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Craniopharyngioma

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https://www.readbyqxmd.com/read/28918496/recurrent-papillary-craniopharyngioma-with-brafv600e-mutation-treated-with-neoadjuvant-targeted-therapy
#1
Elham Rostami, Petra Witt Nyström, Sylwia Libard, Johan Wikström, Olivera Casar-Borota, Olafur Gudjonsson
Craniopharyngiomas are histologically benign but locally aggressive tumors in the sellar region that may cause devastating neurological and endocrine deficits. They tend to recur following surgery with high morbidity; hence, postoperative radiotherapy is recommended following sub-total resection. BRAFV600E mutation is the principal oncogenic driver in the papillary variant of craniopharyngiomas. Recently, a dramatic tumor reduction has been reported in a patient with BRAFV600E mutated, multiply recurrent papillary craniopharyngioma using a combination therapy of BRAF inhibitor dabrafenib and MEK inhibitor trametinib...
September 16, 2017: Acta Neurochirurgica
https://www.readbyqxmd.com/read/28917091/recovery-from-diabetes-insipidus-and-preservation-of-thyroid-function-after-craniopharyngioma-removal-and-pituitary-stalk-sectioning
#2
Yoshikazu Ogawa, Kuniyasu Niizuma, Teiji Tominaga
OBJECTIVES: Craniopharyngioma is a slow-growing tumor, but long-term tumor control with maintenance of quality of life is sometimes very difficult to achieve, and hypothalamic disturbance should be strictly avoided in the treatment. However, management of the pituitary gland and/or pituitary stalk varies among surgeons and institutions. PATIENTS AND METHODS: This retrospective review identified 44 patients, 24 males and 20 females with craniopharyngiomas who were initially treated by surgery through the extended transsphenoidal approach with pituitary stalk sectioning at a single institute...
September 9, 2017: Clinical Neurology and Neurosurgery
https://www.readbyqxmd.com/read/28914872/-the-efficacy-of-desmopressin-in-the-treatment-of-central-diabetes-insipidus-after-resection-of-chiasmo-sellar-region-tumors
#3
L I Astaf'eva
Central diabetes insipidus (CDI) is a neuroendocrine disease, the pathogenesis of which is associated with abnormal secretion of the antidiuretic hormone. One of the specific causes of CDI is neurosurgical resection of chiasmatic-sellar region tumors. AIM: to study the efficacy and safety of desmopressin in CDI patients after resection of chiasmatic-sellar region (CSR) tumors. MATERIAL AND METHODS: Examination and treatment of patients were performed at a hospital for 7-14 days after surgery and then were continued after discharge...
2017: Zhurnal Voprosy Neĭrokhirurgii Imeni N. N. Burdenko
https://www.readbyqxmd.com/read/28893008/spontaneous-intra-parenchymal-rupture-of-craniopharyngioma-a-rare-phenomenon
#4
Vivakaran Thanga Tirupathi Rajan, Asogan Vaishnavi Janani, Aiyappan Senthil Kumar, Ilangovan Vijay Sundar
Craniopharyngioma is a relatively benign intracranial tumour that involves sellar and supra-sellar regions. Spontaneous rupture of craniopharyngioma into the sub-arachnoid space or into the ventricles is a rare phenomenon and few cases are reported in literature. We hereby report a case of sellar-suprasellar craniopharyngioma with focal intra-parenchymal rupture into brain causing aggravation of headache in a 12-year-old female child. This complication is a relatively rare phenomenon, which requires attention for early intervention...
July 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28883874/treatment-of-cystic-craniopharyngioma-with-intracystic-stereotactic-instillation-of-phosphorus-32
#5
Sohrab Shahzadi, Ahmad Soltani, Andia Shahzadi, Khosrow Parsa
OBJECTIVE: Cystic craniopharyngiomas are considered the most common intracranial nonglial tumor in children with the tendency for cyst formations. The aim of this study was to evaluate the effect of intracystic phosphorus 32 (P32) therapies on controlling the growth of the cystic component of craniopharyngioma. MATERIALS & METHODS: This clinical study was conducted on 47 patients with cystic craniopharyngioma from March 1998 to June 2012 at Shohada Tajrish Hospital, Tehran, Iran...
2017: Iranian Journal of Child Neurology
https://www.readbyqxmd.com/read/28882980/the-metabolic-syndrome-and-its-components-in-178-patients-treated-for-craniopharyngioma-after-16-years-of-follow-up
#6
Mark Wijnen, Daniel S Olsson, Marry M van den Heuvel-Eibrink, Casper Hammarstrand, Joseph A M J L Janssen, A J Van der Lely, Gudmundur Johannsson, Sebastian J C M M Neggers
OBJECTIVE: Patients with craniopharyngioma are at increased risk for cardio- and cerebrovascular mortality. The metabolic syndrome (MetS) is an important cardiometabolic risk factor, but barely studied in patients with craniopharyngioma. We aimed to investigate the prevalence of and risk factors for the MetS and its components in patients with craniopharyngioma. DESIGN: Cross-sectional study with retrospective data. METHODS: We studied the prevalence of and risk factors for the MetS and its components in 110 Dutch (median age 47 years, range 18-92) and 68 Swedish (median age 50 years, range 20-81) patients with craniopharyngioma with ≥3 years of follow-up (90 females [51%]; 83 patients with childhood-onset craniopharyngioma [47%]; median follow-up after craniopharyngioma diagnosis 16 years [range 3-62])...
September 7, 2017: European Journal of Endocrinology
https://www.readbyqxmd.com/read/28878739/precocious-puberty-or-premature-thelarche-analysis-of-a-large-patient-series-in-a-single-tertiary-center-with-special-emphasis-on-6-to-8-year-old-girls
#7
Tero Varimo, Heta Huttunen, Päivi Johanna Miettinen, Laura Kariola, Johanna Hietamäki, Annika Tarkkanen, Matti Hero, Taneli Raivio
INTRODUCTION: We describe the etiology, MRI findings, and growth patterns in girls who had presented with signs of precocious puberty (PP), i.e., premature breast development or early menarche. Special attention was paid to the diagnostic findings in 6- to 8-year-olds. MATERIALS AND METHODS: We reviewed the medical records of 149 girls (aged 0.7-10.3 years) who had been evaluated for PP in the Helsinki University Hospital between 2001 and 2014. RESULTS: In 6- to 8-year-old girls, PP was most frequently caused by idiopathic gonadotropin-releasing hormone (GnRH)-dependent PP (60%) and premature thelarche (PT; 39%)...
2017: Frontiers in Endocrinology
https://www.readbyqxmd.com/read/28871375/walter-e-dandy-his-contributions-to-pituitary-surgery-in-the-context-of-the-overall-johns-hopkins-hospital-experience
#8
Andrea Corsello, Giulia Di Dalmazi, Fabiana Pani, Paulina Chalan, Roberto Salvatori, Patrizio Caturegli
BACKGROUND: Walter E. Dandy (1886-1946) was an outstanding neurosurgeon who spent his entire career at the Johns Hopkins Hospital. After graduating from medical school in 1910, he completed a research fellowship in the Hunterian laboratory with Harvey Cushing and then joined the Department of Surgery as resident, rising to the rank professor in 1931. Dandy made several contributions that helped building the neurosurgical specialty, most famously the introduction of pneumo-ventriculography to image brain lesions for which he received a Nobel prize nomination...
September 4, 2017: Pituitary
https://www.readbyqxmd.com/read/28869375/treatment-strategies-for-craniopharyngiomas
#9
Malte Ottenhausen, Kavelin Rumalla, Emanuele LA Corte, Andrew Alalade, Prakash Nair, Jonathan Forbes, Atef Ben Nsir, Theodore H Schwartz
Craniopharyniomas arise from the sellar region and are particularly challenging because of their close proximity to critical neurovascular structures, including cranial nerves, brainstem, internal carotid arteries, posterior cerebral arteries, hypothalamus, and the pituitary gland. The tumors are benign on histology but can cause serious symptoms by compression of surrounding vital structures. While radical surgery and gross total resection (GTR) remains the first line treatment, choosing the optimal surgical approach is critical for the minimization of complications and post-operative morbidity...
September 4, 2017: Journal of Neurosurgical Sciences
https://www.readbyqxmd.com/read/28867358/radiation-induced-large-vessel-cerebral-vasculopathy-in-pediatric-patients-with-brain-tumors-treated-with-proton-radiation-therapy
#10
Stephen F Kralik, Gordon A Watson, Chie-Schin Shih, Chang Y Ho, Whitney Finke, Jeffrey Buchsbaum
PURPOSE: The purpose of this research was to evaluate the incidence, time to development, imaging patterns, risk factors, and clinical significance of large vessel cerebral vasculopathy in pediatric patients with brain tumors treated with proton radiation therapy. METHODS AND MATERIALS: A retrospective study was performed on 75 consecutive pediatric patients with primary brain tumors treated with proton radiation therapy. Radiation-induced large vessel cerebral vasculopathy (RLVCV) was defined as intracranial large vessel arterial stenosis or occlusion confirmed on magnetic resonance angiography, computed tomographic angiography, catheter angiography, or a combination of these within an anatomic region with previous exposure to proton beam therapy and not present before radiation therapy...
July 12, 2017: International Journal of Radiation Oncology, Biology, Physics
https://www.readbyqxmd.com/read/28859712/long-term-olfactory-outcome-after-nasoseptal-flap-reconstructions-in-midline-skull-base-surgery
#11
Michael B Soyka, Carlo Serra, Luca Regli, Eva Meier, David Holzmann
BACKGROUND: The nasoseptal flap (NSF) is a universally used surgical technique to cover defects of the skull base after extended sinonasal surgery. Long-term follow-up of complications, sequelae, and smell function is largely unknown. OBJECTIVE: The main objective of this study was to investigate monorhinal olfaction after transsphenoidal skull base surgery. METHODS: Patients who underwent skull base surgery for midline lesions (craniopharyngioma and pituitary tumors) by using a NSF, also referred to as the Haddad flap, were assessed for their long-term outcome...
September 1, 2017: American Journal of Rhinology & Allergy
https://www.readbyqxmd.com/read/28859336/molecular-analyses-reveal-inflammatory-mediators-in-the-solid-component-and-cyst-fluid-of-human-adamantinomatous-craniopharyngioma
#12
Andrew M Donson, John Apps, Andrea M Griesinger, Vladimir Amani, Davis A Witt, Richard C E Anderson, Toba N Niazi, Gerald Grant, Mark Souweidane, James M Johnston, Eric M Jackson, Bette K Kleinschmidt-DeMasters, Michael H Handler, Aik-Choon Tan, Lia Gore, Alex Virasami, Jose Mario Gonzalez-Meljem, Thomas S Jacques, Juan Pedro Martinez-Barbera, Nicholas K Foreman, Todd C Hankinson
Pediatric adamantinomatous craniopharyngioma (ACP) is a highly solid and cystic tumor, often causing substantial damage to critical neuroendocrine structures such as the hypothalamus, pituitary gland, and optic apparatus. Paracrine signaling mechanisms driving tumor behavior have been hypothesized, with IL-6R overexpression identified as a potential therapeutic target. To identify potential novel therapies, we characterized inflammatory and immunomodulatory factors in ACP cyst fluid and solid tumor components...
September 1, 2017: Journal of Neuropathology and Experimental Neurology
https://www.readbyqxmd.com/read/28843459/site-and-type-of-craniopharyngiomas-impact-differently-on-24-hour-circadian-rhythms-and-surgical-outcome-a-neurophysiological-evaluation
#13
M Foschi, L Sambati, M Zoli, G Pierangeli, A Cecere, F Mignani, G Barletta, C Sturiale, M Faustini-Fustini, L Milanese, P Cortelli, D Mazzatenta, F Provini
This study aimed to quantify 24h body core temperature (BcT°) and sleep-wake cycle rhythm alterations in craniopharyngioma (CP) patients and to identify markers related to the postsurgical outcomes. Ten consecutive CP patients underwent neuroradiological, endocrinological and ophthalmological evaluations, 24h BcT° and sleep-wake cycle recordings before and after endoscopic endonasal surgery. The sample included four women and six men. Nocturnal sleep efficiency was pathologically reduced in eight patients before surgery...
August 18, 2017: Autonomic Neuroscience: Basic & Clinical
https://www.readbyqxmd.com/read/28840946/high-resolution-melting-and-immunohistochemical-analysis-efficiently-detects-mutually-exclusive-genetic-alterations-of-adamantinomatous-and-papillary-craniopharyngiomas
#14
Koji Yoshimoto, Ryusuke Hatae, Satoshi O Suzuki, Nobuhiro Hata, Daisuke Kuga, Yojiro Akagi, Takeo Amemiya, Yuhei Sangatsuda, Nobutaka Mukae, Masahiro Mizoguchi, Toru Iwaki, Koji Iihara
Craniopharyngioma consists of adamantinomatous and papillary subtypes. Recent genetic analysis has demonstrated that the two subtypes are different, not only in clinicopathological features, but also in molecular oncogenesis. Papillary craniopharyngioma (pCP) is characterized by a BRAF mutation, the V600E (Val 600 Glu) mutation. Adamantinomatous craniopharyngioma (aCP) can be distinguished by frequent β-catenin gene (CTNNB1) mutations. Although these genetic alterations can be a diagnostic molecular marker, the precise frequency of these mutations in clinical specimens remains unknown...
August 25, 2017: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/28840085/-pure-suprasellar-schwannoma-presented-with-communicating-hydrocephalus-a-case-report
#15
Tomoya Oishi, Seiya Takehara, Yasuhiro Yamamura, Mamoru Tomida, Shinji Ito, Ken Kuriki, Hiroki Namba
Schwannoma is a benign peripheral nerve sheath tumor originating from Schwann cells. Most intracranial schwannomas arise from vestibular nerve and schwannoma in the suprasellar region is extremely rare. A 64-year-old man presented with walking disturbance and blurred vision for three months. Lateral hemianopsia in the left eye and brachybasia were observed. Magnetic resonance imaging revealed a suprasellar tumor with strong contrast enhancement associated with communicating hydrocephalus. The cerebrospinal fluid tap test improved gait disturbance...
July 2017: NMC Case Rep J
https://www.readbyqxmd.com/read/28838874/youtcomes-following-endoscopic-endonasal-resection-of-craniopharyngiomas-in-the-pediatric-population
#16
Vishal S Patel, Andrew Thamboo, Jennifer Quon, Jayakar V Nayak, Peter H Hwang, Michael Edwards, Zara M Patel
BACKGROUND: Craniopharyngiomas have traditionally been treated via open, transcranial approaches. More recently, endoscopic, transsphenoidal approaches have been increasingly utilized; however very few case-series exist in the pediatric population. METHODS: A retrospective review of patients (<18 years) undergoing endoscopic transsphenoidal resection of craniopharyngiomas between 1995 and 2016 was performed. Preoperative data included presenting symptoms, tumor size, location, and components...
August 21, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28821944/the-2017-world-health-organization-classification-of-tumors-of-the-pituitary-gland-a-summary
#17
REVIEW
M Beatriz S Lopes
The 4th edition of the World Health Organization (WHO) classification of endocrine tumors has been recently released. In this new edition, major changes are recommended in several areas of the classification of tumors of the anterior pituitary gland (adenophypophysis). The scope of the present manuscript is to summarize these recommended changes, emphasizing a few significant topics. These changes include the following: (1) a novel approach for classifying pituitary neuroendocrine tumors according to pituitary adenohypophyseal cell lineages; (2) changes to the histological grading of pituitary neuroendocrine tumors with the elimination of the term "atypical adenoma;" and (3) introduction of new entities like the pituitary blastoma and re-definition of old entities like the null-cell adenoma...
August 18, 2017: Acta Neuropathologica
https://www.readbyqxmd.com/read/28781761/recent-advances-in-molecular-pathology-of-craniopharyngioma
#18
REVIEW
Sarah Larkin, Niki Karavitaki
Craniopharyngiomas are rare epithelial tumours arising along the path of the craniopharyngeal duct. Two major histological subtypes have been recognised, the papillary and the adamantinomatous. Craniopharyngiomas remain challenging tumours to manage and are associated with significant morbidities and mortality. Recent advances in the molecular pathology of these neoplasms have identified BRAF mutations in the papillary variant, offering promising options for targeted pharmacological treatment. The involvement of β-catenin and the Wnt pathway in the tumorigenesis of the adamantinomatous subtype has been previously established with the identification of stabilising mutations in exon 3 of CTNNB1...
2017: F1000Research
https://www.readbyqxmd.com/read/28774763/anatomic-dissection-of-arachnoid-membranes-encircling-the-pituitary-stalk-on-fresh-non-formalin-fixed-specimens-anatomo-radiologic-correlations-and-clinical-applications-in-craniopharyngioma-surgery
#19
P Ciappetta, L Pescatori
BACKGROUND: The anatomy of the arachnoid membranes and cisternal spaces around the pituitary stalk has not been yet exhaustively described and understood. In this study we performed a detailed anatomic study on fresh non formalin fixed cadavers of the arachnoid membranes encircling the pituitary stalk and correlate our anatomic findings with MRI. METHODS: Ten fresh, non formalin-fixed non silicon-injected adult cadaveric heads were analyzed in this study. The membrane and cisterns that have been studied for our study are: - The diaphragma sellae and its dural components; - The basal arachnoid membrane; - The Lilequist's membrane with its diencephalic and mesencephalic portion; - The medial carotid membrane; - The chiasmatic cistern; - The pituitary stalk...
July 31, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28766000/-pediatric-brain-tumors
#20
REVIEW
W Reith, S Bodea, R Mühl-Benninghaus
Brain tumors differ between children and adults both in histology and localization. Malignant gliomas and meningiomas predominate in adults while medulloblastomas and low-grade astrocytomas are the most frequent brain tumors in children. More than one half (50-70%) of pediatric brain tumors have an infratentorial location but only approximately 30% in adults. Brain tumors can be recognized in sonography, cranial computed tomography (CCT) and magnetic resonance imaging (MRI) by their space-consuming character and by their divergent density and intensity in comparison to normal brain parenchyma...
August 1, 2017: Der Radiologe
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