Tumors of the angle of Treitz are a rare entity. Only 3%-5% of gastrointestinal stromal tumors (GISTs) occur at the level of the duodenum, and their location at the duodenojejunal junction is very uncommon. Surgery is the treatment of choice, while adjuvant medical therapy is used on the basis of the degree of radicality of the excision and the tumor’s proliferative profile. These factors primarily influence the prognosis. Due to the frailty of the vascular viability of the left duodenum, which can be injured during surgery, it is generally recommended to perform digestive reconstruction at the level of the right portion of the duodenum. We here report the case of a patient with a large GIST located at the duodenojejunal junction behind the ligament of Treitz. We found reconstructive digestive anastomosis at the level of the third part of the duodenum to be a safe procedure.
Tumori 2016; 102(Suppl. 2): e71 - e73
Article Type: CASE REPORT
AuthorsFrancesco Caruso, Marco Nencioni, Arianna Zefelippo, Giorgio Rossi, Lucio Caccamo
- • Accepted on 17/06/2015
- • Available online on 22/07/2015
- • Published online on 11/11/2016
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Gastrointestinal stromal tumors (GISTs) are a rare entity with an incidence of around 1/100,000/year (1). They may occur in the entire length of the digestive tract. The stomach (40%-60%) and small intestine (30%-40%) are the most common locations, while duodenal sites are very rare (3%-5%) (2). The mainstay of treatment of GISTs is radical resection, while chemotherapy is reserved for cases of incomplete surgical excision and depending on the proliferative profile of the disease; the outcome is influenced primarily by these 2 factors (3).
We report the case of a patient with a large GIST located at the duodenojejunal junction behind the ligament of Treitz. Our report aims to show the safety of using the third part of the duodenum as the site for digestive reconstruction after radical resection.
A 73-year-old man with a history of chronic HCV-related liver disease (sustained viral response after antiviral therapy in 2005), hiatal hernia, diverticulosis of the colon, hypertension, glaucoma and multiple cysts of the liver presented to our center with anemia and melena. Computed tomography (CT) of the large intestine showed an 8-cm hypervascularized non-occlusive mass close to the distal portion of the duodenum/angle of Treitz (
Preoperative CT scan. White line: GIST of the angle of Treitz; black star: superior mesenteric artery; black dot: superior mesenteric vein; white square: pancreas; white arrow: duodenum.
A diagnostic laparoscopy was performed for tumor staging. It confirmed the presence of a large mass arising from the duodenojejunal junction behind the ligament of Treitz, without infiltration of the inferior surface of the pancreas and including the first part of the jejunum. A second, small, similar nodule was found on the first jejunal loop 10 cm distally from the main mass. Peritoneal and liver surface metastases were ruled out. Open access was required to minimize blood loss and avoid rupture of the tumor capsule, and also in view of the characteristics of the main tumor (size, location close to the superior mesenteric axis and pancreatic body, presence of arterial neovascularization). After an upper midline incision, the lesions were radically resected en bloc with the fourth part of the duodenum and the first jejunal loop (resected bowel was about 30 cm long), leaving a duodenal stump on the left side of the mesenteric vessels. The excision was safely carried out using an ultrasound dissector to minimize vascular damage as well as the need for dissection. Bowel continuity was re-established by means of a mechanic circular (EEA Auto suture 28 mm – Covidien Mansfield, MA, USA) end-to-side anastomosis between the third part of the duodenum and a jejunal loop, after careful evaluation of the vascularization of both intestinal stumps. The jejunal stump was closed with a linear stapler and a retro-anastomotic drain was placed. The surgery lasted 260 minutes with blood loss of 1,000 mL.
The postoperative course was uneventful, with oral nutrition being started on the fourth postoperative day. The patient was discharged on the eighth postoperative day. Histopathological examination confirmed a bifocal GIST (lesion 1: diameter 9.5 cm, 6 mitoses/50 HPF, Ki-67 10%, focal necrosis, CD117+, CD34±/–, S100–, actin–, desmin–; lesion 2: diameter 0.8 cm, 1 mitosis/50 HPF, Ki-67 2%, CD117+; 5 lymph nodes negative). Postoperative follow-up showed regular endoscopic patency at the level of the anastomosis. On the basis of the histopathological findings and the high malignant potential of the disease according to the Miettinen classification (4), the patient received adjuvant treatment with imatinib. Four months’ follow-up with CT scan showed no local or distance recurrence.
Tumors of the angle of Treitz are a rare entity. The symptoms are often unspecific (anemia, vague abdominal pain, dyspepsia), with bowel occlusion or upper gastrointestinal bleeding occurring as late symptoms (5). Duodenal tumors account for 3%-5% of all GISTs (3), and those localized at the duodenojejunal junction are very infrequent.
The diagnosis of these tumors by conventional endoscopy may be challenging as the duodenojejunal region is not easily reached. Contrast-enhanced abdominal CT clarifies the stage of the tumor and the anatomical relationships between the mass and the surrounding structures. In all cases it is fundamental to obtain a histological diagnosis preoperatively. However, performing an endoscopic biopsy in patients with small lesions may be difficult due to the possible submucosal location of GISTs.
In our patient operation resulted in en bloc tumor resection including the fourth part of the duodenum and the first jejunal loop, with a mechanic duodenojejunal end-to-side anastomosis ensuring digestive continuity. The duodenum was sectioned about 2 cm to the left of the superior mesenteric vessels. The jejunal loop was sectioned about 30 cm from the large mass and about 20 cm from the second nodule. The bowel resections were performed while preserving the vascularization of the intestinal stumps by minimizing their mobilization using an ultrasonic scalpel.
Some authors advocate digestive reconstruction at the level of the right portion of the duodenum to avoid the risk of surgical damage to its terminal portion (6). This recommendation is based on the knowledge that the third and fourth parts of the duodenum are vascularized by minor arteries deriving from the superior mesenteric artery, and these vessels can be injured during surgical exposure of the angle of Treitz. We believe this frail area can be safeguarded by limiting the surgical exposure with the help of the “no-touch” technique. The use of innovative technological tools allows to both reduce the aggressiveness of anatomical dissection and facilitate technical reconstruction. As shown by the present case, limited surgical mobilization, careful evaluation of the vascular supply to the bowel stumps, and use of mechanical staplers to design the new digestive tract all contributed to achieving a regular clinical course without the need for complex digestive reconstructions.
In conclusion, GIST of the angle of Treitz is a rare entity which is best treated by radical resection and adjuvant imatinib. Duodenojejunal anastomosis to the left of the superior mesenteric vessels is a safe and feasible option for digestive reconstruction.
The authors would like to thank Paolo Sabatino for his precious support.
- Caruso, Francesco [PubMed] [Google Scholar] , * Corresponding Author (firstname.lastname@example.org)
- Nencioni, Marco [PubMed] [Google Scholar]
- Zefelippo, Arianna [PubMed] [Google Scholar]
- Rossi, Giorgio [PubMed] [Google Scholar]
- Caccamo, Lucio [PubMed] [Google Scholar]
HBP Surgery and Liver Transplantation, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico Milano, Milan - Italy