昆明医科大学第二附属医院 肝胆胰外科，云南 昆明 650000
Department of Hepatobiliary and Pancreatic Surgery, the Second Affiliated Hospital of Kunming Medical University, Kunming 650000, China
背景与目的 胰腺肝样腺癌（PHC）是一种罕见的易发生于胰腺体尾部的具有肝细胞癌（HCC）样分化特征的特殊类型腺癌，其发病率低，恶性程度高，侵袭性强，早期易发生淋巴转移和远处转移，疾病发展快，预后较差。PHC临床表现和影像学特征缺乏特异性，术前诊断困难，但多数患者可高表达甲胎蛋白（AFP），为其诊断提供一定依据，而最终疾病的确诊需进行病理学检查。本文回顾性分析总结1例PHC并腹腔多发脏器转移的患者临床资料特点及诊治过程，并对国内外相关文献进行复习，旨在增加临床医生对PHC的认识，并不断完善疾病的治疗方案。方法 回顾性分析昆明医科大学第二附属医院肝胆胰外科收治的1例PHC并腹腔多发脏器转移患者的临床资料及诊治过程，并结合国内外相关文献对该病的发生机制、临床特点、疾病诊断及治疗方案等进行分析总结。结果 患者为63岁男性，因出现腹胀腹痛伴乏力、纳差3个月余入院，检查提示肝脏、胰腺、脾脏、胃等多脏器占位性病变，性质待排，后行姑息性减瘤手术治疗。术后病理结果提示为PHC，实性型，组织学分级3级。术后3个月出现肝内转移灶，行奥沙利铂130 mg+氟尿嘧啶200 mg肝动脉灌注化疗及栓塞治疗，后病灶转移至肺，于术后10个月因多器官功能衰竭死亡。结论 PHC是一种罕见的病因不明的具有HCC样分化特征的特殊类型腺癌，缺乏典型的临床及影像学表现，该病恶性程度高，早期易发生淋巴转移和远处转移，出现症状时多达中晚期，已丧失根治性手术切除机会；PHC发病机制不详，但病变表现为特征性的HCC样分化，可高度表达AFP及HCC免疫组织化学标志物，病理学检查是其诊断金标准；目前国内外尚无PHC的诊治共识，对于有手术切除机会的患者应积极手术治疗，切除病灶，改善预后；而对于无法根治性切除的患者可采取辅助治疗，目前认为放疗对其无效，而化疗是疾病的独立预后因素，但在化疗方案上仍存在争议；因此，有条件者可取材活检明确诊断后选择合适的化疗方案，提高患者治疗的客观缓解率，不能明确诊断者可参照消化道系统疾病治疗方案处理。
Background and Aims Pancreatic hepatoid adenocarcinoma (PHC) is a rare and aggressive adenocarcinoma with hepatocellular carcinoma (HCC)-like characteristics, primarily found in the tail of the pancreas. It has a low incidence rate, high malignancy, strong invasiveness, early propensity for lymphatic and distant metastasis, rapid disease progression, and poor prognosis. Clinical and radiological features of PHC lack specificity, making preoperative diagnosis challenging. However, most patients exhibit elevated alpha-fetoprotein (AFP) levels, which serves as a diagnostic clue. Nevertheless, the definitive diagnosis of the disease requires pathological examination. This article retrospectively analyzes and summarizes the clinical characteristics and treatment process of one patient with PHC and multiple intra-abdominal organ metastases, as well as reviews relevant literature from both domestic and international sources to enhance clinicians' understanding of PHC and continually improve treatment strategies for the disease.Methods The clinical data and treatment process of one patient with PHC and multiple intra-abdominal organ metastases treated in the Department of Hepatobiliary and Pancreatic Surgery at the Second Affiliated Hospital of Kunming Medical University were retrospectively analyzed. The pathogenesis, clinical characteristics, diagnostic methods, and disease treatment options were analyzed and summarized in combination with a review of relevant domestic and international literature.Results The patient was a 63-year-old male admitted to the hospital due to abdominal distension, abdominal pain, fatigue, and poor appetite for over three months. Examination revealed space-occupying lesions in multiple organs, including the liver, pancreas, spleen, and stomach, with the nature of the lesions yet to be determined. The patient underwent palliative tumor resection surgery. Postoperative pathological results supported the diagnosis of PHC, with a solid type and a histological grade of 3. Three months after surgery, intrahepatic metastases were detected, and the patient underwent hepatic arterial infusion chemotherapy with oxaliplatin (130 mg), fluorouracil (200 mg), and embolization therapy. Subsequently, the lesions metastasized to the lungs, and the patient died 10 months after surgery due to multiple organ failure.Conclusion PHC is a rare, poorly understood adenocarcinoma with HCC-like differentiation, lacking typical clinical and radiological features. It exhibits high malignancy early lymphatic and distant metastasis and is often diagnosed in advanced stages, and the opportunity for radical surgical resection has already been lost. The pathogenesis of PHC remains unclear, but it presents with characteristic HCC-like differentiation and often shows high levels of AFP and HCC immunohistochemical markers, making pathological examination the gold standard for diagnosis. Currently, there is no consensus on diagnosing and treating PHC at home and abroad. Aggressive surgical treatment is recommended for patients eligible for surgical resection to improve prognosis. However, adjuvant therapy should be considered for those who cannot undergo radical resection. Radiotherapy is generally considered ineffective, and chemotherapy is an independent prognostic factor, though the optimal chemotherapy regimen remains debated. Therefore, if conditions permit, an appropriate chemotherapy regimen can be selected after biopsy diagnosis to improve patients' objective remission rate. For those with an unclear diagnosis, treatment can follow protocols used for gastrointestinal system diseases.