颅内孤立性纤维性肿瘤/血管周细胞瘤伴肾脏等多发转移临床病理分析

Intracranial solitary fibrous tumours with metastasis of kidney and other organs: a clinicopathologic analysis

目的探讨颅内孤立性纤维性肿瘤/血管周细胞瘤(SFT/HPC)伴肾脏等多器官转移4例的临床病理学特征、组织学分级、诊断与鉴别诊断及预后情况。方法收集解放军总医院2005—2017年诊断为颅内SFT/HPC伴肾脏等多发转移患者4例,观察其临床、影像及病理学特征及预后,结合文献复习对其临床表现、病理形态学特点及鉴别诊断进行分析。结果 4例患者中女性2例,男性2例,发生肾脏等多发转移的中位年龄45.5岁(29～53岁)。临床主要症状为体检发现肾脏占位入院;影像学检查显示为肾、肺、肝等血供丰富的肿块。组织学表现为富含分支血管的短梭形细胞肿瘤,细胞形态大小较一致,细胞核卵圆形,具有非典型性,核分裂象多少不等。免疫组化染色显示肿瘤细胞典型的STAT6胞核强(+)以及CD34、PR、vimentin、bcl-2不同程度(+),网织纤维染色可见丰富的网状纤维围绕单个肿瘤细胞或肿瘤细胞团。结论 1例患者脑膜SFT/HPC术后6年复发,1例患者脑膜SFT/HPC术后11年(在转移之后)复发,2例患者无复发。4例患者分别于脑膜SFT/HPC术后13、10、12、5年发生包括肾脏等多发脏器转移。颅内SFT/HPC具有高复发和颅外转移的风险。外科手术完整切除辅以术后放疗为该肿瘤的首选治疗方案。术后需长期密切随访,以尽早发现,尽早治疗。

To study the clinicopathological features,histological classification,differential diagnosis and prognosis of four intraeranial solitary fibrous tumours/haemangiopericytomas (SFT/HPC)with kidney and other organs metastasis. Methods Four cases of SFT/HPCs with kidney and other organs metastasis were analyzed on clinical manifestations,MR images,histomorphological characteristics and prognosis.Results In the 4 cases,2 males and 2 female were included, aged 29 to 53 years with median age of 43.25 years.Renal masses were the main clinical presentation.The MR images showed masses with rich blood supply in kidneys,lungs,liver,etc.The histopathologic feature of SFF/HPCs was a short spindle cell tumor,showing rich branching vascular pattern,the more consistent cell morphological size,and different pathologic mitosis.Immunohistochemical staining showed the tumor ceils were strongly positive for STAT6 D34,PR, vimentin and bcl-2,CD99 and prominent reticulin staining surrounding individual or small clusters of cells was also observed.One patient had recurrence 6 years after surgery,and one patient had recurrence 11 years after metastasis,and two patients had no recurrence.All four patients underwent multiple organ metastasis,including kidney and other organs, after 13,10,12 and 5 years,respectively.Conclusions As high risk of recurrence and extracranial metastasis seems unavoidable,radical surgical resection followed by radiation therapy can be considered as the optimal management policy for this tumour,and long-term follow-up with serial imaging should be considered in all cases.