hrp0084fc11.5 | Neuroendocrinology | ESPE2015

Endocrinopathy after Intracranial Germ Cell Tumours (IGCT) is Disease Not Radiation-Related: Two Decades of Surveillance in a Large Tertiary Paediatric Cohort

Dimitrakopoulou Eftychia Ioanna , Chow Jasmine , Spoudeas Helen , Ederies Ash

Background: Childhood IGCT are rare, malignant tumours of the pituitary stalk and pineal region, highly curable (>90%) by multimodal therapies. Neuroendocrine outcomes are thus important. Deficits increase over time but, without longitudinal studies, it remains unclear whether they are primarily disease or treatment related.Objective and hypotheses: To determine, by longitudinal retrospective analysis in survivors, tumour- and treatment-related facto...

hrp0089p2-p313 | Pituitary, Neuroendocrinology and Puberty P2 | ESPE2018

Neuroendocrine Consequences of Hypothalamic Hamartoma and their Imaging (MRI) and Surgery Correlates

Corredor Beatriz , Caredda Elisabetta , Ederies Ash , Tisdall Martin , Cross Helen , Spoudeas Helen A.

Background: Hypothalamic hamartomas(HH) are rare heterotopic congenital malformations causing central precocious puberty(CPP) and/or resistant epilepsy whose natural history is unknown.Aim: To describe clinical and imaging features, and the risk of developing endocrine deficits, particularly after surgery.Method: Retrospective case note and imaging review of all HH diagnosed by MRI between 30.08.1991 and 24.11.17, analysed by initi...

hrp0086rfc3.1 | Pituitary | ESPE2016

Endocrinopathy in Childhood Intracranial Germ Cell Tumours is Predicted by Disease Location not Treatment: 30 year Experience from a Single Tertiary Centre

Serra-Caetano Joana , Dimitrakopoulou Eftychia , Ederies Ash , Phipps Kim , Spoudeas Helen Alexandra

Background: Childhood pineal and/or suprasellar intracranial germ cell tumours (IGCT) are highly curable (>90%) with neuraxial radiation alone; international (SIOP) trials have aimed to decrease late radiation-induced neuroendocrine morbidity by substituting chemotherapy. However, without longitudinal study, disease and treatment contributions to long term outcomes remain unknown.Objective: To define tumour and treatment factors implicated in neuroen...

hrp0082p1-d2-30 | Autoimmune Endocrine Disease | ESPE2014

Managing Children with Thickened Pituitary Stalk and/or Idiopathic Central Diabetes Insipidus: a Single Centre Experience on 63 Children

Cerbone Manuela , Ederies Ash , Losa Laura , Moreno Carolina , Sun Kristi , Spoudeas Helen A

Background and objective: Children with Thickened pituitary stalk (TPS) and/or Idiopathic central diabetes insipidus (ICDI) present to different (endocrine, oncology, and ophthalmology) specialists. Their rarity, absence of agreed radiological criteria or consensus guidance, make their management problematic. Biopsy is too dangerous and cases may remain undiagnosed or evolve over decades. We aimed: i) to longitudinally characterize a large childhood cohort presenting with TPS ...

hrp0084p3-1148 | Puberty | ESPE2015

Endocrine Dysfunction in Hypothalamic Hamartoma Depends on Presentation (Endocrine or Epileptogenic), Radiological Characteristics and Surgery

Caredda Elisabetta , Wilkinson Robert , Ederies Ash , Improda Nicola , Varadkar Sophia , Cross Helen , Tisdall Martin , Spoudeas Helen A

Background: Hypothalamic hamartomas(HH)-rare heterotopic congenital malformations (incidence 1:200 000)-present with central precocious puberty (CPP) or gelastic seizures (GS) but their natural history and best treatment strategy are unknown. Given their proximity to the hypothalamus–pituitary axis, wider endocrine dysfunction may be expected.Objective and hypotheses: To describe clinical features and any evolving endocrinopathies in HH patients, by...

hrp0086rfc3.5 | Pituitary | ESPE2016

Prospective Dynamic Evaluation of Hypothalamo-Pituitary Function in 30 Cases of Paediatric Craniopharyngioma, by Hypothalamic Injury and Treatment; A Single Centre Series

Guzzetti Chiara , Losa Laura , Improda Nicola , Pang Gloria , Phatarakijnirund Voraluck , Gan Hoong-Wei , Hayward Richard , Aquilina Kristian , Ederies Ash , Spoudeas Helen A.

Background: Craniopharyngiomas are the commonest pituitary tumours of childhood. Though benign histologically, their localisation and invasive tendency can cause significant neuroendocrine morbidity and late mortality.Objective and hypotheses: To prospectively determine risk factors for neuroendocrine morbidity by longitudinal survival analysis.Method: All children with craniopharyngioma newly presenting to our quaternary centre be...

hrp0082p2-d3-494 | Endocrine Oncology | ESPE2014

Three Year Experience of a National Interdisciplinary Initiative to Improve Outcomes for Children with Hypothalamic Pituitary Axis Tumours (HPATs) Using Multi-site Videoconferencing for Decision Making on Behalf of the UK HPAT Interest Group

Perelberg Daniel , Morillon Paul , Ederies Ash , Aquilina Kristan , Dorward Neil , Michalski Anton , Hargrave Darren , Chang Yen-Ching , Bozorgi Nillofar , James Samantha , Korbonits Marta , Drake William , Akker Scott , Mallucci Connor , Pizer Barry , Blair Jo , Kamaly Ian , Clayton Peter , Spoudeas Helen

Background: Childhood tumours of the hypothalamic pituitary axis (HPATs) are very rare and hence any single centre experience is limited. Without evidence-based guidance, treatment is individualised on a case basis. Survival rates are high, but at the expense of significant morbidity. Centralised care or wider multi-professional consultation may improve neuroendocrine and visual outcomes.Objective and hypotheses: i) To facilitate multi-professional dialo...