Auger M, Raney B, Callender D, Eifel P, Ordóñez N G
Division of Pathology, University of Texas M. D. Anderson Cancer Center, Houston 77030.
Pediatr Pathol. 1994 Sep-Oct;14(5):763-70. doi: 10.3109/15513819409037673.
A 27-month-old boy of Hispanic background developed multiple cranial nerve palsies, difficulty swallowing, bloody nasal discharge, and irritability. Radiographic evaluations showed extensive destruction of the clivus by a large tumor that invaded the sphenoid bone, left cavernous sinus, ethmoid sinus, nasal cavity, and left orbit. Multiple pulmonary nodules were also noted. The bone marrow and spinal fluid showed no evident tumor cells. Transnasal biopsy revealed a chordoma. Treatment was initiated with a combination of ifosfamide, mesna, and etoposide along with radiation therapy to the cranial tumor. Shifting pulmonary densities were noted on serial films. Despite some clinical improvement, the child developed rapidly progressive hypoxemia 3 weeks after admission and died. Autopsy showed persistent viable tumor in the primary site and massive pulmonary arteriolar tumor emboli, infarcts, and widespread lung parenchymal metastases. No other sites of tumor involvement were discovered. This is the second child reported with intracranial chordoma, pulmonary metastases at diagnosis, and early death attributed to pulmonary tumor emboli.
一名27个月大的西班牙裔男孩出现了多条颅神经麻痹、吞咽困难、鼻出血和易怒症状。影像学评估显示，一个大肿瘤广泛破坏斜坡，侵犯蝶骨、左侧海绵窦、筛窦、鼻腔和左侧眼眶。还发现了多个肺结节。骨髓和脑脊液未发现明显的肿瘤细胞。经鼻活检显示为脊索瘤。开始采用异环磷酰胺、美司钠和依托泊苷联合治疗，并对颅内肿瘤进行放射治疗。系列胸片显示肺部密度改变。尽管临床症状有所改善，但患儿入院3周后出现快速进展性低氧血症并死亡。尸检显示原发部位存在持续存活的肿瘤，并有大量肺小动脉肿瘤栓子、梗死灶和广泛的肺实质转移。未发现其他肿瘤累及部位。这是第二例报告的诊断时伴有颅内脊索瘤、肺转移且早期死于肺肿瘤栓子的儿童病例。
