A 53-year-old woman with a history of hip fractureectomy and neurinoma in the left cervical region in 1986.
She was admitted for asthenia and pruritus of recent onset, without abdominal pain or alterations in the physical examination.
Laboratory tests revealed FA 1499 IU/l, GGT 1163 IU/l, total bilirubin 1.62 mg/dl and CA-19.9 533 IU/ml (normal < 37 IU/ml).
Doppler ultrasound and abdominal CT showed the existence of a mass of 6 cm in diameter, located in the head of the pancreas, with dilatation of the intrahepatic and extrahepatic bile ducts.
The lesion presented cystic areas, with septa and solid nipples inside, some of which captured contrast intensely ; without data of vascular invasion or adenopathies.
With the diagnostic impression of cystic neoplasm of the pancreas without surgical and resectable contraindication, a duodenopancreatectomy was performed.
The surgical specimen showed a solid, whitish-colored and elastic consistency mass, located in the head of the pancreas, not encapsulated but with well-defined contours, easily separated from the pancreas.
The immunohistochemical study showed positivity for vimentin marker, and negativity for cytokeratins (AE1-AE3), S-100, chromogranin, CD 34, C-KIT and actin-cell malignancy with a very suggestive sarcoma grade.
After two years of follow-up the patient remains asymptomatic and without recurrence of the disease.
