Natural killer (NK) cell enteropathy is a lymphoproliferative disorder, initially described by Mansoor and colleagues, that presents in the gastrointestinal tract, and is often mistaken for extranodal NK/T-cell lymphoma on first assessment. This population of cells in this process have an NK-cell phenotype (CD3, CD56, CD2, CD7), lacks evidence of Epstein-Barr virus infection, has germline rearrangement of the T-cell receptor, and a very indolent clinical course. Indeed, many of such patients had been originally diagnosed as having an NK/T-cell lymphoma, and subsequently received chemotherapy. We report a unique case where an indolent lymphoproliferative disorder with features that resemble NK-cell enteropathy is encountered for the first time outside the gastrointestinal tract, specifically in the female genitourinary tract. We provide morphologic, immunophenotypic, and molecular documentation of such, in association with a completely indolent clinical behavior of this type of process.

SUMMARYFew studies have looked at erectile function recovery (EFR) rates in men undergoing non-nerve sparing resection during radical prostatectomy (RP). Existing studies show great variation in EFR rates owing to multiple factors that minimize their utility in counselling RP patients. We investigated the EFR rate and its predictors in unilateral cavernous nerve resection and bilateral cavernous nerve resection patients 24 months after RP. We conducted a population-based, prospective cohort study of 966 patients who underwent RP at a tertiary cancer centre from 2008 to 2012. Cavernous nerve condition was evaluated on a 4-point nerve sparing score and assigned to one of three groups: bilateral sparing, unilateral resection (UNR) and bilateral nerve resection (BNR). EF was assessed pre-RP and 24-30 months post-op using a validated 5-point patient-reported scale (1 = fully rigid; 5 = no tumescence). EFR was defined as a post-op EF grade of 1-2. Statistical analysis included descriptive statistics, ANOVA, chi-square, Fisher's exact test and logistic regression. Mean baseline EF was 1.84 AE 1.3 and 2.74 AE 1.5 for UNR and BNR patients respectively. Thirty-three percent of UNR patients and 13% of BNR patients exhibited EFR. Age, baseline EF were predictors of EFR. Multivariable analysis showed baseline EF was a significant predictor of EFR at 24 months for UNR. For BNR patients, pre-RP EF was the only factor predictive of EFR. Patients undergoing nerve resection still have a significant chance of achieving true EFR, with UNR surgery patients showing more potential for improvement than patients undergoing BNR surgery. Age and baseline EFR characterize recovery prospects in these two groups. Physicians should thus measure and account for baseline EF in addition to age and the degree of nerve resection when advising patients about expectations for successful EF following RP.

IntroductionPredictors of non-response in mismatch repair deficiency cancers are poorly understood. Upregulation of the canonical Wnt pathway has been associated with decreased immune cell infiltration in many cancer types. The relationship between Wnt/β-catenin pathway activation and the programmed death-ligand 1 axis in endometrial cancer remains poorly characterized. This study evaluates β-catenin expression in a well characterized cohort of endometrial cancers by mismatch repair status and programmed death-ligand 1 expression.MethodsWhole sections of formalin-fixed, paraffin embedded tissue from 23 Lynch syndrome-associated carcinomas, 20 mutL homolog-1 (MLH1) promoter hypermethylated carcinomas, and 19 mismatch repair intact carcinomas were evaluated. Immunohistochemistry staining for β-catenin and programmed death-ligand 1 was performed on all cases. Programmed death-ligand 1 expression was scored in both the tumor and the peri-tumoral immune compartment. Tumor staining was classified as positive when membranous (programmed death-ligand 1) staining was present in ≥1% of tumor cells. Immune stromal staining was scored as positive when ≥5% of peritumoral and intratumoral immune cells (including lymphocytes and macrophages) showed reactivity.ResultsSix tumors (6/62, 9.7%) demonstrated nuclear expression of β-catenin (4 were Lynch syndrome-associated, 1 was MLH1 methylated, 1 was mismatch repair intact). The majority of tumors with nuclear β-catenin expression demonstrated concomitant tumoral programmed death-ligand 1 expression (5/6, 83.3%) and were more likely to demonstrate tumoral programmed death-ligand 1 expression compared to tumors without nuclear β-catenin expression (83.3% vs 39.3%, p=0.04). Both tumoral and immune cell expression of programmed death-ligand 1 was statistically significantly associated with mismatch repair deficient tumors.DiscussionTumors demonstrating nuclear β-catenin expression were more likely to express tumoral programmed death-ligand 1 staining than tumors without nuclear β-catenin expression. Nuclear β-catenin expression could be a potential predictive biomarker for non-response to immune checkpoint inhibition in mismatch repair deficient tumors. Nuclear β-catenin expression status should be considered as a translational endpoint in future clinical trials of immune checkpoint inhibition in endometrial cancer.

Objective To investigate the multiparametric prostate magnetic resonance imaging (mpMRI) findings in patients treated with testosterone replacement therapy (TRT) while on active surveillance (AS) for low-risk prostate cancer. Methods We retrospectively reviewed 12 patients who underwent mpMRI before and after TRT while on AS. Changes in serum testosterone level, prostate specific antigen (PSA), prostate biopsy findings, prostate volume and Prostate Imaging Reporting and Data System Version 2 (PI-RADSv2) score before and after TRT were summarized. Results Following TRT, there was a significant increase in serum testosterone (516.5 ng/dl vs. 203.0 ng/dl), PSA (4.2 ng/ml vs. 3.3 ng/ml) and prostate volume (55.2 cm3 vs. 39.4 cm3). Two patients had biopsy progression during the study periods. The PI-RADSv2 scores before and after TRT were unchanged in 10/12 patients; none of these demonstrated biopsy progression on post TRT. The PI-RADSv2 scores increased after TRT in 2/12 patients; both showed Gleason score upgrade on follow-up biopsy. One of these two patients underwent radical treatment due to clinical progression. The area under the curve calculated from PI-RADSv2 score after TRT was 0.90, which was better than that calculated from post TRT PSA level (0.48). Conclusions After TRT, mpMRI findings remained stable in patients without biopsy progression, while PI-RADSv2 score increase was identified in patients with Gleason score upgrade on follow-up biopsy.

Objectives To evaluate the concordance between second‐trimester anatomic ultrasound and fetal echocardiography in detecting minor and critical congenital heart disease in pregnancies meeting American Heart Association criteria. Methods We conducted a retrospective cohort study of pregnancies in which a second‐trimester fetal anatomic ultrasound examination (18–26 weeks) and fetal echocardiography were performed between 2012 and 2018 at our institution based on American Heart Association recommendations. Anatomic ultrasound studies were interpreted by maternal‐fetal medicine specialists and fetal echocardiographic studies by pediatric cardiologists. Our primary outcome was the proportion of critical congenital heart disease (CCHD) cases not detected by anatomic ultrasound but detected by fetal echocardiography. The secondary outcome was the proportion of total congenital heart disease cases missed by anatomic ultrasound but detected by fetal echocardiography. Neonatal medical records were reviewed for all pregnancies when obtained and available. Results Overall, 722 studies met inclusion criteria. Anatomic ultrasound and fetal echocardiography were in agreement in detecting cardiac abnormalities in 681(96.1%) studies (κ = 0.803; P < .001). The most common diagnosis not identified by anatomic ultrasound was a ventricular septal defect, accounting for 9 of 12 (75%) missed congenital heart defects. Of 664 studies with normal cardiac findings on the anatomic ultrasound examinations, no additional instances of CCHD were detected by fetal echocardiography. No unanticipated instances of CCHD were diagnosed postnatally. Conclusions With current American Heart Association screening guidelines, automatic fetal echocardiography in the setting of normal detailed anatomic ultrasound findings provided limited benefit in detecting congenital heart defects that would warrant immediate postnatal interventions. More selective use of automatic fetal echocardiography in at‐risk pregnancies should be explored.