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BACKGROUND: Ewing sarcoma (ES) is a malignant neoplasm of neuroectodermal origin and is commonly observed in children and young adults. The musculoskeletal system is the main body system impacted and ES is rarely seen in the visceral organs particularly the adrenal gland. AIM: To present a comprehensive review of primary adrenal ES, with emphasis on diagnosis, therapy and oncological outcomes. METHODS: A systematic review of the literature was performed according to the Preferred Reporting Items for Systematic reviews and Meta-Analyses 2020. PubMed/ MEDLINE, EMBASE and Google Scholar bibliographic databases were searched to identify articles from 1989 to 2022 and included patients with ES/primitive neuroectodermal tumor (PNET) of the adrenal gland. PubMed, Google Scholar and EMBASE medical databases were searched, combining the terms "adrenal", "ES" and "PNET". Demographic, clinical, pathological and oncological data of patients were analyzed by SPSS version 29.0. RESULTS: A total of 52 studies were included for review (47 case reports and 5 case series) with 66 patients reported to have primary adrenal ES. Mean age at diagnosis was 26.4 ± 15.4 years (37.9% males, 57.6% females, sex not reported in 3 cases). The most frequent complaint was abdominal/flank pain or discomfort (46.4%) followed by a palpable mass (25.0%), and the average duration of symptoms was 2.6 ± 3.1 mo. The imaging modality of choice was computed tomography scan (81.5%), followed by magnetic resonance imaging (20.4%). Preoperative staging revealed that 17 tumors (27.9%) were metastatic and 14 patients had inferior vena cava or renal vein neoplastic thrombus at initial diagnosis. Open adrenalectomy was performed in the majority of cases (80.0%), of which 27.9% required more extensive resection. Minimally invasive surgery was attempted in 8.2% of tumors. Complete surgical resection was achieved in 89.4% of the patients. Adjuvant therapy was administered to 32 patients, in the form of chemotherapy (62.5%), radiotherapy (3.1%) or combination (34.4%). Median overall survival was 15 mo and 24-mo overall survival was 40.5%. Median disease-free survival was 10 mo and 24-mo disease-free survival was 33.3%. CONCLUSION: The significant progress in molecular biology and genetics of ES does not reflect on patient outcomes. ES remains an aggressive tumor with a poor prognosis and high mortality.
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Hepatic small vessel neoplasm (HSVN) is a recently described vascular neoplasm of the liver. It demonstrates an infiltrative growth pattern and lacks cytologic atypia and mitotic activity. So far, no cases of metastasis or disease recurrence after excision have been reported in the literature. In this report, we present the case of a 31-year-old woman with a lesion in segments VII-VIII of the liver who was referred to our surgical department due to right lumbar pain. She underwent an atypical wedge hepatectomy (segments VII, VIII) and cholecystectomy. The histopathology of the resected specimen confirmed a 40mm HSVN. The patient did not receive any adjuvant therapy and is scheduled for follow-up with serial magnetic resonance imaging (MRI) scans over the next five years due to the unknown malignant potential of the tumor.
RESUMO
Background The present study assesses the educational value of laparoscopic cholecystectomy videos on YouTube regarding the correct application of the critical view of safety (CVS), and evaluates surgical trainees' perceptions of the CVS criteria in a simulated, operative decision-making exercise. Methods YouTube was systematically searched for laparoscopic cholecystectomy videos, explicitly reporting a satisfactory CVS. The top 30 most popular videos, by number of views, were identified and scored on the 6-point scale by three experienced consultants. After watching a training module on CVS rationale and criteria, 10 trainees, blinded to the consultants' assessment, were instructed to view the videos, score each criterion and answer the binary question "Would you divide the cystic structures?" by "yes" or "no." Results An inadequate CVS was found in 30% of the included videos. No statistical association was noted between number of views, likes, or dislikes with successful CVS rates. Inter-observer agreement between consultants and trainees ranged from minimal to moderate ( k = 0.07-0.60). Discrepancy between trainees' CVS scores and their simulated decision to proceed to division of the cystic structures was found in 15% of assessments, with intra-observer agreement ranging from minimal to excellent ( k = 0.27-1.0). For the CVS requirements, inter-observer agreement was minimal for the dissection of the cystic plate ( k = 0.26) and triangle clearance ( k = 0.39) and moderate for the identification of two and only two structures ( k = 0.42). Conclusion The CVS is central to the culture of safety in laparoscopic cholecystectomy. Surgical videos are a useful training tool as simulated, operative decision-making exercises. However, public video platforms should be used judiciously, since their content is not peer-reviewed or quality-controlled.