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A Case of Papillary Craniopharyngioma Mimicking Rathke's Cleft Cyst.

ABSTRACT: Craniopharyngioma (CP) and Rathke's cleft cyst (RCC) are both suprasellar lesions. They are sometimes difficult to distinguish due to their similar findings. We report a case of papillary craniopharyngioma (pCP) with the clinical findings suggesting RCC. A 42-year-old female with intellectual disability presented to our hospital with severe visual dysfunction. Preoperative images revealed a suprasellar cystic lesion without calcification. We performed transsphenoidal surgery. Since the cyst had condensed-milk-like content suggesting RCC, we performed cyst fenestration and wash without removal of the cyst wall. Thereafter, we found fish-egg-like structures on the cyst wall. The histopathological analysis revealed that they had papillary structures surrounded by hyperplastic squamous epithelium with parakeratosis. Immunostaining for BRAF V600E was positive, leading to the diagnosis of pCP. After the surgery, her visual function improved and follow-up Magnetic resonance imaging at 18 months postoperatively showed no apparent recurrence. The presence of condensed-milk-like content suggests a likelihood of RCC indicating that aggressive resection may not be necessary. In contrast, the existence of fish-egg-like structures suggests pCP and requires careful follow-up.

SUBMITTER: Harada H

PROVIDER: S-EPMC11345118 | biostudies-literature | 2024

REPOSITORIES: biostudies-literature

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A Case of Papillary Craniopharyngioma Mimicking Rathke's Cleft Cyst.

Harada Hideyuki H Takeuchi Kazuhito K Nagata Yuichi Y Inoshita Naoko N Ito Eiji E Okumura Eriko E Kondo Tatsuma T Sato Yoshiki Y Saito Ryuta R

NMC case report journal 20240727

Craniopharyngioma (CP) and Rathke's cleft cyst (RCC) are both suprasellar lesions. They are sometimes difficult to distinguish due to their similar findings. We report a case of papillary craniopharyngioma (pCP) with the clinical findings suggesting RCC. A 42-year-old female with intellectual disability presented to our hospital with severe visual dysfunction. Preoperative images revealed a suprasellar cystic lesion without calcification. We performed transsphenoidal surgery. Since the cyst had ...[more]

PMID: 39183799

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Project description:Cilia are highly conserved organelles, which serve critical roles in development and physiology. Motile cilia are expressed in a limited range of tissues, where they principally regulate local extracellular fluid dynamics. In contrast, primary cilia are expressed by many vertebrate cell types during interphase, and are intimately involved in the cell cycle and signal transduction. Notably, primary cilia are essential for vertebrate hedgehog pathway activity. Improved detection of motile cilia may assist in the diagnosis of some pathologic entities such as Rathke's cleft cysts, whereas characterizing primary cilia in neoplastic tissues may implicate cilia-dependent signaling pathways as critical for tumorigenesis. We show that immunohistochemistry for the nuclear transcription factor FOXJ1, a master regulator of motile ciliogenesis, robustly labels the motile ciliated epithelium of Rathke's cleft cysts. FOXJ1 expression discriminates Rathke's cleft cysts from entities in the sellar/suprasellar region with overlapping histologic features such as craniopharyngiomas. Co-immunohistochemistry for FOXJ1 and markers that highlight motile cilia such as acetylated tubulin (TUBA4A) and the small GTPase ARL13B further enhance the ability to identify diagnostic epithelial cells. In addition to highlighting motile cilia, ARL13B immunohistochemistry also robustly highlights primary cilia in formalin-fixed paraffin-embedded sections. Primary cilia are present throughout the neoplastic epithelium of adamantinomatous craniopharyngioma, but are limited to basally oriented cells near the fibrovascular stroma in papillary craniopharyngioma. Consistent with this differing pattern of primary ciliation, adamantinomatous craniopharyngiomas express significantly higher levels of SHH, and downstream targets such as PTCH1 and GLI2, compared with papillary craniopharyngiomas. In conclusion, motile ciliated epithelium can be readily identified using immunohistochemistry for FOXJ1, TUBA4A, and ARL13B, facilitating the diagnosis of Rathke's cleft cysts. Primary cilia can be identified by ARL13B immunohistochemistry in routine pathology specimens. The widespread presence of primary cilia in adamantinomatous craniopharyngioma implicates cilia-dependent hedgehog signaling in the pathogenesis of adamantinomatous craniopharyngioma.

Project description:BackgroudRecurrence of Rathke's cleft cyst (RCC) is not uncommon after surgery, and the associated factors and incidence of relapse deserve a systematic summary.MethodsThis study was conducted in accordance with Preferred Reporting Items for Systematic Review and Meta-Analysis (PRISMA) guidelines. The Pubmed, Embase, Cochrane, and Web of Science databases were searched until September 12, 2022. Studies with significant results of recurrent factors or specific incidences of RCC recurrence and mean/median follow-up time were included. Based on a protocol of a 2-year interval grouping, included studies were categorized into four groups with follow-up periods <24 months, 24-48 months, 48-72 months, and ≥72 months, respectively. Quality assessment was performed using the NOS score. Pooled estimations were computed by using a random-effects model in the STATA "metaprop" command. Publication bias was assessed visually through a funnel plot and statistically through Egger's linear regression test and Begg's correlation test.ResultsA total of 44 studies were included containing 2,539 cases. Squamous metaplasia was the most commonly reported factor, followed by the extent of cyst removal. The other factors were reported individually. The pooled overall incidences of RCC recurrence after surgery in four groups of the follow-up period were 7.4% (95%CI = 4.1-11.3%) in <24 months, 13.1% (95%CI = 9.7-17.0%) in 24-48 months, 13.7% (95%CI = 7.7-21.0%) in 48-72 months, and 33.8% (95%CI = 19.6-49.6%) in ≥72 months. The pooled symptomatic incidences were 2.3% (95%CI = 0.4-5.1%) in <24 months, 5.6% (95%CI = 3.6-7.9%) in 24-48 months, 5.9% (95%CI = 2.4-10.6%) in 48-72 months, and 14.1% (95%CI = 6.0-24.5%) in ≥72 months. A dramatic increase in recurrent incidence was observed when the follow-up period was more than 72 months in both overall and symptomatic recurrence. A similar trend of recurrence was found in subgroup analyses stratified by publication year, cohort size, and cyst resection strategy.ConclusionThis study systematically reviewed recurrent factors and described the profile of trends in RCC recurrent incidence after surgery with a follow-up time based on a protocol of a 2-year interval, finding a dramatic increase in recurrent rates with a follow-up period of more than 72 months. This encouraged us to put forward a recommendation of at least a 6-year follow-up after surgery for patients with RCC.Systematic review registrationhttps://www.crd.york.ac.uk/prospero/, identifier: CRD42021278970.

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Dataset Information

A Case of Papillary Craniopharyngioma Mimicking Rathke's Cleft Cyst.

Publications

A Case of Papillary Craniopharyngioma Mimicking Rathke's Cleft Cyst.

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