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Acinar Cell Carcinoma of the Pancreas: Overview of Clinicopathologic Features and Insights into the Molecular Pathology.

La Rosa S, Sessa F, Capella C - Front Med (Lausanne) (2015)

Bottom Line: Acinar cell carcinomas (ACCs) of the pancreas are rare pancreatic neoplasms accounting for about 1-2% of pancreatic tumors in adults and about 15% in pediatric subjects.They show different clinical symptoms at presentation, different morphological features, different outcomes, and different molecular alterations.This heterogeneous clinicopathological spectrum may give rise to difficulties in the clinical and pathological diagnosis with consequential therapeutic and prognostic implications.

View Article: PubMed Central - PubMed

Affiliation: Department of Pathology, Ospedale di Circolo , Varese , Italy.

ABSTRACT
Acinar cell carcinomas (ACCs) of the pancreas are rare pancreatic neoplasms accounting for about 1-2% of pancreatic tumors in adults and about 15% in pediatric subjects. They show different clinical symptoms at presentation, different morphological features, different outcomes, and different molecular alterations. This heterogeneous clinicopathological spectrum may give rise to difficulties in the clinical and pathological diagnosis with consequential therapeutic and prognostic implications. The molecular mechanisms involved in the onset and progression of ACCs are still not completely understood, although in recent years, several attempts have been made to clarify the molecular mechanisms involved in ACC biology. In this paper, we will review the main clinicopathological and molecular features of pancreatic ACCs of both adult and pediatric subjects to give the reader a comprehensive overview of this rare tumor type.

No MeSH data available.


Related in: MedlinePlus

Acinar cell carcinoma in a 1-year-old boy characterized by a trabecular growth (A) resembling that of a pancreatic neuroendocrine tumor. However, tumor cells are strongly immunoreactivity for trypsin (B).
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Figure 10: Acinar cell carcinoma in a 1-year-old boy characterized by a trabecular growth (A) resembling that of a pancreatic neuroendocrine tumor. However, tumor cells are strongly immunoreactivity for trypsin (B).

Mentions: Acinar cell carcinomas in children show the same histological features and immunohistochemical reactivity of those of adults (Figure 10). Among the 29 cases reported in the literature, 22 were pure ACCs, 4 MANECs, 1 acinar cell cystoadenocarcinoma, 1 mixed acinar-ductal carcinoma, and 1 mixed acinar-neuroendocrine-ductal adenocarcinoma. The cases of ACCs associated with Cushing’s syndrome were characterized by a surprisingly scant neuroendocrine component weakly immunoreactive for ACTH and general neuroendocrine markers including chromogranin A and synaptophysin (11, 68, 69).


Acinar Cell Carcinoma of the Pancreas: Overview of Clinicopathologic Features and Insights into the Molecular Pathology.

La Rosa S, Sessa F, Capella C - Front Med (Lausanne) (2015)

Acinar cell carcinoma in a 1-year-old boy characterized by a trabecular growth (A) resembling that of a pancreatic neuroendocrine tumor. However, tumor cells are strongly immunoreactivity for trypsin (B).
© Copyright Policy
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4469112&req=5

Figure 10: Acinar cell carcinoma in a 1-year-old boy characterized by a trabecular growth (A) resembling that of a pancreatic neuroendocrine tumor. However, tumor cells are strongly immunoreactivity for trypsin (B).
Mentions: Acinar cell carcinomas in children show the same histological features and immunohistochemical reactivity of those of adults (Figure 10). Among the 29 cases reported in the literature, 22 were pure ACCs, 4 MANECs, 1 acinar cell cystoadenocarcinoma, 1 mixed acinar-ductal carcinoma, and 1 mixed acinar-neuroendocrine-ductal adenocarcinoma. The cases of ACCs associated with Cushing’s syndrome were characterized by a surprisingly scant neuroendocrine component weakly immunoreactive for ACTH and general neuroendocrine markers including chromogranin A and synaptophysin (11, 68, 69).

Bottom Line: Acinar cell carcinomas (ACCs) of the pancreas are rare pancreatic neoplasms accounting for about 1-2% of pancreatic tumors in adults and about 15% in pediatric subjects.They show different clinical symptoms at presentation, different morphological features, different outcomes, and different molecular alterations.This heterogeneous clinicopathological spectrum may give rise to difficulties in the clinical and pathological diagnosis with consequential therapeutic and prognostic implications.

View Article: PubMed Central - PubMed

Affiliation: Department of Pathology, Ospedale di Circolo , Varese , Italy.

ABSTRACT
Acinar cell carcinomas (ACCs) of the pancreas are rare pancreatic neoplasms accounting for about 1-2% of pancreatic tumors in adults and about 15% in pediatric subjects. They show different clinical symptoms at presentation, different morphological features, different outcomes, and different molecular alterations. This heterogeneous clinicopathological spectrum may give rise to difficulties in the clinical and pathological diagnosis with consequential therapeutic and prognostic implications. The molecular mechanisms involved in the onset and progression of ACCs are still not completely understood, although in recent years, several attempts have been made to clarify the molecular mechanisms involved in ACC biology. In this paper, we will review the main clinicopathological and molecular features of pancreatic ACCs of both adult and pediatric subjects to give the reader a comprehensive overview of this rare tumor type.

No MeSH data available.


Related in: MedlinePlus