Pancreaticoduodenectomy for locally advanced colon cancer in hereditary nonpolyposis colorectal cancer

Abstract

Background: Hereditary nonpolyposis colorectal cancer (HNPCC), or Lynch syndrome, accounts for 3% of newly diagnosed cases of colorectal cancer. While a partial or subtotal colectomy is indicated for early stage disease, there is a paucity of data addressing locally advanced disease involving the foregut.

Case presentation: We report two patients with hereditary nonpolyposis colorectal cancer presenting with locally advanced colon cancer surgically managed by pancreaticoduodenectomy with en bloc partial colectomy and a review of the literature.

Conclusions: Locally advanced colorectal cancer in HNPCC is a rare clinical entity that requires special surgical consideration. Multidisciplinary treatment, including multi-visceral resection, offers the best long-term outcome.

Fig. 1

Computed tomography with intravenous and oral contrast from case 2. Coronal section of the abdomen and pelvis. Arrow points to 14.0 × 9.8 × 10.8 cm heterogeneous mass in the right upper quadrant abutting the ascending colon and duodenum. Oral contrast is seen within the mass. The fat plane between the mass and the second segment of the duodenum is obscured

Fig. 2

Endoscopy images, gross pathology, and histopathology from case 2. a Colonoscopy revealed an ulcerated, partially obstructing, large mass in the transverse colon (marked by asterisk). The pediatric colonoscope could not completely traverse this large mass due to tumor obstruction and alteration in the normal colon anatomy. b Upper endoscopy showed a large malignant ulcerated mass (marked by asterisk) with no bleeding in the second part of the duodenum. c Part of the surgical specimen including colon (marked by single arrow), duodenum (marked by double arrow), with the mass (marked by asterisk) with a diffusely mucinous appearance. d Hematoxylin and eosin stain of primary colonic adenocarcinoma (viewed at ×40) with large pools of mucin evident. The tumor was 14 cm, low grade mucinous adenocarcinoma of the colon with loss of mismatch repair proteins and high microsatellite instability. There was mild to moderate intratumoral lymphocytes, mild to moderate peritumor lymphocytes, with low grade tumor budding, and no lymphovascular invasion. Margins were negative and 22 lymph nodes were negative (not shown)