- (2009) Volume 10, Issue 2
Vikas Gupta1, Virendra Singh2, Naveen Kalra3, Kim Vaiphei4
Departments of 1General Surgery, 2Hepatology, 3Radiodiagnosis, 4Histopathology, Postgraduate Institute of Medical Education and Research. Chandigarh, India
Received: 24 May 2004 Accepted: 8 June 2004
Context Benign proliferative changes of the Brunner's glands account for about 10% of neoplasias of the duodenal bulb. Case report The authors present a case of giant hamartoma of Brunner’s glands. The patient presented clinically with vague epigastric discomfort. A pancreas-sparing duodenal resection was performed. The patient made an uneventful recovery and is well at a one year follow-up. The diagnosis was established on histopathology. Conclusion Duodenal hamartomas are rare and may become very large, mandating surgical removal. A pancreas-sparing duodenal resection is one of the modalities of treating such lesions.
Brunner Glands; Duodenal Neoplasms; Hamartoma; Hyperplasia; Pancreas; Polyps
Brunner’s glands are mucus secreting acinar glands situated in the deep mucosa and submucosa of the duodenum which empty into the crypts of Lieberkühn [1]. Brunner’s gland hyperplasia in the duodenum is a rare, benign, hamartomatous, proliferative disorder of the duodenum and accounts for about 10% of neoplasias of the duodenal bulb [1, 2]. It is characterized by the proliferation of the acinar glands [1, 2, 3]. Owing to its rarity, these lesions can be mistaken for malignancy on radiological and endoscopic examination s [2, 4, 5, 6]. Small masses can present with bleeding, intussusceptions and are rarely asymptomatic [2, 7, 8]. Large masses may cause obstructive and compressive symptoms which may depend upon the location of the tumour [4, 5, 9]. These lesions sometimes become enormous and are called giant brunneromas [4, 5, 9]. A variety of endoscopic and surgical approaches have been described to tackle these lesions based on their size and location [4, 5, 8, 9]. The present report describes successful surgical management of a large lesion by an organ-sparing procedure.
Clinical Presentation
A 28-year-old woman presented with pain in the upper abdomen and occasional vomiting of 3 months duration. General physical, systemic and abdominal examinations were normal. Her biochemical, hematological and coagulation parameters were normal. She underwent an esophagogastroduodenoscopy, which revealed an extramucosal mass lesion in the gastric antrum, and the first and second parts of the duodenum, displacing the lumen eccentrically. Duodenal biopsy showed non specific inflammation.
Imaging
A contrast-enhanced computerized tomogram of the abdomen revealed an exophytic mass arising from the medial wall of the first and second parts of the duodenum displacing the pancreas medially. However, the fat plane between the mass and the pancreas was preserved (Figure 1).
Figure 1. Axial CECT section shows a large heterogeneous mass lesion in relation to the antropyloric region of the stomach and the C loop of the duodenum. Fat plane with the head of the pancreas is preserved.
Initial exploration was performed by a bilateral subcostal incision. A generous Kocher’s maneuver was performed to facilitate the examination. There was a 8x10 cm exophytic mass arising from the antrum of the stomach and medial wall of the duodenum (1st and 2nd parts) extending beyond the papilla (Figure 2). The tumour could easily be separated from the pancreas. Therefore, the decision to perform a pancreas-sparing resection was taken. The gastric antrum was divided as is done in a classical pancreaticoduodenectomy. The proximal jejunum was transected about 15 cm distal to the ligament of Treitz. The jejunum and the duodenum were separated from the uncinate process as is done in a standard pancreaticoduodenectomy. Cholecystectomy was performed in a retrograde fashion and a catheter was introduced through the cystic duct stump to identify the papilla. The dissection was continued in the interface between the pancreas and the duodenum by ligating and dividing all the vessels. Upon reaching the major papilla, the duodenum was made taut and the papilla was divided. During the course of the dissection, the minor papilla was ligated.
Figure 2. Operative image showing a large tumour mass occupying the distal antrum, and the first and second parts of the duodenum.
Sphincteroplasty and septotomy were performed to achieve a wide anastomosis with the jejunum as has previously been described by Sarmiento et al. [10]. The end of the jejunum was stapled. Along the antimesenteric border of the jejunum, the major papilla was anastomosed in a mucosa to mucosa fashion using a fine absorbable suture. The knots of the posterior layer were placed inside while those of the anterior layer were placed outside. The seromuscular layer of the jejunum was sutured to the pancreatic substance with a non-absorbable suture. A 6 Fr external stent was placed in the pancreatic duct and an 8 Fr external stent was placed in the common bile duct. Enteric anastomosis was performed 15 cm downstream from the papillary anastomosis.
She made an uneventful recovery and is well at a one year follow-up.
The gross specimen revealed a large polypoidal mass occupying most of the resected specimen (Figure 3). Histopathology of the specimen from the polypoidal lesion showed collections of Brunner’s glands in the submucosa. The Brunner’s glands showed a lobular pattern of arrangement and each lobule appeared to have a separate draining duct. Strands of fibrous tissue and smooth muscles were also seen between the lobules (Figure 4).
Figure 3. Gross specimen large and small multiple intraluminal polypoidal lobulated masses occupying the duodenum.
Figure 4. a. Low power photomicrograph to show stretched-out duodenal mucosa overlying the mass lesion (H&E, x60). b. High power photomicrograph to show lobular collection of normal looking mucous glands with a draining intralobular duct (H&E, x240).
Benign tumors of the duodenum are very rare. In a large series of more than 215,000 autopsies, only 0.008% was benign duodenal tumours and those arising from Brunner’s glands accounted for 11% of these benign lesions [3]. The term Brunner’s gland hyperplasia has been used interchangeably with Brunner’s gland hamartoma, Brunner’s gland adenoma and brunneroma. The latter term is a misnomer as the lesion does not contain any neoplastic element but it is, in fact, a hamartoma [1, 2, 3, 4, 5, 11, 12]. The exact incidence of the condition is difficult to determine because of the heterogeneity of nomenclature. In an analysis of 2,800 duodenal specimens, Feyrter [13] classified Brunner’s gland hyperplasia into three types based on the gross morphological features. However, the various types could represent the different stages of evolution of the same lesion.
The exact pathogenesis of these lesions is not well known but is thought to be more often associated with Helicobacter pylori infections and chronic pancreatitis [11, 14]. The present case did not have any of these underlying risk factors.
The most common location of these lesions is the posterior wall of the first and second parts of the duodenum. In a study of 27 patients, Levine et al. [15] found 70% of the lesions located in the duodenal bulb and 26% to be located in the second part of the duodenum. However, the occurrence of these lesions as far as the distal ileum has also been described [16]. The present case is peculiar as to the extent of involvement which started from the gastric antrum and extended into the duodenum beyond the papilla.
Despite their rare occurrence, these lesions tend to be clinically significant as 45% of them bleed and 51% cause enteric obstruction [17]. However, with the advent of endoscopy and barium examination of the gastrointestinal tract, asymptomatic small lesions are detected more often. Occasionally, these lesions are a rare cause of gastric outlet [18], duodenal [19] or small bowel obstruction [16, 20] or upper gastrointestinal hemorrhage [21], and require excision. Concomitant biliary and pancreatic obstruction and consequent pancreatitis caused by these lesions has been described [12, 22]. The occurrence of surgical jaundice due to the mass lesion produced by the tumour in the periampullary location has been described [4]. For the most part, these lesions are benign but the coexistence of malignancy has also been described. In an analysis of more than seven hundred cases with Brunner’s gland hyperplasia, Sakuri et al. [2] reported coexisting dysplasia, and invasive carcinoma in only 2% cases.
All symptomatic lesions should be treated. Small lesions are amenable to endoscopic removal. Various strategies such as snare polypectomy, submucosal resections and hemoclip application have been described depending on the individual situation [9, 11, 17, 21, 23, 24]. Large lesions require removal as it is difficult to rule out malignancy in the majority of cases. Moreover, proven cases of malignancy developing in Brunner’s hyperplasia have also been described [2]. Coriat et al. [9] reported the endoscopic removal of a large lesion. Others have also reported endoscopic polypectomy removal of a large lesion. Obha et al. [25] described a modified endoscopic submucosal dissection technique to remove a large Brunner’s gland hyperplasia.
Laparoscopic removal of small and large lesions has been described [8, 26]. Iusco et al. [5] resorted to radical resection in a large Brunner’s gland hamartoma where it was difficult to rule out malignancy. Hol et al. [4] performed a pancreaticoduodenectomy for a periampullary giant lesion with a suspicion of coexisting malignancy. The present report is the first of its kind where a pancreas-sparing resection has been performed for a large brunneroma. Owing to the voluminous size of the lesion and uncertainty in the diagnosis, resectional treatment was mandated.
Since the first description in 1984, pancreas-sparing duodenal resection has been widely used for various benign and malignant conditions of the duodenum [26, 27]. These include familial adenomatous polyposis, trauma, lymphoma, leiomyosarcoma, corrosive injury and duodenal bleeding [10, 27, 28, 29, 30, 31, 32]. The proposed potential advantages are the restoration of near normal continuity of the gastrointestinal tract, and organ preservation leads to a better long-term outcome. In a series of 21 pancreas-sparing resections, Mackey et al. [28] found it to be a safe method for treating patients with Spigelman’s stage IV duodenal polyposis. Sarmiento et al. [10] described a good absorptive capacity, weight gain and quality of life in patients with duodenal polyposis undergoing a pancreas-sparing resection. Suzuki et al. [29] performed pancreas sparing resection of the third and fourth portions of the duodenum in a patient with a large leiomyosarcoma. Absence of the contiguous involvement of the pancreas and lymph nodal metastasis were the factors dictating this type of resection [10, 27, 28, 29].
Once the resection is performed, the reconstruction can be tailored according to the anatomy and involvement of the pancreaticobiliary complex. The choice lies between transplanting the duodenal button including the papilla into the jejunum or excising the major papilla and forming a neo-pancreaticobiliary junction [10, 27, 28, 29, 30, 31, 32]. The former technique is simple to perform but carries the risk of leaving behind the diseased duodenal mucosa. The latter is more technically demanding [10, 27, 28, 29, 30, 31, 32]. In the present case, a neopancreaticobiliary junction was created which was anastomosed to the jejunum.
In conclusion, a duodenal hamartoma is a rare disorder in which the hamartoma may become very large, mandating surgical removal. A pancreas-sparing duodenal resection is one of the modalities of treating such lesions.
The authors have no potential conflicts of interest
