Acinar cell cystadenocarcinoma of the pancreas: Report of rare case and review of the literature

ArticleinHuman Pathlogy 35(12):1568-71 · January 2005with4 Reads
DOI: 10.1016/j.humpath.2004.08.004 · Source: PubMed
Most exocrine pancreatic tumors are of ductal origin, whereas acinar cell adenocarcinomas are unusual (1% to 2% of all exocrine pancreatic neoplasms). We recently found a cystic adenocarcinoma of the pancreatic body whose cells had the characteristics of acinar cells, which we term acinar cell cystadenocarcinoma. Macroscopically, this tumor consists of a large multilocular cystic mass with a pseudocapsule and a spongy appearance on the cut surface. Microscopically, the cysts are lined by a single layer of cuboid/columnar cells. The cytoplasm has the characteristics of acinar cells, with eosinophilic granules in the apex and prominent nucleoli. Immunohistochemically, the cells express alpha1-antitrypsin, trypsin, and lipase in their cytoplasm, thus confirming the acinar origin of the tumor. A review of the literature revealed only 5 other cases of this tumor reported since its first description in 1981. Follow-up data are available for 4 of these; all of the affected patients had metastases at presentation or a few months later, and 2 died of the disease, at 13 and 37 months after diagnosis. Although this variant of adenocarcinoma of the pancreas is not prognostically different from the classic solid type (few patients survive more than 5 years), we believe that it is important because of its extreme rarity.
    • "Acinar cystadenocarcinoma is extremely rare with fewer than 10 cases reported in the literature. these tumors typically present with abdominal pain and a multilocular cystic lesion [24]. of ovarian-like stroma (Figure 2A) [10]. mcns usually appear smooth, well-defined, and unilocular or with a few septations (Figure 2B). "
    [Show abstract] [Hide abstract] ABSTRACT: This article reviews the current strategies and challenges of diagnosing pancreatic cystic lesions, and presents an overview of molecular tools that are available to enhance diagnostic accuracy. Specifically, we highlight the emergence of microRNAs (miRNAs) as diagnostic markers. miRNA signatures have been reported for both solid tissue and biofluid specimens, including cyst fluid, collected from patients with solid and cystic pancreatic lesions. These miRNA signatures offer the opportunity to improve molecular characterization of pancreatic lesions, to help guide clinical management through early diagnosis and informed prognosis, and to provide novel therapeutic targets for pancreatic cancer.
    Full-text · Article · Jan 2014
    • "The pathological classification of these various types of cystic pancreatic neoplasms is still evolving, but the most common types include IPMN, SCN, MCN, and SPN [2e6]. Rarer cystic neoplasms include acinar cell cystadenocarcinoma [9], acinar cell cystadenoma [10], cystic neuroendocrine tumor [11] and cystic mesenchymal tumor [12]. However, it should always be borne in mind that the differential diagnoses of cystic neoplasms of the pancreas should also include PDA with cystic change. "
    [Show abstract] [Hide abstract] ABSTRACT: Pancreatic ductal adenocarcinoma (PDA) with cystic change is classified into several types according to the features of the cysts; however, those tumors do not constitute a uniform group, and the classification is controversial. In this study, we have described a series of cystic PDAs that show distinctive and previously unreported morphologic and immunohistochemical features. We analyzed 200 cases of PDA treated surgically at a single institution, and extracted the clinical and histopathological features of 7 tumors showing multiple large cystic (MLC) structure. Preoperative radiographic images revealed a multilocular mass in the pancreas which was similar to intraductal papillary mucinous neoplasm or mucinous cystic neoplasm. These tumors were associated with more than 5 large cystic structures and numerous intratumoral microcysts lined by epithelial cells with various degrees of atypia. The average maximal diameter of the cysts (3.7 cm) was much larger than that of previously reported. Immunohistochemically, the cyst-lining epithelia were almost negative for mucin core protein (MUC) 1, MUC2, and MUC6, and showed only focal staining for MUC5AC. Maspin, CEA, and p53 were strongly positive, and the Ki-67 labeling index was high in both cells in solid areas and cyst-lining epithelia. We considered the MLC structures in PDA to be a mixture of ectatic neoplastic glands and retention cysts with ductal cancerization or pancreatic intraepithelial neoplasia (PanIN); however, they might represent a new entity of cystic PDA because of the unusually large size of the dilated cysts.
    Full-text · Article · Jul 2013
    • "The cystic form of acinar cell tumor is rare [22]. It could be present in a benign form as acinar cell cystadenoma, in addition to the malignant form known as acinar cell cystadenocarcinoma [23, 24]. This rare tumor entity should not be confused with solid pseudopapillary tumor of the pancreas in which the cystic component is rather a degenerative process than true cyst as in the cystic form of acinar cell tumors [25]. "
    Full-text · Chapter · Sep 2011 · Pancreatology
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