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伴有眼球突出的鼻窦恶性外间充质瘤。

Malignant ectomesenchymoma of the paranasal sinuses with proptosis.

作者信息

Vinck A S, Lerut B, Hermans R, Nuyts S, Sciot R, Jorissen M

机构信息

Department of Otorhinolaryngology, Head and Neck Surgery, University Hospitals Leuven, Belgium.

出版信息

B-ENT. 2011;7(3):201-4.

PMID:22026142
Abstract

PROBLEM

A 36-year-old woman presented with a feeling of pressure in the right orbit and proptosis of the right eye after a "common cold".

METHODOLOGY

Computed tomography (CT) of the maxillofacial region revealed, and endoscopy confirmed, a mass in the right ethmoid sinus, eroding the lamina papyracea and extending into the orbit. Pathology of multiple biopsies revealed a nasal neoplasm composed of neuroectodermal and mesenchymal neoplastic elements, suggestive of a malignant ectomesenchymoma (MEM). Magnetic resonance imaging was used for MEM staging. Computed tomography of the chest and abdomen show no evidence of distant metastases.

RESULTS

Due to the intracranial and intraorbital extension of the tumour, radical surgery was not an option. Appropriate chemotherapy (6 cycles of vincristine/ifosfamide/adriamycin and 2 cycles of vincristine/ifosfamide/cisplatin) and intensity-modulated radiation therapy were administered.

CONCLUSION

Twenty-eight months after treatment, there was no evidence of residual or metastatic disease.

摘要

问题

一名36岁女性在“普通感冒”后出现右眼眶部压迫感及右眼突出。

方法

颌面计算机断层扫描(CT)显示并经内镜证实右侧筛窦有一肿物,侵蚀纸样板并延伸至眼眶。多次活检病理显示为一种由神经外胚层和间充质肿瘤成分组成的鼻腔肿瘤,提示为恶性外胚间叶瘤(MEM)。采用磁共振成像进行MEM分期。胸部和腹部CT显示无远处转移证据。

结果

由于肿瘤向颅内和眶内扩展,无法进行根治性手术。给予了适当的化疗(长春新碱/异环磷酰胺/阿霉素6个周期及长春新碱/异环磷酰胺/顺铂2个周期)和调强放射治疗。

结论

治疗28个月后,无残留或转移性疾病证据。

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