Diagnosis and management of urinary bladder paragangliomas: A Sino-American-European retrospective observational study.

OBJECTIVE: Paragangliomas of the urinary bladder (UBPGLs) are rare neuroendocrine tumours and pose a diagnostic and surgical challenge. It remains unclear what factors contribute to a timely presurgical diagnosis. The purpose of this study is to identify factors contributing to missing the diagnosis of UBPGLs before surgery. DESIGN, PATIENTS AND MEASUREMENTS: A total of 73 patients from 11 centres in China, and 51 patients from 6 centres in Europe and 1 center in the United States were included. Clinical, surgical and genetic data were collected and compared in patients diagnosed before versus after surgery. Logistic regression analysis was used to identify clinical factors associated with initiation of presurgical biochemical testing. RESULTS: Among all patients, only 47.6% were diagnosed before surgery. These patients were younger (34.0 vs. 54.0 years, p < .001), had larger tumours (2.9 vs. 1.8 cm, p < .001), and more had a SDHB pathogenic variant (54.7% vs. 11.9%, p < .001) than those diagnosed after surgery. Patients with presurgical diagnosis presented with more micturition spells (39.7% vs. 15.9%, p = .003), hypertension (50.0% vs. 31.7%, p = .041) and catecholamine-related symptoms (37.9% vs. 17.5%, p = .012). Multivariable logistic analysis revealed that presence of younger age (<35 years, odds ratio [OR] = 6.47, p = .013), micturition spells (OR = 6.79, p = .007), hypertension (OR = 3.98, p = .011), and sweating (OR = 41.72, p = .013) increased the probability of initiating presurgical biochemical testing. CONCLUSIONS: Most patients with UBPGL are diagnosed after surgery. Young age, hypertension, micturition spells and sweating are clues in assisting to initiate early biochemical testing and thus may establish a timely presurgical diagnosis.

Androgen deprivation therapy plus apalutamide as neoadjuvant therapy prior radical prostatectomy for patients with unresectable prostate cancer.

Whether neoadjuvant therapy confers a survival benefit in advanced prostate cancer (PCa) remains uncertain. The primary endpoints of previous retrospective and phase II clinical studies that used neoadjuvant therapy, including androgen deprivation therapy combined with new-generation androgen receptor signaling inhibitors or chemotherapy, were pathological downstaging, progression-free survival, prostate-specific antigen relief, and local symptom improvement. To the best of our knowledge, no studies have explored the efficacy and safety of neoadjuvant therapy in improving the surgical resection rate in cases of unresectable primary tumors of PCa. We first designed this retrospective study to evaluate the potential value of apalutamide as neoadjuvant therapy in improving the resectability rate of radical prostatectomy (RP). We initially reported 7 patients with unresectable primary lesions who underwent neoadjuvant apalutamide treatment for a median of 4 months, and all of them successfully underwent RP treatment. Our study supported apalutamide as neoadjuvant therapy, which helped improve RP's success rate and did not significantly increase perioperative complications, and the neoadjuvant therapy was controllable. Our findings' clinical value and benefit for survival still need further clinical research to confirm.

Somatic IDH1 Hotspot Variants in Chinese Patients With Pheochromocytomas and Paragangliomas.

CONTEXT: IDH1 is a pheochromocytoma/paraganglioma (PPGL) susceptibility gene; however, its role, especially in the Chinese population, has not been characterized. OBJECTIVE: To determine the prevalence of somatic IDH1 hotspot variants in a large cohort of Chinese patients with PPGLs and to summarize associated phenotypes. METHODS: This retrospective cross-sectional study was based on a main cohort of 1141 patients with PPGLs from 2 tertiary-care centers in China. We included 50 cases with urinary bladder paragangliomas (UBPGLs), of whom 29 were part of the main cohort and 21 were from other centers. Two additional cases with IDH1 hotspot variants not part of the main cohort were also included for summarizing IDH1-associated phenotypes. Next-generation sequencing of tumor DNA was used to analyze a customized panel of genes. RESULTS: The overall prevalence of IDH1 hotspot variants in the main cohort was 0.5% (6/1141). Among those PPGLs without mutations in 15 common driver genes, the prevalence of IDH1 variants was 0.9% (4/455). When restricted to paraganglioma (PGL) without mutations, the prevalence reached 4.7% (4/86). Among UBPGLs, IDH1 hotspot variants accounted for 8% (4/50). Together, all 10 patients (9 PGLs and 1 pheochromocytoma) with IDH1 hotspot variants, including 3 females with concurrent EPAS1 hotspot variants, had apparently sporadic tumors, without metastasis or recurrence. There were 3 patients with biochemical data, all showing a non-adrenergic phenotype. CONCLUSIONS: The somatic IDH1 hotspot variants cause PPGL development in some Chinese patients, especially among those apparently sporadic PGLs with a non-adrenergic phenotype and without mutations in major PPGL driver genes.

Current Treatment for Low-Risk Prostate Cancer in China: A National Network Survey.

Objective: To analyze the current treatment for low-risk prostate cancer (LRPC) in China. Methods: A national questionnaire survey titled "A survey of current treatment of LRPC" was designed and released nationally through the network from July 16 to August 3, 2017. Results: A total of 1,116 valid questionnaires were recovered. The percentages of preferred treatment by active surveillance (AS) or radical prostatectomy (RP) were 29.21% and 45.61%, respectively. A correspondence analysis showed that the physician in charge was more inclined to choose AS than RP. Respondents from different institution types, hospitals with different annual numbers of newly admitted patients with prostate cancer, and with different familiarity with the LRPC definition presented a significant difference in the preferred treatments (p < 0.05). Urologists chose AS or not for the following reasons: tumor progression (52.51%), potential medical disputes (42.56%) (i.e., medical disputes from patients or their relatives when urologists choose AS to treat patients with LRPC and the patient has a poor outcome), fear of cancer (41.94%), and surgical risk (39.07%). These reasons were ubiquitous, and there was no significant difference among urologists for these concerns (p > 0.05). Personal skills, surgical risk, and tumor progression were the most common factors that influenced whether AS or RP was preferred (p < 0.05). Concern about the medical disputes brought about by AS was a key factor for not choosing AS (p < 0.05). Conclusions: LRPC is still dominated by RP in China, followed by AS. Personal skills, surgical risk, and concern about tumor progression were the common factors influencing whether AS or RP was preferred. In addition, medical disputes brought by AS are another key factor for not choosing AS. There will be more Chinese data in the future to guide treatment of LRPC.

Bladder leiomyosarcoma in a patient with chronic ketamine abuse: A case report.

Bladder leiomyosarcoma is a rare mesenchymal tumour, accounting for less than 0.5% of all primary bladder malignancies. Adult women of reproductive age have the higher incidence of bladder leiomyosarcoma. Exposure to local pelvic radiotherapy or systemic chemotherapy, especially cyclophosphamide therapy, is also a significant risk factor. We describe a case of a 31-year-old male who developed urinary bladder leiomyosarcoma. The patient had no history of radiotherapy, systemic chemotherapy, or other significant event, except a 5-year history of ketamine abuse. The tumour was found on the left bladder wall and was definitively diagnosed by transurethral resection of the bladder tumour. A partial cystectomy was performed. There are no known reports of urinary bladder leiomyosarcoma associated with chronic ketamine abuse; therefore, we speculate that chronic ketamine abuse may be a factor in the development of this infrequent bladder malignancy.