The epithelial-mesenchymal transition induces aggressivity of mucinous cystic neoplasm of the pancreas with neuroendocrine component: An immunohistochemistry study

Background

Pancreatic mucinous cystic neoplasms (MCN) are rare tumors that are usually diagnosed in females.

Materials and methods

In our department, only four of the 109 consecutive cases of pancreatic tumors (3.67%) were diagnosed as MCNs. In this report, we present the characteristics of these four specific cases which also showed unusual HER-2 positivity and neuroendocrine differentiation.

Results

The four MCNs were diagnosed in patients with ages between 46 and 75 years. Other clinical particularities were the following: one benign case, splenic rupture as result of a giant cystic tumor on the tail of the pancreas directly invading the spleen in the second one, metastases in the accessory spleen in the third one and invasion of the abdominal vessels in the fourth case. In all of these cases, the ovarian-like stroma tested positivity for calretinin, progesterone receptor (PR) and, in cases 2 and 3, for AE1/AE3 keratin. The malignant tumor cells were marked by carcinoembryonic antigen, HER-2, maspin, PR and the neuroendocrine markers synaptophysin, CD56, and neuron-specific enolase.

Conclusions

These cases highlight the unusually aggressive behavior of pancreatic MCN with invasive carcinomas that share mixed exo- and endocrine components and show epithelial-mesenchymal transition.