scholarly journals Esophageal Rhabdoid-Like Tumor: A Rare Disease With Aggressive Clinical Behavior

2020 ◽  
Vol 7 ◽  
Author(s):  
Andrea Lovece ◽  
Daniele Bernardi ◽  
Barbara Bruni ◽  
Emanuele Asti ◽  
Claudio Clemente ◽  
...  
Keyword(s):  
Minimally Invasive ◽  
Minimally Invasive Esophagectomy ◽  
Rhabdoid Tumor ◽  
Esophageal Wall ◽  
Malignant Rhabdoid Tumor ◽  
Clinical Behavior ◽  
Invasive Esophagectomy ◽  
Poorly Differentiated ◽  
Rhabdoid Tumors ◽  
Aggressive Clinical Behavior

Background: Malignant rhabdoid tumor is a kidney childhood tumor with aggressive clinical behavior and a wide spectrum of histologic, immunophenotypic, and cytogenetic findings. Extra-renal rhabdoid tumors have been reported in the brain, breast, liver, pancreas, bladder, vulva, prostate, and colon. To date, only nine cases of esophageal rhabdoid tumors have been described, all in patients over 50-year old. We add to the current literature the case of an esophageal, poorly differentiated rhabdoid tumor occurring in a young man.Case Report: A 24-year-old man was referred for progressive dysphagia, retrosternal pain, nausea, and food regurgitation. Esophagogastroduodenoscopy showed an obstructing neoplastic lesion of the distal esophagus associated with Barrett's esophagus. Biopsies revealed undifferentiated esophageal cancer with epithelial morphology and immunohistochemistry positive for CK pan, CK 7 e CK 8-18. Minimally invasive esophagectomy and extended lymphadenectomy was performed. Histopathology showed a poorly differentiated tumor, with morphologic characteristics of rhabdoid tumor, central necrosis and transmural infiltration of the esophageal wall. Definitive immunohistochemistry was positive for vimentin, CD34, synaptophysin, and INI1.Conclusion: Esophageal rhabdoid tumor is extremely rare and highly aggressive, with only few patients alive at 1 year follow-up, according to our review. Immunohistochemistry characterization is critical for diagnosis. Minimally invasive esophagectomy is an appealing and possibly less morbid option compared to open surgery. However, further research is needed to investigate the potential role of targeted immunotherapy.

Epithelioid Sarcoma Arising in a Long-Term Survivor of an Atypical Teratoid/Rhabdoid Tumor in a Patient With Rhabdoid Tumor Predisposition Syndrome

2021 ◽  
pp. 109352662098649
Author(s):  
Tiffany G Baker ◽  
Michael J Lyons ◽  
Lee Leddy ◽  
David M Parham ◽  
Cynthia T Welsh
Keyword(s):  
Epithelioid Sarcoma ◽  
Rhabdoid Tumor ◽  
Malignant Rhabdoid Tumor ◽  
Atypical Teratoid Rhabdoid Tumor ◽  
Diagnostic Challenge ◽  
Patient Demographics ◽  
Immunohistochemical Markers ◽  
Genetic Aberration ◽  
Atypical Teratoid ◽  
Rhabdoid Tumors

Rhabdoid tumor predisposition syndrome (RTPS) is defined as the presence of a SMARCB1 or SMARCA4 genetic aberration in a patient with malignant rhabdoid tumor. Patients with RTPS are more likely to present with synchronous or metachronous rhabdoid tumors. Based on the current state of rhabdoid tumor taxonomy, these diagnoses are based largely on patient demographics, anatomic location of disease, and immunohistochemistry, despite their nearly identical histologic and immunohistochemical profiles. Thus, the true distinction between such tumors remains a diagnostic challenge. Central nervous system atypical teratoid/rhabdoid tumor (AT/RT) is a rare, aggressive, primarily pediatric malignancy with variable histologic features and a well documented association with loss of SMARCB1 expression. Epithelioid sarcoma (ES) is a rare soft tissue tumor arising in patients of all ages and characteristically staining for both mesenchymal and epithelial immunohistochemical markers while usually demonstrating loss of SMARCB1 expression. To our knowledge we herein present the first documented case of a patient with RTPS who presented with metachronous AT/RT and ES.

scholarly journals European consensus on essential steps of Minimally Invasive Ivor Lewis and McKeown Esophagectomy through Delphi methodology

2021 ◽  
Author(s):  
Yassin Eddahchouri ◽  
◽  
Frans van Workum ◽  
Frits J. H. van den Wildenberg ◽  
Mark I. van Berge Henegouwen ◽  
...  
Keyword(s):  
Minimally Invasive ◽  
Expert Panel ◽  
Minimally Invasive Esophagectomy ◽  
Round Table ◽  
Round Table Discussion ◽  
Ivor Lewis ◽  
Delphi Methodology ◽  
Invasive Esophagectomy ◽  
European Consensus ◽  
Mckeown Esophagectomy

Abstract Background Minimally invasive esophagectomy (MIE) is a complex and technically demanding procedure with a long learning curve, which is associated with increased morbidity and mortality. To master MIE, training in essential steps is crucial. Yet, no consensus on essential steps of MIE is available. The aim of this study was to achieve expert consensus on essential steps in Ivor Lewis and McKeown MIE through Delphi methodology. Methods Based on expert opinion and peer-reviewed literature, essential steps were defined for Ivor Lewis (IL) and McKeown (McK) MIE. In a round table discussion, experts finalized the lists of steps and an online Delphi questionnaire was sent to an international expert panel (7 European countries) of minimally invasive upper GI surgeons. Based on replies and comments, steps were adjusted and rephrased and sent in iterative fashion until consensus was achieved. Results Two Delphi rounds were conducted and response rates were 74% (23 out of 31 experts) for the first and 81% (27 out of 33 experts) for the second round. Consensus was achieved on 106 essential steps for both the IL and McK approach. Cronbach’s alpha in the first round was 0.78 (IL) and 0.78 (McK) and in the second round 0.92 (IL) and 0.88 (McK). Conclusions Consensus among European experts was achieved on essential surgical steps for both Ivor Lewis and McKeown minimally invasive esophagectomy.

Survival and quality of life after minimally invasive esophagectomy: a single-surgeon experience

2011 ◽  
Vol 26 (1) ◽  
pp. 168-176 ◽  
Author(s):  
Abhishek Sundaram ◽  
Juan C. Geronimo ◽  
Brittany L. Willer ◽  
Masato Hoshino ◽  
Zachary Torgersen ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Minimally Invasive ◽  
Minimally Invasive Esophagectomy ◽  
Invasive Esophagectomy ◽  
Surgeon Experience

Drivers of Cost Associated with Minimally Invasive Esophagectomy

2021 ◽  
Author(s):  
Nikhil Panda ◽  
Larisa Shagabayeva ◽  
Cameron E. Comrie ◽  
Nicole Phan ◽  
Philicia Moonsamy ◽  
...  
Keyword(s):  
Minimally Invasive ◽  
Minimally Invasive Esophagectomy ◽  
Invasive Esophagectomy

Short- and long-term outcomes in patients operated with total minimally invasive esophagectomy for esophageal cancer

2021 ◽  
Author(s):  
Tobias Hauge ◽  
Dag T Førland ◽  
Hans-Olaf Johannessen ◽  
Egil Johnson
Keyword(s):  
Overall Survival ◽  
Esophageal Cancer ◽  
Minimally Invasive ◽  
Minimally Invasive Esophagectomy ◽  
University Hospital ◽  
Long Term Results ◽  
Resection Margins ◽  
Invasive Esophagectomy ◽  
Short And Long Term

Summary At our hospital, the main treatment for resectable esophageal cancer (EC) has since 2013 been total minimally invasive esophagectomy (TMIE). The aim of this study was to present the short- and long-term results in patients operated with TMIE. This cross-sectional study includes all patients scheduled for TMIE from June 2013 to January 2016 at Oslo University Hospital. Data on morbidity, mortality, and survival were retrospectively collected from the patient administration system and the Norwegian Cause of Death Registry. Long-term postoperative health-related quality of life (HRQL) and level of dysphagia were assessed by patients completing the following questionaries: EORTC QLQ-OG25, QLQ-C30, and the Ogilvie grading scale. A total of 123 patients were included in this study with a median follow-up time of 58 months (1–88 months). 85% had adenocarcinoma, 15% squamous cell carcinoma. Seventeen patients (14%) had T1N0M0, 68 (55%) T2-T3N0M0, or T1-T2N1M0 and 38 (31%) had either T3N1M0 or T4anyNM0. Ninety-eight patients (80%) received neoadjuvant (radio)chemotherapy and 104 (85%) had R0 resection. Anastomotic leak rate and 90-days mortality were 14% and 2%, respectively. The 5-year overall survival was 53%. Patients with tumor free resection margins of >1 mm (R0) had a 5-year survival of 57%. Median 60 months (range 49–80) postoperatively the main symptoms reducing HRQL were anxiety, chough, insomnia, and reflux. Median Ogilvie score was 0 (0–1). In this study, we report relatively low mortality and good overall survival after TMIE for EC. Moreover, key symptoms reducing long-term HRQL were identified.

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