Sellar and parasellar lesions in the transition age: a retrospective Italian multi-centre study

Feola, Tiziana; Pirchio, Rosa; Puliani, Giulia; Pofi, Riccardo; Crocco, Marco; Sada, Valentina; Sesti, Franz; Verdecchia, Federica; Gianfrilli, Daniele; Appetecchia, Marialuisa; Iorgi, Natascia Di; Jaffrain-Réa, M. L.; Pivonello, Rosario; Isidori, Andrea M.; Grossman, Ashley; Sbardella, Emilia

doi:10.1007/s40618-022-01900-9

Cited by 3 publications

(2 citation statements)

References 42 publications

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“…Blood tests for hormonal assays and testicular US were performed at each evaluation. Transition age was defined as the phase between the end of puberty and young adulthood: therefore, we included in the transition age group subjects who had achieved Tanner stage V and completed linear growth ( 32 , 33 ); subjects ages 26 years and older were included in the adult group.…”

Section: Methodsmentioning

confidence: 99%

Testicular Dysfunction in 47,XXY Boys: When It All Begins. A Semilongitudinal Study

Pozza

Sesti

Tenuta

et al. 2023

The Journal of Clinical Endocrinology &Amp; Metabolism

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Objective Klinefelter syndrome is the most common chromosomal disorder in males, and the most common cause of hypergonadotropic hypogonadism. We describe the natural history of testicular dysfunction in patients with Klinefelter syndrome through the integration of clinical, hormonal and quantitative ultrasound data in a life-course perspective. Design Prospective semi-longitudinal study. Methods We included 155 subjects with 47, XXY karyotype (age range: 7 months - 55 years) naïve to testosterone replacement therapy. Subjects were divided according to pubertal stage and age group (transition age and adults). Serial clinical, hormonal and testicular ultrasound assessments were performed. Results Testicular development progresses until Tanner stage 4, with subsequent regression, whereas Sertoli and germ cell impairment is not hormonally detected before Tanner stages 3-4, as reflected by normal inhibin B values until stage 4 and the fall in the inhibin B/FSH ratio thereafter. The Testosterone/LH ratio peaks during Tanner stages 2-3 and declines from Tanner stage 4 onward, preceding the development of overt hypogonadism. US echotexture progressively worsens until transition age, reflecting ongoing gonadal compromise, whereas quantitative US echotexture measures and the presence of both hypoechoic lesions and microlithiasis independently and significantly predict a lower circulating testosterone level. Conclusions The findings from this large prospective study contribute to our understanding of the natural history of testicular dysfunction in Klinefelter syndrome, underlining the importance of quantitative testicular US in infancy and childhood, as well as during pubertal development and transition age, for the optimal care of Klinefelter syndrome patients.

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Section: Methodsmentioning

confidence: 99%

Testicular Dysfunction in 47,XXY Boys: When It All Begins. A Semilongitudinal Study

Pozza

Sesti

Tenuta

et al. 2023

The Journal of Clinical Endocrinology &Amp; Metabolism

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“…Apart from scattered case reports, only two studies have reported the clinical presentation of patients with pituitary gigantism diagnosed during the transition age [ 39 , 41 , 42 ]: a single-centre study by Colao et al conducted on 13 patients diagnosed between 15 and 20 years, and a multi-centre study by Rostomyan et al investigating more than 200 patients with a wider age at diagnosis (median 21 years, interquartile range 15.5–27) [ 39 ]. Tall stature is usually the first clinical sign that leads to medical attention, thus initiating the diagnostic process for gigantism.…”

Section: Clinical Presentation Of Gigantism During the Transition Agementioning

confidence: 99%

Tall stature and gigantism in transition age: clinical and genetic aspects—a literature review and recommendations

Sada,

Puliani,

Feola

et al. 2023

J Endocrinol Invest

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Purpose Tall stature is defined as height greater than the threshold of more than 2 standard deviations above the average population height for age, sex, and ethnicity. Many studies have described the main aspects of this condition during puberty, but an analysis of the characteristics that the physician should consider in the differential diagnosis of gigantism—tall stature secondary to a pituitary tumour—during the transition age (15–25 years) is still lacking. Methods A comprehensive search of English-language original articles was conducted in the MEDLINE database (December 2021-March 2022). We selected all studies regarding epidemiology, genetic aspects, and the diagnosis of tall stature and gigantism during the transition age. Results Generally, referrals for tall stature are not as frequent as expected because most cases are familial and are usually unreported by parents and patients to endocrinologists. For this reason, lacking such experience of tall stature, familiarity with many rarer overgrowth syndromes is essential. In the transition age, it is important but challenging to distinguish adolescents with high constitutional stature from those with gigantism. Pituitary gigantism is a rare disease in the transition age, but its systemic complications are very relevant for future health. Endocrine evaluation is crucial for identifying conditions that require hormonal treatment so that they can be treated early to improve the quality of life and prevent comorbidities of individual patient in this age range. Conclusion The aim of our review is to provide a practical clinical approach to recognise adolescents, potentially affected by gigantism, as early as possible.

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Pediatric pituitary neuroendocrine tumors–a 13-year experience in a tertiary center

Li,

Deng,

Zhang

et al. 2023

Front. Oncol.

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IntroductionPediatric pituitary neuroendocrine tumor is a rare condition, and despite previous research focusing on this specific group, the main factors influencing the surgical cure rate have not been identified.MethodsWe conducted a single-center retrospective study on pediatric pituitary neuroendocrine tumor patients who visited Peking Union Medical College Hospital between 2010 and 2023. We collected data on their clinical characteristics, imaging features, surgical outcomes, and follow-up information. Additionally, we used multiple-factor logistic regression to investigate the factors affecting the surgical cure rate of pediatric pituitary neuroendocrine tumor.Results232 patients were diagnosed with pediatric pituitary neuroendocrine tumors, with a higher incidence in females. The most common type was ACTH-secreting adenoma (90/232), followed by prolactin-secreting adenoma (63/232), and growth hormone-secreting adenoma (41/232). The majority of pediatric adenomas were macroadenomas (139/232), and some tumors were associated with cystic changes or hemorrhage (58/232), while a few exhibited invasion of the cavernous sinus (33/232). The results of the multivariate analysis indicated that the different hormone secretion types, macroadenoma or the presence of cystic changes or hemorrhage were not significant risk factors for the cure rate after the first surgery. However, the invasion of the cavernous sinus was found to be an important factor influencing the postoperative cure rate. Most pediatric pituitary neuroendocrine tumors with cavernous sinus invasion were macroadenomas, and some displayed characteristics of refractory pituitary neuroendocrine tumors, with some patients experiencing irreversible complications after surgery.ConclusionPediatric pituitary neuroendocrine tumors are complex, and the postoperative cure rate is particularly poor for tumors with cavernous sinus invasion. Although macroadenoma itself does not significantly impact the postoperative cure rate, it is still recommended to diagnose and treat early to avoid unnecessary surgery or surgical complications.

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Sellar and parasellar lesions in the transition age: a retrospective Italian multi-centre study

Cited by 3 publications

References 42 publications

Testicular Dysfunction in 47,XXY Boys: When It All Begins. A Semilongitudinal Study

Testicular Dysfunction in 47,XXY Boys: When It All Begins. A Semilongitudinal Study

Tall stature and gigantism in transition age: clinical and genetic aspects—a literature review and recommendations

Pediatric pituitary neuroendocrine tumors–a 13-year experience in a tertiary center

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