TITLE:
Synchronic Duodenal Gastrointestinal Stromal Tumor and Neuroendocrine Tumor in Association with Neurofibromatosis Type 1: A Case Report
AUTHORS:
Priscila Doria, Claudio Almeida Quadros, Marcos Lyra, Luciano Espinheira
KEYWORDS:
Gastrointestinal Stromal Tumor, GIST, Neuroendocrine Tumor, Neurofibromatosis, NF1, Von Recklinghausen’s Disease
JOURNAL NAME:
Journal of Cancer Therapy,
Vol.9 No.8,
August
9,
2018
ABSTRACT: Introduction: The coexistence of synchronic duodenal gastrointestinal stromal tumor
(GIST) and neuroendocrine tumor in a patient with neurofibromatosis type 1
(NF1) is extremely rare, and only eight cases were described in the literature. Clinical Case: This is a rare case of a 38-year-old female patient with
NF1 who developed synchronic GIST and neuroendocrine tumor, which were both in
the second portion of the duodenum. The first symptoms were abrupt digestive
bleeding and anemia. Upper digestive endoscopy revealed two tumors, sizes 2.5
and 3.0 cm, in the second portion of duodenum, with biopsies identifying a GIST
and a neuroendocrine tumor. Therapeutic decision was to proceed to surgical
resection, and Whipple’s procedure was indicated. Surgical procedure was performed
with good outcome. Currently the patient has excellent quality of life and
maintains follow up for thirty months without recurrence. Discussion: Long-term disease-free survival and excellent quality of life are reported when these tumors are fully resected in this
context. However, it is not always easy to access the gastrointestinal tract,
especially the small intestine, and proceed to the histopathologic diagnosis of
these tumors. Conclusion: It is important to be aware of the possibility
of the coexistence of various tumors in the NF1 scenario for adequate
screening, staging, and surgical treatment of these patients, as good prognosis
can be achieved when such tumors are detected and treated properly.