[2] It is usually solitary and found in the body or tail of the pancreas, and may be associated with von Hippel–Lindau syndrome.

There are some exceptions; rare case reports have described isolated malignant serous cystadenocarcinomas.

This latter classification scheme is useful because it highlights the range of appearances and the clinical associations of these neoplasms.

[citation needed] These lesions rarely require surgery unless they are symptomatic or the diagnosis is in question.

[6] In selected cases the surgery can be performed using minimally invasive techniques such as laparoscopy.