Clinical presentation and outcome of pituitary adenomas in teenagers

431

583

Background: Pituitary tumours are common and easily treated by surgery or medical treatment in most cases. However, a small subset of pituitary tumours does not respond to standard medical treatment and presents with multiple local recurrences (aggressive pituitary tumours) and in rare occasion with metastases (pituitary carcinoma). The present European Society of Endocrinology (ESE) guideline aims to provide clinical guidance on diagnosis, treatment and follow-up in aggressive pituitary tumours and carcinomas. Methods: We decided upfront, while acknowledging that literature on aggressive pituitary tumours and carcinomas is scarce, to systematically review the literature according to the GRADE (Grading of Recommendations Assessment, Development and Evaluation) system. The review focused primarily on first-and second-line treatment in aggressive pituitary tumours and carcinomas. We included 14 single-arm cohort studies (total number of patients = 116) most on temozolomide treatment (n = 11 studies, total number of patients = 106). A positive treatment effect was seen in 47% (95% CI: 36-58%) of temozolomide treated. Data from the recently performed ESE survey on aggressive pituitary tumours and carcinomas (165 patients) were also used as backbone for the guideline. In patients responding to first-line temozolomide, we suggest continuing treatment for at least 6 months in total. Furthermore, the guideline offers recommendations for patients who recurred after temozolomide treatment, for those who did not respond to temozolomide and for patients with systemic metastasis.

Section: Special Circumstances a Paediatricmentioning

confidence: 99%

European Society of Endocrinology Clinical Practice Guidelines for the management of aggressive pituitary tumours and carcinomas

Raverot

Burman

McCormack

et al. 2018

431

583

“…Previous studies have typically been small (%21 cases (2-5)), with the exception of a few larger studies with 26-44 patients (6)(7)(8)(9), which often comprised of patients from multiple centres. The two largest studies (containing 136 (10) and 150 (11) patients) have described children with adenomas who have undergone surgery and represent a different patient group.…”

Section: Introductionmentioning

confidence: 99%

“…Previous studies have tended to report either the clinical features at presentation (2,8,11) or the results of surgery (3,10,14) with outcomes described in terms of visual field deficits, normalisation of hormones (in secreting tumours), loss of pituitary hormone function, remission/cure or the need for further treatment (radiotherapy or repeat surgery) for residual tumour (6,7). In addition to these factors, we were also interested in investigating long-term sequelae, such as impaired fertility, which has been described in both adult patients with pituitary adenomas (15) and in those with hypopituitarism from various causes (e.g.…”

Section: Introductionmentioning

confidence: 99%

“…Additionally, the definition of adolescence is variable between studies, with the upper limit for age ranging from 17 to 20 years. Owing to the ambiguity in defining adolescence and the fact that symptoms related to pituitary adenomas have been reported to begin 2-5 years before diagnosis (6,12,13), we retrospectively reviewed the hospital records of patients who were diagnosed with a pituitary adenoma up until the age of 21.…”

Section: Introductionmentioning

confidence: 99%

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Pituitary adenomas in childhood, adolescence and young adulthood: presentation, management, endocrine and metabolic outcomes

Steele

MacFarlane

Blair

et al. 2010

Objective: To elucidate the long-term outcomes of pituitary adenomas diagnosed in childhood and adolescence, knowledge of which remains sparse. Design and methods: A retrospective review of patients aged %21 years at diagnosis of pituitary adenoma, attending a neuroendocrine service in Liverpool, UK, between 1984UK, between -2009. Results: There were 41 patients (33 female), mean age at diagnosis 17.3 years (range 11-21) and mean follow-up 9.6 years; 29 patients had prolactinomas (15 macroprolactinomas), 6 non-functioning pituitary adenomas (NFPAs), 5 Cushing's disease (CD) and 1 acromegaly. All prolactinoma patients received dopamine agonists (DAs) and three also underwent pituitary surgery. Furthermore, ten patients underwent surgery: five with CD, one with acromegaly and four with NFPA. Four received radiotherapy after surgery. Another ten patients received hormone replacement: nine hydrocortisone, five thyroxine, seven sex steroids and five GH; another seven had severe asymptomatic GH deficiency. Three female patients were treated for infertility (two successfully). Thirteen patients gained significant weight (body mass index (BMI) increase O2 kg/m 2 ) since diagnosis and 16 in total are now obese (BMIO30 kg/m 2 ). Five were treated with orlistat and one attended a weight management service. Two received antihypertensive medications, two had type 2 diabetes and four were treated for dyslipidaemia. Conclusions: This is one of the largest reviews of patients aged 21 or younger at diagnosis of pituitary adenoma followed up by a single service. Two-thirds had prolactinomas, all were treated with DAs and three underwent surgery. Increased cardiovascular risk factors (obesity and dyslipidaemia) and infertility are important sequelae and active identification and treatment are necessary.

“…Paediatric CD may also present with typical Cushingoid features. As ACTH-secreting adenomas are usually small (!5 mm in diameter), symptoms such as headache and visual field defects due to tumour growth rarely occur (9), in contrast to NFPA which are typically larger (5,10). As in adults, transsphenoidal surgery is the treatment of choice in children with CD, as well as in those with NFPA requiring treatment due to local symptoms (11).…”

Section: Introductionmentioning

confidence: 99%

Comorbidity and cardiovascular risk factors in adult GH deficiency following treatment for Cushing's disease or non-functioning pituitary adenomas during childhood

Ragnarsson

Höybye

Jönsson

et al. 2012

ObjectiveCushing's disease (CD) and non-functioning pituitary adenoma (NFPA) are rare in paediatric patients. The aim of this study was to describe long-term consequences in adults with GH deficiency (GHD) treated for CD or NFPA during childhood.Design, patients and methodsThis was a retrospective analysis of data from KIMS (Pfizer International Metabolic Database). Background characteristics, anthropometry and comorbidity were studied in 47 patients diagnosed with childhood-onset (CO)-CD and 62 patients with CO-NFPA. Data from 100 ACTH-sufficient patients with CO-idiopathic hypopituitarism (CO-Idio) were used for comparison. Cardiovascular risk profile was analysed at baseline and at 1 year on GH treatment in a subgroup of patients (17 CO-CD, 24 CO-NFPA and 55 CO-Idio) not receiving GH treatment at study entry.ResultsThe median age at diagnosis of pituitary tumour was 14.0 years (range 10–17) in patients with CO-CD and 13.7 years (range 8–17) in CO-NFPA. In addition to GHD, 41% of patients with CO-CD had three or four other pituitary hormone deficiencies compared with 78% of patients with CO-NFPA (P<0.001). Eighty-nine per cent of patients with CO-CD had height SDS lower than 0 compared with 61% of patients with CO-NFPA (P=0.002). Hypertension was more common in CO-CD compared with CO-Idio (23 vs 9%,P=0.018). At 1 year on GH treatment, total- and low-density lipoprotein-cholesterol decreased significantly in CO-CD but not in CO-NFPA.ConclusionAdult patients with GHD following treatment for paediatric CD and NFPA have long-term adverse consequences. Despite more severe hypopituitarism in CO-NFPA, patients with CO-CD have more frequently compromised final stature.