Das Liza, Vaiphei Kim, Rai Ashutosh, Ahuja Chirag Kamal, Singh Paramjeet, Mohapatra Ishani, Chhabra Rajesh, Bhansali Anil, Radotra Bishan Dass, Grossman Ashley B, Korbonits Márta, Dutta Pinaki
Department of Endocrinology, Postgraduate Institute of Medical Education and Research, (PGIMER), Chandigarh, India.
Department of Histopathology, PGIMER, Chandigarh, India.
Endocr Connect. 2021 Apr;10(4):387-400. doi: 10.1530/EC-20-0621.
Posterior pituitary tumours (PPTs) are rare neoplasms with the four recognised subtypes unified by thyroid transcription factor -1 (TTF-1) expression, according to the 2017 WHO classification. Though traditionally defined as low-grade neoplasms, a substantial proportion of them show recurrence/persistence following surgery.
We selected patients with PPTs in our cohort of 1760 patients operated for pituitary tumours over the past 10 years (2010-2019). The clinical, radiological, hormonal, histopathological profiles and long-term outcomes of the three cases identified (two pituicytomas and one spindle cell oncocytoma, SCO) were analysed. Following a literature review, data of all published cases with documented TTF-1 positive pituicytomas and SCOs were analysed to determine the predictors of recurrence/persistence in these tumours.
Patients presented with compressive features or hypogonadism. Two had sellar-suprasellar masses. One had a purely suprasellar mass with a pre-operative radiological suspicion of pituicytoma. Two were operated by transsphenoidal surgery and one transcranially guided by neuronavigation. Histopathology confirmed spindle cells in a storiform arrangement and low Ki67 index. Immunohistochemistry showed positive TTF-1, S-100 expression and variable positivity for EMA, vimentin and GFAP. Re-evaluation showed recurrence/persistence in two patients. A literature review of recurrent/persistent pituicytoma (n = 17) and SCO (n = 9) cases revealed clinical clues (headache for pituicytomas, male gender for SCO), baseline tumour size (≥20.5 mm with sensitivity exceeding 80%) and longer follow-up duration as determinants of recurrence/persistence.
PPTs are rare sellar masses with quintessential TTF-1 positivity. Recurrent/persistent disease following surgery is determined by greater tumour size at baseline and duration of follow-up. This warrants intensive and long-term surveillance in these patients.
根据2017年世界卫生组织分类，垂体后叶肿瘤（PPTs）是罕见肿瘤，有四种公认的亚型，由甲状腺转录因子-1（TTF-1）表达统一。尽管传统上被定义为低级别肿瘤，但其中很大一部分在手术后会出现复发/持续存在。
我们在过去10年（2010 - 2019年）接受垂体肿瘤手术的1760例患者队列中选择了PPTs患者。分析了确诊的3例患者（2例垂体细胞瘤和1例梭形细胞嗜酸细胞瘤，SCO）的临床、放射学、激素、组织病理学特征及长期预后。在文献回顾后，分析了所有已发表的TTF-1阳性垂体细胞瘤和SCO病例的数据，以确定这些肿瘤复发/持续存在的预测因素。
患者表现为压迫症状或性腺功能减退。2例有鞍内 - 鞍上肿块。1例有单纯鞍上肿块，术前放射学怀疑为垂体细胞瘤。2例经蝶窦手术，1例在神经导航引导下经颅手术。组织病理学证实为呈束状排列的梭形细胞，Ki67指数低。免疫组织化学显示TTF-1、S-100表达阳性，EMA、波形蛋白和GFAP呈不同程度阳性。重新评估显示2例患者复发/持续存在。对复发性/持续性垂体细胞瘤（n = 17）和SCO（n = 9）病例的文献回顾显示，临床线索（垂体细胞瘤为头痛，SCO为男性）、基线肿瘤大小（≥20.5 mm，敏感性超过80%）和更长的随访时间是复发/持续存在的决定因素。
PPTs是罕见的鞍区肿块，典型的TTF-1阳性。手术后复发/持续存在的疾病由基线时更大的肿瘤大小和随访时间决定。这需要对这些患者进行强化和长期监测。
