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Craniopharyngioma
Published in David A. Walker, Giorgio Perilongo, Roger E. Taylor, Ian F. Pollack, Brain and Spinal Tumors of Childhood, 2020
Hermann L. Müller, Claire Alapetite, Jeffrey Wisoff
Craniopharyngioma is a non-glial intracranial tumor derived from neoplastic transformation of embryonic tissue.5,53–56 Several observations, including cytokeratin expression profiles, indicate that craniopharyngiomas arise from neoplastic transformation of ectodermal-derived epithelial cell remnants of Rathke’s pouch and the craniopharyngeal duct. Epithelial cell rests have been reported to occur between the roof of the pharynx and the floor of the third ventricle, most frequently along the anterior infundibulum and the anterior superior surface of the adenohypophysis – sites of the previous Rathke pouch and involuted duct that links these structures. Metaplasia of cells derived from the tooth primordium leads to the adamantinomatous subtype, whereas metaplastic changes in cells derived from buccal mucosa primordium give rise to the squamous papillary variant.57 Further support for the origin of craniopharyngioma from Rathke’s pouch epithelium is the occasional occurrence of mixed tumors with characteristics of craniopharyngioma and Rathke cleft cysts.
Endocrinology, growth and puberty
Published in Rachel U Sidwell, Mike A Thomson, Concise Paediatrics, 2020
Rachel U Sidwell, Mike A Thomson
This is one of the most common supratentorial tumours in children. It arises from a remnant of the connection between Rathke’s pouch and the oral cavity. It is often large and cystic and 50% occur under the age of 20 years. Calcification is seen in most cases on skull X-ray.
Neurology
Published in Stephan Strobel, Lewis Spitz, Stephen D. Marks, Great Ormond Street Handbook of Paediatrics, 2019
Fenella Kirkham, Adnan Manzur, Stephanie Robb
This tumour arises from the Rathke pouch (buccal epithelium). It often comprises solid and cystic components. The tumour is slow growing but may be diagnosed late if visual acuity (Fig. 8.3) and fields (Figs 8.1, 8.2) are not tested in children with headache. Surgical excision is curative if the tumour is not large; radiotherapy is required if complete excision would jeopardise the carotid arteries, optic nerves or hypothalamus. Survival is excellent if total removal is achieved, but children often have endocrine sequelae (e.g. diabetes insipidus, hypothyroidism or inadequate corticosteroid production), requiring appropriate replacement.
Ophthalmic Manifestations of Rathke’s Cleft Cyst and Its Association to Radiological Characteristics
Published in Current Eye Research, 2021
Hyeshin Jeon, Hie Bum Suh, Woohyun Chung, Hee-young Choi
Rathke’s cleft cyst (RCC) is a benign epithelial-lined cyst formed by the failure in the obliteration of the embryonic cleft of the Rathke’s pouch located in the sellar and suprasellar region1; however, its pathogenesis remains controversial.2 Most RCCs are usually small, asymptomatic, and remain stable during life, and therefore, are usually incidental findings.1,3 Though symptoms with RCC are rare, where they do occur then headache, endocrine dysfunction, and visual disturbance are the commonest presenting symptoms. Symptoms occur if the cyst enlarges and compresses the surrounding structures.4–6 Ophthalmic manifestations include decreased visual acuity, visual field defect, and diplopia, and the incidence varies between 14% and 90%, possibly due to differences in the inclusion criteria among the reported studies and inconsistencies in objective ophthalmic evaluations.6–8
Jefferson, Antony Andrew (1922-2017) Neurosurgeon; the Last of a Medical Dynasty!
Published in British Journal of Neurosurgery, 2018
In 1956, he was made Hunterian Professor and delivered a lecture on Pituitary Tumours and Rathke’s Pouch Cyst and subsequently, was invited to deliver the “Cairns Lecture” in Adelaide, South Australia and the “Pybus Lecture” in Newcastle on Tyne. Antony was a great believer in “neurosurgery without state boundaries” and in international co-operation, actively promoting EANS, where he served as vice-president from 1985 to 1987 and lectured on many courses. He was also English Language Section Editor of ACTA Neurochirugica from 1960 to 1975. He served on the neurosurgical SAC committee, including a period as Chairman. Locally he was active in the management of the Royal Infirmary and later the Royal Hallamshire Hospital, Sheffield, serving as secretary and later as chairman of the Medical Staff Committee. He continuously worked on expanding the department and introducing new methods of treatment and diagnostics.
What are the pharmacological considerations for male congenital hypogonadotropic hypogonadism?
Published in Expert Opinion on Pharmacotherapy, 2022
Giulia Rastrelli, Mario Maggi, Giovanni Corona
Genes involved in CHH associated with other pituitary hormone deficiency (Congenital Combined Pituitary Hormone Deficiency; CPHD) overlap with isolated CHH because mutations in FGFR1, FGF8, and PROKR2 have been found in both forms [2]. Besides these, mutations in genes codifying for transcription factors involved in Rathke pouch development may cause congenital CPHD, including LHX3, LHX4, and PROP1 [2].