胰腺腺泡细胞癌诊断与治疗

doi:10.3877/cma.j.issn.2095-3232.2024.04.008

胰腺腺泡细胞癌（PACC）是来源于胰腺上皮的恶性肿瘤，因其发病率较低而在临床上少见，且无特异性临床表现。国内外报道PACC仅占胰腺外分泌肿瘤的1%～2%，占胰腺恶性肿瘤的0.44%。该病具有诊断困难、手术可切除性低、易转移的特点，但预后优于胰腺导管腺癌，认识及掌握PACC的临床诊治可以提高其诊断率并改善预后。

关键词: 胰腺腺泡细胞癌, 胰腺外分泌肿瘤, 外科治疗, 综合治疗

Pancreatic acinar cell carcinoma (PACC) is a malignant epithelial tumor arising from pancreatic epithelium, which is rare in clinical practice due to low incidence, and it has no specific clinical manifestations. Previous studies have reported that PACC accounts for merely 1%-2% of exocrine pancreatic tumors and 0.44% of pancreatic malignant tumors. This disease is challenging to diagnose, has low resection rates, and easily metastasizes. However, the prognosis is better than that of pancreatic ductal adenocarcinoma. Understanding and mastering clinical diagnosis and treatment of PACC can improve diagnostic rate and clinical prognosis.

Key words: Pancreatic acinar cell carcinoma, Pancreatic exocrine tumor, Surgical treatment, Comprehensive treatment

图1 PACC增强CT和MRI表现注：a、b、c分别为增强CT的动脉期、静脉期和延迟期，d、e、f分别为增强MRI的动脉期、静脉期和延迟期；PACC为胰腺腺泡细胞癌

图2 PACC病理学表现注：肿瘤呈腺泡状或实性结构，腺泡状结构由大小不一的细胞组成，嗜酸性颗粒状细胞质（100× HE染色）；PACC为胰腺腺泡细胞癌

表1 单纯性PACC诊断要点

分类	要点
临床表现	非特异性症状：体重减轻、恶心呕吐、黑便、乏力等
	高脂肪酶血症（10%～15%患者）：皮下脂肪坏死多发性结节灶^[7]，骨脂肪坏死导致松质骨硬化性病变引起的多关节痛
实验室检查	血清脂肪酶升高
	肿瘤标志物升高无一致性，部分患者AFP升高
影像学表现	外生性、圆形或椭圆形、边缘清楚、低血管性肿块，早期病灶多为实性，而晚期病灶可出现坏死表现为囊实性改变。增强CT表现为局限和均匀性强化，但强化程度低于周围胰腺
组织病理学	有不同程度的腺泡分化，但细胞排列缺乏整齐和致密性，细胞具有基向核、单个突出的核仁和嗜酸性颗粒状细胞质
	PAS染色和丁酸酯酶染色阳性，抗胰蛋白酶、凝乳蛋白酶、脂肪酶和淀粉酶阳性
	通常腺瘤性大肠息肉病（APC）基因突变和β-catenin通路异常

表1 单纯性PACC诊断要点

分类	要点
临床表现	非特异性症状：体重减轻、恶心呕吐、黑便、乏力等
	高脂肪酶血症（10%～15%患者）：皮下脂肪坏死多发性结节灶^[7]，骨脂肪坏死导致松质骨硬化性病变引起的多关节痛
实验室检查	血清脂肪酶升高
	肿瘤标志物升高无一致性，部分患者AFP升高
影像学表现	外生性、圆形或椭圆形、边缘清楚、低血管性肿块，早期病灶多为实性，而晚期病灶可出现坏死表现为囊实性改变。增强CT表现为局限和均匀性强化，但强化程度低于周围胰腺
组织病理学	有不同程度的腺泡分化，但细胞排列缺乏整齐和致密性，细胞具有基向核、单个突出的核仁和嗜酸性颗粒状细胞质
	PAS染色和丁酸酯酶染色阳性，抗胰蛋白酶、凝乳蛋白酶、脂肪酶和淀粉酶阳性
	通常腺瘤性大肠息肉病（APC）基因突变和β-catenin通路异常

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