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

NMC case report journal Pub Date : 2024-07-27 eCollection Date: 2024-01-01 DOI:10.2176/jns-nmc.2024-0057
Hideyuki Harada, Kazuhito Takeuchi, Yuichi Nagata, Naoko Inoshita, Eiji Ito, Eriko Okumura, Tatsuma Kondo, Yoshiki Sato, Ryuta Saito
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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.

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一例乳头状颅咽管瘤模仿拉斯科氏裂囊肿的病例
颅咽管瘤(CP)和雷斯克氏裂囊肿(RCC)都是鞍上病变。由于两者的检查结果相似,有时难以区分。我们报告了一例乳头状颅咽管瘤(pCP),其临床表现提示为 RCC。一名42岁的女性智障者因严重的视功能障碍来我院就诊。术前影像学检查显示其颅骨上有一个无钙化的囊性病变。我们为她实施了经蝶窦手术。由于囊肿内有凝乳样内容物,提示为 RCC,我们进行了囊肿切开冲洗,但未切除囊壁。此后,我们在囊壁上发现了鱼卵状结构。组织病理学分析显示,这些结构为乳头状结构,周围为增生的鳞状上皮,伴有角化不全。BRAF V600E 免疫染色呈阳性,因此被诊断为 pCP。手术后,她的视功能有所改善,术后 18 个月的磁共振成像随访显示无明显复发。凝乳样内容物的存在表明有可能是 RCC,因此可能没有必要进行积极的切除。相比之下,鱼卵样结构的存在则提示存在良性前列腺癌,需要进行仔细的随访。
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