原发性胆管鳞状细胞癌伴肝转移是罕见但恶性的。

IF 1.4 Q4 GASTROENTEROLOGY & HEPATOLOGY Gastroenterology Research Pub Date : 2023-10-01 Epub Date: 2023-10-21 DOI:10.14740/gr1637
Mohamad Khaled Almujarkesh, Anirudh R Damughatla, Jasdeep Bathla, Kyle Sugg, Dana LaBuda, Samer Alkassis, Mohammed Najeeb Al Hallak
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引用次数: 0

摘要

原发性肝鳞状细胞癌(SCC)非常罕见,据我们所知,文献中报道的病例很少。这种疾病的确切发病机制尚不清楚;然而,据报道,它大多与肝囊肿、Caroli病、肝结石、肝硬化和肝畸胎瘤有关。我们报告了一名50岁女性的病例,她最初没有病史,餐后上腹部和右上腹疼痛持续恶化,并伴有早期饱腹感、恶心和2个月内体重减轻25磅,这促使她的初级保健医生进行进一步评估。一个月后的磁共振成像(MRI)检查显示,右肝下叶有一个8.5×2.4×7.4 cm的大面积不均匀区域,胆囊有不均匀增强和受累,与胆管癌有关。根据放射学检查结果,她接受了计算机断层扫描(CT)引导的肝脏核心活检,结果显示坏死的恶性肿瘤有利于腺癌,还发现有种系BRCA突变。正电子发射断层扫描(PET)显示一个大的部分坏死的氟脱氧葡萄糖(FDG)肿块,可能来自胆囊窝,侵犯了肝脏的两个叶,可能累及了升结肠的一部分。没有明显的证据表明存在远处转移性疾病。患者在开始化疗前接受了分期腹腔镜检查,并进行了另一次活检,结果显示SCC,细胞角蛋白(CK)19、Ber-EP4(Ber-EP4抗体识别的上皮抗原)和P40(DeltaNp63)的免疫组织化学染色呈阳性;而CK7、CK20、尾侧型同源盒2(CDX-2)、配对盒8(PAX-8)和粘液胺呈阴性。由于种系BRCA突变,患者开始了基于铂的化疗。然而,由于与治疗和疾病进展相关的并发症,患者最终选择了临终关怀。原发性SSC仍然是一种未经探索的侵袭性恶性肿瘤,总体预后较差。由于缺乏特定的实验室检查,诊断可能具有挑战性,需要高度的临床怀疑。病理诊断仍然是金标准;然而,它也有自己的挑战。治疗通常以病例为导向,最终方案尚未制定。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

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Primary Squamous Cell Biliary Carcinoma With Liver Metastasis Is Rare but Malicious.

Primary squamous cell carcinoma (SCC) of the liver is quite rare, and to our knowledge, very few cases have been reported in the literature. The exact pathogenesis of the disease is unestablished; however, it is mostly reported to be associated with hepatic cyst, Caroli's disease, hepatolithiasis, hepatic cirrhosis, and hepatic teratoma. We report a case of a 50-year-old woman with no prior medical history initially, who presented with postprandial epigastric and right upper quadrant pain that continued to worsen and was associated with early satiety, nausea, and weight loss of 25 pounds over 2 months, which prompted further evaluation by her primary care physician. Magnetic resonance imaging (MRI) examination a month later revealed a large heterogeneous area measuring 8.5 × 2.4 × 7.4 cm in the inferior right hepatic lobe with heterogeneous enhancement and involvement of the gallbladder, concerning for cholangiocarcinoma. Given radiographic findings, she underwent a computed tomography (CT)-guided core biopsy of the liver, which showed a necrotic malignant tumor favoring adenocarcinoma and was also found to have germline BRCA mutation. A positron emission tomography (PET) scan revealed a large partially necrotic fluorodeoxyglucose (FDG) avid mass, possibly arising from the gallbladder fossa with an invasion of both lobes of the liver and probable involvement of a portion of the ascending colon. There was no gross evidence of distant metastatic disease. The patient underwent staging laparoscopy prior to initiating chemotherapy, and another biopsy was done, which returned in favor of SCC, with immunohistochemical stains being positive for cytokeratin (CK)19, Ber-EP4 (epithelial antigen recognized by Ber-EP4 antibody), and P40 (DeltaNp63); while negative for CK7, CK20, caudal-type homeobox 2 (CDX-2), paired box 8 (PAX-8), and mucicarmine. The patient started platinum-based chemotherapy due to germline BRCA mutation. However, due to complications associated with therapy and the progression of the disease, the patient eventually chose hospice. Primary SSC remains an unexplored aggressive malignancy that carries an overall poor prognosis. Diagnosis can be challenging and requires high clinical suspicion due to the scarcity in specific laboratory workup. Pathological diagnosis remains the gold standard; however, it also carries its own challenges. Treatment is usually case-oriented, and definitive protocols have yet to be established.

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Gastroenterology Research
Gastroenterology Research GASTROENTEROLOGY & HEPATOLOGY-
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