原发于鳞状颞骨的尤文肉瘤伴颅内外延伸：突发性感觉神经性耳聋的罕见病因。
Primary Ewing sarcoma of the squamous temporal bone with intracranial and extracranial extension: A rare cause of sudden sensorineural hearing loss.
机构信息
Department of Otorhinolaryngology and Biomedical Research Institute, Pusan National University Hospital, Busan, Republic of Korea.
Department of Pediatrics and Biomedical Research Institute, Pusan National University Yangsan Hospital, Yangsan, Republic of Korea.
出版信息
Head Neck. 2019 Mar;41(3):E38-E41. doi: 10.1002/hed.25449. Epub 2018 Dec 24.
BACKGROUND
Primary Ewing sarcoma of the cranial bone is rare, accounting for only 1% of all Ewing sarcomas. Primary Ewing sarcoma arising in the squamous temporal bone is particularly rare.
METHODS
A 16-year-old male was seen with signs of sudden sensorineural hearing loss (SSNHL). After 1 week of SSNHL and new-onset headache, imaging studies showed a mass that originated in the left squamous temporal bone with intracranial and extracranial extension. Histopathological study revealed that the mass was a Ewing sarcoma.
RESULTS
The patient manifested the diagnostic EWSR1 mutation and was treated with adjuvant multidrug chemotherapy and focal radiotherapy after surgery according to the Children's Oncology Group interval compression arm of AEWS0031 with a regimen of vincristine, doxorubicin, and cyclophosphamide alternating with ifosfamide/etoposide.
CONCLUSIONS
This case showed an extremely uncommon location, as well as unusual symptoms of primary Ewing sarcoma.
背景
颅骨原发性尤文肉瘤非常罕见，仅占所有尤文肉瘤的 1%。原发于鳞状颞骨的尤文肉瘤则更为罕见。
方法
一名 16 岁男性出现突发性感觉神经性听力损失（SSNHL）的迹象。在出现 SSNHL 后 1 周和新发头痛时，影像学研究显示起源于左鳞状颞骨并向颅内和颅外延伸的肿块。组织病理学研究显示该肿块为尤文肉瘤。
结果
该患者表现出诊断性 EWSR1 突变，并根据儿童肿瘤学组 AEWS0031 的间隔压缩臂，采用长春新碱、多柔比星和环磷酰胺与异环磷酰胺/依托泊苷交替的方案，在手术后接受辅助多药化疗和局部放疗。
结论
本例表现出非常罕见的位置，以及原发性尤文肉瘤不常见的症状。
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