Single-institution Series Research Articles

Partial versus total adrenalectomy for pheochromocytoma: a population-based comparison of outcomes

The literature assessing outcomes of partial adrenalectomy (PA) among patients with pheochromocytoma patients is largely limited to isolated, single-institution series. We aimed to perform a population-level comparison of outcomes between patients undergoing PA versus those undergoing total adrenalectomy (TA). The Surveillance, Epidemiology, and End Results (SEER) database (1975-2016) was queried to identify adults with pheochromocytoma who underwent either PA or TA. Survival was assessed using multivariable Cox proportional hazards regression, Fine and Gray competing-risks regression, propensity score matching, Kaplan-Meier analysis, and cumulative incidence plots. 286 patients (PA: 101, TA: 185) were included in this study. As compared to those undergoing TA, patients undergoing PA had fewer tumors ≥ 8cm in size (28.7% versus 42.7%, p = 0.048) and were more likely to have localized disease (61.4% versus 44.3%, p = 0.01). In multivariable analysis, patients undergoing PA demonstrated similar all-cause mortality (HR = 0.71, 95% CI 0.44-1.14, p = 0.16) and cancer-specific mortality (HR = 0.64, 95% CI 0.35-1.17, p = 0.15) compared to those who underwent TA. Following 1:1 propensity score matching, Kaplan-Meier analysis revealed no difference in overall survival between PA and TA groups (p = 0.26) nor was there a difference in the cumulative incidence of cancer-specific mortality (p = 0.29). In this first population-level comparison of outcomes among patients with pheochromocytoma undergoing PA and those undergoing TA, we found no long-term differences in any survival metric between groups. PA circumvents the need for lifelong corticoid replacement therapy and remains a promising option for patients with bilateral or recurrent pheochromocytoma.

Combined Treatment of High-Flow Scalp Arteriovenous Malformations.

Appropriate treatment of scalp arteriovenous malformations (SAVMs) remains largely unclear given the rarity of reported cases. This single-institution case series presents consecutive patients with extracranial SAVMs and long-term follow up.The primary aim of this study was to review treatment decisions, evaluate clinical outcomes, and compare our experience to available literature in order to better understand SAVMs and improve future outcomes. A retrospective review of consecutive patients with extracranial SAVMs between January 2015 and December 2019 was performed. Treatment factors of interest included embolization method, embolic agents, and decision to proceed with surgical resection. Relevant clinical outcomes included recurrence rates, cure rates, and complications. Seven patients were included in the present series. Satisfactory outcomes were ultimately achieved for all 7 patients using embolization with or without combined surgical resection. Embolizations performed in combination with resection were curative in 4 of 4 cases (100%). Embolizations performed without resection were curative in 0 of 4 cases (0%), satisfactory in 2 of 4 cases (50%), and unsatisfactory in 2 of 4 cases (50%). All SAVM patients in this series were treated satisfactorily using embolization with or without operative resection. This study strongly suggests that embolization combined with surgical resection during the same admission yields the best treatment outcomes for SAVMs, specifically demonstrating lower rates of recurrence and embolization-related complications. Decisions on whether or not to proceed with resection should be made by the interventional radiologist, the plastic surgeon, and the patient together.

Predicting Strict Trifecta Outcomes after Robot-Assisted Partial Nephrectomy: Comparison of RENAL, PADUA, and C-Index Scores.

Nephrometry scores are designed to characterize tumors and stratify the surgical complexity. It remains unclear as to which nephrometry score can accurately predict the surgical outcomes. We aimed to assess the utility of radius, exophytic/endophytic, nearness, anterior/posterior, location (RENAL), preoperative aspects and dimensions used for anatomic classifications (PADUA), and centrality index (C-index) nephrometry scores for predicting the strict Trifecta achievement from a single institution series robotic-assisted partial nephrectomy (RAPN). We retrospectively identified the prospectively maintained robotic surgery database records of 91 patients who underwent RAPN between June 2015 and September 2020 in Antalya Training and Research Hospital. The main outcome of the study was the achievement of strict Trifecta (negative surgical margin, no major urologic complications, warm ischemia time ≤25 min, and ≥85% preservation of estimated glomerular filtration rate). A multivariable analysis was performed to identify the factors of strict Trifecta success. The mean patient age was 55.82 ± 13.37 years with a median clinical tumor size of 3.5 cm (IQR 2.5–4.9). The median RENAL, PADUA, and C-index score were 7(IQR 6–8), 8(IQR 7–10), and 2.01(IQR 1.64–2.72), respectively. A strict Trifecta could be achieved in 54 patients (59.3%). Clinical tumor size (P = 0.011), RENAL risk groups (low:reference; intermediate; P = 0.040; high; P = 0.009), PADUA risk groups (low:reference; intermediate; P = 0.044; high; P = 0.001) and C-index risk groups (low:reference; high; P = 0.015) were the independent predictors of strict Trifecta attainment in the multivariate analysis. None of the nephrometry scores were a superior predictor compared to other nephrometry scores in comparative analysis. RENAL, PADUA, and C-index scores were all independent predictors of a strict Trifecta achievement. Our comprehensive comparison of the three scores identified that none of the nephrometry scores proved to be inferior to others nephrometry scores.

P-194: Pomalidomide-based therapy in Relapsed/Refractory Multiple Myeloma: a real-world single-center experience

P-194: Pomalidomide-based therapy in Relapsed/Refractory Multiple Myeloma: a real-world single-center experience

MP48-16 PERIOPERATIVE AND ONCOLOGIC OUTCOMES AFTER CONCURRENT CYSTECTOMY AND NEPHROURETERECTOMY

You have accessJournal of UrologyBladder Cancer: Upper Tract Transitional Cell Carcinoma II (MP48)1 Sep 2021MP48-16 PERIOPERATIVE AND ONCOLOGIC OUTCOMES AFTER CONCURRENT CYSTECTOMY AND NEPHROURETERECTOMY Grayden Cook, Jeffrey Howard, Vitaly Margulis, Arthur Sagalowsky, Ganesh Raj, Solomon Woldu, Aditya Bagrodia, and Yair Lotan Grayden CookGrayden Cook More articles by this author , Jeffrey HowardJeffrey Howard More articles by this author , Vitaly MargulisVitaly Margulis More articles by this author , Arthur SagalowskyArthur Sagalowsky More articles by this author , Ganesh RajGanesh Raj More articles by this author , Solomon WolduSolomon Woldu More articles by this author , Aditya BagrodiaAditya Bagrodia More articles by this author , and Yair LotanYair Lotan More articles by this author View All Author Informationhttps://doi.org/10.1097/JU.0000000000002074.16AboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookLinked InTwitterEmail Abstract INTRODUCTION AND OBJECTIVE: Radical nephroureterectomy (RNU) and cystectomy (RC) are commonly performed in urologic oncology, but their concurrent use is rare. Here, we report the perioperative and oncologic outcomes of a single-institution series of concurrent RNU and RC. METHODS: We retrospectively reviewed our institutional RNU database to identify patients who underwent concurrent RNU/RC for malignancy between 2006 and 2020. Patient demographic and clinical factors, perioperative parameters, and oncologic outcomes were obtained by chart review. RESULTS: Twenty-seven patients underwent concurrent RNU/RC during the study period, of whom 22 (82%) were male. Median (IQR) patient age was 71 (66-76) years. The indication for concurrent RNU was upper tract urothelial carcinoma (UTUC) in 15 cases (56%) and nonfunctional kidney in 12 cases (44%). Eleven patients (41%) were rendered anephric by the procedure.Thirteen (48%) cases were performed by an open approach, nine (33%) via a robotic approach, and the remaining five (19%) by a hybrid (laparoscopic kidney / open bladder) approach. Median (IQR) estimated blood loss was 600 (250-1150) mL and operative time was 381 (290-408) minutes. Median (IQR) postoperative length of stay was 6 (4-9) days. Two patients (7.4%) experienced early postoperative mortality, one due to a myocardial infarction and the other due to sepsis from a deep surgical site infection. Notably, these deaths were in the two patients with the highest preoperative Charlson-Deyo comorbidity scores (9 and 10). Eight patients (30%) experienced a Clavien-Dindo grade 3 or 4 complication.Fourteen (52%) were alive at last follow-up, with median follow up of 33 months. Median progression-free (PFS) and overall survival (OS) were 32 and 47 months, respectively. Notably, 5 out of 22 male patients (23%) experienced a urethral recurrence at a median of 19 months postoperatively. CONCLUSIONS: We present one of the largest published series of outcomes following concurrent RNU/RC. There is a high risk of perioperative death in comorbid patients. A substantial proportion of patients do achieve long-term survival following concurrent RNU/RC, making it an appropriate option in management of carefully selected patients. Close surveillance of the remnant urethra is important in male patients given high rates of urethral recurrence. Source of Funding: This study was funded internally by the UTSW Department of Urology © 2021 by American Urological Association Education and Research, Inc.FiguresReferencesRelatedDetails Volume 206Issue Supplement 3September 2021Page: e871-e871 Advertisement Copyright & Permissions© 2021 by American Urological Association Education and Research, Inc.MetricsAuthor Information Grayden Cook More articles by this author Jeffrey Howard More articles by this author Vitaly Margulis More articles by this author Arthur Sagalowsky More articles by this author Ganesh Raj More articles by this author Solomon Woldu More articles by this author Aditya Bagrodia More articles by this author Yair Lotan More articles by this author Expand All Advertisement Loading ...

Shuntogram utility in predicting future shunt failures.

Shunt failure remains a challenging diagnosis for neurosurgeons, particularly when patient symptoms suggest shunt malfunction but radiographic evidence is lacking. In such situations, shuntograms are sometimes employed to guide medical decision-making. In this study, the authors aimed to investigate the utility of shuntograms in aiding patient management, particularly in terms of a negative result. This retrospective single-institution series comprises patients who underwent a shuntogram procedure to evaluate shunt patency over a roughly 6-year period. The medical records of patients were reviewed to determine the findings of the shuntogram procedure, the type of obstruction, and whether a subsequent operation for a shunt revision took place either within 30 days or up to 1 year after the procedure. Statistical analysis was completed by calculating the sensitivity, specificity, negative predictive value (NPV), and positive predictive value (PPV) of the shuntogram as compared with the revision surgery. Of the 98 patients who underwent a shuntogram, 95 patients were included in the statistical analysis. A ventriculoperitoneal shunt was assessed in 81% of patients (77/95). The PPV of the procedure was 100%. The NPV for predicting a subsequent shunt revision within 30 days was 68.3% (false-negative rate of 31.7%) or within 365 days was 61.2% (false-negative rate of 38.8%). The sensitivity and specificity of the shuntogram were 55.8% and 100% within 30 days and 51.9% and 100% within 365 days, respectively. The most common intervention at the time of surgery following a negative shuntogram was a valve replacement in 38.5% of patients (10/26). With an NPV of 68.3% for predicting revision within 30 days in our series of 95 patients, shuntograms remained a useful tool in the clinical decision-making process when evaluating potential shunt failure.

Endolymphatic sac tumor: single-institution series of seven cases with updated review of literature.

Endolymphatic sac tumour (ELST) is a rare low-grade locally aggressive neoplasm arising from the endolymphatic duct or sac. It presents mostly with vestibulo-cochlear symptoms either sporadically or as part of von Hippel-Lindau (VHL) syndrome. Micro-neurosurgical excision remains the cornerstone of therapy with the role of radiotherapy (RT) being controversial. This is a clinico-pathological analysis of consecutive ELST patients presenting to a single-institution in India. Neuropathology database of a tertiary-care comprehensive cancer centre was searched electronically to identify consecutive patients with histopathological diagnosis of ELST registered at the institute over last one decade. Data regarding demographic profile, clinical presentation, histopathological features, treatment details and outcomes were retrieved from electronic medical records for this retrospective analysis. Electronic search identified seven unique patients with biopsy-proven ELST registered at the institute between 2009 and 2020. Median age of the study cohort was 39years (range 24-65years) with strong male predilection (5:2 ratio) and left-sided preponderance (71%). Most common presenting symptoms were hearing loss (86%) and earache (71%) on affected side followed by headache (43%). All patients underwent maximal safe resection at initial diagnosis and were followed-up closely with periodic surveillance imaging. Two patients underwent salvage RT using high-precision conformal techniques at recurrence/progression. ELST is a rare low-grade locally aggressive neoplasm that arises generally as part of VHL syndrome or sometimes sporadically. Gross total resection provides the best chance of cure with RT being reserved for unresectable disease, large residue, medical inoperability, or as salvage therapy for recurrent/progressive tumor.

Clinical Features of Pineal Parenchymal Tumors of Intermediate Differentiation (PPTID): A Single-Institution Series

Pineal parenchymal tumors of intermediate differentiation (PPTID) are rare tumors of the pineal gland. Their treatment is often heterogeneous due to the lack of literature to compile standardized treatments. Although no single institution has large numbers of cases, our experience has been that the clinical course is more varied and complicated than reported. We reviewed the clinical data for all patients with pathology found to be consistent with PPTID at our institution between the years 2006 and2019. Nine patients were identified. At initial diagnosis, all were treated with surgery and 4 of 9 patients underwent gross total resection. Adjuvant radiation therapy to the resection bed was administered in 6 of 9 patients. Mean follow-up time was 95.3 months. Mean progression-free survival was 50.5 months, with a tendency to be longer for male sex and after gross total resection. Seven patients developed a recurrence. Five of 6 known locations of first recurrences had either distant metastases or dissemination of disease. First recurrences were treated with radiation alone in 5 patients, craniospinal radiation with multiagent chemotherapy in 1 patient, and surgery with radiation therapy in 1. At last follow-up, 2 patients had died. Herein, we report clinical patterns of disease progression and treatment patterns of PPTID. Many patients progressed during the follow-up period. Disseminated disease was the most common presentation at recurrence. Ultimately, given the risk of recurrence and dissemination at recurrence, more aggressive treatment strategies should be considered. Specifically, our series suggests a benefit of adjuvant radiation at initial diagnosis for grade II patients.

Carotid Stent Explant Indications and Outcomes

Carotid artery stenting is an alternative to carotid endarterectomy, especially in patients deemed to have significant anatomic or medical risk for the latter. There is scant literature, however, on indications for and outcomes of carotid stent (CAS) explant. We sought to determine indications and outcomes of CAS explant at our institution. We queried a prospectively maintained institutional vascular surgery database as well as hospital records to identify patients undergoing carotid stent explant from 2010-2020. Ten patients were identified. Their charts were reviewed to obtain demographic, comorbidity, procedural, and outcomes data. Data of particular interest were preoperative atherosclerotic factors, indications for CAS explant, carotid repair strategy, and 30-day post-procedural complications. Data were analyzed for ranges and means. Ten patients were identified. Comorbidity was common: all patients had at least 1 atherosclerotic risk factor, with hypertension and hypercholesterolemia being the most common. Seven (70.0%) patients had recurrent stenosis as the indication for explant. Seven (70.0%) presented symptomatically. Five (50.0%) cases of restenosis had been refractory to angioplasty. There were 3 (30.0%) instances of CAS explant due to infection. Only 1 (12.5%) index CAS was performed at our institution. There was a mean hospital length of stay of 4.5 days. One patient had vocal cord paralysis requiring no intervention. There was no (0%) 30-day mortality, stroke, or postoperative wound infections. Our series had 0% 30-day mortality, stroke, and postoperative wound infection suggesting that CAS explant may be performed safely. Our series is small but represents the largest single institution series to date. This procedure may become increasingly common in the coming years with the more frequent use of CAS; additional data is needed to rigorously understand outcomes.

One-Year Outcomes of Micropulse Cyclophototherapy for Primary Open-angle Glaucoma.

In primary open-angle glaucoma (POAG), micropulse trans-scleral cyclophototherapy (MPTCP) is effective in lowering intraocular pressure (IOP), but its effects are not permanent. Hence, it can serve as a temporizing measure before definitive glaucoma surgery. There is limited data on MPTCP in POAG. This is the first study that looks at MPTCP treatment specifically in POAG patients. This is an interventional, single-institution exploratory case series with 55 eyes of 48 patients with POAG. Data was collected from clinical records, including patient demographics, clinical information, number of glaucoma medications, MPTCP laser settings, complications, and clinical outcomes. Patients had a mean age of 67.3±14.1 years with a preponderance of males. IOP was 24.8±1.0 mm Hg before MPTCP and decreased to 19.7±1.1, 21.9±1.1, and 21.8±1.1 mm Hg at postoperative month 3, 6, and 12 respectively. IOP remained below pretreatment levels throughout the postoperative period (P<0.05). Visual acuity and mean deviation remained stable before and after MPTCP. No eyes had complications. Number of glaucoma medications remained the same after MPTCP. Four eyes required additional oral acetazolamide at postoperative month 1 for IOP control. Seventeen eyes subsequently required further surgical intervention after 9.84 months. Maximal IOP decrease was greater when there were higher power settings, higher preoperative IOP, and better preoperative visual acuity. The IOP lowering effect of MPTCP treatment in patients with POAG was found to be modest and transient with a similar medication burden, and definitive glaucoma surgery was needed in a number of patients.

Combination of AST to ALT and neutrophils to lymphocytes ratios as predictors of locally advanced disease in patients with bladder cancer subjected to radical cystectomy: Results from a single-institutional series.

Bladder cancer (BC) staging is challenging. There is an important need for available and affordable predictors to assess, in combination with imaging, the presence of locally-advanced disease. To determine the role of the De Ritis ratio (DRR) and neutrophils to lymphocytes ratio (NLR) in the prediction of locally-advanced disease defined as the presence of extravescical extension (pT ⩾ 3) and/or lymph node metastases (LNM) in patients with BC treated with radical cystectomy (RC). We retrospectively analyzed clinical and pathological data of 139 consecutive patients who underwent RC at our institution. Logistic regression models (LRMs) were fitted to test the above-mentioned outcomes. A total of 139 consecutive patients underwent RC at our institution. Eighty-six (61.9%) patients had a locally-advanced disease. NLR (2.53 and 3.07; p = 0.005) and DRR (1 and 1.17; p = 0.01) were significantly higher in patients with locally-advanced disease as compared to organ-confined disease. In multivariable LRMs, an increasing DRR was an independent predictor of locally-advanced disease (OR = 3.91; 95% CI: 1.282-11.916; p = 0.017). Similarly, an increasing NLR was independently related to presence of locally-advanced disease (OR = 1.28; 95% CI: 1.027-1.591; p = 0.028). In univariate LRMs, patients with DRR > 1.21 had a higher risk of locally advanced disease (OR = 2.83; 95% CI: 1.312-6.128; p = 0.008). Similarly, in patients with NLR > 3.47 there was an increased risk of locally advanced disease (OR = 3.02; 95% CI: 1.374-6.651; p = 0.006). In multivariable LRMs, a DRR > 1.21 was an independent predictor of locally advanced disease (OR = 2.66; 95% CI: 1.12-6.35; p = 0.027). Similarly, an NLR > 3.47 was independently related to presence of locally advanced disease (OR = 2.24; 95% CI: 0.95-5.25; p = 0.065). No other covariates such as gender, BMI, neoadjuvant chemotherapy or diabetes reached statistical significance. The AUC of the multivariate LRM to assess the risk of locally advanced disease was 0.707 (95% CI: 0.623-0.795). Limitations include the retrospective nature of the study and the relatively small sample size.

PD-0910 Radical radiotherapy in lymph node or bone metastatic prostate cancer: a single institution series

PD-0910 Radical radiotherapy in lymph node or bone metastatic prostate cancer: a single institution series

When laparoscopic repair is feasible for diaphragmatic hernia in adults? A retrospective study and literature review.

Diaphragmatic hernia (DH), congenital or traumatic, is uncommon but sometimes can lead to a serious surgical emergency. There are no clinical guidelines or approved recommendations for the management of this condition, and most data are from retrospective, single-institution series. The aim is to analyze the management of the DH at our institution and review the indications for laparoscopic repair. A retrospective serie of patients diagnosed of DH with surgical treatment at our institution between 2009 and 2019. Literature review was carried out to establish the current indications of laparoscopic repair in each type of DH. Surgery was carried out in 15 patients with DH, 5 congenital and 10 traumatic hernias. Traumatic hernias were classified as acute (n = 2) and chronic (n = 8). 53.4% of all cases (8 patients) required urgent surgery using an abdominal approach (5 open and 3 laparoscopic) and elective surgery was performed in 46.6% of all cases (7 patients) with an abdominal approach (3 open and 4 laparoscopic) and 2 patients with a combined approach. Primary repair was performed in 4 patients (26.6%), closure and mesh reinforcement in 9 cases (60%) and only mesh placement in 2 patients (13.4%). Postoperative morbidity and mortality were 20% and 0%, respectively. No recurrences were detected. DH may pose different scenarios which require urgent or elective surgical treatment. Laparoscopic approach may be a first option in elective surgery; and in emergency setting taking into account hemodynamic stability and associated injuries.

Experience With Ventriculoperitoneal and Lumboperitoneal Shunting for the Treatment of Idiopathic Intracranial Hypertension: A Single Institution Series.

CSF shunting is among the most widely utilized interventions in patients with idiopathic intracranial hypertension (IIH). Ventriculoperitoneal shunting (VPS) and lumboperitoneal shunting (LPS) are 2 possible treatment modalities. To evaluate and compare complications, malfunction, infection, and revision rates associated with VPS compared to LPS. Electronic medical records were reviewed to identify baseline and treatment characteristics for patients diagnosed with IIH treated with VPS or LPS. A total of 163 patients treated with either VPS (74.2%) or LPS (25.8%) were identified. The mean follow-up was 35 mo. Shunt revision was required in 40.9% of patients. There was a nonsignificant higher rate of revision with LPS (52.4%) than VPS (36.4%, P=.07). In multivariate analysis, increasing patient age was associated with higher odds of shunt revision (P=.04). LPS had higher odds of shunt revision, yet this association was not significant (P=.06). Shunt malfunction was the main indication for revision occurring in 32.7%, with a significantly higher rate with LPS than VPS (P=.03). In total, 15 patients had shunt infection (9.4% VPS vs 12.2% LPS P=.50). The only significant predictor of procedural infection was the increasing number of revisions (P=.02). The incidence of shunt revision was 40.9%, with increasing patient age as the sole predictor of shunt revision. The incidence of shunt malfunction was significantly higher in patients undergoing LPS, while there was no significant difference in the incidence of shunt infection between the 2 modalities.

Laser interstitial thermal therapy (LITT) for intracranial lesions: a single-institutional series, outcomes, and review of the literature

Introduction Laser interstitial thermal therapy (LITT) is a minimally invasive treatment method in managing primary brain neoplasms, brain metastases, radiation necrosis, and epileptogenic lesions, many of which are located in operative corridors that would be difficult to address. Although the use of lasers is not a new concept in neurosurgery, advances in technology have enabled surgeons to perform laser treatment with the aid of real-time MRI thermography as a guide. In this report, we present our institutional series and outcomes of patients treated with LITT. Methods We retrospectively evaluated 19 patients (age range, 28–77 years) who underwent LITT at one or more targets from 2015 to 2019. Primary endpoint observed was mean progression free survival (PFS) and overall survival (OS). Results Seven patients with glial neoplasms and 12 patients with metastatic disease were reviewed. Average hospitalization was 2.4 days. Median PFS was 7 and 4 months in the metastatic group and primary glial neoplasm group, respectively (p = 0.01). Median OS from time of diagnosis was 41 and 32 months (p = 0.02) and median OS after LITT therapy was 25 and 24 months (p = 0.02) for the metastatic and primary glial neoplasm groups, respectively. One patient experienced immediate post-procedural morbidity secondary to increased intracerebral edema peri-lesionally while one patient experienced post-operative mortality and expired secondary to hemorrhage 1-month post-procedure. Median follow-up was 10 months. Conclusion Laser interstitial thermal therapy (LITT) is a safe, minimally invasive treatment method that provides surgeons with cytoreductive techniques to treat neurosurgical conditions. Both PFS and OS appear to be more favorable after LITT in patients with metastatic disease. In properly selected patients, this modality offers improved survival outcomes in conjunction with other salvage therapies.

Dysembryoplastic neuroepithelial tumors: A single-institutional series with special reference to glutamine synthetase expression

Dysembryoplastic neuroepithelial tumors (DNT) is a benign (World Health Organisation, WHO, grade I) glioneuronal tumor and it represent one of the most frequent neoplasm in patient affected by seizures. The epileptic neuronal activity can be determined by abnormal synchronization, excessive glutamate excitation and\\or inadequate GABA inhibition. Increasing evidence suggests that the astrocytes might be involved in this process even if neurons play a relevant role. In particular astrocytes promote the clearance of glutamate, a potent excitatory neurotransmitter of the central nervous system. Indeed, elevated concentrations of extracellular glutamate may determine iper-excitability and seizures as well as other neurological disorders. So, astrocytes, converting glutamate into glutamine via the enzyme glutamine synthetase (GS), could play a protective anti-seizures role. In the present study, we analyzed the immunohistochemical expression of GS in 20 DNTs specimens documenting a constant immunoistochemical expression of GS in astrocytes of the lesional tissue and of the cerebral cortex.

Only SF3B1 mutation involving K700E independently predicts overall survival in myelodysplastic syndromes.

SF3B1 mutations (SF3B1mut ) in myelodysplastic syndromes (MDS) frequently involve codon K700E and have a favorable prognosis. The prognostic effect of non-K700E SF3B1mut is uncertain. The authors analyzed the clinicopathological features and outcomes of a single-institution series of 94 treatment-naive SF3B1mut MDS patients (18%) and 415 treatment-naive SF3B1wt MDS patients and explored the differences between K700E and non-K700E SF3B1mut MDS. Fifty-five patients (59%) carried K700E. Recurrent non-K700E mutations (39 [41%]) included R625, H662, and K666. Compared with SF3B1mut K700E patients, non-K700E patients had a lower median absolute neutrophil count (1.8 vs 2.4; P = .005) and were frequently "high" according to the Revised International Prognostic Scoring System (19% vs 4%; P = .031). Non-K700E MDS was associated frequently with RUNX1 (26% vs 7%; P = .012) and exclusively with BCOR, IDH2, and SRSF2 mutations. A splicing analysis showed the differential distribution of alternatively spliced events and gene expression profiles between K700 and non-K700E MDS patients. The majority (at least 80%) of SF3B1mut K700E, SF3B1mut non-K700E, and SF3B1wt patients were treated with hypomethylating agents. Over a median follow-up of 16 months, SF3B1mut had superior overall survival (OS) in comparison with SF3B1wt in all MDS patients (not reached vs 25.2 months; P = .0003), in patients with low-grade MDS, and in patients with myelodysplastic syndromes with ring sideroblasts (MDS-RS). Compared with SF3B1wt , SF3B1mut K700E had superior outcomes in all MDS (median OS, 25 months vs not reached; P = .0001), in low-grade MDS (median OS, 41.3 months vs not reached; P = .0015), and in MDS-RS (median OS, 22.3 months vs not reached; P = .0001), but no significant difference was seen between non-K700E and SF3B1wt MDS. By multivariable analysis, the absence of SF3B1mut K700E mutations was independently associated with the prognosis. This study highlights the importance of the SF3B1 mutation subtype in MDS risk assessment. Myelodysplastic syndromes (MDS) with SF3B1 mutations are regarded as having a favorable prognosis by both the World Health Organization and the International Working Group for the Prognosis of Myelodysplastic Syndromes. However, this article shows that only MDS patients with SF3B1 K700E mutations have a favorable prognosis (and not MDS patients with SF3B1 mutations involving other codons). This has important implications for refining future MDS subclassification and risk assessment criteria.

Renal oncocytoma with adverse pathologic features: a clinical and pathologic study of 50 cases.

Renal oncocytoma is the most common benign epithelial renal neoplasm. Several adverse features that would typically increase the stage of renal cell carcinomas are not uncommon in renal oncocytoma, including perinephric, sinus fat, or renal vein invasion. Herein, we report the largest single institutional series of renal oncocytoma with adverse pathologic features. The cohort comprised 50 patients, 38 were men (76%) and 12 were women (24%), with a mean age of 68 years (range, 50-87 years). All cases were diagnosed on nephrectomy specimens. No laterality predilection was noted. The tumors ranged in size from 1.5-15.7 cm (mean, 5.3 cm). Adverse pathologic features included perinephric fat invasion (n = 25; 50%), renal sinus fat invasion (n = 9; 18%), and renal vein invasion (n = 5; 10%). More than one adverse feature was seen in 11 tumors (22%). All tumors showed diffuse reactions to KIT (n = 40; 100%) and cyclin D1 (n = 27; 100%). Keratin 7 highlighted rare (<5%) scattered cells, as well as entrapped renal tubules (n = 21; 100%). Reaction to DOG1 was patchy in three tumors (n = 27; 11%) while reactions to vimentin (n = 31) and Hale colloidal iron special stain (n = 30) were negative. On follow-up, no tumor recurrence or metastasis was observed over a follow-up range of 1-144 months (mean, 54 months; median, 60 months). Our data suggest that adverse pathologic features in renal oncocytoma do not alter their benign course.

Outcome after treatment of pediatric supratentorial ependymoma: long-term follow-up of a single consecutive institutional series of 26 patients

Background Long-term outcome after surgical treatment of supratentorial ependymoma (STE) in children has not been extensively reported. Findings We identified 26 children who underwent primary tumor resection of STE between 1953 and 2011, with at least 8 years follow-up. Ten patients (38%) had anaplastic and 16 had low grade ependymoma. Four of 15 children (26%) treated in the years 1953–1976 survived more than 5 years, but the observed 10-year survival was only 7%. One patient lived for 37 years, and second surgery for a local recurrent lesion disclosed a glioblastoma, possibly secondary to radiotherapy. In contrast, the observed 5-year survival rate for 11 children treated in the years 1992–2011 was 8/11 (73%) and observed 10- and 25-year survival rates were 70% and 66%, respectively. Eight patients were alive and tumor-free with follow-up periods of 8–27 (median 18) years, all treated after 1992. Five of these long-term survivors were 23–39 years old with full-time (n = 3) or part-time (n = 2) work. The last three patients were still children (9–12 years old): one with good function and two with major neurological deficits. The majority of patients (n = 18) received adjuvant radiotherapy and eight children no adjuvant treatment. Repeated resections for residual or recurrent tumor were necessary in 11 patients (42%), mostly due to local disease with progressive clinical symptoms. Eight patients underwent only one repeat resection, whereas three patients had two or more repeat resections within 18 years after initial surgery. Four patients were tumor-free after repeated resections at the latest follow-up, 2–13 years after last surgery. Conclusion Pediatric STE has a marked risk for local recurrence even after gross total resection and postoperative radiotherapy, but survival has increased following the introduction of modern treatment in recent years. Repeated surgery is an important part of treatment and may lead to persistent tumor control.

P0904 - How to follow patients with negative targeted prostate biopsy but positive multi-parametric MRI? Results from a large, single institution series

P0904 - How to follow patients with negative targeted prostate biopsy but positive multi-parametric MRI? Results from a large, single institution series