Lottin Marine, Escande Alexandre, Bauchet Luc, Albert-Thananayagam Marie, Barthoulot Maël, Peyre Matthieu, Boone Mathieu, Zouaoui Sonia, Guyotat Jacques, Penchet Guillaume, Pallud Johan, Dufour Henry, Emery Evelyne, Lefranc Michel, Freppel Sébastien, Namaki Houman, Gueye Edouard, Lemaire Jean-Jacques, Muckensturm Bertrand, Srour Robin, Derrey Stéphane, Monfilliette Apolline, Constans Jean-Marc, Maurage Claude-Alain, Chauffert Bruno, Penel Nicolas
Department of Oncology, Amiens University Hospital, 80054 Amiens, France.
Department of Radiotherapy, Lille Oscar Lambret Centre, 59000 Lille, France.
Cancers (Basel). 2023 Jan 24;15(3):704. doi: 10.3390/cancers15030704.
Intracranial solitary fibrous tumour (iSFT) is an exceptional mesenchymal tumour with high recurrence rates. We aimed to analyse the clinical outcomes of newly diagnosed and recurrent iSFTs.
We carried out a French retrospective multicentre ( = 16) study of histologically proven iSFT cases. Univariate and multivariate Cox models were used to estimate the prognosis value of the age, location, size, WHO grade, and surgical extent on overall survival (OS), progression-free survival (PFS), and local recurrence-free survival (LRFS).
Eighty-eight patients were included with a median age of 54.5 years. New iSFT cases were treated with gross tumour resection (GTR) ( = 75) or subtotal resection (STR) ( = 9) and postoperative radiotherapy (PORT) ( = 32, 57%). The median follow-up time was 7 years. The median OS, PFS, and LRFS were 13 years, 7 years, and 7 years, respectively. Forty-two patients experienced recurrence. Extracranial metastasis occurred in 16 patients. Median OS and PFS after the first recurrence were 6 years and 15.4 months, respectively. A higher histological grade was a prognosis factor for PFS ( = 0.04) and LRFS ( = 0.03). GTR influenced LRFS ( = 0.03).
GTR provided benefits as a first treatment for iSFTs. However, approximately 40% of patients experienced relapse, which remains a challenging state.
颅内孤立性纤维瘤（iSFT）是一种罕见的间充质肿瘤，复发率高。我们旨在分析新诊断和复发性iSFT的临床结局。
我们对16个中心组织学确诊的iSFT病例进行了一项法国回顾性多中心研究。使用单变量和多变量Cox模型评估年龄、位置、大小、世界卫生组织分级和手术范围对总生存期（OS）、无进展生存期（PFS）和无局部复发生存期（LRFS）的预后价值。
纳入88例患者，中位年龄54.5岁。新诊断的iSFT病例接受了肿瘤全切术（GTR）（n = 75）或次全切除术（STR）（n = 9）以及术后放疗（PORT）（n = 32，57%）。中位随访时间为7年。中位OS、PFS和LRFS分别为13年、7年和7年。42例患者出现复发。16例患者发生颅外转移。首次复发后的中位OS和PFS分别为6年和15.4个月。较高的组织学分级是PFS（P = 0.04）和LRFS（P = 0.03）的预后因素。GTR影响LRFS（P = 0.03）。
GTR作为iSFT的首选治疗方法具有益处。然而，约40%的患者出现复发，这仍然是一个具有挑战性的情况。
