Introduction:The group of so-called “sellar-region masses” consists of a heterogeneous group of neoplasms and tumor-mimicking lesions, whose differential diagnosis may be challenging due to the overlapping of clinical and radiological features, which can be found both in “common” and “uncommon” lesions. The choice of a correct treatment strategy is still arduous and requires histological analysis. Gamma Knife Radiosurgery (GKRS) has already been reported as a safe and effective treatment in these cases. The objective of this study is to evaluate single-center pre-operative data, post-operative outcomes, and long-term follow-up in patients treated with GKRS for unusual sellar tumors.Methods:We retrospectively identified and analyzed nine patients treated with GKRS from 2004 to 2015, according to a standard protocol. Lesions consist of hypothalamic hamartoma (HH), Rathke’s cleft cist (RCC), Langerhans cell histiocytosis (LCH), spindle cell oncocytoma (SCO), choroid plexus papilloma (CPP), and ossifying fibroma (OF). The diagnosis was histologically confirmed in six patients that underwent surgery, while in three patients, diagnosis was based on characteristic clinical and radiological findings (two HH and one RCC). Pre-operative and post-operative data were retrieved from medical archives, and long-term follow-up was obtained through clinical and neuroradiological periodic examination.Results:In our series, all the “rare” sellar lesions treated, had a successful radiographic and clinical response in a medium-long follow-up period.Conclusions:The long-term follow-up results suggest that GKRS is a safe and effective treatment in rare sellar lesions, with very low toxicity. To the best of our knowledge, this report represents the largest series of unusual sellar lesions treated with GKRS in a single high-volume center, suggesting that GKRS might be an effective non-invasive adjuvant treatment option. Further studies and a larger number of patients are needed to confirm if residuals of these rare sellar lesions might regress on their own without treatment or if other non-invasive treatments could be as effective as GKRS.
引言：所谓“鞍区占位性病变”是一组异质性肿瘤及类肿瘤病变，其鉴别诊断因临床与影像学特征的重叠而颇具挑战性，这一特点在“常见”与“罕见”病变中均存在。制定正确的治疗策略仍较为困难，通常需要组织学分析作为依据。已有研究报道伽玛刀放射外科（GKRS）在此类病例中是一种安全有效的治疗手段。本研究旨在评估接受GKRS治疗的罕见鞍区肿瘤患者的单中心术前数据、术后结果及长期随访情况。 方法：我们根据标准方案回顾性识别并分析了2004年至2015年间接受GKRS治疗的9例患者。病变类型包括下丘脑错构瘤（HH）、拉克氏囊肿（RCC）、朗格汉斯细胞组织细胞增生症（LCH）、梭形细胞嗜酸细胞瘤（SCO）、脉络丛乳头状瘤（CPP）及骨化性纤维瘤（OF）。其中6例经手术患者获得组织学确诊，另3例（2例HH及1例RCC）基于特征性临床与影像学表现确诊。术前及术后数据均从医疗档案中提取，长期随访通过定期临床与神经影像学检查完成。 结果：在本系列病例中，所有接受治疗的“罕见”鞍区病变在中长期随访期间均获得良好的影像学与临床反应。 结论：长期随访结果表明，GKRS对罕见鞍区病变是一种安全有效的治疗方法，且毒性反应极低。据我们所知，本报告呈现了单一高手术量中心采用GKRS治疗罕见鞍区病变的最大病例系列，提示GKRS可能是一种有效的非侵入性辅助治疗选择。未来需要更多研究及更大样本量来验证这些罕见鞍区病变的残留病灶是否可能自行消退，或其他非侵入性治疗是否具有与GKRS相当的疗效。
The Role of Gamma Knife Surgery in the Treatment of Rare Sellar Neoplasms: A Report of Nine Cases
肿瘤(癌症)患者之家