We report the case of a 59-year-old man with neurofibromatosis type 1 with a history of malignant schwannoma excision in the popliteal hollow, supracondylar ischial amputation of the lower limb three years later.
In a study of asthenia of 3 months duration due to severe iron deficiency anemia with no evidence of bleeding.
Gastroscopy: distal strictures of the duodenum show bleeding remnants coming from the small intestine.
Gastrointestinal transit: An image of the first portion of the gallstone, very close to the angle of Treitz.
TAC: A heterogeneous mass adjacent to the first loops of the institution shows communication with them, of 9.3 x 7 x 7.4 cm.
Surgery was performed, finding a very vascularized tumor a few centimeters from the Treitz angle, about 10 cm in diameter.
There are two other pedunculated lesions of 2-3 cm, one above and another about 30 cm distally.
Likewise, the entire small intestine shows multiple milimetric lesions with a "neurofibromatous" appearance.
The intraoperative study of the main tumor is reported as a neoplasm of probable mesenchymal origin.
Intestinal resection was performed, including the three main lesions, and end-to-end anastomosis.
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The initial postoperative period is normal, resuming intestinal transit and tolerating feeding.
On the seventh day, she suddenly presented syncope episode and respiratory distress, which did not respond to CPR and exitus.
The autopsy showed right pulmonary thromboembolism.
The pathology report reports multiple gastrointestinal stromal tumor (GIST).
The main measurement is 10.5 cm and is located at the mesenteric edge, below the muscularis propria, causing continuity solution in the mucosa.
It is positive for c-kit (CD117), CD34, vimentin, S-100 and focally for actin.
She was negative.
Due to its characteristics it is a high risk GIST malignancy/aggressive behavior.
The other two lesions have the same immunohistochemical behavior.
The most proximal one measures 1.8 cm and presents less than 5 mitosis/50 CGA (GIST of very low risk of malignant potential) and the distal one measures 2.5 cm and presents less than 5 mitoses/50 CGA (G).
Additionally, the serous surface of the cerebellum identifies several smaller lesions of 5 mm that are histologically and immunohistochemically identical to the previous ones (very low risk of malignant potential).
