Harada Hideyuki, Takeuchi Kazuhito, Nagata Yuichi, Inoshita Naoko, Ito Eiji, Okumura Eriko, Kondo Tatsuma, Sato Yoshiki, Saito Ryuta
Department of Neurosurgery, Nagoya University, Nagoya, Aichi, Japan.
Department of Pathology, Moriyama Memorial Hospital, Tokyo, Japan.
NMC Case Rep J. 2024 Jul 27;11:191-194. doi: 10.2176/jns-nmc.2024-0057. eCollection 2024.
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.
颅咽管瘤（CP）和拉克氏囊肿（RCC）均为鞍上病变。由于它们的表现相似，有时难以区分。我们报告一例乳头状颅咽管瘤（pCP），其临床表现提示为RCC。一名42岁智力残疾女性因严重视力障碍就诊于我院。术前影像学检查显示鞍上有一个无钙化的囊性病变。我们进行了经蝶窦手术。由于囊肿内容物呈凝乳状提示RCC，我们进行了囊肿开窗和冲洗，未切除囊肿壁。此后，我们在囊肿壁上发现了鱼卵样结构。组织病理学分析显示，它们具有被增生性鳞状上皮伴不全角化包围的乳头状结构。BRAF V600E免疫染色呈阳性，从而诊断为pCP。手术后，她的视力功能有所改善，术后18个月的随访磁共振成像显示无明显复发。凝乳状内容物的存在提示可能为RCC，表明可能无需进行积极切除。相比之下，鱼卵样结构的存在提示为pCP，需要密切随访。
