Kim Jun Won, Hong Chang-Ki, Cha Yoon Jin, Kim Se Hoon, Suh Chang-Ok, Lee Kyu-Sung
Department of Radiation Oncology, Gangnam Severance Hospital, Yonsei University College of Medicine, Seoul, Korea.
Department of Neurosurgery, Gangnam Severance Hospital, Yonsei University College of Medicine, Seoul, Korea.
World Neurosurg. 2018 May;113:103-107. doi: 10.1016/j.wneu.2018.02.041. Epub 2018 Feb 14.
Chordomas are rare tumors showing locally aggressive nature and high rates of local recurrences. Distant metastases are infrequently reported and cardiac metastases are extremely rare.
A 32-year-old woman had been diagnosed with a large chordoma involving the clivus. She received a total removal of the tumor and adjuvant intensity-modulated radiotherapy. Four and a half years after the surgery, she presented with multifocal lesions in the ventricles and the right atrium, although an open thoracotomy revealed chronic inflammation. Two years after finding the cardiac lesions, thoracoscopic biopsy of the chest wall lesion revealed metastatic chordoma. She received palliative imatinib for 2 weeks before she died of tumor progression.
This case illustrates the metastatic potential of clivus chordoma, and the heart can be a site of metastasis.
脊索瘤是一种罕见的肿瘤，具有局部侵袭性，局部复发率高。远处转移报道较少，心脏转移极为罕见。
一名32岁女性被诊断为累及斜坡的巨大脊索瘤。她接受了肿瘤全切及辅助调强放疗。术后四年半，她出现脑室和右心房的多灶性病变，尽管开胸手术显示为慢性炎症。发现心脏病变两年后，胸壁病变的胸腔镜活检显示为转移性脊索瘤。她在死于肿瘤进展前接受了两周的姑息性伊马替尼治疗。
本病例说明了斜坡脊索瘤的转移潜能，心脏可作为转移部位。
