Synchronous multiple adenocarcinomas of the colon in a patient with von Recklinghausen disease: a case report

Abstract

SUMMARY
The type-I neurofibromatosis (von Recklinghausen disease)
(NF-1), is a dominantly inherited syndrome with varying
disease manifestations with the consistent feature that
tissues derived from the neural crest is most commonly
affected. In addition to the nearly uniform development of
neurofibromas, NF1 patients are at elevated risk of developing
pheochromocytomas, schwannomas, neurofibrosarcomas, and
primary brain tumours.
Although gastrointestinal tumours have been well described
in patients with von Recklinghausen disease, and numerous
case studies have been reported, colonic cancer is rarely
associated with the disease.
We report a case of multiple synchronous colonic tumours,
in a patient who fulfilled two of the seven criteria proposed
by the NIH (National Institute of Health) for the diagnosis
of NF1.
Key words: neurofibromatosis-1, von Recklinghausen
disease, multiple adenocarcinoma of colon, phacomatosis