scholarly journals Adamantinomatous craniopharyngioma

2021 ◽  
Author(s):  
Frank Gaillard
Keyword(s):  
Adamantinomatous Craniopharyngioma

Interrater reliability of a method to assess hypothalamic involvement in pediatric adamantinomatous craniopharyngioma

2020 ◽  
Vol 25 (1) ◽  
pp. 37-42 ◽  
Author(s):  
Ros Whelan ◽  
Eric Prince ◽  
David M. Mirsky ◽  
Robert Naftel ◽  
Aashim Bhatia ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Brain Tumors ◽  
Interrater Reliability ◽  
Statistical Evaluation ◽  
Grading System ◽  
Reliability Study ◽  
Postoperative Mri ◽  
Mri Scans ◽  
Adamantinomatous Craniopharyngioma

OBJECTIVEPediatric adamantinomatous craniopharyngiomas (ACPs) are histologically benign brain tumors that confer significant neuroendocrine morbidity. Previous studies have demonstrated that injury to the hypothalamus is associated with worsened quality of life and a shorter lifespan. This insight helps many surgeons define the goals of surgery for patients with ACP. Puget and colleagues proposed a 3-tiered preoperative and postoperative grading system based on the degree of hypothalamic involvement identified on MRI. In a prospective cohort from their institution, the authors found that use of the system to guide operative goals was associated with decreased morbidity. To date, however, the Puget system has not been externally validated. Here, the authors present an interrater reliability study that assesses the generalizability of this system for surgeons planning initial operative intervention for children with craniopharyngiomas.METHODSA panel of 6 experts, consisting of pediatric neurosurgeons and pediatric neuroradiologists, graded 30 preoperative and postoperative MRI scans according to the Puget system. Interrater reliability was calculated using Fleiss’ κ and Krippendorff’s α statistics.RESULTSInterrater reliability in the preoperative context demonstrated moderate agreement (κ = 0.50, α = 0.51). Interrater reliability in the postoperative context was 0.27 for both methods of statistical evaluation.CONCLUSIONSInterrater reliability for the system as defined is moderate. Slight refinements of the Puget MRI grading system, such as collapsing the 3 grades into 2, may improve its reliability, making the system more generalizable.

Clinical features and operative technique of transinfundibular craniopharyngioma

2020 ◽  
Vol 133 (1) ◽  
pp. 119-128 ◽  
Author(s):  
Bin Tang ◽  
ShenHao Xie ◽  
GuanLin Huang ◽  
ZhiGang Wang ◽  
Le Yang ◽  
...  
Keyword(s):  
Clinical Features ◽  
Operative Technique ◽  
Third Ventricle ◽  
Radical Resection ◽  
The Third ◽  
Expanded Endonasal Approach ◽  
Sequencing Studies ◽  
Characteristic Features ◽  
Mri Study ◽  
Adamantinomatous Craniopharyngioma

OBJECTIVETransinfundibular craniopharyngioma (TC) is one of the 4 subtypes of suprasellar craniopharyngioma. In this study, the authors analyzed the clinical features of and operative technique for TC.METHODSA total of 95 consecutive cases of suprasellar craniopharyngioma that had been resected via the endoscopic expanded endonasal approach were retrospectively reviewed. Patients were divided into 2 groups: 34 in the TC group and 61 in the nontransinfundibular craniopharyngioma (NC) group. Clinical and radiographic features, intraoperative findings, histopathological and genetic findings, and surgical outcomes were analyzed and compared between groups.RESULTSCompared with NC, TC was mostly seen in adult patients (97.1%); it was rare in children (2.9%). Clinical presentations tended toward headache, hydrocephalus, and diabetes insipidus. The relatively smaller volume, midline location (consistent with the stalk position), unidentifiable stalk, no shift of the third ventricle, and greater likelihood to involve the third ventricle and cause hydrocephalus were the characteristic features of TC in the preoperative MRI study. According to the degree of vertical extension of the tumor, the 34 TCs could be classified into 3 subtypes: type 1, entity was limited to stalk (n = 2, 5.9%); type 2, tumor extended up to the third ventricle (type 2a) or down to the subdiaphragmatic cavity (type 2b) (n = 23, 67.6%); and type 3, tumor extended in both directions (n = 9, 26.5%). For TC resection, the chiasm–pituitary corridor, lamina terminalis corridor, and pituitary corridor could be used separately or jointly. Most of the TCs originated from the infundibulum–tuber cinereum, grew within and along the long axis of the infundibulum, and the pituitary stalk was not usually preserved in TCs (20.6%), whereas the rate of preservation was higher (80.3%) in NCs. Bilateral hypothalamic injury was found in nearly all TCs if radical resection was performed, whereas the relationship between NCs and hypothalamus was either compression (32.8%) or unilateral invasion (67.2%). Meanwhile, the postoperative endocrine and neuropsychological function outcomes in patients with TC were worse than in patients with NC. The genetic analysis with whole-exome sequencing studies showed no differential mutations of CTNNB1 (β-catenin) and BRAF (V600E) between TC and NC subtypes, but there was a difference between adamantinomatous craniopharyngioma and papillary craniopharyngioma.CONCLUSIONSTC is a special subtype of suprasellar craniopharyngioma, which is remarkably different from NC. Identification of this type of tumor preoperatively is essential for the planning of appropriate surgical approach and degree of excision.

Enhancement of the Canonical Wnt Pathway in Rathke's Pouch Results in Pituitary Tumours Reminiscent of Human Adamantinomatous Craniopharyngioma.

2010 ◽  
pp. OR38-3-OR38-3
Author(s):  
Carles Gaston-Massuet ◽  
Cynthia L Andoniadou ◽  
Massimo Signore ◽  
Sajutha Jayakody ◽  
Nicoletta Charolidi ◽  
...  
Keyword(s):  
Wnt Pathway ◽  
Pituitary Tumours ◽  
Rathke’S Pouch ◽  
Rathke's Pouch ◽  
Canonical Wnt Pathway ◽  
Adamantinomatous Craniopharyngioma ◽  
Canonical Wnt

Expression of Insulin-like Growth Factor Type 1 Receptor is Linked to Inflammation in Adamantinomatous Craniopharyngioma

2021 ◽  
Author(s):  
Lang Yang ◽  
Kai Li ◽  
Weizhao Li ◽  
Chaohu Wang ◽  
Yi Liu ◽  
...  
Keyword(s):  
Stem Cell ◽  
Growth Factor ◽  
High Expression ◽  
Inflammatory Factors ◽  
Insulin Like Growth Factor ◽  
Factor Type ◽  
Adamantinomatous Craniopharyngioma ◽  
The Relationship ◽  
Igf1r Expression

Introduction Insulin-like growth factor type 1 receptor (IGF1R) is overexpressed in various malignant tumors, which relates to their transformation and recurrence. Craniopharyngioma is a benign tumor with malignant results, often accompanied by a severe inflammatory reaction. However, the relationship between IGF1R expression and the inflammatory response of craniopharyngioma is unclear. Methods We enrolled 85 patients with adamantinomatous craniopharyngioma (ACP) in a study to explore the relationship between IGF1R expression and clinical features of this disease. Results Patients in the IGF1R high expression group had a significantly higher incidence of hypopituitarism, higher recurrence rate and lower progression-free survival. Beta-catenin can further regulate expression of the stem cell marker, CD44, by regulating IGF1R. Using immunofluorescence, we found that tumor stem cell–like cells did not express phosphorylated (p)-ERK, although p-ERK activation was evident in the surrounding cells. Picropodophyllin, a specific inhibitor of IGF1R, increased the expression of p-ERK protein, and decreased the transcription level of interleukin-6. Conclusions High expression of IGF1R might promote inflammation of ACP, which might be an unfavorable factor for pituitary function and prognosis. The high expression of IGF1R in tumor cell stem-like cells might inhibit the expression of p-ERK and promote the generation of inflammatory factors. Insulin-like growth factor type 1 receptor plays a stemness maintenance role in ACP and regulates the production of inflammatory factors through a p-ERK pathway, which suggests that targeting IGF1R and p-ERK might provide a new direction for alleviating tumor inflammation.

scholarly journals THER-33. TOCILIZUMAB AS A POTENTIAL NOVEL THERAPY IN PATIENTS DIAGNOSED WITH ADAMANTINOMATOUS CRANIOPHARYNGIOMA

2019 ◽  
Vol 21 (Supplement_2) ◽  
pp. ii120-ii121
Author(s):  
Sydney Grob ◽  
Andrew Donson ◽  
Eric Prince ◽  
Trinka Vijmasi ◽  
Nicholas Foreman ◽  
...  
Keyword(s):  
Novel Therapy ◽  
Adamantinomatous Craniopharyngioma

scholarly journals Second-hit APC mutation in a familial adamantinomatous craniopharyngioma

2020 ◽  
Vol 22 (6) ◽  
pp. 889-891 ◽  
Author(s):  
Alexander Gorelyshev ◽  
Nadia Mazerkina ◽  
Olga Medvedeva ◽  
Evgeny Vasilyev ◽  
Vasily Petrov ◽  
...  
Keyword(s):  
Second Hit ◽  
Adamantinomatous Craniopharyngioma ◽  
Apc Mutation

scholarly journals Harvey Cushing and pituitary Case Number 3 (Mary D.): the origin of this most baffling problem in neurosurgery

2016 ◽  
Vol 41 (1) ◽  
pp. E6 ◽  
Author(s):  
José María Pascual ◽  
Ruth Prieto
Keyword(s):  
Great Influence ◽  
Pituitary Surgery ◽  
Multiple Sources ◽  
Tumor Registry ◽  
Neurosurgical Procedure ◽  
Harvey Cushing ◽  
History Of ◽  
Seminal Article ◽  
Adamantinomatous Craniopharyngioma ◽  
Case Number

From the very beginning of his career, Harvey Williams Cushing (1869–1939) harbored a deep interest in a complex group of neoplasms that usually developed at the infundibulum. These were initially known as “interpeduncular” or “suprasellar” cysts. Cushing introduced the term “craniopharyngioma” for these lesions, which he believed represented one of the most baffling problems faced by neurosurgeons. The patient who most influenced Cushing's thinking was a 16-year-old seamstress named “Mary D.,” whom he attended in December 1901, exactly the same month that Alfred Fröhlich published his seminal article describing an adiposogenital syndrome in a young boy with a pituitary cyst. Both Cushing's and Fröhlich's patients showed similar symptoms caused by the same type of tumor. Notably, Cushing and Fröhlich had met one another and became good friends in Liverpool the summer before these events took place. Their fortunate relationship led Cushing to realize that Fröhlich's syndrome represented a state of hypopituitarism and provided a useful method of diagnosing interpeduncular cysts. It is noteworthy that Cushing's very first neurosurgical procedure on a pituitary tumor was performed in the case of Mary D.'s “interpeduncular cyst,” on February 21, 1902. Cushing failed to remove this lesion, which was later found during the patient's autopsy. This case was documented as Pituitary Case Number 3 in Cushing's masterpiece, The Pituitary Body and Its Disorders, published in 1912. This tumor was considered “a teratoma”; however, multiple sources of evidence suggest that this lesion actually corresponded to an adamantinomatous craniopharyngioma. Unfortunately, the pathological specimens of this lesion were misplaced, and this prompted Cushing's decision to retain all specimens and documents of the cases he would operate on throughout his career. Accordingly, Mary D.'s case crystallized the genesis of the Cushing Brain Tumor Registry, one of Cushing's major legacies to neurosurgery. In this paper the authors analyze the case of Mary D. and the great influence it had on Cushing's conceptions of the pituitary gland and its afflictions, and on the history of pituitary surgery.

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