Ruengwanichayakun Poosit, Marrari Andrea, Mazzatenta Diego, Baldi Giacomo Giulio, Bertolini Patrizia, Piwchan Setthachai, Bercich Luisa, Facco Carla, Turri-Zanoni Mario, Silini Enrico Maria, Spinnato Paolo, Gentilini Francesca, Gianfranco Vornetti, Riccardo Draghi, Tonon Caterina, Gambarotti Marco, Lodi Raffaele, Foschini Maria Pia, Asioli Sofia, Righi Alberto
Department of Pathology, Faculty of Medicine, Naresuan University, Phitsanulok, Thailand.
Innovative Therapy Unit, Osteoncology, Soft Tissue and Bone Sarcomas, IRCCS Istituto Ortopedico Rizzoli, Bologna, Italy.
Head Neck Pathol. 2025 May 15;19(1):64. doi: 10.1007/s12105-025-01801-2.
Skull base osteosarcoma is an exceedingly rare malignancy. Unlike maxillofacial osteosarcomas, there are only few cases of skull base tumors reported in literature, and their clinical behavior and treatment outcome are not well defined. This study aims to characterize the clinical features and outcomes of primary skull base osteosarcoma based on our experiences of 9 cases and to review available literature data.
A retrospective analysis was done on 9 cases of skull base osteosarcoma diagnosed at IRCCS Istituto Ortopedico Rizzoli, Bologna, Italy, from 1995 to 2023. Clinicopathologic features were reported, and survival outcomes were analyzed. Literature review was performed by searching the PubMed database for published cases of skull base osteosarcoma. Kaplan-Meier survival analysis was used to calculate 1-year, 3-year, and 5-year survival rates.
Patients were 5 males and 4 females, with an age range of 5-72 years (mean: 39 years). Tumor location was frontal (3), sphenoid/ethmoid (3), occipital (2), and temporal (1) bone. Seven patients had subtotal resections; Two had total resection, but only one achieved negative margin. Follow-up duration ranged from 4 to 256 months (median: 28 months). Local recurrence occurred in 4 patients. At last follow-up, 5 patients were alive, with 3 had no evidence of disease (NED) and 2 alive with disease (AWD), while 4 patients died of disease (DOD). One-year, 3-year, and 5-year survival were 88.9%, 76.2%, and 57.1%, respectively. Literature review showed 1-year, 3-year, and 5-year survival of 50.3%, 22.6%, and 6.7%, respectively.
Skull base osteosarcoma is a rare malignancy with limited data on its clinical behavior. Surgical resection with negative margins is critical for better outcomes but is challenging due to the complex anatomy. Multidisciplinary treatment approaches are essential to improve our understanding of the tumor and survival outcomes.
颅底骨肉瘤是一种极其罕见的恶性肿瘤。与颌面骨肉瘤不同，文献中报道的颅底肿瘤病例很少，其临床行为和治疗结果尚不明确。本研究旨在根据我们9例病例的经验，描述原发性颅底骨肉瘤的临床特征和结果，并回顾现有文献数据。
对1995年至2023年在意大利博洛尼亚IRCCS里佐利骨科研究所诊断的9例颅底骨肉瘤进行回顾性分析。报告临床病理特征，并分析生存结果。通过在PubMed数据库中搜索已发表的颅底骨肉瘤病例进行文献综述。采用Kaplan-Meier生存分析计算1年、3年和5年生存率。
患者男性5例，女性4例，年龄范围为5至72岁（平均：39岁）。肿瘤位于额骨（3例）、蝶骨/筛骨（3例）、枕骨（2例）和颞骨（1例）。7例患者行次全切除；2例行全切除，但只有1例切缘阴性。随访时间为4至256个月（中位数：28个月）。4例患者发生局部复发。在最后一次随访时，5例患者存活，其中3例无疾病证据（NED），2例带瘤存活（AWD），4例患者死于疾病（DOD）。1年、3年和5年生存率分别为88.9%、76.2%和57.1%。文献综述显示，1年、3年和5年生存率分别为50.3%、22.6%和6.7%。
颅底骨肉瘤是一种罕见的恶性肿瘤，其临床行为的数据有限。切缘阴性的手术切除对于获得更好的结果至关重要，但由于解剖结构复杂，具有挑战性。多学科治疗方法对于提高我们对肿瘤的认识和生存结果至关重要。
