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青年成人的巨大纤维板层状癌:一例报告。

Voluminous fibrolamellar carcinoma in a young adult: A case report.

作者信息

Atallah Aziz, Slama Sana Ben, Guelbi Mohamed, Hadrich Zied, Megdiche Sadok, Omrani Sahir

机构信息

Department of Surgery, Mongi Slim Hospital, Marsa, Tunisia.

Department of Pathology, Mongi Slim Hospital, Marsa, Tunisia.

出版信息

Int J Surg Case Rep. 2025 Apr 23;131:111355. doi: 10.1016/j.ijscr.2025.111355.

DOI:10.1016/j.ijscr.2025.111355
PMID:40288144
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12059704/
Abstract

INTRODUCTION

Fibrolamellar carcinoma (FLC) is a rare primary liver cancer, typically presenting as a solitary nodule in young adults without underlying liver disease. Surgical resection is currently the only curative treatment.

PRESENTATION OF CASE

We report a 27-year-old woman with a 6-month history of moderate epigastric pain, right upper quadrant heaviness, and a 20-kg weight loss. Imaging studies (ultrasound, CT, and MRI) revealed an 11-cm mass in the right liver featuring a central scar and calcifications, highly suggestive of FLC. A liver biopsy confirmed the diagnosis. The patient subsequently underwent a right hepatectomy with en bloc resection of an adherent diaphragmatic collar and lymphadenectomy. Her postoperative course was uneventful, leading to discharge on postoperative day 10.

DISCUSSION

FLC accounts for less than 1 % of primary liver tumors and is distinct from conventional hepatocellular carcinoma, primarily due to its occurrence in non-cirrhotic, younger patients. Characteristic radiologic findings include a well-circumscribed, large lesion with a central fibrous scar and occasional calcifications. Although the prognosis post-resection is generally favorable, recurrence rates exceed 60 %, emphasizing the need for aggressive surgical management and vigilant long-term follow-up.

CONCLUSION

FLC is a distinct clinical entity with improved surgical outcomes compared to classical hepatocellular carcinoma. Nonetheless, its high recurrence potential necessitates continued surveillance and further research to optimize treatment strategies.

摘要

引言

纤维板层癌(FLC)是一种罕见的原发性肝癌,通常表现为年轻成人无潜在肝脏疾病的孤立性结节。手术切除是目前唯一的治愈性治疗方法。

病例介绍

我们报告一名27岁女性,有6个月中度上腹部疼痛、右上腹沉重感病史,体重减轻20公斤。影像学检查(超声、CT和MRI)显示右肝有一个11厘米的肿块,有中央瘢痕和钙化,高度提示纤维板层癌。肝活检确诊。患者随后接受了右肝切除术,整块切除粘连的膈肌环并进行淋巴结清扫。她的术后过程顺利,术后第10天出院。

讨论

纤维板层癌占原发性肝肿瘤的比例不到1%,与传统肝细胞癌不同,主要是因为它发生在非肝硬化的年轻患者中。典型的影像学表现包括边界清晰的大病变,有中央纤维瘢痕,偶尔有钙化。虽然切除术后的预后通常良好,但复发率超过60%,强调需要积极的手术管理和长期密切随访。

结论

纤维板层癌是一种独特的临床实体,与经典肝细胞癌相比,手术效果有所改善。尽管如此,其高复发潜力需要持续监测和进一步研究以优化治疗策略。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/fd1a/12059704/7ea29a12af4a/gr5.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/fd1a/12059704/821fa6b6a32f/gr1.jpg
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https://cdn.ncbi.nlm.nih.gov/pmc/blobs/fd1a/12059704/177b88f6ac1b/gr3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/fd1a/12059704/2efea920de09/gr4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/fd1a/12059704/7ea29a12af4a/gr5.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/fd1a/12059704/821fa6b6a32f/gr1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/fd1a/12059704/52592b6cfd4f/gr2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/fd1a/12059704/177b88f6ac1b/gr3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/fd1a/12059704/2efea920de09/gr4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/fd1a/12059704/7ea29a12af4a/gr5.jpg

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