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Pheochromocytoma/neuroblastoma composite tumors are rare entities for which little is known. We report an atypical case of a 39-year-old man with secondary bone locations of a composite tumor, 7 years after resection of adrenal neuroblastoma, with constitutional alteration of SWI/SNF-related, matrix-associated, actin-dependent regulator of chromatin, subfamily A, member 4 whose role is unknown. The diagnosis of a peripheral neuroblastic tumor in adulthood is difficult and even more so when it is a composite tumor. In the absence of a standard of care, management is varied and discussions about treatment modalities for these patients are complex.

Purpose: This study was undertaken to describe the Adolescents and Young Adults' (AYA-aged patients') neuro-oncology care pathways at Grenoble Alpes University Hospital according to the French health recommendations. Methods: A retrospective monocentric observational study was conducted between 2020 and 2021, on patients 13-29 years of age, diagnosed from January 2013 to 2019 in our institute with a primary brain tumor, excluding meningiomas, schwannomas, and neurinomas. The detailed analysis took into account the type of care unit (adult or pediatric), whether or not a dedicated AYA support team was involved, and various pretherapeutic actions. Results: Sixty patients were included in this study. Three of the 9 key stages of the care process were completed regardless of the type of management. Management in a pediatric oncology department seems to improve multidisciplinary discussions and access to the AYA team. Collaboration with an AYA team seems to improve the recourse to pediatric advice and supportive care. Conclusions: Increased vigilance at all key steps as defined in the recommendations of the Directorate General of Healthcare Services would significantly improve the overall quality of care for this specific population. In the absence of a dedicated AYA hospitalization unit, interdisciplinary cooperation between the different professionals of the adult and pediatric services is essential, and the involvement of the AYA mobile team is a factor that favors links and exchanges and ultimately an overall improvement in patient care and life. We propose our vision of an ideal AYA neuro-oncology care pathway.

To observe the effects of androgen replacement on neuropsychological measures in menopausal women, healthy menopausal women already using replacement estrogen were studied in a randomized, double-blind, active placebo-controlled, crossover comparison between two 8-week periods of treatment with (1) 0.625 mg oral esterified estrogen (E) alone and (2) in combination with 1.25 mg oral methyltestosterone (meT). After an initial baseline session, data were gathered at the end of two treatment periods. Scores on standardized psychological tests and computerized reaction times were compared between treatments, as was an overall outcome score that combined all measures. Added meT significantly improved scores on a test of complex information processing, the Switching Attention Test, but not on other tests. Mean outcome score showed no net change and wide variation. Fourteen subjects had outcome scores >1 SD from the mean, and 21 had no change. In the estrogen alone condition, three measures predicted favorable outcome with added meT: surgically compromised ovarian function, fewer physical symptoms, and higher score on a self-image measure of creativity. Added meT treatment may improve complex information processing. Despite wide disparities in outcome, an increased chance of overall improvement may be predicted by specific pretreatment measures.

During a double-blind comparison of menopausal replacement therapy with estrogen alone compared with estrogen plus methyltestosterone (meT), subjects who had been on conjugated equine estrogen (CEE) said they felt better when placed on esterified estrogen (EE). We, therefore, tested whether these estrogen treatments differed in their neuropsychological effects. Subjects were 34 healthy menopausal respondents to advertisements younger than age 66 who were on CEE at baseline. Each was randomized into the EE condition, either immediately after baseline or after they first took EE plus added meT for 8 weeks. We compared neuropsychological measures between these two conditions. Data included cognitive performance test results and symptom self-ratings. Multivariate techniques were used to adjust for the effects of treatment order. Compared with prior CEE treatment, EE treatment was associated with significantly improved scores on the Zung Self-Rated Depression Scale and on Switching Attention Test performance. Further investigation is warranted to determine if different forms of estrogen replacement induce different neuropsychological effects.

Previous economic evaluations of hormone replacement therapy (HRT) have restricted positive effects to alleviation of postmenopausal symptoms and negative effects to drug side effects. We studied the association between HRT use and postmenopausal women's valuation of both health-related quality of life and potential treatment side effects. Postmenopausal women with either a documented first vertebral fracture within the past 5 years or no history of osteoporotic fractures were recruited from Olmsted County, Minnesota, and from Dartmouth-Hitchcock Medical Center in New Hampshire to participate in a study to assess quality of life and women's attitudes toward osteoporosis prevention. Women's valuations of their current health and potential HRT-related side effects were quantified as quality-adjusted life years (QALYs) assessed by an automated utility assessment instrument (U-Titer) and the time tradeoff technique, by a vertical rating scale, and by estimated quality of well-being (QWB) scores. Health status was measured using the Medical Outcomes Study SF-36. Regression methods were used to assess the impact of current HRT use on health-related quality of life and valuation of side effects. There were 106 women with vertebral fracture and 180 with no history of hip, wrist, or vertebral fractures. Altogether, 116 (40.6%) women were currently taking HRT, 64 (22.2%) had taken HRT in the past, and 106 (37.1%) women had never taken HRT. Current HRT users had higher time tradeoff QALYs than never and past HRT users, with gains ranging from 15.0 to 83.7 days per year for current users relative to the others. Benefits were largest for women with a vertebral fracture and limitations in activities. The secondary QALY measures also showed significantly higher values for current HRT users compared with other women, as did SF-36 subscales for general health, physical function, role-emotional function, and vitality. There was substantial variability in women's perceptions of HRT side effects. Overall, the proportion of women willing to trade time to avoid bleeding was largest, at 95.5%, followed by breast tenderness, weight gain, and endometrial biopsy at 90.4%, 87.4%, and 82.7%, respectively. Current HRT users had higher health-related quality of life than past or never users according to all measures studied. Women's perceptions of potential side effects were highly variable and should be considered by physicians when prescribing an HRT regimen. If, as our results suggest, postmenopausal therapy has positive effects beyond the immediate postmenopausal years, previous economic studies may have underestimated the value of HRT.

During reproductive life, ovarian steroid biosynthesis is gonadotropin dependent and occurs in theca and granulosa cells. In the menopausal ovary, there is atresia of ovarian follicles, with sparing of the androgen-producing theca-interstitial cell component. The aging ovary, therefore, produces significantly reduced amounts of estrogen, with continued, though decreased, androgen production. After menopause, ovarian estradiol biosynthesis is minimal, with circulating estrogen being derived principally from peripheral aromatization of ovarian and adrenal androgens. Androgen biosynthesis from the adrenal gland, in addition to that from the ovary, decreases with age. Although ovarian androgen production declines with age, there is not an abrupt decrease as is seen with ovarian estrogen levels at the time of menopause. The biological activity of these steroids, either before or after menopause, depends on the amount of steroid available in the unbound fraction. To this end, sex hormone-binding globulin (SHBG) levels are an important determinant of hormone action. Not only does the concentration of SHBG influence the biological effect of testosterone and estradiol, but these steroids also regulate SHBG concentrations.