Maharajan Karthikeyan, Hey Hwee Weng Dennis, Tham Ivan, Thamboo Thomas Paulraj, Wong Alvin, Khan Irfan Sagir, Kumar Naresh
Aarhus University Hospital, Aarhus, Denmark.
University Orthopaedics, Hand and Reconstructive Microsurgery Cluster, National University Health System, Singapore.
J Spine Surg. 2017 Jun;3(2):287-293. doi: 10.21037/jss.2017.06.06.
Primary clear cell carcinoma of liver (PCCCL) is an uncommon variant of primary hepatocellular carcinoma. Though the literature describes a better prognosis in relation to the proportion of clear cells in the tumour when compared to the other variants, there is no general consensus in the management due to its rarity and unclear clinicopathological and prognostic factors. There is dearth of evidence with regard to the metastasizing nature of PCCCL and its management. In addition, the management of recurrent spinal tumours both primary and metastatic is not clear as the available evidence is mostly based on case reports. We describe an unusual presentation of PCCCL with solitary spinal metastasis and further complicated by tumour recurrence in a 71-year-old male. Such presentation has never been described before. He presented with low back pain and incomplete neurological deficits involving both lower limbs. On detailed evaluation, he was found to have a solitary metastasis at L3 vertebra secondary to PCCCL. He underwent radical excision of tumour and reconstruction for the solitary metastasis at L3 vertebral body and trans arterial chemo embolisation (TACE) for the hepatic lesion. Pt was asymptomatic until 9 months post operatively when he developed tumour recurrence at L3 vertebra. Patient subsequently underwent 2 stage palliative surgery followed by radiotherapy and chemotherapy. At his latest follow-up (1 year), the patient's overall general condition has improved with residual neurological deficits in the lower limb. PCCCL is a rare type of hepatocellular carcinoma which can present as "solitary metastasis" to the spine. Although the literature suggests a good prognosis for this histological type, this case did not have a good outcome. In addition to providing information for the management of similar cases in the future, this case report highlights that every patient has to be managed on a case-by-case basis.
肝原发性透明细胞癌（PCCCL）是原发性肝细胞癌的一种罕见变体。尽管文献表明，与其他变体相比，该肿瘤中透明细胞的比例预示着更好的预后，但由于其罕见性以及临床病理和预后因素尚不明确，在治疗方面尚未达成普遍共识。关于PCCCL的转移特性及其治疗，目前证据不足。此外，原发性和转移性复发性脊柱肿瘤的治疗尚不明确，因为现有证据大多基于病例报告。我们描述了一名71岁男性患者，其PCCCL表现为罕见的孤立性脊柱转移，并进一步并发肿瘤复发。此前从未有过此类病例报告。他因腰背痛和双下肢不完全神经功能缺损就诊。经过详细评估，发现他因PCCCL在L3椎体有孤立性转移。他接受了肿瘤根治性切除术，并对L3椎体的孤立性转移灶进行了重建，同时对肝脏病变进行了经动脉化疗栓塞（TACE）。术后9个月前患者无症状，之后L3椎体出现肿瘤复发。患者随后接受了两阶段姑息性手术，随后进行了放疗和化疗。在最近一次随访（1年）时，患者的整体一般状况有所改善，但下肢仍有残余神经功能缺损。PCCCL是一种罕见的肝细胞癌类型，可表现为脊柱“孤立性转移”。尽管文献表明这种组织学类型预后良好，但该病例的结局并不理想。除了为未来类似病例的治疗提供信息外，本病例报告还强调，每个患者都必须根据具体情况进行个体化治疗。
