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Cheng et al. Military Medical Research (2021) 8:39 https://doi.org/10.1186/s40779-021-00332-5 RESEARCH Open Access Clinical features, radiological profiles, pathological features and surgical outcomes of pituicytomas: a report of 11 cases and a pooled analysis of individual patient data Jian-Hua Cheng1†, Ding Nie1†, Bin Li2, Song-Bai Gui1, Chu-Zhong Li2, Ya-Zhuo Zhang2, Luigi Maria Cavallo3 and Peng Zhao1* Abstract Background: Pituicytoma is an extremely rare low-grade glial tumor that is closely related to the neurohypophysis axis. Most studies of pituicytomas include only several cases. To better understand this disease, we reviewed a series of cases of pituicytomas. The diagnosis and treatment of pituicytoma must be further elucidated. Methods: Eleven patients with pituicytoma admitted to Beijing Tiantan Hospital from 2012 to 2019 were selected. The clinical features, including radiological and histological examination, surgical records and prognosis were reviewed. Sixty-eight other previously published cases of pituicytoma also were used to analyze the predictive factors for the results. The Cox regression model was used for univariate and multivariate analyses. Results: Our patients included 5 males (45.5%) and 6 females (54.5%), with a mean age of 49.3 years. The tumor was located in the suprasellar region in 5 patients (45.5%), intrasellar region in 4 patients (36.4%), and intrasellar- suprasellar region in 2 patients (18.2%). All patients were misdiagnosed with other common tumors in the sellar region before the operation. During the operation, gross total resection (GTR) of the tumor was achieved in 6 patients (54.5%), and subtotal resection (STR) was achieved in 5 patients (45.5%). The mean progression-free survival (PFS) time was 29.82 months. Tumor progression after surgical resection occurred in 4 patients (36.4%). Among them, 60.0% of the patients (cases 4, 5, 7) with STR experienced progression, while 16.7% of the patients (case 2) with GTR experienced progression. Combined with the 68 cases in the literature, GTR was an independent risk factor for PFS time (P < 0.05). * Correspondence: [email protected] †Jian-Hua Cheng and Ding Nie contributed equally to this work. 1Neurosurgical Department, Beijing Tiantan Hospital, Capital Medical University, No. 119 South Fourth Ring West Road, Fengtai District, Beijing 100070, China Full list of author information is available at the end of the article © The Author(s). 2021 Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data. Cheng et al. Military Medical Research (2021) 8:39 Page 2 of 9 Conclusions: Pituicytomas are more common in middle-aged people and the sellar region. The clinical manifestations of pituicytomas are different, but no diagnostic clinical features have been identified other than an abnormally abundant blood supply. Currently, GTR is the best approach for the treatment of pituicytomas. More patients and longer follow-up periods were needed to further elucidate the biological features of pituicytomas. Keywords: Pituicytoma, Endoscopic transsphenoidal surgery, Craniotomy, Posterior pituitary lobe tumor Background definite or suspected cerebrospinal fluid leakage during Pituicytoma is a benign fusiform astrocytoma that origi- ETS. nates from the neurohypophysis or pituitary stalk [1–3]. It is a localized, generally solid, low-grade glial tumor Pathological examination that consists of bipolar fusiform cells arranged in bun- The tumor specimens of all patients were sent for a dles or striations [4]. Pituicytomas are exceedingly rare. pathological evaluation, including hematoxylin and eosin Currently, approximately 119 cases of pituicytoma have (HE) staining and immunohistochemical staining. been reported [5, 6]. Postoperative immunohistochemical staining was per- Pituicytoma is easily confused with other tumors in formed with antibodies against glial fibrillary acidic pro- the sellar region. However, it has been differentiated tein (GFAP, Abcam, UK), S100 (Abcam, UK), vimentin from other tumors in the sellar region based on micro- (Abcam, UK), thyroid transcription factor-1 (TTF-1, scopic cell morphology and immunohistochemistry [7]. Abcam, UK), endomysial antibody (EMA, Abcam, UK), The treatment of pituicytoma generally involves surgical CD34 (Abcam, UK), MIB-1 (Abcam, UK), progesterone resection [8]. Here, this study reports 11 patients with receptor (PR, Abcam, UK), smooth muscle actin (SMA, pathologically proven pituicytoma and their long-term Abcam, UK). Pituicytomas were diffusely positive for the follow-up results. To the best of our knowledge, this S100 and vimentin proteins, weakly to moderately posi- study involves the largest number of patients with pitui- tive for GFAP, and displayed nuclear immunoreactivity cytoma from a single center. We believe that the clinical, for TTF-1 and a punctate distribution of EMA-positive radiological, and pathological features of pituicytomas staining in the cytoplasm [8]. studied here will facilitate the better diagnosis and treat- ment of pituicytomas in the future. Literature research The literature on pituicytoma published from January Methods 1958 to January 2020 was searched on PubMed using Clinical data the keyword “pituicytoma”. The following selection cri- This study retrospectively analyzed the clinical features teria were used: (1) patients with pituicytoma confirmed and surgical results of patients with histologically diag- by an operation and pathology; (2) patients who had nosed pituicytoma who were treated at Beijing Tiantan been followed for more than half a year; and (3) cases re- Hospital from 2012 to 2019. This study was approved by ported in English literature. The exclusion criteria were the Ethics Committee of Beijing Tiantan Hospital as follows: (1) patients with pituicytoma and other intra- (KYSQ2019–228-01). All patients underwent cranial cranial tumors; (2) patients with other space-occupying le- contrast magnetic resonance imaging (MRI) and testing sions in the sellar region. The information from each of their hormone levels before the operation. The clin- patient was carefully reviewed by two authors (JHC and ical data were collected by reviewing clinical case infor- DN) and any repeated patients were excluded. The infor- mation and telephone follow-up and included clinical mation collected included the general condition of the symptoms, radiographic examinations, surgical results, patients (sex, age at the time of diagnosis, and location of immunohistochemical results, and follow-up results. the tumor), the treatment (surgical approach, extent of tumor resection, radiotherapy and chemotherapy), and Surgical treatment outcome (tumor recurrence or progression). All the steps The preoperative examination was completed, and con- of the process followed the PRISMA flow diagram as pre- traindications were excluded. All patients underwent sented in Fig. 1. Sixty-eight cases of pituicytoma reported endoscopic transsphenoidal surgery (ETS) or craniotomy in the literature are listed in Supplementary Table 1. under general anesthesia. The extent of tumor resection was divided into gross total resection (GTR) and sub- Follow-up total resection (STR) based on an analysis of the postop- The clinical data analyzed in this study, including clinical erative cranial contrast MRI and surgical records. symptoms, radiographic examination, surgical results, im- Mucosal flap repair was performed in all patients with munohistochemical results, and follow-up results, were Cheng et al. Military Medical Research (2021) 8:39 Page 3 of 9 Fig. 1 PRISMA flow-chart collected by reviewing clinical case information and tele- first operation, while the pathological diagnosis obtained phone follow-up. Among these parameters, the clinical in the second operation was pituicytoma. history of the patients was collected by a retrospective chart review. Potential risk factors assessed here included Patient’s imaging characteristics age, sex, tumor size and location, surgical resection extent, Suprasellar lesions were diagnosed in 5 patients (45.5%), and other adverse predictors, such as progression-free sur- intrasellar lesions in 4 patients (36.4%), and intrasellar- vival (PFS). suprasellar lesions in 2 patients (18.2%) (Fig. 2a-c, Table 1). MRI showed a clear boundary of the tumor in 10 pa- tients (90.9%) and an unclear boundary in 1 patient Statistical analysis (9.1%). Contrast-enhanced MRI showed homogeneous The Cox regression model was used for univariate and enhancement in 6 patients (54.5%), inhomogeneous en- multivariate