A 38-year-old woman with a history of NF1 manifested in childhood with abundant epheloids throughout the body, axillary and inguinal region and mild neurofibromas.
The patient presented at the Emergency Unit of the Hospital de La Serena with a clinical picture of 7 days of evolution with intense abdominal pain, initially epigastric and subsequently diffuse.
He was admitted in septic shock and poor general condition.
After stabilization, we decided to perform a surgical intervention for acute abdomen of unknown origin and secondary origin.
No radiological study was performed because our hospital does not have a shift radiologist or an emergency ultrasound or tomography.
The abdominal approach was performed through a midline laparotomy.
A cyst tumor of 15 cm in diameter was aspirated from the purulent exudate and in the exploration it was evidenced that it originated in the A- portion of the duodenum, perforated, adhered to the colon.
The tumor was resected en bloc with the omentum and the A- portion of the duodenum.
Intestinal transit was reconstructed by termino-terminal anastomosis between the 3rd duodenum and the common bile duct.
The postoperative course was torpid, with complications: infection of the superficial surgical site, prolonged intestinal ileus and nosocomial pneumonia.
After 37 days of hospitalization, the patient was discharged in good condition.
The histological study showed a solid-cystic, partially necrotic tumor with low mitotic index (1 to 2 mitosis per 50 fields of increased).
Epithelioid and fusiform cells were observed, positive for CD117, CD34 and desmin.
The immunohistochemical reaction was negative for S100, vimentin and smooth muscle tone.
The size of the tumor and the clinical presentation (necrosis and perforation) are indications for imatinib therapy and was referred to the Regional Oncological Committee to initiate appropriate treatment.
The patient is asymptomatic and has been under follow-up for 11 months.
