Primary temporal bone chondrosarcoma: experience with 10 cases

Abstract

Background: Temporal bone chondrosarcoma (TBC) are uncommon primary temporal bone malignancies, and clinicians lack experience in its diagnosis and treatment. The optimal management of patients with tumor of TBC also remains a topic of debate and controversy.

Objectives: This article summarizes the experience of diagnosis and treatment of TBC, in order to improve the quality of life of cancer patients in the future.

Material and methods: We conducted a retrospective analysis of 10 patients who were referred to our hospital from June 2009 to June 2018 for the treatment of TBC.

Results: There were 3 males and 7 females. The most common presenting symptoms were facial paresis (50%) and hearing loss (40%), whereas otalgia (10%), vertigo (10%) and headache (10%) were less common. All tumors originated from the temporal bone, and 80% involved the jugular foramen area. All patients survived without evidence of disease at a median time of follow up of 28.8 months.

Conclusions and significance: TBC mostly originated in the middle ear mastoid area, and easily extended to the jugular foramen area. An individualized surgical procedure that removes tumors integrally with minimal nerve and blood vessel damage provides long-term cancer control and minimal morbidity in most cases.

背景：颞骨软骨肉瘤（TBC）是罕见的原发性颞骨恶性肿瘤；临床医生缺乏对其诊断和治疗的经验。对TBC肿瘤患者的最佳治疗仍然是一个辩论和争议的话题。

目的：本文总结了TBC诊断和治疗的经验, 以期提高未来癌症患者的生活质量。

材料和方法：我们对2009年6月至2018年6月到我院诊治的10例TBC患者进行了回顾性分析。

结果：男性3例, 女性7例。最常见的症状是面部麻痹（50％）和听力损失（40％）, 而耳痛（10％）、眩晕（10％）和头痛（10％）不太常见。所有肿瘤均源发于颞骨, 80％涉及颈静脉孔区域。所有患者在随访28.8个月的中位时间存活期间, 没有疾病迹象。

结论和意义：TBC主要源发于中耳乳突区, 易于扩展到颈静脉孔区。在大多数情况下, 个人化的外科手术可以在最小的神经和血管损伤的情况下整体移除肿瘤, 从而保证大多数病例的长期癌症控制和最小发病率。

Keywords:

• Chondrosarcoma
• temporal bone
• diagnosis
• treatment

Acknowledgments

The authors thank the contributors of the Department of Medical History, Eye, Otolaryngology Hospital affiliated to Fudan University. We also thank the Departments of Imaging and Pathology for input regarding the imaging, radiotherapy, and pathology contents of the article, respectively. In addition, we also would like to acknowledge the helpful comments on this paper received from our reviewers.

Ethics approval and consent to participate

This study does not contain any studies with human participants or animals performed by any of the authors. Since all TBC dataset included in this manuscript were obtained from the department of Medical History, Eye, Otolaryngology Hospital affiliated to Fudan University, therefore, additional approval by an Ethics Committee is not necessary. In addition, the procedures of this manuscript were in accordance with the Helsinki declaration of 1964 and its later amendments.

Availability of data and materials

The datasets used during the present study are available from the corresponding author upon reasonable request.

Disclosure statement

The authors declare that they have no competing interests.

Authors' contributions

Kun Zhang and Peng Qu acquired the data and created a draft of the manuscript; ED Zhang and CF Dai conducted and further performed the study, processed and analyzed the data; CF Dai and Bin Chen revised and approved the final version of the manuscript. All authors read and approved the manuscript and agree to be accountable for all aspects of the research in ensuring that the accuracy or integrity of any part of the work are appropriately investigated and resolved.

Additional information

Funding

The present study was supported in part by the National Natural Science Foundation of China (no. 81822011, 81870726, 81771013).