Craniopharyngioma is a common sellar region tumor occurring in children. It usually manifests as endocrinological deficits such as short stature, delayed puberty, and obesity. Patients with craniopharyngioma commonly present with visual deficits and hydrocephalus. The authors present the case of a child who presented with short stature and clinical evidence of Cushing disease (CD) associated with a suprasellar tumor. The patient underwent insertion of an Ommaya reservoir into the tumor's cystic portion. High adrenocorticotropic hormone (ACTH) levels were demonstrated within the cyst's fluid and in the serum. After adequate decompression of the tumor, the patient underwent total resection. The tumor pathology was compatible with an adamantinomatous craniopharyngioma and immunohistochemical studies failed to show staining for ACTH. Panhypopituitarism developed postoperatively in the patient and he received hormone substitution therapy with final adequate height and normal-high weight. The neurosurgical implications of CD along with a possible mechanism for this patient's presentation are discussed in detail on the basis of the pertinent literature.
Download full-text PDF |
Source |
|---|---|
| http://dx.doi.org/10.3171/ped.2005.102.3.0318 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Cushing syndrome.
Nat Rev Dis Primers
January 2025
Endocrine Division, Department of Medicine, Centre hospitalier de l'Université de Montréal (CHUM), Montreal, Québec, Canada.
Cushing syndrome (CS) is a constellation of signs and symptoms caused by excessive exposure to exogenous or endogenous glucocorticoid hormones. Endogenous CS is caused by increased cortisol production by one or both adrenal glands (adrenal CS) or by elevated adrenocorticotropic hormone (ACTH) secretion from a pituitary tumour (Cushing disease (CD)) or non-pituitary tumour (ectopic ACTH secretion), which stimulates excessive cortisol production. CS is associated with severe multisystem morbidity, including impaired cardiovascular and metabolic function, infections and neuropsychiatric disorders, which notably reduce quality of life.
View Article and Find Full Text PDFCushing's syndrome related to higher rates and earlier onset of cataract: A nationwide retrospective cohort study.
Graefes Arch Clin Exp Ophthalmol
January 2025
School of Medicine, Faculty of Medical and Health Sciences, Tel Aviv University, Tel Aviv, Israel.
Purpose: To investigate the risk of cataract in patients with Cushing's syndrome (CS) and evaluate whether disease onset occurs at an earlier age compared to general population.
Methods: A nationwide retrospective matched-cohort study including individuals diagnosed with endogenous CS from 2000 to 2023. Patients with CS were matched in a 1:5 ratio with a control group individually matched for age, sex, socioeconomic status, and body mass index.
Outcomes of a Population-Based Congenital Cytomegalovirus Screening Program.
JAMA Pediatr
January 2025
Department of Pediatrics, Children's Hospital of Eastern Ontario, University of Ottawa, Ottawa, Ontario, Canada.
Importance: Detection of congenital cytomegalovirus (cCMV) infection has previously relied on targeted screening programs or clinical recognition; however, these approaches miss most cCMV-infected newborns and fail to identify those infants who are asymptomatic at birth but at risk for late-onset sensorineural hearing loss.
Objective: To determine the feasibility of using routinely collected newborn dried blood spots (DBS) in a population-based cCMV screen to identify infants at risk for hearing loss and describe outcomes of infants screened.
Design, Setting, And Participants: This diagnostic study of a population-based screening program in Ontario, Canada, took place from July 29, 2019, to July 31, 2023.
Approach to Endocrine Hypertension: A Case-Based Discussion.
Curr Hypertens Rep
January 2025
Department of Endocrinology, Kasturba Medical College, Manipal Academy of Higher Education, Manipal, 576104, India.
Purpose Of Review: Hypertension remains a major chronic disease morbidity across the world, even in the twenty-first century, affecting ≈40% of the global population, adversely impacting the healthcare budgets in managing the high incidence of cardiovascular disease (CVD) complications and mortality because of elevated blood pressure (BP). However, evaluation and management of endocrine hypertension are not optimal in clinical practice. With three unique clinical case scenarios, we update the evidence base for diagnostic evaluation and management of endocrine hypertension in this review to inform appropriate day-to-day clinical practice decisions.
View Article and Find Full Text PDFMetabolic, hormonal profiles and comorbidities in pituitary Cushing's syndrome, adrenal Cushing's syndrome and mild autonomous cortisol secretion: a comparative study.
Postgrad Med
January 2025
Department of Endocrinology, Ankara City Hospital, Ankara, Türkiye.
Purpose: Our study aimed to discern disparities in metabolic, hormonal profiles, and comorbidities among patients with pituitary Cushing (PC), adrenal Cushing (AC), and Mild autonomous cortisol secretion (MACS).
Methods: We conducted a retrospective analysis involving 76 patients diagnosed with PC ( = 26), AC ( = 21), and MACS ( = 29) at our clinic. We compared the groups' demographic data, clinical characteristics, biochemical profiles, hormonal analyses, and surgical interventions.
Want AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!