Goblet Cell Carcinoid in a Patient with Neurofibromatosis Type 1 : A Rare Combination

Abstract EN

Neuroendocrine tumors are rare tumors primarily located in the gastrointestinal tract.

Goblet cell carcinoid is a rare subgroup of neuroendocrine tumors located in the appendix.

Neurofibromatosis type 1 is an autosomal dominant disorder caused by a mutation in the NF1 gene.

Patients with neurofibromatosis type 1 have an increased incidence of typical neuroendocrine tumors, but it is unknown if this is the case with goblet cell carcinoids.

We describe a patient with both neurofibromatosis type 1 and goblet cell carcinoid, that according to literature would occur in 0.00017 per million per year.

This may suggest a previously unknown association between neurofibromatosis type 1 and goblet cell carcinoids.