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肝腺瘤病:病因及治疗的重新评估

Liver adenomatosis: re-evaluation of aetiology and management.

作者信息

Veteläinen Reeta, Erdogan Deha, de Graaf Wilmar, ten Kate Fiebo, Jansen Peter L M, Gouma Dirk J, van Gulik Thomas M

机构信息

Department of Surgery, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands.

出版信息

Liver Int. 2008 Apr;28(4):499-508. doi: 10.1111/j.1478-3231.2008.01669.x.

Abstract

BACKGROUND

Liver adenomatosis (LA) is a rare benign parenchymal liver disease with unknown aetiology. In recent reports, LA has been associated with hepatic steatosis, with potential implications for the management of this disease. The aim of this study was to determine prognosis and optimal management of patients with LA.

METHODS

Clinical presentation, diagnostic studies and management of patients with LA were analysed in our centre. Furthermore, a Medline search of all published case reports and series of LA patients was performed.

RESULTS

Ninety-four patients with LA have been reported in the literature. Fifty-two per cent of females had a history of oral contraceptive use. Eighteen per cent of patients had steatosis in nontumoral tissue. In our own series, five of six patients had histologically confirmed steatosis. Forty-three per cent of patients presented with acute pain, of whom 46% had a haemorrhagical complication, in contrast to 2% of nonsymptomatic patients. Tumours <5 cm tended to increase in size during follow-up and only in four patients tumour regression was observed.

CONCLUSION

Liver adenomatosis is a progressive, benign parenchymal disease mainly occurring in females. There is a potential link with hepatic steatosis with implications for the management of patients with LA. Noninvasive diagnosis is difficult because of the variety of tumoral and nontumoral components. Management should primarily be conservative.

摘要

背景

肝腺瘤病(LA)是一种病因不明的罕见良性肝实质疾病。在最近的报道中,LA与肝脂肪变性有关,这可能对该疾病的治疗产生影响。本研究的目的是确定LA患者的预后和最佳治疗方法。

方法

对我们中心LA患者的临床表现、诊断研究和治疗进行了分析。此外,还对Medline上所有已发表的LA患者病例报告和系列研究进行了检索。

结果

文献中报道了94例LA患者。52%的女性有口服避孕药史。18%的患者在非肿瘤组织中有脂肪变性。在我们自己的系列研究中,6例患者中有5例经组织学证实有脂肪变性。43%的患者出现急性疼痛,其中46%有出血并发症,无症状患者的这一比例为2%。<5 cm的肿瘤在随访期间往往会增大,只有4例患者观察到肿瘤缩小。

结论

肝腺瘤病是一种主要发生在女性的进行性良性肝实质疾病。它与肝脂肪变性可能存在联系,这对LA患者的治疗有影响。由于肿瘤和非肿瘤成分的多样性,无创诊断较为困难。治疗应以保守为主。

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