Craniopharyngioma
David A. Walker, Giorgio Perilongo, Roger E. Taylor, Ian F. Pollack in Brain and Spinal Tumors of Childhood, 2020
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.
Skull base tumors
Michael Y. Wang, Andrea L. Strayer, Odette A. Harris, Cathy M. Rosenberg, Praveen V. Mummaneni in Handbook of Neurosurgery, Neurology, and Spinal Medicine for Nurses and Advanced Practice Health Professionals, 2017
The initial neurosurgical consultant offered a limited resection. Another consultant deemed the tumor inoperable and suggested no intervention. Several radiation options were also discussed: intratumoral seed radiation, cystic aspiration, or radiation alone. The family sought a third opinion from a neurosurgeon specializing in skull base surgery. This surgeon offered an attempt at gross total resection, which would be the most likely approach to result in a cure. Since craniopharyngiomas have a high recurrence rate, many experts believe the first attempt at surgery offers the best hope for a cure (Cheng et al., 2016). Ultimately, the patient and her parents opted for maximal safe resection with the hope for a cure, or if not possible, for a delay in recurrence.
The size and nature of the problem
Lindy Burton in Care of the Child Facing Death, 1974
In the field of childhood malignancy, tumours of the brain are the most numerous (Table 2). They are of various types and found in several different sites, and although all present problems of diagnosis and treatment, some carry a relatively good outlook. The onset is usually insidious. Fits, a common presenting symptom of cerebral tumours in adults, are much less frequent, and occur later, when a child is the victim. This is probably due to the fact that most childhood tumours lie in the brainstem, whereas in adults the majority of growths occur in the cerebral hemispheres. The occurrence of persistent headaches, often accompanied by vomiting, may indicate that the intra-cranial pressure is raised, but occurs whether the cause is a tumour, an abscess, meningitis or hydrocephalus, and is in no way specific. Highly malignant tumours of the brainstem are inoperable and rapidly fatal, with sometimes a limited response to radiotherapy. Tumours in the cerebellum may disturb balance and limb movements, but are sometimes slowly growing, relatively benign, and susceptible to complete removal leaving varying degrees of handicap. An unusual variety of tumour, the craniopharyngioma, arises from an embryological remnant in the region of the pituitary gland, and by local pressure produces impairment of the hormonal regulation of growth and sexual development, as well as interfering with vision. Although it is not usually malignant, it can be extremely difficult to remove because its situation is almost inaccessible without serious damage to vital structures. Pineal gland tumours are rare, but may present as precocious puberty, also due to a disturbance of the endocrine system.
Do Anxiety and Mood Vary among Disparate Sleep Profiles in Youth with Craniopharyngioma? A Latent Profile Analysis
Published in Behavioral Sleep Medicine, 2022
Sara M. Witcraft, Molly E. Wickenhauser, Kathryn M. Russell, Belinda N. Mandrell, Heather M. Conklin, Thomas E. Merchant, Valerie McLaughlin Crabtree
Craniopharyngioma is a rare brain tumor that arises in adults and children. Although histologically benign, its association with critical neurovascular structures and cerebrospinal fluid pathways results in significant morbidity at the time of presentation and later following surgery or radiation therapy (Cohen et al., 2011; Jacola et al., 2016). Long-term survival exceeds 90% in most series, regardless of treatment (Müller et al., 2001); however, the majority of patients (i.e., 52% – 87%) experience significant complications from tumor and treatment (Jacola et al., 2016). Psychosocial impairment (Klages et al., 2019; Müller, 2019), deficits in memory (Waber et al., 2006) and cognitive functioning (Fournier-Goodnight et al., 2017), vision loss (Merchant et al., 2002), and sleep dysregulation and excessive daytime sleepiness (EDS; Cohen et al., 2011; Jacola et al., 2016) are common in those diagnosed with craniopharyngioma and may compound disease- and treatment-related fatigue and/or negatively impact quality of life (Cohen et al., 2011).
A Latent Profile Analysis of Sleep, Anxiety, and Mood in Youth with Craniopharyngioma
Published in Behavioral Sleep Medicine, 2022
Sara M. Witcraft, Molly E. Wickenhauser, Kathryn M. Russell, Belinda N. Mandrell, Heather M. Conklin, Thomas E. Merchant, Valerie McLaughlin Crabtree
Craniopharyngioma is a rare brain tumor that arises in adults and children. Although histologically benign, its association with critical neurovascular structures and cerebrospinal fluid pathways results in significant morbidity at the time of presentation and later following surgery or radiation therapy (Cohen et al., 2011; Jacola et al., 2016). Long-term survival exceeds 90% in most series, regardless of treatment (Müller et al., 2001); however, the majority of patients (i.e., 52–87%) experience significant complications from tumor and treatment (Jacola et al., 2016). Psychosocial impairment (Klages et al., 2019; Müller, 2019), deficits in memory (Waber et al., 2006) and cognitive functioning (Fournier-Goodnight et al., 2017), vision loss (Merchant et al., 2002), and sleep dysregulation and excessive daytime sleepiness (EDS; Cohen et al., 2011; Jacola et al., 2016; Mandrell et al., 2020) are common in those diagnosed with craniopharyngioma and may compound disease- and treatment-related fatigue and/or negatively impact quality of life (Cohen et al., 2011).
Actigraphy versus Polysomnography to Measure Sleep in Youth Treated for Craniopharyngioma
Published in Behavioral Sleep Medicine, 2020
Kristin Niel, Kayla N. LaRosa, Kimberly L. Klages, Thomas E. Merchant, Merrill S. Wise, Sara M. Witcraft, Donna Hancock, Mary Caples, Belinda N. Mandrell, Valerie Mclaughlin Crabtree
Craniopharyngioma is a rare, benign epithelial brain tumor that occurs along an axis extending from the sella turica, through the pituitary stalk, and into the hypothalamus with 0.5–2.5 new cases per million each year (Poretti, Grotzer, Ribi, Schönle, & Boltshauser, 2004) representing 6 to 13% of all childhood brain tumors (Cohen, Guger, & Hamilton, 2011; Garrė & Cama, 2007). Often, as the tumor enlarges it invades nearby anatomical structures, and both the tumor and its resection and treatment can be associated with significant neurological and endocrinological complications (Cohen et al., 2011). Although the long-term survival of youth with craniopharyngioma is high, their quality of life (QoL) after neurosurgical intervention is often marked by excessive daytime sleepiness (EDS), decreased physical activity, hypothalamic obesity, mood changes, and weak performance on neurocognitive measures and academic fluency (Cohen et al., 2011; Fournier-Goodnight et al., 2017). In 52–87% of cases, the location of the tumor has been found to cause disruption of the hypothalamic-pituitary-adrenal (HPA) axis (Muller, 2008); notably, the HPA axis is an area of the brain that plays a central role in the regulation of sleep and wake (Muller, 2016). Given the functions of the brain that are associated with the location of the tumor, youth undergoing resection have substantially decreased sleep latency, and higher likelihood of EDS and narcolepsy (Jacola et al., 2016; Muller, 2010; Muller et al., 2006; Muller, Handwerker, Wollny, Faldum, & Sorensen, 2002), all of which interfere with psychosocial function and academic QoL (Cohen et al., 2011).
Related Knowledge Centers
- Neoplasm
- Pituitary Gland
- Quadrantanopia
- Bitemporal Hemianopsia
- Optic Chiasm
- Prevalence
- Rathke'S Cleft Cyst
- Benign Tumor
- Headache
- Hydrocephalus