A 52-year-old woman with a history of NF1.
She presented with a 2-month history of intermittent fever, associated with a 5 kg weight loss. During the last week prior to admission, she developed jaundice and choluria.
She was hospitalized for study.
Physical examination revealed mild jaundice and multiple brown spots on the skin.
Examinations were performed to confirm a highly obstructive malignancy with total bilirubin 7 mg/dl. A magnetic resonance imaging of the abdomen and pelvis showed dilatation of the biliary tract of 25 mm and of the infected Wirsung duct of Wirsung.
Upper endoscopy confirmed the periampullary lesion. A biopsy of the lesion showed moderate enteritis with mild vascular ectasia with no elements of specificity.
A negative dissemination study was performed.
Although the biopsy was negative, surgery was decided due to the high clinical suspicion.
1.
The patient underwent laparotomy, a Kocher maneuver and palpation of the head of the pancreas were performed.
A 2-3 cm mass located in the periampullary region was found.
There was no peritoneal or hepatic dissemination.
In the stomach, multiple 2-10 mm nodular flat lesions were found in the anterior and posterior surfaces. A sample was sent for contemporary biopsy which was reported as a fusocellular tumor.
A pacreatoduodenectomy was performed without pyloric preservation and subsequent reconstruction without incidents.
In the postoperative evolution, the patient presented a low output pancreatic fistula that was managed conservatively by drainage and was discharged on postoperative day 14.
Biopsy reported that in the pemphigus-ribular region, in the duodenum, there was a well-differentiated neuroendocrine carcinoma combined with well-differentiated tubular adenocarcinoma affecting the submucosa and the distal muscle of the duodenum x 1 x 1 x 1 cm pancreatic tail.
Immunohistochemistry was positive for synaptophysin, chromogranin and CEA.
Biopsy of the nodules of the gastric serosa was compatible with a gastrointestinal stromal tumor of 1 x 1 x 0.5 cm, with positive reaction for CD 117 and negative for pro-tein S-100 and actin.
1.
The case was discussed in an outpatient committee and it was decided to observe and control GIST tumors in the stomach.
Currently the patient is in good condition without recurrence four months after surgery.
