Thranitz M, Berg T, Kneifel C, Stock K, Knipping S
Klinik für Hals-, Nasen- und Ohrenheilkunde, Kopf- und Halschirurgie, Plastische Operationen, Städtisches Klinikum Dessau, Auenweg 38, 06847, Dessau-Roßlau, Deutschland.
HNO. 2014 Mar;62(3):202-6. doi: 10.1007/s00106-013-2780-6.
Sarcomas count among the very rare malignancies of the orbit. Epithelioid sarcomas typically occur in the subcutaneous tissue of younger patients; an affected orbit constitutes a rarity.
Only three cases of this extremely rare disease of the orbit, which is associated with marked aggressiveness and a very poor prognosis, have been analysed in the worldwide current literature.
We present a case report together with a literature review. A rapidly progressing painful proptosis with visual loss occurred in a 30-year-old patient. Imaging and invasive diagnostic procedures were initiated as a consequence of this.
Diagnostic imaging revealed an infiltrative process of the right orbit. Following endonasal decompression of the orbit and probe acquisition, histological and immunohistochemical tests showed the presence of a proximal type epithelioid sarcoma. Therapy consisted of orbit exenteration together with implantation of titanium miniplates for orbital prosthesis. During the course of disease, osseous metastases developed. The patient died during palliative chemotherapy, 14 months after the initial diagnosis.
Epithelioid sarcomas are extremely rare, difficult to treat tumours in the head and neck region. The associated mortality rates are high. Aggressive surgical therapy with intensive follow-up is recommended. The prognosis depends upon the resection status.
肉瘤是眼眶极为罕见的恶性肿瘤。上皮样肉瘤通常发生于年轻患者的皮下组织；累及眼眶者实属罕见。
在全球现有文献中，仅分析过3例这种与显著侵袭性和极差预后相关的极为罕见的眼眶疾病。
我们报告1例病例并进行文献复习。1例30岁患者出现迅速进展的疼痛性眼球突出并伴有视力丧失。因此启动了影像学及侵入性诊断程序。
诊断性影像学检查显示右侧眼眶有浸润性病变。经鼻行眼眶减压并获取组织样本后，组织学及免疫组化检查显示为近端型上皮样肉瘤。治疗包括眼眶内容物剜除术及植入微型钛板以制作眼眶假体。在病程中出现了骨转移。患者在初次诊断14个月后于姑息化疗期间死亡。
上皮样肉瘤是头颈部极为罕见且难以治疗的肿瘤。相关死亡率很高。建议采取积极的手术治疗并进行密切随访。预后取决于切除情况。
