Congenital Cyanotic Heart Disease and the Association with Pheochromocytomas and Paragangliomas.

PURPOSE OF REVIEW: Pheochromocytomas and paragangliomas (PPGLs) are rare neuroendocrine tumors that commonly produce excess catecholamines causing significant morbidity and mortality. Patients with cyanotic congenital heart disease (CCHD) develop PPGLs at a higher frequency than the general population. This review will summarize recent research in the association of PPGL and CCHD. RECENT FINDINGS: Advances in molecular genetics have provided new insights into a variety of germline mutations and somatic mutations related to PPGLs. In the CCHD population, mutations can occur in the hypoxia signaling pathway with gain-of-function somatic mutations in EPAS1, which prevent degradation of hypoxia-inducible factor-2 alpha. These mutations are implicated in oncogenesis. PPGLs associated with CCHD develop as early as age 15 years and have predominantly noradrenergic secretion. Surgical removal is considered the first line of therapy, although belzutifan, a HIF-2α inhibitor, is currently being tested as a potential therapy. Early screening with plasma metanephrines may assist in identifying PPGLs in patients with CCHD.

Testosterone Treatment of Men with Unequivocal Hypogonadism Following Treatment of Organ-Confined Prostate Cancer.

OBJECTIVE: To determine if testosterone treatment of men with unequivocal hypogonadism and organ-confined prostate cancer is associated with recurrence of the cancer. The testosterone dependence of metastatic prostate cancer has made physicians reluctant to treat hypogonadal men with testosterone even after treatment of prostate cancer. Prior studies of testosterone treatment of men with treated prostate cancer have not documented that the men were unequivocally hypogonadal. METHODS: A computerized search of electronic medical records from January 1, 2005, to September 20, 2021, identified 269 men aged ≥50 years who were diagnosed with prostate cancer and hypogonadism. We reviewed the individual records of these men and identified those treated by radical prostatectomy and had no evidence of extraprostatic extension. We then identified men who were hypogonadal prior to the diagnosis of prostate cancer based on at least 1 morning serum testosterone concentration of ≤220 ng/dL, discontinued testosterone treatment when the prostate cancer was diagnosed, resumed testosterone treatment within 2 years after treatment of the cancer, and were monitored for cancer recurrence, defined by a prostate-specific antigen level of ≥0.2 ng/mL. RESULTS: Sixteen men met the inclusion criteria. Their baseline serum testosterone concentrations were 9-185 ng/dL. The median duration of testosterone treatment and monitoring was 5 years (range, 1-20 years). None of the 16 men had biochemical recurrence of prostate cancer during this period. CONCLUSION: Testosterone treatment of men with unequivocal hypogonadism whose organ-confined prostate cancer is treated by radical prostatectomy may be safe.