Abstract
A 49-year-old woman consulted her general practitioner (GP) regarding epigastric pain
that she had experienced for 2 months. Physical examination and laboratory results
were unremarkable. An abdominal ultrasound indicated a solid pancreatic tumor, which
was confirmed on subsequent CT and MRI. Endoscopic ultrasound (EUS) showed a well-defined
heterogeneous, predominantly hypoechoic mass in the pancreatic body, so a neuroendocrine
tumor (NET) was suspected. However, EUS-guided fine-needle aspiration (EUS-FNA) was
performed and based on (immuno-)histochemical findings, the extremely rare diagnosis
of a perivascular epithelioid cell tumor (PEComa) of the pancreas was made. Due to
the malignant potential of pancreatic PEComas, laparoscopic left-sided pancreatectomy
was performed. We present a case diagnosed by preoperative EUS-FNA highlighting the
clinical and endosonographic features which help to distinguish it from its most important
differential diagnosis, neuroendocrine tumors (NETs) of the pancreas.