A clinical approach to parasellar lesions in the transition age

Sbardella, Emilia; Puliani, Giulia; Feola, Tiziana; Pofi, Riccardo; Pirchio, Rosa; Sesti, Franz; Verdecchia, Federica; Gianfrilli, Daniele; Moffat, Daniel; Isidori, Andrea M.; Grossman, Ashley

doi:10.1111/jne.12995

Cited by 4 publications

(7 citation statements)

References 249 publications

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“…Among non-endocrine lesions, it is worth noting that craniopharyngiomas, which are the most common sellar masses in paediatric patients, are relatively infrequent in the transition age, only 2% of the whole series and 14.8% of non-endocrine lesions. Instead, germinomas and RCCs were the most prevalent in accordance with a previous systematic review indicating germinomas as the most common brain neoplasm during adolescence [ 3 ]. Of note, inflammatory lesions are rare in this age group but virtually any type of hypophysitis may be encountered, in particular LCH—which is now classified as a neoplastic condition, and lymphocytic hypophysitis—which may reveal an underlying germinoma [ 22 ].…”

Section: Discussionsupporting

confidence: 86%

“…Sellar region tumours represent 20% of intracranial tumours in the paediatrics, comprising mainly tumours arising from adenohypophyseal cells (78%), also now often referred to as pituitary neuroendocrine tumours (Pit-NETs), followed by craniopharyngiomas (18%) and germ cell tumours (2%) [1][2][3][4].…”

Section: Introductionmentioning

confidence: 99%

“…Craniopharyngiomas are the second most frequent sellar region tumour during childhood and adolescence, with a peak incidence occurring in the paediatric age range (5–14 years) for the most common adamantinomatous type [ 2 , 3 , 15 ], whereas papillary craniopharyngiomas typically occur in adults [ 1 ].…”

Section: Introductionmentioning

confidence: 99%

“…Intracranial germ cell tumours have also a significant prevalence during childhood and adolescence, particularly germinomas with pineal and suprasellar localisation, showing a peak incidence (28.7% of brain tumours) between 15 and 19 years of age [ 15 ]. Other sellar/parasellar lesions occurring in this age group include Rathke’s cleft cysts (RCC), Langerhans cell histiocytosis (LCH) and optic gliomas [ 3 ].…”

Section: Introductionmentioning

confidence: 99%

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

Feola

Pirchio

Puliani

et al. 2022

J Endocrinol Invest

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Background Sellar/parasellar lesions have been studied in the adult and paediatric age range, but during the transition age their epidemiology, clinical manifestations, management and treatment outcomes have been poorly investigated. Materials and methods An Italian multicentre cohort study, in which hospital records of patients with diagnosis of sellar/parasellar lesions during the transition age and young adulthood (15–25 years), were reviewed in terms of prevalence, clinical and hormonal features at diagnosis, and outcomes where available. Both pituitary neuroendocrine tumours (pituitary tumours, Group A) and non-endocrine lesions (Group B) were included. Results Among Group A (n = 170, 46.5% macroadenomas), the most frequent were prolactin and GH-secreting tumours, with a female predominance. Among Group B (n = 28), germinomas and Rathke cells cysts were the most common. In Group A, the most frequent hormonal deficiency was gonadal dysfunction. Galactorrhoea and amenorrhoea were relatively common in female patients with prolactinomas. Pre-surgical diabetes insipidus was only seen in Group B, in which also hormone deficiencies were more frequent and numerous. Larger lesions were more likely to be seen in Group B. Patients in Group B were more frequently male, younger, and leaner than those of Group A, whereas at last follow-up they showed more obesity and dyslipidaemia. In our cohort, the percentage of patients with at least one pituitary deficiency increased slightly after surgery. Conclusions The management of sellar/parasellar lesions is challenging in the transition age, requiring an integrated and multidisciplinary approach. Hormone and metabolic disorders can occur many years after treatment, therefore long-term follow-up is mandatory.

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Section: Discussionsupporting

confidence: 86%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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

Feola

Pirchio

Puliani

et al. 2022

J Endocrinol Invest

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“…In women with panhypopituitarism, rhGH substitution—in addition to gonadotropins, LT4 and hydrocortisone—may be essential for obtaining pregnancy [ 47 ]. Current knowledge on GH’s influence on ovaries has recently been discussed by Devesa and Caicedo [ 48 ], and interactions between GH/IGF-1 and testicular function by Tenuta et al [ 49 ]. However, GH secreted by the pituitary gland of the fetus seems to have no evident effects on intrauterine growth, as the birth sizes of newborns with congenital GHD are, in general, within the normal range [ 1 , 8 , 9 , 18 ].…”

Section: Discussionmentioning

confidence: 99%

Delayed Diagnosis of Congenital Combined Pituitary Hormone Deficiency including Severe Growth Hormone Deficiency in Children with Persistent Neonatal Hypoglycemia—Case Reports and Review

Smyczyńska

Pawelak

Hilczer

et al. 2022

IJMS

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Apart from stimulation of human growth and cell proliferation, growth hormone (GH) has pleiotropic metabolic effects in all periods of life. Severe GH deficiency is a common component of combined pituitary hormone deficiency (CPHD). CPHD may be caused by mutations in the genes encoding transcription factors and signaling molecules involved in normal pituitary development; however, often its genetic cause remains unknown. Symptoms depend on which hormone is deficient. The first symptom of GH or adrenocorticotropic hormone (ACTH) deficiency may be persistent hypoglycemia in apparently healthy newborns, which is often neglected. Diagnosing CPHD is based on decreased concentrations of hormones secreted by the anterior pituitary and peripheral endocrine glands. Findings in magnetic resonance imaging vary widely, including anterior pituitary hypoplasia/aplasia or pituitary stalk interruption syndrome (PSIS). Delayed diagnosis and treatment can be life-threatening. GH therapy is necessary to recover hypoglycemia and to improve auxological and psychomotor development. We present two girls, diagnosed and treated in our departments, in whom the diagnosis of CPHD was delayed, despite persistent neonatal hypoglycemia; and a review of similar cases, with attention paid to progress in the genetic assessments of such patients, since the introduction of whole exome sequencing that is especially important for PSIS.

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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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A clinical approach to parasellar lesions in the transition age

Cited by 4 publications

References 249 publications

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

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

Delayed Diagnosis of Congenital Combined Pituitary Hormone Deficiency including Severe Growth Hormone Deficiency in Children with Persistent Neonatal Hypoglycemia—Case Reports and Review

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

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