Bumber Zeljko, Krizanac Simun, Janjanin Sasa, Bilic Mario, Bumber Boris
University Department of ENT, Head & Neck Surgery, Zagreb University Hospital Center, Salata 4, 10000 Zagreb, Croatia.
Auris Nasus Larynx. 2009 Feb;36(1):116-9. doi: 10.1016/j.anl.2007.12.009. Epub 2008 Mar 21.
Chordomas are rare, malignant, slowly growing neoplasms which develop from vestigial remnants of the fetal notochord. Most chordomas arise in the sacrococcygeal and spheno-occipital region. Extranotochordal chordomas are extremely unusual. A case of extranotochordal chordoma with extralaryngeal localization is described. A 73-year-old male presented with swallowing difficulties and hoarseness. Contrast-enhanced magnetic resonance imaging of the neck revealed a well-encapsulated tumor mass that was well enhanced and located in the left retrolaryngeal space at the level of C4, dislocating the larynx to the right. Left radical neck dissection and tumor extirpation were performed. The tumor had not invaded cervical vertebra and the surrounding soft tissue but superficial erosions of the ossificated thyroid and cricoid cartilage were found. High-power pathologic examination and immunohistochemistry defined the lesion as a dedifferentiated type of chordoma. The patient received adjuvant radiotherapy. Four years after the surgery, the patient has been free from tumor recurrence.
脊索瘤是一种罕见的恶性肿瘤，生长缓慢，起源于胎儿脊索的残余遗迹。大多数脊索瘤发生在骶尾部和蝶枕区域。非脊索性脊索瘤极为罕见。本文描述了一例发生于喉外的非脊索性脊索瘤病例。一名73岁男性因吞咽困难和声音嘶哑就诊。颈部增强磁共振成像显示一个边界清晰的肿瘤肿块，强化明显，位于C4水平的左喉后间隙，将喉推向右侧。行左侧根治性颈清扫术及肿瘤切除术。肿瘤未侵犯颈椎及周围软组织，但发现钙化的甲状腺和环状软骨有浅表侵蚀。高倍病理检查及免疫组化将病变定义为去分化型脊索瘤。患者接受了辅助放疗。术后四年，患者无肿瘤复发。
