[Prostate cancer of elderly patients: Place and role of geriatric assessment]. / Cancer de prostate des sujets âgés : place et rôle de l'évaluation gériatrique.

INTRODUCTION: The aim of this study was to appreciate the place and role of geriatric assessment in elderly patients with prostate cancer. MATERIALS AND METHODS: We performed a retrospective analysis of prostate cancer patients who underwent geriatric assessment during the therapeutic management from 2008 to 2014. Patient, tumor, treatment characteristics and their associated toxicity as well as the parameters of geriatric assessment were studied. The occurrence of geriatric assessment within the 3 months preceding a therapeutic decision was reviewed. RESULTS: Data of seventy-four patients were analyzed with a median follow-up of 15.6 years. The average age at diagnosis was 74.3 and 80.6 at the geriatric assessment. At the time of the geriatric assessment 64 patients had metastatic disease, 39 were in poor condition more than 50% of patients had walking ability disorders. Thirteen patients underwent radical surgery, 28 received radiotherapy, 30 patients had chemotherapy and hormonotherapy was prescribed for 72 patients. The geriatric assessment, requested on average 15 years after diagnosis, was not carried out within the 3 months preceding treatment decision for 55 patients. CONCLUSION: The recourse to geriatric assessment is predominantly used to endorse a decision of supportive care for elderly patients with prostate cancer. An early intervention by a geriatrician consultant for the initial management and then at each therapeutic event is a sine qua non condition for efficient personalized therapeutic management suitable to every patient according to physiological age. LEVEL OF EVIDENCE: 4.

A man who inherited his SRY gene and Leri-Weill dyschondrosteosis from his mother and neurofibromatosis type 1 from his father.

We report on a man with neurofibromatosis type 1 (NF1) and Leri-Weill dyschondrosteosis (LWD). His father had NF1. His mother had LWD plus additional findings of Turner syndrome (TS): high arched palate, bicuspid aortic valve, aortic stenosis, and premature ovarian failure. The proband's karyotype was 46,X,dic(X;Y)(p22.3;p11.32). Despite having almost the same genetic constitution as 47,XXY Klinefelter syndrome, he was normally virilized, although slight elevation of serum gonadotropins indicated gonadal dysfunction. His mother's karyotype was mosaic 45,X[17 cells]/46,X,dic(X;Y)(p22.3;p11.32)[3 cells].ish dic(X;Y)(DXZ1 +,DYZ1 + ). The dic(X;Y) chromosome was also positive for Y markers PABY, SRY, and DYZ5, but negative for SHOX. The dic(X;Y) chromosome was also positive for X markers DXZ1 and a sequence < 300 kb from PABX, suggesting that the deletion encompassed only pseudoautosomal sequences. Replication studies indicated that the normal X and the dic(X;Y) were randomly inactivated in the proband's lymphocytes. LWD in the proband and his mother was explained by SHOX haploinsufficiency. The mother's female phenotype was most likely due to 45,X mosaicism. This family segregating Mendelian and chromosomal disorders illustrates extreme sex chromosome variation compatible with normal male and female sexual differentiation. The case also highlights the importance of karyotyping for differentiating LWD and TS, especially in patients with findings such as premature ovarian failure or aortic abnormalities not associated with isolated SHOX haploinsufficiency.