Osteosarcoma of the skull base: An analysis of 19 cases and literature review

Guo, Zhifei; Hu, Ke; Zhao, Bing; Bian, Erbao; Song, Ning; Wan, Jinghai

doi:10.1016/j.jocn.2017.06.014

Cited by 21 publications

(13 citation statements)

References 62 publications

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“…Dural involvement is not uncommon and in cases with dural involvement, the dura needs to be resected [ 23 , 29 , 50 ]. Although wide surgical margins seem to improve survival [ 17 , 36 ], this is commonly difficult to achieve because of adjacent critical structures [ 22 , 50 ]. This study also showed a 31.5% GTR rate.…”

Section: Discussionmentioning

confidence: 99%

“…However, these often include early cases before the chemotherapy era. Metastasis of skull OSC is uncommon and less frequent compared to extracranial sites [ 22 , 42 ]. Metastasis may occur to the lungs or brain; similar locations compared to extracranial OSC [ 8 , 10 , 42 , 46 ].…”

Section: Discussionmentioning

confidence: 99%

“…In case of metastasis, survival is very poor [4, 26]. Recurrence is however more common, probably due to difficulty in achieving radical margins, especially in the skull base [10, 22, 23]. Recurrence is a poor prognosticator, commonly followed by death [10, 22].…”

Section: Discussionmentioning

confidence: 99%

“…To date, literature on OSC and EWS primarily arising from the skull has been limited to case reports or small case series [ 14 , 22 , 23 , 39 , 40 ]. This is primarily due to the low frequency of this tumor site.…”

Section: Introductionmentioning

confidence: 99%

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Treatment and survival of osteosarcoma and Ewing sarcoma of the skull: a SEER database analysis

et al. 2018

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Background Common primary bone tumors include osteosarcomas (OSC) and Ewing sarcomas (EWS). The skull is a rare site, and literature about their treatment and survival is scarce. Using the Surveillance, Epidemiology, and End Results (SEER) database, this study aims to assess the treatment and survival of skull OSC and skull EWS, as well as predictors for survival. Methods Skull OSC and EWS cases were obtained from the SEER database. Patient and tumor characteristics, treatment modalities, and survival were extracted. Overall survival (OS) was assessed using multivariable Cox proportional hazard regression stratified by tumor histology. Kaplan-Meier curves were constructed for OS comparing OSC and EWS, as well as histological subtypes in OSC. Results A total of 321 skull OSC and 80 skull EWS patients were registered from 1973 to 2013. EWS was more common in younger patients ( p < 0.001). Resection was the predominant treatment strategy (80.1%), frequently in combination with adjuvant radiotherapy (30.4%). The 5-year survival rate varied significantly between OSC and EWS (51.0% versus 68.5%, p = 0.02). Kaplan-Meier curves show that EWS had a significantly better survival compared to OSC. Comparing histological subtypes of skull OSC, chondroblastic OSC had the best OS, Paget OSC the worst. Older age, tumor advancement, no surgical treatment, and the use of radiotherapy were identified as independent predictors of decreased OS in skull OSC. Conclusion Overall prognosis is better for EWS compared to OSC. Chondroblastic OSC have the best overall survival, while OSC associated with Paget’s disease of the bone has the poorest overall survival.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Treatment and survival of osteosarcoma and Ewing sarcoma of the skull: a SEER database analysis

et al. 2018

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“…Osteosarcomas are highly aggressive lesions caused by osteoid-producing malignant cells. They primarily affect the metaphysis of long bones but 6–10% of them may take place within the skull [ 61 ]. Being a tumour of mesenchymal origin, osteosarcoma shows positivity to vimentin.…”

Section: Resultsmentioning

confidence: 99%

The identification of H3F3A mutation in giant cell tumour of the clivus and the histological diagnostic algorithm of other clival lesions permit the differential diagnosis in this location

et al. 2018

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BackgroundGiant Cell Tumour of Bone (GCT) is a locally aggressive primary bone tumour that usually occurs at the epiphyses of the long bones of the appendicular skeleton with a tendency to recurrence. Recurrent somatic H3F3A mutations have been described in 92% of GCT cases. GCTs involving the Clivus are extremely rare lesions and less than 15 cases are described in the literature. They represent a surgery challenge and are easily misdiagnosed. Our aim was to reveal if the genetic bases underlying Clival GCTs were the same of GCTs of long bones to improve the diagnosis and treatment.MethodsThe targeted somatic sequencing of GCT-related genes (H3F3A, H3F3B, IDH1, IDH2 and ZNF687) was performed on Clival GCT biopsies of two different cases. Histological analyses on the same tissues were used to detect the neoplastic population and its expression profile.ResultsSanger sequencing revealed that both patients were positive for the p.Gly34Trp mutation in the H3F3A gene. Immunofluorescence assay using monoclonal antibody, specifically detecting the mutant H3.3, highlighted that the mutation only involved the mononuclear cell population and not the multinucleated giant cells. Moreover, immunohistochemistry assay showed that RANKL was highly expressed by the stromal cells within Clival GCT, mimicking what happens in GCT of the long bones. In addition, systematic literature review allowed us to generate a histology-based diagnostic algorithm of the most common clival lesions.ConclusionsWe conclude that the Clival GCT is genetically defined by somatic mutation in the H3F3A gene, linking it to the GCT of long bones. The similarity with GCTs of long bones let us to hypothesize the utility of Denosumab therapy (already effective for GCTs) in these surgically challenging cases. Moreover, H3F3A genetic screening can be combined to the histological analysis to differentiate GCTs from morphologically similar giant cell-rich sarcomas, while the histological diagnostic algorithm could help the differential diagnosis of other clival lesions.

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