Chen Nelson, Slater Kellee
Department of Hepatobiliary and Pancreatic Surgery, Princess Alexandra Hospital, 199 Ipswich Road, Woolloongabba, QLD, 4102, Australia.
World J Surg Oncol. 2017 Jan 18;15(1):27. doi: 10.1186/s12957-017-1102-y.
Solitary fibrous tumours (SFT) are neoplasms of mesenchymal origin that predominantly arise from the pleura. SFT of the liver (SFTL) are a rare occurrence with little number of cases reported in English literature. Malignant cases of hepatic SFT are an even rarer occurrence. For this reason, the prognostic evaluation of SFTLs is unknown and difficult to measure.
A search on English literature on "Solitary Fibrous Tumour of the Liver" was conducted on common search engines (PubMed, Google). All published articles, case reports and literature reviews and their reference lists were reviewed.
This paper presents a 61-year-old male who was referred to a tertiary hospital in April 2010 with marked hepatomegaly. USS, CT and MRI scans were suggestive of a neoplasm, and the patient underwent a subsegmental IVb resection in June 2010. The specimen demonstrated histological and immunohistochemical features of malignant SFTL with clear resection margins. The patient was followed up regularly for 3 years with imaging and no suggestion of recurrence. Six years after the initial surgery, the patient represented with worsening right upper quadrant pain and dyspnoea secondary to extensive tumour recurrence adjacent to the resection site and metastatic deposits in the pleura. The patient was managed symptomatically and discharged for community follow-up after palliative involvement.
SFTL are rare with only 84 cases reported in the English Literature including the present case. The average age of patients is 57.1 and occurs in females more than males (1.4:1). Most SFTLs follow a benign course, however, 17.9% of cases displayed malignant histological features. Only three cases including the current case are reported to have both local recurrence and metastasis. Surgical resection remains the mainstay of treatment and appears to be curative of most cases. The rarity of this tumour makes it difficult to evaluate its prognosis and natural course.
孤立性纤维瘤(SFT)是间叶组织来源的肿瘤,主要起源于胸膜。肝孤立性纤维瘤(SFTL)极为罕见,英文文献中报道的病例数量很少。肝SFT的恶性病例更是罕见。因此,SFTL的预后评估尚不清楚且难以衡量。
在常用搜索引擎(PubMed、谷歌)上搜索关于“肝孤立性纤维瘤”的英文文献。对所有已发表的文章、病例报告和文献综述及其参考文献列表进行了审查。
本文介绍了一名61岁男性,2010年4月因明显肝肿大被转诊至一家三级医院。超声、CT和MRI扫描提示为肿瘤,该患者于2010年6月接受了IVb段次全切除术。标本显示为恶性SFTL的组织学和免疫组化特征,切缘清晰。对该患者进行了为期3年的定期影像学随访,未发现复发迹象。初次手术后6年,患者因切除部位附近广泛肿瘤复发及胸膜转移灶,出现右上腹疼痛加重和呼吸困难。对患者进行了对症处理,姑息治疗后出院接受社区随访。
SFTL很罕见,英文文献中包括本病例在内共报道了84例。患者的平均年龄为57.1岁,女性多于男性(1.4:1)。大多数SFTL病程呈良性,但17.9%的病例显示出恶性组织学特征。据报道,只有3例(包括本病例)出现局部复发和转移。手术切除仍然是主要的治疗方法,似乎对大多数病例具有治愈性。这种肿瘤的罕见性使得难以评估其预后和自然病程。