Synchronous duodenal neuroendocrine neoplasm and congenital factor XIII deficiency: case report and review of the literature.

ABSTRACT

Background: Neuroendocrine neoplasms (NENs) are uncommon, with duodenal NENs (dNENs) being particularly rare in clinical practice. Congenital factor XIII deficiency (FXIIID) is also an extremely rare hematological disease in which poor wound healing may occur due to coagulopathy. The concurrent occurrence of these two rare diseases has not been reported before, which increases the difficulty of diagnosis and treatment. This is the first report of dNEN concomitant with Congenital FXIIID, which can present as a reference for clinicians who may encounter similar situations in the future. Case Description We report a 33-year-old woman with bleeding diathesis since childhood who complained of digestive tract bleeding for 7 years. She was finally diagnosed as duodenal neuroendocrine neoplasm combined with congenital factor XIII deficiency. The patient underwent surgery, and pathological findings confirmed neuroendocrine tumor. After surgery she received cryoprecipitate and fresh frozen plasma (FFP) therapy. No tumor recurrence has been observed nor recurrence of digestive tract bleeding during the 2-year follow-up. Conclusions: Our report suggests when gastrointestinal bleeding is difficult to explain, more general examinations in addition to gastroscopy should be performed. In situations where digestive tract bleeding cannot be fully explained by a single disease, the possibility of concomitant disease, such as hematological disorders, should be considered to avoid the missed diagnosis of rare co-morbidities.