ESPE Abstracts

Background: Craniopharyngioma is a benign, slow-growing epithelial tumor, in children it contributes to 5–10% of all brain tumors. Its incidence is 0.5-2 per 1,000,000 person-years. 30–50% of these tumors are recognized in childhood, most often in ages 5–14, no sex predilection is observed. It is located mainly in the sellar/parasellar region, in children adamantinomatous variant, with tendency to recur, is the most common type. Views on the usefulness of immuno...

Introduction: Craniopharyngiomas are slow growing epithelial tumors located in the sellar or suprasellar region of the brain. Adamantinomatous subtype affects mainly children and accounts for 5–10% of all intracranial paediatric tumors. Diagnosed antenatal and neonatal craniopharyngiomas are very seldom, about 40 such cases have been detected to date. They are characterised by large size, progressive hydrocephalus and a poor prognosis.Aim: The aim o...

Tricho-rhino-phalangeal syndrome Type I (TRPS I) is a rare autosomal dominant genetic disorder clinically characterized by craniofacial and skeletal abnormalities, associated with cone-shaped epiphyses, brachydactyly and short stature. Although patients with TRPS I present various degrees of short stature, there are only four reports of growth hormone deficiency in patients with TRPS I. We present the case of TRPS I and partial GH deficiency. A 15-year-old female was referred ...

Background: TSH-secreting pituitary adenomas are rare, accounting for less than 2% of all pituitary adenomas. Their diagnosis may be difficult when a coexistence of other diseases masks the typical clinical and biochemical manifestations of TSH-hypersecretion.Objective: To report a case of a TSH-adenoma without signs/symptoms of hyperthyroidism due to underlying autoimmune thyroid disease.Results: Patient is a 17 year old male who ...

Background: Pituitary gigantism is a rare condition that occurs due to excessive GH secretion during childhood, usually associated with a pituitary adenoma. We describe a case that required the full spectrum of standard therapeutic options available.Case report: A 15-year-old boy presented with a 3-year history of intractable occipital headaches and being psychologically distressed by his tall stature (203 cm). He had mild clinical features of GH excess....

Background: The 12q14.3q15 microdeletion syndrome is a rare entity of which only 16 new cases have been described to date. The syndrome consists of the association of severe pre- and postnatal growth retardation, proportional short stature, psychomotor retardation and osteopoikilosis. The phenotypic appearance of these patients poses a differential diagnosis with Silver-Russell syndrome, among other entities.Clinical description: A 10-month-old boy was r...

Background: Craniopharyngiomas are rare embryogenic malformations of the sellar area with low-grade histological malignancy. Its incidence is 0.5–2.0 cases per million persons per year, 30–50% of all cases become apparent in children.Objective and hypotheses: The aim of the study was to analyse the clinical symptoms and treatment efficacy in children diagnosed with Craniopharyngioma.Method: A retrospective analysis includ...

Background: Brain tumours constitute the second most common tumours in childhood after leukaemia. Infra-tentorial tumours are more common. Most of the supra-tentorial tumours (STT) are in the supra or para-sellar regions. Malignant tumours are rare. The survival is 50–90% with appropriate management. However, STT and/or treatment may lead to traumatic brain injury (TBI) with endocrinopathic sequel.Methods: This is a retrospective hospital based stud...

Background: Congenital combined pituitary hormone deficiency (CPHD) may be associated with pituitary/extra pituitary abnormalities. Well-known causes are mutations in pituitary transcription factor genes. Agenesis of internal carotid artery (ICA) is a rare vascular anomaly that has been associated with CPHD.Case presentation: The patient is a 2-year old male, born at term to non-consanguineous parents, birth wt 3.2 kg, length 47 cm, no gestational or per...

Background: Macroprolactinomas are rare during childhood. Hypopituitarism is a common feature and recovery of pituitary function was reported following cabergoline therapy.Objective: We present a case of 13 year-old boy with macroprolactinoma who responded to cabergoline therapy with a complete regression of the tumor with a partial empty sella.Results: He complained of a decreased of right vision since 1 year. Evaluation of anteri...