Selläre Neoplasien und tumorähnliche Läsionen

Salzman, Karen L.; Osborn, Anne G.; Grigoryev, Maria

doi:10.1016/b978-3-437-21041-9.00025-x

Cited by 2 publications

(15 citation statements)

References 21 publications

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“…CP is overall rare accounting for 1.2-4.6% of all brain tumors; [2,13] yet, its incidence is higher in children, accounting for 5-13% of all pediatric brain tumors [14][15][16]. The overall incidence of CP was reported as 0.13 per 100,000 person-years in the USA with no difference between genders or races [17].…”

Section: Epidemiologymentioning

confidence: 99%

Craniopharyngioma

Kurt¹,

Aslan²

2023

Central Nervous System Tumors - Primary and Secondary

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Craniopharyngioma (CP) is a rare, benign, slow-growing, but clinically aggressive tumor located mainly in the sellar and suprasellar regions. While it occurs equally in children and adults, there are two peaks in the age distribution: first in 5–14 years of age and second in 45–74 years of age. The clinical presentation varies according to the age of patients, while the predominant symptoms are visual disturbances, headache, and endocrine dysfunctions. CPs are topographically classified in several subgroups based on the relationship of the tumor to the sella, diaphragma sellae, optic chiasm, stalk, and third ventricle; whereas the pathological classification includes two types: adamantinomatous (aCP) and papillary (pCP). Distinctive features of aCP are cysts with content of “motor-oil” fluid, calcification, wet keratin, peripheral palisading of basal cells, stellate reticulum, and mutations in CTNNB1/β-catenin gene; and those of Pcp are regular stratified squamous epithelium, devoid of cilia, papillary projections, no calcification, rare cyst with a clear fluid, and mutations in BRAF V600E. The surgical approaches include transcranial (subfrontal, pterional, transcallosal, and transcortical-transventricular) and transsfenoidal approaches, having different selection criteria, advantages, and disadvantages. Despite complete resection and radiotherapy, CPs are inclined to recur causing high morbidity and mortality.

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Section: Epidemiologymentioning

confidence: 99%

Craniopharyngioma

Kurt¹,

Aslan²

2023

Central Nervous System Tumors - Primary and Secondary

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“…The persistence of the fonticulus frontalis or pre-nasal space beyond the eighth week of gestation is responsible for a delayed ossification of the “crista galli” and cribriform plate and is related to the occurrence of cephaloceles and naso-facial lipomas, both potentially associated with syndromic frontonasal dysplasia (FND), as well as Pai syndrome [ 1 , 3 , 7 , 14 , 19 ]. The generic term of cephaloceles also includes meningoencephaloceles and meningoceles, which in the context of Pai syndrome, generally involve the midline facial skeleton and the nasal cavity.…”

Section: Clinical Featuresmentioning

confidence: 99%

“…Frontoethmoidal, or sincipital, type involves the herniation of the brain tissue into forehead, dorsum of the nose, or orbit. Within the sincipital group, frontonasal, naso-ethmoidal, and naso-orbital variant has been reported to have an incidence of 60%, 30%, and 10%, respectively [ 19 , 20 ]. On computerized tomography (CT) scan, nasal cephaloceles are well demarcated, heterogeneous, and have a mixed density, whereas they are isointense, show a direct contiguity with basal cortex on magnetic resonance imaging (MRI), and do not enhance after gadolinium, as opposed to certain types of primary brain tumors [ 19 – 21 ].…”

Section: Clinical Featuresmentioning

confidence: 99%

“…Nasal lipomas are benign masses that can be usually excised for cosmetic rather than clinical reasons, and this is usually performed in the context of the surgical correction of other midline facial defects [ 1 ]. Furthermore, lipomas classically become hypointense with fat suppression [ 19 ].…”

Section: Clinical Featuresmentioning

confidence: 99%

“…CNS lipomas are thought to derive from an over-proliferation of the fat cells of the leptomeninges, and two types have been reported. The most common is the tubulonodular one, usually located anteriorly and associated with extensive callosal and frontonasal anomalies, in particular, agenesis or dysgenesis of the corpus callosum with a less favorable prognosis, while the curvilinear lipoma is posteriorly located and usually thinner than the tubulonodular type [ 19 , 23 ]. When pericallosal lipomas are isolated they are usually benign and remain asymptomatic, with a good prognosis [ 22 ].…”

Section: Clinical Featuresmentioning

confidence: 99%

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Pai syndrome: a review

et al. 2020

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Background Pai syndrome is a rare idiopathic developmental condition characterized by midline craniofacial abnormalities. It was originally described as the presence of a median cleft lip, cutaneous polyps of the nasal mucosa and face, and midline lipomas of the central nervous system, mostly at the corpus callosum. However, there is great phenotypical variability and these characteristics are rarely all present at once. Objective The aim of this review was to analyze the available evidence regarding Pai syndrome in order to better delineate this rare condition and its features. Methods We analyzed the PubMed database using the words “Pai syndrome”, “frontonasal dysplasia”, “cleft lip”, “nasal polyp”, “facial polyp”, and “corpus callosum lipoma”, including reviews, case reports and case series. Conclusion There is no consensus regarding the diagnostic criteria of Pai syndrome up to date. It is usually diagnosed at birth, and its incidence is often underestimated. At present, the etiology of Pai syndrome is unknown. Several hypotheses regarding its genetic background have been made; however, there are not enough data yet to elucidate this point. An improved awareness could help in diagnosing the condition and performing the necessary investigations. These patients should have a multidisciplinary follow-up.

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Selläre Neoplasien und tumorähnliche Läsionen

Cited by 2 publications

References 21 publications

Craniopharyngioma

Craniopharyngioma

Pai syndrome: a review

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