Spontaneous rupture of recurrent gastrointestinal stromal tumor associated with neurofibromatosis type 1.

The incidence of gastrointestinal stromal tumor (GIST) among neurofibromatosis type 1 (NF-1) patients is approximately 3.9-25%, and this relationship is generally considered to be non-coincidental. We report a patient with NF-1 who underwent laparotomy 3 times due to recurrent intra-abdominal tumor rupture with internal bleeding in the space of 13 years. The pathologic diagnoses were schwannoma, malignant peripheral nerve sheath tumor and GIST. Because of the similar histologic features of these tumors, we considered them to be of the same nature. Immunohistochemical staining can help in the differential diagnosis. We suggest that NF-1 patients with gastrointestinal symptoms receive further survey to rule out GISTs.