFTs), as well as to evaluate which is the most appropriate treatment, especially as PSFTs represent a rare occurrence in the thoracic pathology. Material and Method: A retrospective study was conducted on a group of 45 patients submitted to surgery between January 2015 and December 2019. In most cases, the diagnosis was established through imaging studies—thoracic computed tomography (CT) scan with or without contrast—but also using magnetic resonance imaging (MRI) or positron emission tomography (PET) scans when data from CT scans were scarce. All patients were submitted to surgery with curative intent. Results: Most patients included in this study were asymptomatic, with this pathology being more common in patients over 60 years of age, and more common in women. The occurrence of malignant PSFT in our study was 17.77% (8 cases). All cases were submitted to surgery with curative intent, with a single case developing further recurrence. In order to achieve complete resection en bloc resection of the tumor with the chest wall, resection was performed in two cases, while lower lobectomy, pneumectomy, and hemidiaphragm resection, respectively, were needed in each case. Postoperative mortality was null. Conclusion: Thoracic CT scan remains the most important imagistic investigation in diagnosing. MRI is superior to thoracic CT, especially in cases that involved the larger blood vessels within the thorax, spinal column, or diaphragm. Complete surgical resection is the gold standard in treatment of PSFT, and the prognosis in benign cases is very good.


2020 ◽  
Vol 2020 (6) ◽  
Author(s):  
Kit-fai Lee ◽  
Randolph H L Wong ◽  
Howard H W Leung ◽  
Eugene Y J Lo ◽  
Charing C N Chong ◽  
...  

Abstract A 56-year-old man presented with an 11-cm hepatocellular carcinoma (HCC) at segment 7 of liver. To induce left liver hypertrophy, a sequential transarterial chemoembolization (TACE) and portal vein embolization before right hepatectomy were adopted. However, the tumor further increased in size despite TACE and invaded through the diaphragm to the right lung base. Anterior approach right hepatectomy with en bloc wedge resection of the involved right lower lobe of lung by endovascular staplers via transdiaphragmatic approach was performed. The diaphragmatic defect was closed with Goretex mesh. Patient made an uneventful recovery. Pathology confirmed a 12.5 cm poorly differentiated HCC invading through diaphragm to lung. During follow-up, patient developed a 6 cm recurrence at left lung base 17 months after surgery for which he received sorafenib therapy. However, the lung mass further increased in size with new liver recurrence at segment 3 despite treatment. He succumbed 2 years and 3 months after surgery.


2021 ◽  
Vol 34 (Supplement_1) ◽  
Author(s):  
John V Reynolds ◽  
Jessie A Elliott ◽  
Noel Donlon ◽  
Claire Donohoe ◽  
Narayanasamy Ravi ◽  
...  

Abstract   The ECCG developed a standardized platform for reporting operative complications, with consensus definitions, and DUCA adopted these definitions and have reported a comparison against these benchmarks. The aim of this study was to report five year complications data using the standardized definitions of the Esophageal Complications Consensus Group (ECCG), and to compare with published ECCG benchmark studies from the collaborative group and from the Dutch Upper Gastrointestinal Cancer Audit (DUCA). Methods All patients undergoing multimodal therapy or surgery with curative intent from 2014 to 2018 inclusive were studied. All data were recorded prospectively and maintained internally as well as entered onto a secure online database (Esodata.org) from 2015. Statistical analysis was performed using SPSS® (version 18.0). Results 219 patients (mean age 67; 77% male) underwent open resection, 66.6% via transthoracic en bloc resection. 30-day and 90-day mortality were 0.0 and 0.9%, respectively. The anastomotic leak rate was 5.4%, and chyle leak 5.4%. Pneumonia was recorded in 18.2%, respiratory failure 10.9%, and ARDS in 2.7%. Atrial dysrhythmia occurred in 22.8%, recurrent nerve injury 3.1%, and delirium in 5.0%. Compared with both ECCG and DUCA, where MIE constituted 47% and 86% of surgical approaches, respectively, overall complications were similar in this open series, as was complications severity, however anastomotic leak rate were several-fold less, and mortality rates were lower. Conclusion In this unselected consecutive series and comparative audit with benchmark averages from the ECCG and DUCA publication, a low mortality and anastomotic leak rate were the key differential findings. Although not risk-stratified or directly matched, the severity of complications from this ‘open’ series is consistent with series containing large numbers of total or hybrid MIE, highlighting a need to adhere to these strictly defined definitions in further prospective research and randomized studies.


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