Segmentally Distributed Neurofibromatosis Associated with Adenocarcinoma of the Colon

Abstract

Neurofibromatosis can be associated with various malignancies, but an association with adenocarcinoma is extremely rare. A 61-year-old man who had been diagnosed with adenocarcinoma of the hepatic flexure of the colon was referred for segmentally located, multiple café-au-lait spots and tumors on his back and axillary freckles for 40 years. Histopathologic examination of the tumor was consistent with the neurofibromatosis. We report an unusual case of type I neurofibromatosis associated with adenocarcinoma of the colon that was clinically similar to segmental neurofibromatosis.

Colonic Vascular Anomalies and Colon Cancer in Neurofibromatosis: Report of a Case

“…This is the fourth report in the medical literature describing adenocarcinoma of the colon in the presence of neurofibromatosis. [2][3][4] There also have been seven case reports in the literature that indicate an association between neurofibromatosis and small-bowel adenocarcinoma. 5 It has been suggested that there is an association with adenocarcinoma of the stomach, 6 oesophagus, 7 and pancreas.…”

“…There are, however, only three case reports that indicate an association with adenocarcinoma of the colon. [2][3][4] This case report provides further evidence of a link between neurofibromatosis and adenocarcinoma of the colon.…”

Synchronous multiple colonic adenocarcinomas arising in patient with neurofibromatosis type 1

“…We reviewed clinical characteristics based on published case series (Table 1). There were nine patients including ours [2,3,4,5,6,7,8]. Eight were male [2,3,4,6,7,8].…”

“…In a large population-based cohort, 42 cases of colorectal cancer were observed among 8,003 people with NF type 1 and 2 [1]. Upon review of the literature, only eight cases, two of which were described in Korea [2,3], have been reported till now [4,5,6,7,8]. Hereby, we present a case of synchronous multiple colon adenocarcinomas in a patient with NF1 and provide characteristic clinical features based on the reported case series.…”

A Case of Segmental Neurofibromatosis

“…In the literature, segmental neurofibromatosis associated with skeletal abnormatilies, soft tissue hypertrophy, visceral neurofibromas, unilateral renal agenesis, renal angiomyolipoma and malignancies such as Hodgkin's lymphoma, colon carcinoma, gastric carcinoma, lung carcinoma, and peripheral nerve sheath tumour have been reported. 3,7,8,[12][13][14][15] The prevalence of segmental neurofibromatosis has been estimated to range between 0,0014% to 0,002%. We think that most of the cases are underdiagnosed because of the absence of symptoms.…”