Salvia R, Festa L, Butturini G, Tonsi A, Sartori N, Biasutti C, Capelli P, Pederzoli P
Department of Surgery, University of Verona, Verona, Italy.
Minerva Chir. 2004 Apr;59(2):185-207.
Cystic tumors of the pancreas are less frequent than other tumors in neoplastic pancreatic pathology, but in recent years the literature has reported an increasing number. After the first report by Becourt in 1830, cystic tumors were classified into 2 different types by Compagno and Oertel in 1978: benign tumors with glycogen-rich cells and mucinous cystic neoplasms with overt and latent malignancy. The WHO classification of exocrine tumors of the pancreas, published in 1996, is based on the histopathological features of the epithelial wall, which are the main factor in differential diagnosis with cystic lesions of the pancreas. Thanks to the knowledge acquired up to now, a surgical procedure is not always required because the therapeutic choice is conditioned by the correct classification of this heterogeneous group of tumors. Clinical signs are not really useful in the clinical work up, most patients have no symptoms and when clinical signs are present, they may help us to pinpoint the organ of origin but never to identify the type of pathology. In the last few years, the great improvement in imaging has enabled us not only to discriminate cystic from solid lesions, but also to identify the features of the lesions and label them preoperatively. More invasive diagnostic procedures such as fine needle aspiration and intracystic fluid tumor marker level are not really useful because they are not sensitive and the cystic wall can show different degrees of dysplasia and de-epithelialization. These are the reasons for sending the entire specimen to pathology. Good cooperation between surgeons, pathologists, radiologists and gastroenterologists is mandatory to increase the chances of making a proper diagnosis. Therefore, we must analyze all the information we have, such as age, sex, clinical history, location of the tumor and radiological features, in order to avoid the mistake of treating a cystic neoplasm as a benign lesion or as a pseudocyst, as described in the literature. Except for inoperable cases due to the critical condition of the patient or non-resectable lesions, surgical treatment differs with the diagnosis. Cystic tumors of the pancreas, therefore, are a heterogeneous group of tumors, with a real problem regarding differential diagnosis between neoplastic and inflammatory lesions. Even with a proper work up, some perplexity may remain about the nature of the lesion and in these cases the surgical procedure has a therapeutic value as well as playing a diagnostic role. The role of surgery is central in the treatment of these tumors because it could be curative when complete resection is possible. In this way, the lack of good therapeutic results with chemotherapy and radiotherapy force the surgeon to go ahead with the procedure. Intraductal papillary mucinous neoplasms represent a new and, from the epidemiological point of view, important chapter in the world of cystic tumors. The margin of resection is important and the surgeon has to be aware that in order to have a curative resection, total pancreatectomy is sometimes required. In the last few years the therapeutic approach has changed thanks to new knowledge of the biological behavior of these tumors. In fact, from a surgical approach in all cases, we are now discussing the possibility of a follow-up not only for asymptomatic serous cystadenomas but also for the little branch side intraductal papillary mucinous neoplasms (IPMNs) in critical patients. A follow-up could be planned even for solid pseudopapillary tumors but it seems risky to leave untreated big tumors in young patients without a certain diagnosis and with so few studies reported in the literature.
胰腺囊性肿瘤在胰腺肿瘤性病变中比其他肿瘤少见,但近年来文献报道的数量有所增加。1830年贝库尔首次报告后,1978年孔帕尼奥和奥特尔将囊性肿瘤分为2种不同类型:富含糖原细胞的良性肿瘤和具有明显及潜在恶性的黏液性囊性肿瘤。1996年发布的世界卫生组织胰腺外分泌肿瘤分类基于上皮壁的组织病理学特征,这是与胰腺囊性病变进行鉴别诊断的主要因素。由于目前已掌握的知识,并非总是需要进行外科手术,因为治疗选择取决于对这一异质性肿瘤组的正确分类。临床体征在临床检查中并非真正有用,大多数患者没有症状,而当出现临床体征时,它们可能帮助我们确定起源器官,但无法确定病理类型。在过去几年中,影像学的巨大进步使我们不仅能够区分囊性和实性病变,还能识别病变特征并在术前进行标记。更具侵入性的诊断程序,如细针穿刺和囊内液体肿瘤标志物水平检测,并非真正有用,因为它们不敏感,且囊壁可显示不同程度的发育异常和去上皮化。这些就是将整个标本送检病理的原因。外科医生、病理学家、放射科医生和胃肠病学家之间的良好合作对于提高正确诊断的几率至关重要。因此,我们必须分析所掌握的所有信息,如年龄、性别、临床病史、肿瘤位置和放射学特征,以避免如文献中所述将囊性肿瘤误诊为良性病变或假性囊肿的错误。除因患者病情危急或病变不可切除而无法手术的情况外,手术治疗因诊断不同而有所差异。因此,胰腺囊性肿瘤是一组异质性肿瘤,在肿瘤性和炎性病变的鉴别诊断方面存在实际问题。即使进行了适当的检查,对于病变的性质可能仍会存在一些困惑,在这些情况下,外科手术不仅具有诊断作用,还具有治疗价值。手术在这些肿瘤的治疗中起着核心作用,因为如果能够完全切除,手术可能治愈。这样一来,化疗和放疗效果不佳迫使外科医生继续进行手术。导管内乳头状黏液性肿瘤代表了囊性肿瘤领域中一个新的且从流行病学角度来看很重要的篇章。切除边缘很重要,外科医生必须意识到,为了实现根治性切除,有时需要进行全胰切除术。在过去几年中,由于对这些肿瘤生物学行为的新认识,治疗方法发生了变化。事实上,从过去对所有病例都采用手术方法,我们现在不仅在讨论对无症状浆液性囊腺瘤进行随访的可能性,还在讨论对病情危急的患者中较小分支型导管内乳头状黏液性肿瘤(IPMN)进行随访的可能性。甚至对于实性假乳头状肿瘤也可以计划进行随访,但对于年轻患者中未明确诊断且文献报道研究较少的大肿瘤不进行治疗似乎存在风险。