• Clinical course and management of adrenal endocrine hypertension: A single-center experience.
    2 days ago
    Objectives. Endocrine etiologies of secondary hypertension (HTN) are frequent and increasingly diagnosed according to the learned societies. Adrenal causes as primary aldosteronism (PA), pheochromocytomas (PHEO), and ACTH-independent Cushing's syndrome (CS) are widely described separately regarding their clinical, biochemical, and morphological aspects and only a few studies have developed the particular aspect of associated HTN. For this reason, we conducted a retrospective observational descriptive study at a tertiary referral center. Subjects. Fifty-five subjects were included (PA: 52.8%, PHEO: 29.1%, and CS: 18.1%). We found a slight female preponderance (60%). The average age of participants was 47.0±14.9 years. First-degree family history of HTN was described in up to two thirds of cases. Regarding HTN characteristics, subjects with PA presented significantly younger age at diagnosis and had more severe and resistant profile and required higher number of antihypertensive drugs. HTN-mediated organ damage (HMOD) was mainly cardiovascular and ocular. Patients with PHEO were significantly older at diagnosis and had more severe and paroxysmal HTN. Patients with CS had less severe HTN and less HMOD. Specific treatments were indicated in all cases aiming complete remission of HTN. Subjects with PA were treated medically with mineralocorticoid receptor antagonists in 72.4% of cases and HTN remission was achieved in only 21.8% after surgical treatment. All subjects with PHEO were operated with adrenalectomy and the remission was observed in 37.5% of cases, while patients with CS were surgically managed in 50% of cases with 40% of HTN remission. Conclusions. Diagnosis and management of adrenal endocrine HTN diseases are challenging for healthcare professionals and early screening is crucial for prevention of cardiovascular morbidity.
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  • Epidemiology and Survival Determinants of Monomorphic Epitheliotropic T-Cell Lymphoma: A Pooled Patient-Level Dataset Analysis.
    2 days ago
    Monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL) is a rare, aggressive intestinal T-cell lymphoma with poorly defined prognostic factors and limited therapeutic evidence. This study presents a retrospective, pooled patient-level analysis of MEITL cases, including those previously classified as type II enteropathy-associated T-cell lymphoma, consistent with modern MEITL criteria. Among 299 patients, the median age was 60 years, with a male preponderance. Cases clustered predominantly in East Asia. Abdominal pain was the most frequent presentation, and perforation occurred in 31.9% of cases. The canonical CD3+, CD4-, CD5-, CD8+, CD56+ phenotype was present in 65.9% of fully annotated cases. Median OS was 12 months, and median PFS was 5 months. Elevated LDH ≥ 250 U/L (p = 0.002), advanced Lugano stage II2/IIE/IV (p = 0.042), and non-ileal-only disease distribution were associated with inferior OS, whereas isolated ileal involvement was favorable (p = 0.004). In multivariable analysis, advanced Lugano stage, non-ileal-only involvement, and failure to achieve complete or partial response remained independently adverse. An exploratory prognostic score incorporating stage, anatomic distribution, and LDH showed preliminary risk group separation in a small subset of patients (C-index 0.734). Stem cell transplantation (SCT) was associated with improved survival in fixed-time analyses, but this association was attenuated after accounting for transplant timing; exploratory responder-restricted and landmark analyses suggested that any potential SCT benefit may be concentrated among patients with chemotherapy-sensitive disease. MEITL demonstrates poor survival despite multimodality therapy. Disease extent, anatomic distribution, LDH, treatment response, and response-adapted SCT may inform risk stratification and management.
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  • Regional Diversity of Human Papillomavirus Genotypes in Southeastern Brazil: Implications for Cervical Cancer Screening.
    2 days ago
    Cervical cancer remains a major public health challenge in Brazil. A molecular testing for human papillomavirus (HPV) was incorporated into the national screening program, current strategies prioritize HPV-16 and HPV-18, potentially overlooking other high-risk genotypes circulating regionally. This cross-sectional study evaluated HPV prevalence and genotype distribution among 8073 women in routine cervical screening and a separately recruited research cohort of sexually active women aged 15-25 years in 25 municipalities in southern Espírito Santo, Brazil, from September 2024 to September 2025. Cervicovaginal samples were tested using the Allplex HPV28 real-time quantitative PCR assay, which detects 28 HPV genotypes. Sociodemographic and behavioral data were collected via standardized questionnaires and vaccination status was verified for women aged 15-25 years using official records. Associations were analyzed using odds ratios (ORs) and 95% confidence intervals (CIs). Overall HPV prevalence was 27.9%, increasing to 57.0% among those aged 15-25 years. Carcinogenic genotypes comprised 43.4% of infections. The most prevalent relevant genotypes were HPV-53 (3.6%), HPV-68 (3.1%), HPV-52 (2.9%), HPV-16 (2.9%), and HPV-58 (2.3%). Multiple carcinogenic/probable or possible carcinogenic infections occurred in 48.5% of HPV-positive cases, with HPV-52, HPV-53, and HPV-68 forming a central coinfection cluster. Among women aged 15-25 years, 30.4% were vaccinated; no vaccine-covered genotypes were detected in vaccinated women, compared with 6.4% in unvaccinated women (OR, 0.17; 95% CI, 0.009-3.08; p = 0.18). Non-16/18 genotypes predominate in this population, but the two most prevalent (HPV-53 and HPV-68) carry comparatively low attributable risk for invasive cervical cancer under current global classifications, whereas genotypes with established high carcinogenic potential (16, 18, 31, 33, 35, 45, 52, and 58) accounted for only 31.5% of detected infections. These findings support continued regional genotype surveillance and vaccine-impact monitoring but, in the absence of histological or invasive-cancer outcome data from this population, do not by themselves justify changes to screening panels or vaccine composition based on prevalence alone.
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  • TAPESTRY study: impact of prior transurethral resection of the prostate on outcomes after robot-assisted radical prostatectomy: a propensity score-matched cohort study.
    2 days ago
    Prior transurethral resection of the prostate (TURP) may distort bladder neck and apical anatomy and complicate robot-assisted radical prostatectomy (RARP). Its effect on anastomotic recovery and oncological control remains controversial. To evaluate perioperative, anastomotic, pathological, and available PSA outcomes after RARP in patients with and without prior TURP. We retrospectively analyzed a prospectively maintained database of 3,332 consecutive RARP procedures performed between August 2011 and December 2024. Ninety-seven patients with prior TURP were matched 1:3 to 291 TURP-naive controls using propensity scores derived from age, body mass index, clinical stage, ISUP grade, prostate-specific antigen (PSA), positive biopsy cores, and prostate volume. Comparative outcome analyses used available cases within the matched cohort. Baseline characteristics were well balanced after matching. Operative duration was similar between groups (median 135 vs. 139 min; p = .584). Prior TURP was associated with longer catheterization (7 [IQR 4.5-11.5] vs. 6 [4-7] days; p = .004), more cystographies (p = .004), more frequent catheterization beyond 7 days (33.7% vs. 20.1%; p = .011), and a higher rate of complications within 3 months (13.4% vs. 6.2%; p = .030). Cystographic leakage was numerically more frequent after TURP (25.4% vs. 14.8%; p = .061). Pathological stage, nodal positivity, positive surgical margin rate and length, and available PSA outcomes at 1 and 2 years were comparable. After Bonferroni adjustment, catheterization duration and the number of cystographies remained statistically significant. Prior TURP was associated primarily with greater postoperative anastomotic management reflected by longer catheterization and more cystographic follow-up; the observed increase in 3-month complications did not remain significant after correction for multiple comparisons. Available PSA data did not indicate a clear between-group difference, although incomplete follow-up precluded robust conclusions regarding biochemical control.
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  • A systematic review of cancer risks associated with MITF variants.
    2 days ago
    The MITF E318K variant has been associated with melanoma risk, while risk associated with other variants or of other cancers remains uncertain. We performed a systematic review with meta-analysis of 11 retrospective case-control studies to evaluate cancer risks associated with germline MITF variants. Across 8,606 melanoma patients and 17,953 controls, the E318K variant was detected in 2.1% and 0.8% of individuals, respectively, corresponding to a significantly increased melanoma risk (OR 2.55, 95% CI 1.90-3.43). The association was stronger in patients with multiple primary melanomas, with carrier frequencies up to 2.6% compared to 1.0% in single melanoma cases and ORs reaching 4.45 in individual studies. Phenotypic analyses showed enrichment of high nevus burden (> 200 nevi), with ORs up to 12.4 in multiple melanoma cohorts. No consistent association with age at onset or pigmentary traits was observed. Evidence for non-melanoma cancers was limited and heterogeneous: a single study reported increased renal cancer risk (OR 7.64), whereas larger cohorts failed to replicate this finding; an association with pheochromocytoma/paraganglioma was observed (OR 3.19) but lacks confirmation. No other MITF variants demonstrated significant cancer risk. These findings support MITF E318K as a moderate-penetrance melanoma susceptibility allele, particularly associated with multiple primary melanomas.
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  • Parental distress - a predictor of treatment adherence in pediatric neuroblastoma: a cross-sectional study of chinese families.
    2 days ago
    This cross-sectional study examined whether parental distress is associated with treatment adherence in children with neuroblastoma and identified specific parental psychological factors as independent risk markers for non-adherence.

    Three hundred fifty families from four tertiary medical centers in China were enrolled. Parents completed the Parenting Stress Index-Short Form (PSI-SF), Medication Adherence Rating Scale (MARS-5), Beck Depression Inventory-II (BDI-II), and Family Assessment Device-General Functioning (FAD-GF). Path analysis examined associations between parental distress, family functioning, disease risk, and treatment adherence. Common method bias was assessed using Harman's single-factor test. Sensitivity analyses examined MARS-5 cutoff robustness using median split and MARS-5 < 18 thresholds.

    Parental distress was significantly associated with lower treatment adherence (beta = -0.35, p = 0.002). Family functioning was statistically consistent with partial mediation (indirect beta = -0.12, p = 0.003), though causal interpretation is precluded by the cross-sectional design. Paternal depressive symptoms emerged as the strongest independent risk marker for non-adherence (OR = 2.2, 95% CI 1.18-4.09, p = 0.013). Mothers reported significantly higher parenting stress than fathers (Bonferroni-corrected p < 0.008; Cohen's d = 0.37-0.62). Families with poor functioning were 2.8 times more likely to exhibit suboptimal adherence. Harman's single-factor test yielded 8 factors with eigenvalues > 1.0, with the first factor explaining 28.4% of total variance-below the 40% threshold indicative of substantial common method bias. Sensitivity analyses using alternative MARS-5 cutoffs confirmed the robustness of paternal depression and family functioning as predictors.

    Parental distress, particularly paternal depressive symptoms, represents a modifiable risk marker associated with treatment non-adherence. Screening for paternal psychological distress and family dysfunction may support treatment adherence in pediatric neuroblastoma. Given the cross-sectional design, these associations should be interpreted as concurrent rather than causal.
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  • Single-stage tumor resection and immediate CAD/CAM-assisted cranio-orbital reconstruction for spheno-orbital meningiomas: a standardized surgical workflow and outcome analysis.
    2 days ago
    Spheno-orbital meningiomas (SOMs) are surgically challenging lesions characterized by tumor infiltration and hyperostosis of the sphenoid wing and orbit. Surgical treatment requires tumor resection, drilling of hyperostotic bone, orbital decompression and skull base reconstruction. Traditionally, reconstruction has been performed either free hand or as a delayed second-stage procedure, often resulting in suboptimal skull and facial symmetry. Single-stage resection with immediate CAD/CAM-assisted reconstruction offers a feasible alternative but requires meticulous preoperative planning and a standardized surgical workflow.

    In this retrospective study of 34 patients with SOMs, we present our institutional standardized protocol for preoperative CAD/CAM planning and single-stage tumor resection with immediate skull reconstruction technique. Clinical, functional and radiological outcomes were also assessed.

    Exopthalmos was the predominant presenting symptom. Gross-total resection was achieved in 73.5% of cases, resulting in a reduction of exophthalmos in 85% of patients and improvement of visual acuity in approximately 70%. However, postoperative amaurosis occurred in 8.8% and transient diplopia in 55.9%. CAD/CAM implants demonstrated appropriate intraoperative fit without major implant-related complications and with preservation of orbital and facial symmetry.

    Single-stage resection with immediate CAD/CAM-assisted skull reconstruction for SOMs is feasible, safe and reproducible. The standardized workflow by combining tumor resection and patient-specific reconstruction within a single-stage procedure may offer practical advantages in the overall surgical pathway.
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  • Transoral Robotic Surgery (TORS) versus radical radiotherapy following neoadjuvant therapy for oropharyngeal carcinoma: a multicenter, real-world efficacy and safety analysis.
    2 days ago
    This study aimed to compare the efficacy and safety of transoral robotic surgery (TORS) versus radical radiotherapy (RT) following neoadjuvant therapy in patients with oropharyngeal carcinoma (OPC). OPC patients who received neoadjuvant therapy followed by either transoral robotic surgery(TORS) or radiotherapy (RT) between 2018 and 2024 across two centers were included in this study. To address potential confounding and ensure comparability, the overlap weighting (OW) method was employed. Progression-free survival (PFS), overall survival (OS), locoregional relapse-free survival (LRRFS), distant metastasis-free survival (DMFS), and adverse events were evaluated. A total of 367 eligible patients were included (264 in the RT group and 103 in the TORS group). Over a median follow-up of 41 months in the OW-adjusted cohort, no significant differences were observed between TORS and RT groups in 3-year PFS (75.6% vs. 74.4%, p = 0.834), OS (80.5% vs. 84.8%, p = 0.703), LRRFS (76.1% vs. 75.0%, p = 0.909), or DMFS (80.0% vs. 82.0%, p = 0.941). Subgroup analyses demonstrated that the treatment effect remained highly consistent across all subgroups, without significant statistical interactions (all P for interaction > 0.05). Toxicity patterns differed: the RT group had higher rates of xerostomia, mucositis, and leukopenia, while the TORS group had more hemorrhage (including one fatal case) and infection. Following neoadjuvant therapy, survival outcomes were comparable between TORS and RT in the OW-adjusted cohort. Toxicity patterns differ between the groups, underscoring the importance of personalized, multidisciplinary treatment decisions.
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  • Upper- versus lower-extremity synovial sarcoma: distinct surgical pathways and postoperative morbidity in a 24-year cohort.
    2 days ago
    Synovial sarcoma is a rare, late-relapsing soft-tissue sarcoma of the extremities. Whether anatomical location shapes the surgical treatment pathway, rather than prognosis alone, is unclear. We compared upper-extremity (UE) and lower-extremity (LE) tumors with respect to margin status, re-excision, reconstruction, morbidity and long-term oncological events.

    Retrospective single-center cohort of 59 consecutive patients with histologically confirmed extremity synovial sarcoma treated between 2000 and 2023. Co-primary endpoints were a microscopically positive margin (R1) at the index resection and any postoperative complication within 8 weeks (Clavien-Dindo). Metastasis-free survival (MFS) and overall survival were exploratory. Fisher exact, Mann-Whitney U, Kaplan-Meier and log-rank methods were used; odds ratios (OR) are UE relative to LE.

    Twenty-three tumors were UE and 36 LE. R1 at the index resection was more frequent in UE tumors (14/23 [61%] vs. 5/36 [14%]; OR 9.64, 95% CI 2.73-34.1; P < 0.001), as was neoadjuvant therapy (39% vs. 8%; P = 0.007). Definitive R0 was achieved in all patients. Any postoperative complication (13% vs. 53%; OR 0.13, 95% CI 0.03-0.53; P = 0.002) and major complications (9% vs. 39%; P = 0.015) were more frequent after LE surgery. Seven of nine distant metastases occurred in LE tumors, several beyond six years; ten-year MFS was 90% versus 56% (log-rank P = 0.095).

    Upper- and lower-extremity synovial sarcomas followed distinct surgical pathways and carried distinct morbidity profiles, whereas definitive R0 resection was attained in both. The time-to-event findings are exploratory and require multicenter validation.
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  • Role of cytoreductive nephrectomy in metastatic medullary renal cell carcinoma: controlled analysis.
    2 days ago
    To test for differences in overall survival (OS) according to cytoreductive nephrectomy (CN) in medullary metastatic renal cell carcinoma (med-mRCC) relative to most comparable clear cell metastatic RCC (cc-mRCC) patients.

    Within the Surveillance, Epidemiology and End Results (SEER) database (2000-2021), we identified 62 patients with med-mRCC vs. 10,372 with cc-mRCC. Propensity score matching (PSM, ratio 1:3), Kaplan-Meier plots and multivariable Cox regression analyses addressed were used.

    Of 10,434 patients, 62 (0.6%) harbored med-mRCC vs. 10,372 (99.4%) cc-mRCC. Med-mRCC patients were younger (27 vs. 63 years, p < 0.001), more frequently African American (74 vs. 6%, p < 0.001) and more frequently harbored N1 stages (52 vs. 28%, p < 0.001). After 1:3 PSM for age, race/ethnicity, T-stage and N-stage, 62 of 62 (100%) med-mRCC (median OS 4 months) and 186 of 10,372 (2%) cc-mRCC patients (median OS 10 months) remained for further analyses. In med-mRCC, median OS was 9 months with CN vs. 3 months without CN (p = 0.01). In cc-mRCC, median OS was 28 months with CN vs. 5 months without CN (p < 0.001). In med-mRCC, CN independently predicted better OS (Hazard's Ratio [HR] 0.5, p = 0.005), as well as in cc-mRCC (HR 0.4, p < 0.001).

    Med-mRCC patients exhibit a drastically different phenotype relative to their cc-mRCC counterparts. Despite this difference, med-mRCC exposed to CN harbor a similar relative protective survival effect compared to their cc-mRCC counterparts with most comparable age, race/ethnicity and tumor characteristics.
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