Fornelli Adele, Zanini Nicola, De Biase Dario, Lega Stefania, Lombardi Raffaele, Masetti Michele, Jovine Elio, Fabbri Carlo, Larghi Alberto
1 Unit of Anatomic Pathology, Ospedale Maggiore, Bologna, Italy.
2 Unit of Surgery, Ospedale Maggiore, Bologna, Italy.
Int J Surg Pathol. 2019 Feb;27(1):89-93. doi: 10.1177/1066896918784666. Epub 2018 Jul 11.
Carcinoma of the ampulla of Vater is an uncommon neoplasm and represents 0.5% of all gastrointestinal malignancies, being less common than carcinoma of the pancreas and bile ducts. The most common ampullary tumor is the adenocarcinoma with tubular growth pattern. Signet ring cell carcinoma is extremely rare. In this article, we report a case of signet ring cell carcinoma of the ampulla of Vater showing focal neuroendocrine amphicrine differentiation and intestinal phenotype, which occurred in a 49-year-old male who is still alive 7 years after surgery, without evidence of recurrence. This long-term survival might be attributed not only to the early stage of the disease but also to the neuroendocrine differentiation and the absence of genetic alterations.
壶腹癌是一种罕见的肿瘤，占所有胃肠道恶性肿瘤的0.5%，比胰腺癌和胆管癌少见。最常见的壶腹肿瘤是具有管状生长模式的腺癌。印戒细胞癌极为罕见。在本文中，我们报告了一例壶腹印戒细胞癌，其表现为局灶性神经内分泌双相分化和肠型表型，该病例发生在一名49岁男性身上，术后7年仍存活，无复发迹象。这种长期生存可能不仅归因于疾病的早期阶段，还归因于神经内分泌分化和无基因改变。
