Papillary craniopharyngioma
Updates to Article Attributes
Papillary craniopharyngiomas are WHO grade 1 tumours of the pituitary region typically presenting as mostly solid masses in adults. They are an entirely separate entity from the far less common adamantinomatous craniopharyngioma that are found in children 1,2.
Terminology
Until the 5th Edition (2021) of the WHO classification of CNS tumours, adamantinomatous papillary craniopharyngiomas were considered a subtype of craniopharyngiomas along with adamantinomatous craniopharyngioma 1.
Increasing molecular evidence has demonstrated that these adamantinomatous and papillary craniopharyngiomasthey have distinct and mutually exclusive mutations of CTNNB1 (adamantinomatous) and BRAF respective(papillary) 11-3. TheyAs such, they are now, therefore, considered separate entities and are discussed separately.
Epidemiology
Papillary craniopharyngiomas are far less common than adamantinomatous craniopharyngiomas and rare before middle age, identified most commonly between the ages of 40 and 60 years of age 4. No sex or racial predilection has been identified 4.
Importantly, even though adamantinomatous craniopharyngiomas are thought of as tumours of childhood whereas papillary craniopharyngiomas are considered tumours of adults, adamantinomatous craniopharyngiomas are more common than papillary craniopharyngiomas in all but the very elderly 4.
Clinical presentation
Clinical presentation is variable on account of the variable location and size of the tumour. Presenting complaints include:
- headaches and raised intracranial pressure
- visual symptoms
-
hormonal imbalances
- decreased libido
- amenorrhoea
- diabetes insipidus
Pathology
Papillary craniopharyngiomas are formed of masses of monomorphic squamous epithelium with a fibrovascular core and scattered immune cells 5.
Unlike adamantinomatous craniopharyngiomas, "wet keratin" and calcifications are absent 6.
Although cysts do form, they are not a prominent feature, and the tumour is usually mostly solid 6.
This article is currently being written. Like right now!
-<p><strong>Papillary craniopharyngiomas</strong> are WHO grade 1 tumours of the pituitary region typically presenting as mostly solid masses in adults. They are an entirely separate entity from the far less common <a title="Adamantinomatous craniopharyngioma" href="/articles/adamantinomatous-craniopharyngioma">adamantinomatous craniopharyngioma</a> that are found in children<sup> 1,2</sup>. </p><h4>Terminology</h4><p>Until the 5th Edition (2021) of the <a href="/articles/who-classification-of-cns-tumours-1">WHO classification of CNS tumours</a>, adamantinomatous craniopharyngiomas were considered a subtype of craniopharyngiomas along with <a href="/articles/adamantinomatous-craniopharyngioma">adamantinomatous craniopharyngioma</a> <sup>1</sup>. Increasing molecular evidence has demonstrated that these adamantinomatous and papillary craniopharyngiomas have distinct and mutually exclusive mutations of CTNNB1 and BRAF respective<sup> 1-3</sup>. They are now, therefore, considered separate entities. </p><h4>Epidemiology</h4><p>This article is currently being written. Like <em>right now!</em></p>- +<p><strong>Papillary craniopharyngiomas</strong> are WHO grade 1 tumours of the pituitary region typically presenting as mostly solid masses in adults. They are an entirely separate entity from the far less common <a href="/articles/adamantinomatous-craniopharyngioma">adamantinomatous craniopharyngioma</a> that are found in children<sup> 1,2</sup>. </p><h4>Terminology</h4><p>Until the 5th Edition (2021) of the <a href="/articles/who-classification-of-cns-tumours-1">WHO classification of CNS tumours</a>, papillary craniopharyngiomas were considered a subtype of craniopharyngiomas along with <a href="/articles/adamantinomatous-craniopharyngioma">adamantinomatous craniopharyngioma</a> <sup>1</sup>.</p><p>Increasing molecular evidence has demonstrated that they have distinct and mutually exclusive mutations of CTNNB1 (adamantinomatous) and BRAF (papillary) <sup>1-3</sup>. As such, they are now considered separate entities and are discussed separately. </p><h4>Epidemiology</h4><p>Papillary craniopharyngiomas are far less common than adamantinomatous craniopharyngiomas and rare before middle age, identified most commonly between the ages of 40 and 60 years of age <sup>4</sup>. No sex or racial predilection has been identified <sup>4</sup>. </p><p>Importantly, even though adamantinomatous craniopharyngiomas are thought of as tumours of childhood whereas papillary craniopharyngiomas are considered tumours of adults, adamantinomatous craniopharyngiomas are more common than papillary craniopharyngiomas in all but the very elderly <sup>4</sup>. </p><h4>Clinical presentation</h4><p>Clinical presentation is variable on account of the variable location and size of the tumour. Presenting complaints include:</p><ul>
- +<li>headaches and raised intracranial pressure</li>
- +<li>visual symptoms</li>
- +<li>hormonal imbalances<ul>
- +<li>decreased libido</li>
- +<li>amenorrhoea</li>
- +<li><a href="/articles/diabetes-insipidus">diabetes insipidus</a></li>
- +</ul>
- +</li>
- +</ul><h4>Pathology</h4><p>Papillary craniopharyngiomas are formed of masses of monomorphic squamous epithelium with a fibrovascular core and scattered immune cells <sup>5</sup>.</p><p>Unlike adamantinomatous craniopharyngiomas, "wet keratin" and calcifications are absent <sup>6</sup>.</p><p>Although cysts do form, they are not a prominent feature, and the tumour is usually mostly solid <sup>6</sup>. </p><p> </p><p> </p><p>This article is currently being written. Like <em>right now!</em></p>
References changed:
- 1. Louis D, Perry A, Wesseling P et al. The 2021 WHO Classification of Tumors of the Central Nervous System: A Summary. Neuro-Oncology. 2021;23(8):1231-51. <a href="https://doi.org/10.1093/neuonc/noab106">doi:10.1093/neuonc/noab106</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/34185076">Pubmed</a>
- 2. Jastania R, Saeed M, Al-Khalidi H et al. Adamantinomatous Craniopharyngioma in an Adult: A Case Report with NGS Analysis. IMCRJ. 2020;Volume 13:123-37. <a href="https://doi.org/10.2147/imcrj.s243405">doi:10.2147/imcrj.s243405</a>
- 3. Hölsken A, Sill M, Merkle J et al. Adamantinomatous and Papillary Craniopharyngiomas Are Characterized by Distinct Epigenomic as Well as Mutational and Transcriptomic Profiles. Acta Neuropathol Commun. 2016;4(1):20. <a href="https://doi.org/10.1186/s40478-016-0287-6">doi:10.1186/s40478-016-0287-6</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/26927026">Pubmed</a>
- 4. Zacharia B, Bruce S, Goldstein H, Malone H, Neugut A, Bruce J. Incidence, Treatment and Survival of Patients with Craniopharyngioma in the Surveillance, Epidemiology and End Results Program. Neuro-Oncology. 2012;14(8):1070-8. <a href="https://doi.org/10.1093/neuonc/nos142">doi:10.1093/neuonc/nos142</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/22735773">Pubmed</a>
- 5. Sartoretti-Schefer S, Wichmann W, Aguzzi A, Valavanis A. MR Differentiation of Adamantinous and Squamous-Papillary Craniopharyngiomas. AJNR Am J Neuroradiol. 1997;18(1):77-87. <a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8337875">PMC8337875</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/9010523">Pubmed</a>
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