We report the case of a 41 year-old Caucasian male with FAP and metastatic colonic cancer who was referred to our institution. The patient was not under screening although he had a family history of FAP. He presented 2 years ago with epigastric pain and symptoms of obstructive ileus. Abdominal Computerized Tomography (CT) revealed stenosis at the transition of the descending to sigmoid colon, multifocal metastatic disease of the liver and a mesenteric nodal mass (1.9 cm). (Fig. 1) An exploratory laparotomy was performed, in which diffuse peritoneal carcinomatosis was revealed in combination with an obstructing neoplastic mass in the aforementioned by the CT segment of descending colon. A diverting loop ileostomy was created and biopsies were obtained from the liver and the greater omentum.
Colonoscopy after surgery revealed multiple polyps of the rectum, sigmoid and distal descending colon; a total colonoscopy was not achieved due to the neoplastic stenosis of the descending colon. Upper gastrointestinal tract endoscopy showed multiple cystic polyps at the gastric fundus and body and multiple duodenal adenomas at the ampulla of Vater (Fig. 2). Histopathological examination of the surgical specimens revealed moderately differentiated adenocarcinoma. Immunohistochemistry tests were positive for the markers CDX2 and CK20, whereas the CK7 marker was negative. KRAS/BRAF screening of pathological specimens found wild-type KRAS, wild-type NRAS,was negative for BRAF mutations and the tumour was microsatellite stable (MSS).
Chemotherapy (ChT) was given for a period of 15 months, consisting of Oxaliplatin and Bevacizumab regimens. Subsequent CT and Magnetic Resonance Imaging (MRI) revealed an impressive response of metastatic liver disease; only a single metastatic lesion in liver segment VI had remained, which had been downsized from 3.5 cm before ChT to 1.8 cm after ChT. Moreover, multiple small focal lesions were shown in liver segments III and VI, possibly corresponding to “cured” metastases. (Fig. 3). Positron emission tomography–computed tomography (PET- CT) revealed only one hypermetabolic area (SUVmax = 4.2) in liver segment VI. Interestingly, PET-CT showed absence of hypermetabolic neoplastic activity elsewhere in the abdominal cavity, including the already known site of the primary tumor in the descending colon. (Fig. 3).
The hospital’s multidisciplinary tumor board concluded that a second exploratory laparotomy in combination with possible restorative proctocolectomy, liver metastasectomy, peritonectomy and HIPEC was decided as the best option for the patient.
Abdominal cavity exploration revealed seven liver metastases(segment II and right lobe), metastatic lesions to the right hemidiaphragm and three suspicious lesions in the mesentery. Peritoneal carcinomatosis Index (PCI) was 7.
He then underwent total proctocolectomy with J-Pouch formation with diverting ileostomy, liver metastasectomy, part of right hemidiaphragm resection and HIPEC.
The small bowel mesentery was mobilized adequately and J-Pouch was constructed from the terminal 40 cm of small intestine. A side to side anastomosis was performed with multiple firings of a GIA 75 linear stapler via enterotomy at the pouch apex.
The blind loop of the J-pouch was closed with a linear stapler and a second reinforcing overstich line. Insufflation with normal saline confirmed the integrity of the pouch. The IPAA anastomosis was performed with a circular stapler. Care was taken for prevention of twisting of small bowel mesentery. The doughnuts were checked and the air-leak test was negative for leak of the anastomosis. Moreover, a diverting ileostomy was performed.
Completeness of cytoreduction (CCR) score was 0. Open coliseum technique was utilized for HIPEC, suspending the edges of the abdominal wall with a running suture over a self-retaining retractor. HIPEC with mitomycin C was administered for 90 min, at a temperature of 42 °C simultaneously with intravenous administration of 5-Fluoruracil and leucovorin. After HIPEC, washout of the abdomen was performed.
The postoperative course was complicated with persistent fever and resolved after intra-port removal. This prolonged his hospital stay to 15 days.
He then underwent ChT for six months with bevacizumab and capecitabine regimens. Six months after surgery and before closure of the diverting ileostomy, the pouchogram was performed and it was normal (Fig. 4). The patient is free of metastasis (CT, MRI), his pouch is uncomplicated as seen by colonoscopy and is going to reverse his ileostomy.