scholarly journals MANAGEMENT OF ORBITAL RHABDOMYOSARCOMA: ABOUT 2 CASES AND LITERATURE REVIEW

2021 ◽  
Vol 9 (11) ◽  
pp. 1187-1193
Author(s):  
Zenab Alami ◽  
◽  
Ahmed Bennis ◽  
Kaoutar Soussy ◽  
Idrissa Souley ◽  
...  

Rhabdomyosarcoma is the most common primary malignancy in children. It constitutes a therapeutic emergency. The ocular region, particularly the orbital soft tissues, is an important anatomic location for RMS, which is the most common primary orbital malignancy of childhood. Radio-chemotherapy with conservative surgery allows a recurrence-free survival of 87% at 5 years. The aim of this study was to improve the understanding of the clinical features by reviewing the literature and analysing the medical records of patients who were diagnosed with orbital Rhabdomyosarcoma in our hospital. We report 2 cases of patients treated for an orbital Rhabdomyosarcoma at the radiotherapy department of the Hassan II University Hospital of FES.

2021 ◽  
Author(s):  
Pauline Aeschbacher ◽  
Attila Kollár ◽  
Daniel Candinas ◽  
Guido Beldi ◽  
Anja Lachenmayer

Abstract Background Retroperitoneal sarcoma (RPS) is a rare disease often requiring multi-visceral and wide margin resections. Midline incision seems to be the access of choice. Up to now there is no evidence for the best surgical access. This study aimed to analyze the oncological outcome according to the incision used for the resection. Methods All patients treated for RPS between 2007 and 2018 at the Department of Visceral Surgery and Medicine of the University Hospital Bern and receiving a RPS resection in curative intent were included. Patient- and treatment specific factors as well as local recurrence-free survival was analyzed in correlation with the type of surgical access used. Results Thirty-five patients were treated for RPS at our center. The majority received their primary RPS resection at a tertiary medical center (n = 23) the rest of the resection were performed in an external non-tertiary institution (n = 12). Median tumor size was 24 cm. Resections were performed via a midline laparotomy (MI = 31) or flank incision (FI = 4). All FI were performed at a non-tertiary center and received no multi-visceral resection. In ML the macroscopically complete resection (R0/1) rate was 83.9% and 25% in FI (p = 0.021). The local recurrence rate after R0/1 resection was 100% in FI and 61.5% in ML (p = 1.00). Early recurrence or local disease progression was present in 50% of FI compared to 0% in ML (p = 0.01). The median recurrence-free survival after R0/1 resection was lower in FI (p < 0.001) and in patients operated in a non-tertiary center (p = 0.27). Conclusions FI with limited exposure of the retroperitoneum and no access to the visceral organs as well as resection at a non-tertiary center are associated worse outcome in RPS surgery.


2009 ◽  
Vol 40 (2) ◽  
pp. 166-173 ◽  
Author(s):  
Y. Yamamoto ◽  
H. Ojima ◽  
K. Shimada ◽  
H. Onaya ◽  
N. Hiraoka ◽  
...  

Author(s):  
F. Oehme ◽  
S. Hempel ◽  
M. Pecqueux ◽  
B. Müssle ◽  
H. M. Hau ◽  
...  

Abstract Purpose The treatment of choice for patients presenting with obstructive cholestasis due to periampullary carcinoma is oncologic resection without preoperative biliary drainage (PBD). However, resection without PBD becomes virtually impossible in patients with obstructive cholangitis or severely impaired liver cell function. The appropriate duration of drainage by PBD has not yet been defined for these patients. Methods A retrospective analysis was conducted on 170 patients scheduled for pancreatic resection following biliary drainage between January 2012 and June 2018 at the University Hospital Dresden in Germany. All patients were deemed eligible for inclusion, regardless of the underlying disease entity. The primary endpoint analysis was defined as the overall morbidity (according to the Clavien-Dindo classification). Secondary endpoints were the in-hospital mortality and malignancy adjusted overall and recurrence-free survival rates. Results A total of 170 patients were included, of which 45 (26.5%) and 125 (73.5%) were assigned to the short-term (< 4 weeks) and long-term (≥ 4 weeks) preoperative drainage groups, respectively. Surgical complications (Clavien-Dindo classification > 2) occurred in 80 (47.1%) patients, with significantly fewer complications observed in the short-term drainage group (31.1% vs. 52%; p = 0.02). We found that long-term preoperative drainage (unadjusted OR, 3.386; 95% CI, 1.507–7.606; p < 0.01) and periampullary carcinoma (unadjusted OR, 5.519; 95% CI, 1.722–17.685; p-value < 0.01) were independent risk factors for postoperative morbidity, based on the results of a multivariate regression model. The adjusted overall and recurrence-free survival did not differ between the groups (p = 0.12). Conclusion PBD in patients scheduled for pancreatic surgery is associated with substantial perioperative morbidity. Our results indicate that patients who have undergone PBD should be operated on within 4 weeks after drainage.


Diseases ◽  
2021 ◽  
Vol 9 (1) ◽  
pp. 21
Author(s):  
Leilani Lakemeyer ◽  
Silvia Sander ◽  
Mathias Wittau ◽  
Doris Henne-Bruns ◽  
Marko Kornmann ◽  
...  

Colorectal cancer (CRC) is the third most common cancer worldwide. A diagnosis at early stages with enhanced screening methods is vital as metastases and recurrences increase mortality. The aim of this study was to analyze the tumor markers CEA and CA19-9 combined in correlation with diagnostics and prognosis. Therefore, 1487 patients with CRC who were diagnosed and treated between 2000 and 2015 at the University Hospital Ulm, Germany, were retrospectively evaluated. Overall and recurrence-free survival was analyzed in association with preoperative CEA and CA19-9 separately and combined and a multivariate analysis was performed. The 5-year overall survival was significantly shorter in patients with a CEA or CA19-9 level ≥200 compared to patients with an increased, but <200, or normal level (CEA: 69%/44%/7%; CA19-9: 66%/38%/8%). Patients with both tumor markers increased also showed a remarkably shorter 5-year survival rate (CEA+/CA19-9+: 23%). The multivariate analysis emphasizes these results (p-value < 0.0001). Patients with both tumor markers elevated had the shortest 5-year recurrence-free survival rate, followed by patients with either CEA or CA19-9 elevated (CEA-/CA19-9-: 79%; CEA+/CA19-9; CEA-/CA19-9+: 65%; CEA+/CA19-9+: 44%). In conclusion, measuring CEA and CA19-9 preoperatively in CRC patients is reasonable and could be useful as a prognostic factor.


2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii390-iii390
Author(s):  
Kung-Hao Liang ◽  
Kuo-Sheng Wu ◽  
Yi-Yen Lee ◽  
Muh-Lii Liang ◽  
Jun-Jeng Fen ◽  
...  

Abstract BACKGROUND Medulloblastoma is an aggressive pediatric brain tumor with surgery and post-resection radiotherapy plus chemotherapy as the major type of treatment currently. METHODS A cohort of 52 medulloblastoma patients were treated in Taipei Medical University Hospital and Taipei Veterans General Hospital. Among them, 28 (53.85%) are male. The average age at presentation is 7.21 ± 4.15. Genome-wide RNA profiling were performed on fresh-frozen surgical samples. Tumor infiltrating immune cell percentages were inferred by the cibersort immune deconvolution algorithm. RESULTS A total of 13 leading genes, including DLL1, ASIC2, SLC22A17, TRPM3, RPS2P5 and KCNC3, were found to be significantly associated with overall survival (All P &lt; 0.001). A risk score was constructed, which is indicative of overall survival (Hazard Ratio [HR] = 2.720, 95% confidence interval [CI] = 1.798 ~ 4.112, P &lt; 0.001) and recurrence-free survival (HR = 1.645, CI = 1.337 ~ 2.025, P &lt; 0.001). After adjustment of clinical factors, the score remained significantly associated with overall survival (HR = 2.781, CI = 1.762 ~ 4.390, P &lt; 0.001) and recurrence-free survival (HR = 1.604, CI = 1.292 ~ 1.992, P &lt; 0.001). The percentage of Natural Killer and T follicular helper (Tfh) cells were higher in patients with better overall survival (P = 0.046 and 0.001, respectively). Furthermore, the Tfh percentage is also positively associated with mutation burdens in the expressed exonic regions (P &lt; 0.001). CONCLUSION Higher mutation burdens are correlated with higher levels of tumor infiltrating Tfh cells, which is indicative of better post-surgery prognosis.


2019 ◽  
Vol 37 (4_suppl) ◽  
pp. 27-27
Author(s):  
Junseok Park ◽  
Joon Seong Lee ◽  
Jin-Oh Kim ◽  
Seong Ran Jeon ◽  
Hyun Gun Kim ◽  
...  

27 Background: Gastric adenomas are considered premalignant, and some can be interpreted as cancer according to different pathologic guidelines. The dysplastic lesions share histologic characteristics including mucin phenotype that are related prognosis. The prognosis of the lesions has been studied but tends to be researched separately. Moreover, the proposed prognostic factors are mostly derived from the study of surgical specimens, and most of them did not consider the anatomical changes after surgery. Previously researched prognostic factors of gastric dysplasia were evaluated on the recurrence after complete resection with endoscopic procedure. Methods: From 2005 to 2016, 1678 gastric dysplasia were endoscopically removed in Soonchunhyang university hospital, Seoul. They were followed up with endoscopy under a standardized protocol. For the 716 lesions were histologically evaluated including mucin phenotype with immunohistochemical stain of MUC5AC, MUC6, MUC2, and CD10. Recurrence of dysplastic lesions were analyzed for the 688 lesions with at least 1 year’s follow-up. Results: Five-hundred and forty-three malignant lesions including in situ lesions were completely resected with endoscopic procedures. Endoscopic submucosal dissection was performed on 603 lesions and other lesions were removed with endoscopic mucosal resection. Submucosal invasion was on 83 lesions of carcinoma. The mucin phenotype of lesions was immunohistochemically evaluated. During median 40 months of follow-up, there was 89 cases of recurrence (12.9%). Kaplan-Meier analysis of the recurrence-free survival were estimated and the elderly over 65 years of age showed statistical significance (p = 0.039). Conclusions: Completely resected early stage of gastric dysplasia showed relatively low recurrence rate. Previously proposed histologic features did not affect prognosis. However, the age of patient showed statistical significance on recurrence-free survival. Regular surveillance on elderly patients is important to improve the clinical outcome of gastric dysplasia.


2021 ◽  
Vol 72 (6) ◽  
Author(s):  
Francesco Plotti ◽  
Giuseppe Messina ◽  
Corrado Terranova ◽  
Roberto Montera ◽  
Carlo De Cicco Nardone ◽  
...  

Cancers ◽  
2021 ◽  
Vol 13 (4) ◽  
pp. 917
Author(s):  
Jun A ◽  
Baotong Zhang ◽  
Zhiqian Zhang ◽  
Hailiang Hu ◽  
Jin-Tang Dong

Molecular signatures predictive of recurrence-free survival (RFS) and castration resistance are critical for treatment decision-making in prostate cancer (PCa), but the robustness of current signatures is limited. Here, we applied the Robust Rank Aggregation (RRA) method to PCa transcriptome profiles and identified 287 genes differentially expressed between localized castration-resistant PCa (CRPC) and hormone-sensitive PCa (HSPC). Least absolute shrinkage and selection operator (LASSO) and stepwise Cox regression analyses of the 287 genes developed a 6-gene signature predictive of RFS in PCa. This signature included NPEPL1, VWF, LMO7, ALDH2, NUAK1, and TPT1, and was named CRPC-derived prognosis signature (CRPCPS). Interestingly, three of these 6 genes constituted another signature capable of distinguishing CRPC from HSPC. The CRPCPS predicted RFS in 5/9 cohorts in the multivariate analysis and remained valid in patients stratified by tumor stage, Gleason score, and lymph node status. The signature also predicted overall survival and metastasis-free survival. The signature’s robustness was demonstrated by the C-index (0.55–0.74) and the calibration plot in all nine cohorts and the 3-, 5-, and 8-year area under the receiver operating characteristic curve (0.67–0.77) in three cohorts. The nomogram analyses demonstrated CRPCPS’ clinical applicability. The CRPCPS thus appears useful for RFS prediction in PCa.


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