Halaoui Adham, Estrella Melanie, Yan Carol H, Goodwill Vanessa S, Beaumont Thomas L
Department of Neurological Surgery, University of California, San Diego, La Jolla, California.
Department of Neuropathology, University of California, San Diego, La Jolla, California.
J Neurosurg Case Lessons. 2025 Jan 20;9(3). doi: 10.3171/CASE24657.
Rathke cleft cysts (RCCs) are benign sellar/suprasellar lesions that result from mucin-secreting vestigial remnants within the pars intermedia of the pituitary gland. When symptomatic, they can present with retro-orbital headaches, visual field defects, and/or pituitary dysfunction.
A 35-year-old female presented with subacute retro-orbital headache, right ptosis, and blurred vision. Workup revealed panhypopituitarism with central hypothyroidism and adrenal insufficiency. Imaging demonstrated a sellar/suprasellar mass with subacute intralesional hemorrhage, which was thought to represent chronic pituitary apoplexy. The patient underwent an endoscopic endonasal approach in which the initial intraoperative frozen section suggested papillary craniopharyngioma. Subsequent specimens suggested RCC, thus presenting a surgical management conundrum. Hemihypophysectomy with lesionectomy was performed. Final histopathology demonstrated RCC with squamous metaplasia (RCC-SM), rupture, and hemorrhage. BRAF V600E was not detected. However, activating mutations in KRAS and MAP2K1 were identified.
RCC can undergo SM and rupture, leading to a hemorrhagic-appearing cystic sellar/suprasellar mass associated with cranial nerve palsies and hypopituitarism that mimics pituitary apoplexy. Intraoperative frozen sections can be ambiguous due to overlapping histopathological features with craniopharyngioma, complicating surgical decision-making. The authors hypothesize that RCC-SM may represent a transitional state between RCC and craniopharyngioma. Neurosurgeons should be mindful of this transitional entity and be prepared to modify their surgical strategy accordingly. https://thejns.org/doi/10.3171/CASE24657.
拉克氏囊肿（RCCs）是一种良性鞍区/鞍上病变，由垂体中间部分泌黏液的残留遗迹形成。出现症状时，可表现为眶后头痛、视野缺损和/或垂体功能障碍。
一名35岁女性出现亚急性眶后头痛、右眼上睑下垂和视力模糊。检查发现全垂体功能减退伴中枢性甲状腺功能减退和肾上腺功能不全。影像学检查显示鞍区/鞍上肿块伴亚急性病灶内出血，考虑为慢性垂体卒中。患者接受了内镜鼻内入路手术，术中最初的冰冻切片提示为乳头型颅咽管瘤。后续标本提示为RCC，因此带来了手术管理难题。遂行半垂体切除加病灶切除术。最终组织病理学检查显示为伴有鳞状化生（RCC-SM）、破裂和出血的RCC。未检测到BRAF V600E。然而，发现了KRAS和MAP2K1的激活突变。
RCC可发生鳞状化生和破裂，导致出现类似垂体卒中的、伴有颅神经麻痹和垂体功能减退的出血性鞍区/鞍上囊性肿块。由于与颅咽管瘤的组织病理学特征重叠，术中冰冻切片可能不明确，使手术决策复杂化。作者推测RCC-SM可能代表RCC和颅咽管瘤之间的一种过渡状态。神经外科医生应注意这种过渡性实体，并准备相应调整手术策略。https://thejns.org/doi/10.3171/CASE24657 。
