Retrosigmoid ileal conduit without transposition of the left ureter after open radical cystectomy for bladder cancer.

OBJECTIVES: To assess perioperative outcomes, complications, and rate of uretero-ileal anastomotic stricture (UAS) in patients undergoing retrosigmoid ileal conduit after radical cystectomy (RC). PATIENTS AND METHODS: Clinical records of consecutive patients receiving retrosigmoid ileal conduit after open RC for bladder cancer between March 2016 and June 2020 at two academic centres were prospectively collected. Two expert surgeons performed all cases. Operating room (OR) time, estimated blood loss (EBL), transfusion rate, and 90-day postoperative complications classified according to the Clavien-Dindo system, were assessed. In particular, rate of UAS, defined as upper urinary tract dilatation requiring endourological or surgical management, was evaluated. RESULTS: A total of 97 patients were analysed. The median (interquartile range [IQR]) OR time was 245 (215-290) min, median (IQR) EBL was 350 (300-500) mL, and blood transfusions were given to 15 (15.5%) cases. There were no intraoperative complications. There were 90-day postoperative complications in 33 patients (34%), being major (Grade III-V) in 19 (19.6%). Two patients died from early postoperative complications. At a median (IQR) follow-up of 25 (14-40) months, there was only one case (1%) of UAS, involving the right ureter and requiring an open uretero-ileal re-implantation. CONCLUSION: The retrosigmoid ileal conduit is a safe and valid option for non-continent urinary diversion after RC, ensuring a very low risk of UAS at an intermediate-term follow-up.

Perioperative Outcomes of Holmium Laser Enucleation of the Prostate: A Systematic Review.

INTRODUCTION: The aim of the study was to systematically review the literature and describe perioperative complications of holmium laser enucleation of the prostate (HoLEP), including the Clavien-Dindo classification of surgical complications. METHODS: All English language publications on HoLEP were evaluated. We followed the PRISMA (Preferred Reporting Items for Systematic Review and Meta-Analyses) guidelines to evaluate PubMed®, Scopus®, and Web of Science™ databases from January 1, 1998, to June 1, 2020. RESULTS: Fifty-seven studies were included, for a total of 10,371 procedures. We distinguished between intra-, peri-, and postoperative complications. Overall, the rate of complications is 0-7%. Intraoperative complications include incomplete morcellation (2.3%), capsular perforation (2.2%), bladder (2.4%), and ureteric orifice (0.4%) injuries. Perioperative complications include postoperative urinary retention (0.2%), hematuria and clot retention (2.6%), and cystoscopy for clot evacuation (0.7%). Postoperative complications include dysuria (7.5%), stress (4.0%), urge (1.8%), transient (7%) and permanent (1.3%) urinary incontinence, urethral stricture (2%) and bladder neck contracture (1%). CONCLUSIONS: HoLEP is a safe procedure, with a satisfactory low complication rate. The most common reported perioperative complications are not severe (Clavien-Dindo classification grades 1-2). Further randomized studies are certainly warranted to fully determine the predictor of surgical complications in order to prevent them and improve this technique.

Head-to-head comparison between multiparametric MRI, the partin tables, memorial sloan kettering cancer center nomogram, and CAPRA score in predicting extraprostatic cancer in patients undergoing radical prostatectomy.

BACKGROUND: It is unclear whether clinical models including the Partin tables (PT), the Memorial Sloan Kettering Cancer Center nomogram (MSKCCn), and the cancer of the prostate risk assessment (CAPRA) can benefit from incorporating multiparametric magnetic resonance imaging (mpMRI) when staging prostate cancer (PCa). PURPOSE: To compare the accuracy of clinical models, mpMRI, and mpMRI plus clinical models in predicting stage ≥pT3 of PCa. STUDY TYPE: Prospective monocentric cohort study. POPULATION: Seventy-three patients who underwent radical prostatectomy between 2016-2018. FIELD STRENGTH/SEQUENCE: 3.0T using turbo spin echo (TSE) imaging, single-shot echoplanar diffusion-weighted imaging, and T1 -weighted high-resolution-isotropic-volume-examination (THRIVE) contrast-enhanced imaging. ASSESSMENT: We calculated the probability of extraprostatic extension (EPE) using the PT and MSKCC, as well as the CAPRA score. Three readers with 2-8 years of experience in mpMRI independently staged PCa on imaging. STATISTICAL TESTS: Receiver operating characteristics analysis and logistic regression analysis to investigate the per-patient accuracy of mpMRI vs. clinical models vs. mpMRI plus clinical models in predicting stage ≥pT3. The alpha level was 0.05. RESULTS: Median probability for EPE and MSKCCn was 27.3% and 47.0%, respectively. Median CAPRA score was 3. Stage ≥pT3 occurred in 32.9% of patients. Areas under the curve (AUCs) were 0.62 for PT, 0.62 for MSKCCn, 0.64 for CAPRA, and 0.73-0.75 for mpMRI (readers 1-3) (P > 0.05 for all comparisons). Compared with mpMRI, the combination of mpMRI with PT or MSKCCn provided lower AUCs (P > 0.05 for all the readers), while the combination with CAPRA provided significantly higher (P < 0.05) AUCs in the case of readers 1 and 3. On multivariable analysis, mpMRI by reader 1 was the only independent predictor of stage ≥pT3 (odds ratio 7.40). DATA CONCLUSION: mpMRI was more accurate than clinical models and mpMRI plus clinical models in predicting stage ≥pT3, except for the combination of mpMRI and CAPRA in two out of three readers. LEVEL OF EVIDENCE: 2 Technical Efficacy: Stage 2 J. Magn. Reson. Imaging 2019;50:1604-1613.

Interreader agreement of PI-RADS v. 2 in assessing prostate cancer with multiparametric MRI: A study using whole-mount histology as the standard of reference.

BACKGROUND: Most studies assessing interreader agreement of Prostate Imaging Reporting and Data System v. 2 (PI-RADS v2) have used biopsy as the standard of reference, thus carrying the risk of not definitively noting all existent cancers. PURPOSE: To evaluate the interreader agreement in assessing prostate cancer (PCa) of PI-RADS v2, using whole-mount histology as the standard of reference. STUDY TYPE: Monocentric prospective cohort study. POPULATION: In all, 48 patients with biopsy-proven PCa referred for radical prostatectomy, undergoing staging multiparametric magnetic resonance imaging (mpMRI) between May 2016 to February 2017. FIELD STRENGTH/SEQUENCE: 3.0T system using high-resolution T2 -weighted imaging, diffusion-weighted imaging (echo-planar imaging with maximum b-value 2000 sec/mm2 ), and dynamic contrast-enhanced imaging (T1 -weighted high resolution isotropic volume examination; THRIVE) ASSESSMENT: Three radiologists blinded to final histology (2-8 years of experience) analyzed mpMRI images independently, scoring imaging findings in accordance with PI-RADS v2. On a per-lesion basis, we calculated overall and pairwise interreader agreement in assigning PI-RADS categories, as well as assessing malignancy with categories ≥3 or ≥4, and stage ≥pT3. STATISTICAL TESTS: Cohen's kappa analysis of agreement. RESULTS: On 71 lesions found on histology, there was moderate agreement in assigning PI-RADS categories to all cancers (k = 0.53) and clinically significant cancers (csPCa) (k = 0.47). Assessing csPCa with PI-RADS ≥4 cutoff provided higher agreement than PI-RADS ≥3 cutoff (k = 0.63 vs. 0.57). Interreader agreement was higher between more experienced readers, with the most experienced one achieving the highest cancer detection rate (0.73 for csPCa using category ≥4). There was substantial agreement in assessing stage ≥pT3 (k = 0.72). DATA CONCLUSION: We found moderate to substantial agreement in assigning the PI-RADS v2 categories and assessing the spectrum of cancers found on whole-mount histology, with category 4 as the most reproducible cutoff for csPCa. Readers' experience influenced interreader agreement and cancer detection rate. LEVEL OF EVIDENCE: 2 Technical Efficacy: Stage 2 J. Magn. Reson. Imaging 2019;49:546-555.

The Simplified PADUA REnal (SPARE) nephrometry system: a novel classification of parenchymal renal tumours suitable for partial nephrectomy.

OBJECTIVE: To simplify the original Preoperative Aspects and Dimensions Used for an Anatomical (PADUA) classification of renal tumours, generating a new system able to predict equally or better the risk of overall complications in patients undergoing partial nephrectomy (PN); and to test if the addition of the contact surface area (CSA) parameter improves the accuracy of the original PADUA and new Simplified PADUA REnal (SPARE) nephrometry classification systems. PATIENTS AND METHODS: We analysed the clinical records of 531 patients who underwent PN (open, laparoscopic and robot-assisted) for renal tumours at five tertiary academic referral centres from January 2014 to December 2016. The ability of each variable included in the PADUA classification to predict overall complications was tested using binary logistic regression analysis. The variables that were not statistically significant were excluded from the SPARE classification. In addition to the original PADUA and SPARE systems, another two models were generated adding tumour CSA. Receiver operating characteristic curve analysis was used to compare the ability of the four different models to predict overall complications. Binary logistic regression was used to perform both univariable and multivariable analyses looking for predictors of postoperative complications. Linear regression analysis was used to identify independent predictors of absolute change in estimated glomerular filtration rate (eGFR; ACE). RESULTS: The SPARE nephrometry score system including: (i) rim location, (ii) renal sinus involvement, (iii) exophytic rate, and (iv) tumour dimension; showed equal performance in comparison with the original PADUA score (area under the curve [AUC] 0.657 vs 0.664). Adding tumour CSA to the original PADUA (AUC 0.661) or to the SPARE (AUC 0.658) scores did not increase the accuracy of either system to predict overall complications. The SPARE system (odds ratio 1.2, 95% confidence interval 1.1-1.3) was an independent predictor of postoperative overall complications. Age (P < 0.001), body mass index (P < 0.001), Charlson Comorbidity Index (P = 0.02), preoperative eGFR (P < 0.001), and tumour CSA (P = 0.005) were independent predictors of ACE. Limitations include the retrospective design and the lack of central imaging review. CONCLUSIONS: The new SPARE score is comprised of only four variables instead of the original six and its accuracy to predict overall complications is similar to that of the original PADUA score. Addition of tumour CSA was not associated with an increase in prognostic accuracy. The SPARE system could replace the original PADUA score to evaluate the complexity of tumours suitable for PN.

Tumour contact surface area as a predictor of postoperative complications and renal function in patients undergoing partial nephrectomy for renal tumours.

OBJECTIVES: To evaluate the ability of original tumour contact surface area (CSA) to predict postoperative complications and renal function impairment in a series of patients who underwent elective partial nephrectomy (PN) for renal masses. MATERIALS AND METHODS: We analysed the clinical records of 531 consecutive patients who underwent elective PN because of a suspicion of kidney cancer at five academic, high-volume centres between January 2014 and December 2016. Each participating centre evaluated prospectively the radiological images to evaluate the CSA and to assign a PADUA score. Several expert surgeons performed the surgical procedures in each participating centre. Binary logistic regression was used to perform both univariable and multivariable analyses to identify predictors of postoperative complications. Linear regression analysis was used to identify independent predictors of absolute change in estimated glomerular filtration rate (eGFR; ACE). RESULTS: The median (interquartile range) CSA value was 14.2 (7.4-25.1) cm2 . A total of 349 tumours (65.7%) had a CSA ≤ 20 cm2 and the remaining 182 (34.3%) had a CSA > 20 cm2 . PNs were performed using an open approach in 237 (44.6%) cases, a pure laparoscopic approach in 152 cases (28.6%), and a robot-assisted approach in the remaining 142 cases (26.7%). Multivariable analyses found that only age (odds ratio [OR] 1.037, 95% confidence interval [CI] 1.018-1.057) and PADUA score (OR 1.289, 95%CI 1.132-1.469) were independent predictors of postoperative complications. Tumour CSA (OR 1.020, 95%CI 1.010-1.030) was found to be an independent predictor of postoperative complications only when PADUA score was removed from the model. Age (from -0.639 to -0.306; P < 0.001); body mass index (from 0.267 to 1.076; P = 0.001), age-adjusted Charlson score (from -3.193 to -0.259; P = 0.02), preoperative eGFR value (from -0.939 to -0.862; P < 0.001) and tumour CSA (from -0.260 to -0.048; P = 0.005) were found to be independent predictors of ACE. CONCLUSIONS: Tumour CSA is an independent predictor of postoperative renal function. Conversely, at multivariable analysis, PADUA score outperformed tumour CSA to predict postoperative complications after PN. The complexity of The Leslie et al. formula for calculating tumour CSA is a potential limitation with regard to its diffusion and application in clinical practice.

Surgical Treatment of Eosinophilic Cystitis in Adults: A Report of Two Cases and a Literature Review.

Eosinophilic cystitis is a rare disease that presents with either urinary frequency, hematuria, suprapubic pain or urinary retention. Although benign, this entity may progress to diffuse bladder involvement with the need for surgical treatment. We report on 2 cases of advanced disease that required cystectomy with very complex lower urinary tract reconstruction, and review the literature of surgically treated cases.

Perioperative Outcomes and Early Survival in Octogenarians Who Underwent Radical Cystectomy for Bladder Cancer.

OBJECTIVE: To evaluate perioperative outcomes and early survival in a series of octogenarians who underwent radical cystectomy (RC) and urinary diversion for bladder cancer. PATIENTS AND METHODS: We retrospectively evaluated the clinical records of 44 patients aged ≥80 years who underwent open RC and urinary diversion at 2 high-volume centers between July 2013 and December 2015. Estimated blood loss (EBL), transfusion rate, and length of hospital stay (LOS) were evaluated. Ninety-day postoperative complications were stratified according to the type of urinary diversion. Univariable analysis was performed to identify predictors of overall and major complications. Overall survival (OS) was estimated using the Kaplan-Meier method. RESULTS: Median age was 83 years (interquartile range [IQR] 81-85). Age-adjusted Charlson score was ≥4 in 37 (84%) patients, and American Society of Anesthesiologists score was ≥3 in 34 (77%) patients. Ileal conduit (IC) was performed in 21/44 (48%) cases, cutaneous ureterostomy (CU) in 20/44 (45%), and no urinary diversion was required for 3 (7%) dialytic patients. Median EBL was 700 mL (IQR 500-1,000) and 23 (52%) patients required blood transfusion. Median LOS was 13 days (IQR 10-18). Overall complications were recorded in 29 (66%) patients, with major complications observed in 12 (27%), with death occurring in 1. No differences in complications were observed between IC and CU. The 2-year OS estimate was 62.5%. CONCLUSIONS: Open RC in octogenarians has an acceptable rate of major complications and mortality. IC should be considered a good urinary diversion in these patients.

Urethral-fixation technique improves early urinary continence recovery in patients who undergo retropubic radical prostatectomy.

OBJECTIVES: To describe step-by-step an original urethrovesical anastomosis technique (urethral fixation) in patients undergoing retropubic radical prostatectomy (RRP), to compare their early urinary continence recovery with those in a control group receiving a standard anastomosis technique and to identify the predictors of early urinary continence recovery. PATIENTS AND METHODS: We compared 70 patients who underwent RRP with the urethral-fixation technique with a contemporary control group of 51 patients who received RRP with a standard urethrovesical anastomosis. In the urethral-fixation group, the urethrovesical anastomosis was made using eight single sutures. Specifically, to avoid retraction and/or deviations, we fixed the urethral stump laterally to the medial portion of levator ani muscle. Also, to maintain the normal position in the context of the pelvic floor, we fixed the urethral sphincter deeper to the medial dorsal raphe using a 3-0 polydioxanone suture at the 6 o'clock position before completing the incision of the urethral wall. Urinary continence recovery was evaluated at 1, 4, 8 and 12 weeks after catheter removal. Patients self-reporting no urine leak were considered continent. Uni- and multivariable analyses were used to identify predictors of urinary incontinence at the different follow-up time-points. RESULTS: The evaluated groups had comparable preoperative variables. At 1 week after catheter removal, 32 (45.7%) patients in the urethral-fixation group and 10 (19.6%) in the control group were continent (P = 0.01). At 4 weeks after catheter removal, 46 (65.7%) patients in the urethral-fixation group and 16 (31.4%) in the control group were continent (P = 0.001). At 8 weeks after catheter removal, 59 (84.3%) patients in the urethral-fixation group and 21 (41.2%) in the control group were continent (P < 0.001). Finally, at 12 weeks after catheter removal, 63 (90%) patients in the urethral-fixation group and 32 (62.7%) in the control group were continent (P = 0.001). The urethral-fixation technique was an independent predictor of urinary continence recovery at 1 week [odds ratio (OR) 4.305; P = 0.002); 4 weeks (OR 4.784; P < 0.001); 8 weeks (OR 7.678; P < 0.001) and 12 weeks (OR 5.152; P = 0.001) after catheter removal. CONCLUSIONS: The urethral-fixation technique significantly improves early urinary continence recovery in comparison with the standard technique. Moreover, our study confirmed that this surgical technique is an independent predictor of urinary continence recovery at 1, 4, 8 and 12 weeks after catheter removal.

Anatomical study of renal arterial vasculature and its potential impact on partial nephrectomy.

OBJECTIVES: To validate Graves' classification of the intrarenal arteries and to verify the absence of collateral arterial blood supply between different renal segments, in order to maximize peri-operative and functional outcomes of partial nephrectomy. MATERIALS AND METHODS: The study was performed on 15 normal kidneys sampled from eight unembalmed cadavers. Kidneys with the surrounding perirenal fat tissue were removed en bloc with the abdominal segment of the aorta. The renal artery was injected with acrylic and radiopaque resins, with the specimen suspended in water. CT examination of the injected kidneys was performed to analyse the branches located deeply. After imaging acquisition, the specimens were treated with sodium hydroxide for removal of the parenchyma to obtain vascular casts. RESULTS: Ten casts (66.6%) showed the classic subdivision of the main artery into single posterior and anterior branches. With regard to the distribution of the segmental or second-order arteries, only two casts (13%) showed a pattern similar to that described by Graves, characterized by four segmental (second-order) branches coming from the anterior renal artery (apical, superior, middle and inferior). In the remaining 13 kidneys (87%) a different arterial vascular network was detected. In 10 casts (80%) a single renal segment was vascularized by two or more different branches coming from an artery leading to another segment (multiple vascularization). Multiple vascularization was observed in three (20%) apical segments, five (33%) superior segments, six (40%) middle segments, seven (47%) inferior segments and two (13%) posterior segments. CONCLUSIONS: This study shows that in the human kidneys the arterial vasculature is frequently different from that described by Graves. Moreover, in a significant percentage of cases, a single renal segment receives two or more branches that originate from an artery leading to another segment.

Anesthesiologic effects of transperitoneal versus extraperitoneal approach during robot-assisted radical prostatectomy: results of a prospective randomized study.

OBJECTIVES: To compare the effects of CO(2) insufflation on hemodynamics and oxygen levels and on acid-base level during Robot-Assisted Radical Prostatectomy (RARP) with transperitoneal (TP) versus extra-peritoneal (EP) accesses. MATERIALS AND METHODS: Sixty-two patients were randomly assigned to TP (32) and EP (30) to RARP. Pre-operation data were collected for all patients. Hemodynamic, respiratory and blood acid-base parameters were measured at the moment of induction of anesthesia (T0), after starting CO(2) insufflation (T1), and at 60 (T2) and 120 minutes (T3) after insufflation. In all cases, the abdominal pressure was set at 15 mmHg. Complications were reported according to the Clavien-Dindo classification. Student's two-t-test, with a significance level set at p<0.05, was used to compare categorical values between groups. The Mann-Whitney U-test was used to compare the median values of two nonparametric continuous variables. RESULTS: The demographic characteristics of the patients in both groups were statistically comparable. Analysis of intra-operative anesthesiologic parameters showed that partial CO(2) pressure during EP was significantly higher than during TP, with a consequent decrease in arterial pH. Other parameters analysed were similar in the two groups. Postoperative complications were comparable between groups. The most important limitations of this study were the small size of the patient groups and the impossibility of maintaining standard abdominal pressure throughout the operational phases, despite attempts to regulate it. CONCLUSIONS: This prospective randomized study demonstrates that, from the anesthesiologic viewpoint, during RARP the TP approach is preferable to EP, because of lower CO(2) reabsorption and risk of acidosis.

The relationship between characteristics of inguinal lymph nodes and pelvic lymph node involvement in penile squamous cell carcinoma: a single institution experience.

PURPOSE: We determined predictors of pelvic lymph node metastases in patients with penile cancer. MATERIALS AND METHODS: We retrieved a total of 188 node positive inguinal groins from 142 patients treated for penile cancer. Logistic regression models were fitted to test for predictors of pelvic lymph node metastases. The minimum p value method was used to determine the most significant cutoff values of each predictor. RESULTS: Pelvic lymph node metastases were observed in 45 cases (31.7%). The 5-year cancer specific survival rate was 71.0% vs 33.2% in patients with inguinal vs pelvic lymph node metastases. The most significant cutoff values were 3 inguinal lymph node metastases and a metastasis diameter of 30 mm. According to univariable logistic regression models the number of inguinal metastases (OR 1.92, p <0.001), the diameter of the metastases (OR 1.03, p = 0.001) and extranodal extension (OR 8.01, p <0.001) were significant predictors of pelvic lymph node metastases. These variables were also independent predictors of metastases in multivariable logistic regression models (p ≤ 0.012). Patients with 3 or more inguinal lymph node metastases and those with a metastasis diameter of 30 mm or greater were at 4.77 and 2.53-fold higher risk, respectively, of harboring pelvic lymph node metastases (p ≤ 0.006). The proportion of metastases increased significantly from 0% in cases with no risk factors to 57.1% when all 3 risk factors were observed (p <0.001). CONCLUSIONS: The number and diameter of inguinal lymph node metastases as well as extranodal extension are significantly associated with pelvic lymph node metastases. These variables should be considered to determine the need for pelvic lymph node dissection. Patients with no risk factors may be spared this dissection.

Peripheral primitive neuroectodermal tumor of seminal vesicles: is there a role for relatively aggressive treatment modalities?

A 50 year old white man received an incidental ultrasound diagnosis of hypoechoic mass interesting the right seminal vesicle. A CT scan showed the presence of a 7.8 cm roundish cyst, originating from the right seminal vesicle. He had been followed by the removal of the right seminal vesicle and both the cystic lesion. The histological findings of the specimen documented the presence of small round cells compatible with Ewing's sarcoma/PPNET. The patient received also adjuvant chemotherapy and radiation treatment. After 10 years, the follow-up is still negative.

How To Report the Minor Component of a High-grade Pattern in Radical Prostatectomy Specimens: Time To Abandon the "Tertiary" Terminology?

The International Society of Urological Pathology and Genitourinary Pathology Society differ in their recommendations for reporting of minor components of high-grade pattern in prostatectomy specimens. This can affect the grade group assigned, particularly when there are only two Gleason patterns in a cancer nodule. We therefore argue that the term "tertiary" should be changed to "minor" component.

Predictive and prognostic biomarkers in urological tumours.

Advancements in cutting-edge molecular profiling techniques, such as next-generation sequencing and bioinformatic analytic tools, have allowed researchers to examine tumour biology in detail and stratify patients based on factors linked with clinical outcome and response to therapy. This manuscript highlights the most relevant prognostic and predictive biomarkers in kidney, bladder, prostate and testicular cancers with recognised impact in clinical practice. In bladder and prostate cancer, new genetic acquisitions concerning the biology of tumours have modified the therapeutic scenario and led to the approval of target directed therapies, increasing the quality of patient care. Thus, it has become of paramount importance to choose adequate molecular tests, i.e., FGFR screening for urothelial cancer and BRCA1-2 alterations for prostate cancer, to guide the treatment plan for patients. While no tissue or blood-based biomarkers are currently used in routine clinical practice for renal cell carcinoma and testicular cancers, the field is quickly expanding. In kidney tumours, gene expression signatures might be the key to identify patients who will respond better to immunotherapy or anti-angiogenic drugs. In testicular germ cell tumours, the use of microRNA has outperformed conventional serum biomarkers in the diagnosis of primary tumours, prediction of chemoresistance, follow-up monitoring, and relapse prediction.

Spermatic cord sarcoma: our experience and review of the literature.

INTRODUCTION: Spermatic cord tumors represent 4% of scrotal tumors. The most common neoplasms are lipomas. Spermatic cord sarcomas (SCS) of the genitourinary tract account for 2% of all urological tumors. Herein we presented our experience in the treatment of these tumors and a review of the literature. PATIENTS AND METHODS: A review of the literature was performed using the Medline database with no restriction on language and date of published papers. The literature search used the following terms: epidemiology, surgery, chemotherapy, radiotherapy and spermatic cord sarcomas. Four cases treated from December 2009 to May 2010 are described. RESULTS: All patients were treated with radical orchiectomy. The final pathological report showed different types of sarcomas. Two of the patients were treated with adjuvant chemotherapy. 12 months after surgery, 2/4 patients were alive without signs of relapse. CONCLUSION: SCS are very rare tumors with a poor prognosis. SCS's prognostic factors have been identified in grading, size, depth of invasion and surgical margin status. Age and performance status of the patient are however very important. Lymphatic and hematogenous dissemination is uncommon. Surgery is the most important treatment both in the first approach and in local relapse. The role of adjuvant chemotherapy and radiation therapy is still debated.