胆管导管内乳头状肿瘤:临床病例启示与文献综述。

IF 1.7 Q2 MEDICINE, GENERAL & INTERNAL Clinics and Practice Pub Date : 2024-08-27 DOI:10.3390/clinpract14050133
Luca Toti, Tommaso Maria Manzia, Francesca Di Giuliano, Eliseo Picchi, Laura Tariciotti, Domiziana Pedini, Luca Savino, Giuseppe Tisone, Roberta Angelico
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引用次数: 0

摘要

背景:胆管导管内乳头状瘤(IPNB)是一种罕见的癌前病变,对胆管癌(CCA)的发展有一定影响。在临床实践中,识别 IPNB 和处理其复发是一项挑战。我们介绍两个病例。病例 1:一名 60 岁的男性因 8 × 8 × 9 厘米的肝囊肿就诊,起初怀疑是包虫囊肿。切除后的组织学检查发现是一个带有腺癌灶的 IPNB。尽管肿瘤边缘呈阴性,但八年后复发,病灶为rT2N0。在没有辅助化疗的情况下进行了手术切除,患者术后存活了 48 个月。病例 2:一名有认知障碍的 28 岁女性因肺栓塞入院,肝脏病变被诊断为单纯囊肿。随后的评估发现腺癌伴有局部转移、广泛的血管受累和血栓形成。尽管采取了积极的治疗措施,包括血栓切除术和化疗,但患者病情恶化,导致肝功能衰竭,最终死亡:结论:IPNB是一种罕见的恶性肿瘤前亚型,有发展为CCA的倾向。R0手术切除通常可提供良好的肿瘤治疗效果,复发风险极低。对局部可切除复发进行手术干预既安全又可行。跟踪 IPNB 复发情况的国际登记对于加深了解和优化诊断、管理和治疗策略至关重要。
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Intraductal Papillary Neoplasms of the Bile Duct: Clinical Case Insights and Literature Review.

Background: Intraductal papillary neoplasms of the bile duct (IPNB) are rare precancerous lesions with implications for the development of cholangiocarcinoma (CCA). Recognizing IPNB and managing its recurrence pose challenges in clinical practice. We present two cases. Case 1: a 60-year-old man presented with an 8 × 8 × 9 cm hepatic cyst initially suspected to be a hydatid cyst. Histology post-resection revealed an IPNB with foci of adenocarcinoma. Despite negative oncologic margins, recurrence occurred eight years later as an rT2N0 lesion. Surgical resection was performed without adjuvant chemotherapy, resulting in the patient's survival at 48 months post-surgery. Case 2: a 28-year-old female with cognitive impairment was admitted with pulmonary embolism and a liver lesion diagnosed as a simple cyst. Subsequent evaluation revealed adenocarcinoma with local metastases, extensive vascular involvement, and thrombosis. Despite aggressive management, including thrombectomy and chemotherapy, the patient's condition deteriorated, leading to hepatic failure and eventual demise.

Conclusion: IPNB represents a rare premalignant subtype with a propensity for progression to CCA. R0 surgical resection typically offers favorable oncological outcomes with a minimal recurrence risk. Surgical intervention for localized resectable recurrence is both safe and feasible. International registries tracking IPNB recurrence are essential for advancing understanding and optimizing diagnosis, management, and treatment strategies.

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来源期刊
Clinics and Practice
Clinics and Practice MEDICINE, GENERAL & INTERNAL-
CiteScore
2.60
自引率
4.30%
发文量
91
审稿时长
10 weeks
期刊最新文献
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