Acinar Cell Carcinoma With Prominent Intraductal Growth Pattern

Toll, Adam D.; Mitchell, Donald G.; Yeo, Charles J.; Hruban, Ralph H.; Witkiewicz, Agnieszka K.

doi:10.1177/1066896909339912

Cited by 31 publications

(25 citation statements)

References 7 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…This is consistent with earlier suggestions that ACC showing intraductal and/or papillary growth is associated with lower morbidity and mortality than typical ACC. 1,14 Similar results have been reported for PDCs; those with intraepithelial extension exceeding 10 mm in the main pancreatic duct had a better outcome than PDCs without such intraepithelial tumor extension. 12 is an obstruction of the main pancreatic duct, caused by the broken-off tip of the polypoid projection.…”

Section: Discussionsupporting

confidence: 84%

Pancreatic Ducts as an Important Route of Tumor Extension for Acinar Cell Carcinoma of the Pancreas

Ban¹,

Shimada

Sekine

et al. 2010

American Journal of Surgical Pathology

View full text Add to dashboard Cite

Acinar cell carcinoma (ACC) of the pancreas is very rare, which usually grows expansively. Recently, a variant of ACC with predominant growth in the pancreatic ducts has been proposed, and is speculated to have potentially less aggressive behavior. The aim of this study was to investigate how the pancreatic duct system is related to the growth and extension of ACC. We reviewed the detailed gross and histologic features of 13 cases of ACC, of which 7 (54%) showed intraductal polypoid growth (IPG) of the tumor in the large pancreatic ducts with a mean IPG length of 24.8 mm. Tumors with IPG were found to spread characteristically along the pancreatic ducts as extending polypoid projections, filling the ducts and destroying the duct walls, although tumors did not tend to extend beyond the pancreatic parenchyma. Comparison of the clinicopathologic characteristics showed that ACC with IPG had less infiltrative features including lymphatic, venous, and neural invasion, formation of tumor thrombus in the portal vein, nodal metastasis, and invasion beyond the pancreas to the surrounding organs; death in only 1 case (14%) of ACC with IPG was the result of ACC itself. In contrast, ACC without IPG frequently showed more infiltrative growth, and was the cause of death in 50% of patients with this type of tumor. Intraductal dissemination of ACC in pancreatic ducts was proven in 1 case of ACC with IPG. These findings suggest that a significant proportion of ACC shows IPG, which is potentially linked to less aggressive clinicopathologic characteristics.

show abstract

Section: Discussionsupporting

confidence: 84%

Pancreatic Ducts as an Important Route of Tumor Extension for Acinar Cell Carcinoma of the Pancreas

Ban¹,

Shimada

Sekine

et al. 2010

American Journal of Surgical Pathology

View full text Add to dashboard Cite

show abstract

“…Most acinar cell carcinomas are large solid lesions and demonstrate no involvement of the native pancreatic ducts. However, rare cases have been described in which an intraductal growth pattern is present either focally or extensively 64, 68–70 . Fortunately, immunohistochemistry is very helpful.…”

Section: Discussionmentioning

confidence: 99%

Intraductal Tubulopapillary Neoplasm of the Pancreas

Basturk¹,

Adsay

Askan³

et al. 2017

American Journal of Surgical Pathology

View full text Add to dashboard Cite

Background Intraductal tubulopapillary neoplasm (ITPN) is a relatively recently described member of the pancreatic intraductal neoplasm family. Thus, the literature on its histologic and immunohistochemical features, clinical behavior, and its similarities and differences from other pancreatic neoplasms is limited. Design Thirty-three cases of ITPN, the largest series to date, were identified. Immunohistochemical labeling for cytokeratins, glycoproteins, pancreatic enzymes, markers for intestinal and neuroendocrine differentiation, and antibodies associated with genetic alterations previously described in pancreatic neoplasms were performed. Clinicopathologic features and survival was assessed. Results Seventeen patients were female, fourteen were male. Mean age was 55 years (range, 25–79). Median overall tumor size was 4.5 cm (range, 0.5–15). Forty-five percent of the tumors occurred in the head, 32% in the body/tail, and 23% showed diffuse involvement. Microscopically, the tumors were characterized by intraductal nodules composed of tightly packed small tubular glands lined by cuboidal cells lacking apparent mucin. Although it was often challenging to determine its extent, invasion was present in 71%. Almost all tumors labeled for CAM5.2, CK7 and CK19; most expressed CA19.9, MUC1 and MUC6. CDX2, MUC2, trypsin, chymotrypsin, chromogranin and synaptophysin were not expressed. SMAD4 expression was retained in 100%, p16 expression and p53 overexpression was seen in 33% and 27%. Follow-up information was available for twenty-two patients (median follow-up, 45 months; range, 11–173). Two patients with invasive carcinoma died of disease at 23 and 41 months. One patient died of unrelated causes at 49 months. Twelve patients were alive with disease. Seven patients were alive with no evidence of disease. The overall 1-, 3- and 5-year survival rates were 100% in patients without an invasive component and 100%, 91% and 71% in patients with an invasive component (p=0.7). Conclusions ITPN is a distinct clinicopathologic entity in the pancreas. Despite the difficulties of determining the extent of invasive carcinoma in many cases, the overall outcome appears relatively favorable and substantially better than that of conventional ductal adenocarcinoma, even when only the cases with invasive carcinoma are considered.

show abstract

“…Predominantly intraductal growth of acinar cell carcinoma is uncommon but has been reported. 4,5 Microscopically, acinar cell carcinomas are composed of cells with architectural, cytological, and/or immunohistochemical evidence of acinar differentiation. Acinar architecture occurs when pyramidal-shaped cells surround small lumina (Fig.…”

Section: Clinical and Pathological Featuresmentioning

confidence: 99%

Pathology and genetics of pancreatic neoplasms with acinar differentiation

Wood

Klimstra

2014

Seminars in Diagnostic Pathology

View full text Add to dashboard Cite

Pancreatic neoplasms with acinar differentiation, including acinar cell carcinoma, pancreatoblastoma, and carcinomas with mixed differentiation, are distinctive pancreatic neoplasms with a poor prognosis. These neoplasms are clinically, pathologically, and genetically unique when compared to other more common pancreatic neoplasms. Most occur in adults, although pancreatoblastomas usually affect children under 10 years old. All of these neoplasms exhibit characteristic histologic features including a solid or acinar growth pattern, dense neoplastic cellularity, uniform nuclei with prominent nucleoli, and granular eosinophilic cytoplasm. Exocrine enzymes are detectable by immunohistochemistry and, for carcinomas with mixed differentiation, neuroendocrine or ductal lineage markers are also expressed. The genetic alterations of this family of neoplasms largely differ from conventional ductal adenocarcinomas, with only rare mutations in TP53, KRAS, and p16, but no single gene or neoplastic pathway is consistently altered in acinar neoplasms. Instead, there is striking genomic instability, and a subset of cases has mutations in the APC/β-catenin pathway, mutations in SMAD4, RAF gene family fusions, or microsatellite instability. Therapeutically targetable mutations are often present. This review summarizes the clinical and pathologic features of acinar neoplasms and reviews the current molecular data on these uncommon tumors.

show abstract

Acinar Cell Carcinoma With Prominent Intraductal Growth Pattern

Cited by 31 publications

References 7 publications

Pancreatic Ducts as an Important Route of Tumor Extension for Acinar Cell Carcinoma of the Pancreas

Pancreatic Ducts as an Important Route of Tumor Extension for Acinar Cell Carcinoma of the Pancreas

Intraductal Tubulopapillary Neoplasm of the Pancreas

Pathology and genetics of pancreatic neoplasms with acinar differentiation

Contact Info

Product

Resources

About