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Lymphangiomas of the gastrointestinal tract are extremely rare benign tumors. There have been 39 cases of solitary intestinal lymphangiomas reported, 8 of which were found in the duodenum; only 3 of these were seen radiographically. A case of cavernous lymphangioma of the duodenum is presented, the roentgenographic and pathologic findings are described, and the literature is reviewed.
As recently described, Ki-1 anaplastic large cell lymphoma is a distinctive subtype of non-Hodgkin's lymphoma. We report a Ki-1 anaplastic large cell lymphoma of the duodenum in a 62-year-old man. He received modified CHOP therapy and was in complete remission at 18 months. To our knowledge, this is the first report of primary Ki-1 anaplastic large cell lymphoma of the duodenum.
BACKGROUND: Because of the rarity of primary adenocarcinoma of the duodenum, accumulation of natural history data has been difficult. As a result, debate continues over important treatment issues. METHODS: We did a retrospective review of 67 patients with nonampullary adenocarcinoma of the duodenum treated at the University of Texas M.D. Anderson Cancer Center between 1967 and 1991. Presenting symptoms and signs, diagnostic studies, operation performed, surgical pathology, and survival were analyzed. RESULTS: A primary duodenal tumor was demonstrated by upper gastrointestinal radiographs (UGI) in 37 of 42 patients (88%), esophagogastroduodenoscopy (EGD) in 49 of 55 (89%), and computerized tomograms (CT) in 21 of 42 (50%). A curative resection was performed in 36 of the 59 patients who underwent operation (61%); 27 had pancreaticoduodenectomies and nine had wide local excisions. Overall 5-year survival was 29%. The 5-year survival difference between resected and unresected patients was 54% versus 0%, respectively (p < 0.0001). No survival difference was noted between patients who underwent pancreaticoduodenectomy rather than wide local excision. Lymph node metastases were significantly related to the occurrence of distant metastases (p = 0.0034). The 5-year survival for patients with stage I or II tumors was 100% and 52%, respectively, compared to 45% and 0% for stage III and IV (p < 0.0001). CONCLUSIONS: Our data suggest that UGI and EGD are effective for diagnosing duodenal carcinoma. Survival is improved by curative resection and is not compromised by a wide local excision instead of a pancreaticoduodenectomy for lesions of the third and fourth portion. We recommend that adjuvant chemotherapy be considered for stage III disease, because distant failure is the predominant pattern of failure in this group.
A case of primary non-Hodgkin's T-cell lymphoma of the duodenum is presented. A 41-year-old man was hospitalized in 1984 complaining of abdominal distention and vomiting. Hypotonic duodenography showed an encircling filling defect in the second portion of the duodenum, and a biopsy specimen revealed features of malignancy suggestive of either undifferentiated carcinoma or malignant lymphoma. Radical surgery (pancreaticoduodenectomy) was performed, after which chemotherapy was administered. A histological evaluation of the duodenal tumor showed it to be non-Hodgkin's lymphoma. It was a diffuse, large-cell type, which immunohistochemically suggested it to be of T-cell origin. Currently the patient is doing well, with no evidence of disease recurrence 13 years after surgery.
A duodenal duplication cyst in a 45-year-old male is reported. A hypotonic duodenography showed a smooth spherical defect in the descending portion of the duodenum. A fiberoptic duodenoscopy disclosed a smooth well-defined tumor which was located orally from the ampulla of Vater. A retrograde pancreatocholangiography indicated the tumor was not in communication with the pancreatic duct or biliary tract. At operation, a cystic spherical mass, 3 cm in diameter, was located in the posterior wall of the duodenum corresponding to the above described diagnosis. The combination of a greater awareness of this condition as well as improved X-ray and endoscopic techniques has made preoperative diagnosis more accurate.
A 52-year-old man was admitted on February 15, 1990, with hiccups and vomiting. He had been well until 13 days before admission when he stumbled and fell when intoxicated, striking his abdomen. A diagnosis of intramural hematoma was made with computerized tomography and sonography of the abdomen after admission, revealing a mass that was intimately related to the duodenum. Treatment of the intramural duodenal hematoma is controversial. However, this case illustrates the ideal situation where conservative management could be applied with total parenteral nutrition, percutaneous aspiration drainage, and endoscopic balloon catheter dilatation of the narrowed lumen of the duodenum. The patient's subsequent course supports the concept of planned conservative management.
BACKGROUND: Adenoma of the ampulla of Vater and the duodenum are rare tumors. The leading symptoms are unspecific; sometimes they are due to the disturbances of the passage or erosion of the tumor. Early and correct diagnosis and the appropriate therapy are of decisive importance because these tumors are precancerous. PATIENTS AND METHODS: We report the cases of seven patients with periampullary adenoma of the duodenum, in one case with incorporation of the the distal bile duct. The diagnostic steps and the surgical treatment are reported and discussed. In achieving a definitive diagnosis, endoscopic retrograde cholangiopancreatography is of paramount importance. Three patients subsequently underwent partial pancreatoduodenectomy (Whipple's procedure); in three patients transduodenal local tumor excision was performed. Postoperative course was without complications in each case and no operative mortality occurred. One patient was treated by argon-coagulation. RESULTS: The histopathological examination showed a tubular villous adenoma in six cases and in one case a villous adenoma (ranging from low to high grade dysplasia). Because there is evidence of an adenoma-carcinoma-sequence, radical surgical treatment is recommended, even in adenoma with low grade dysplasia, in spite of the absence of malignancy.
Duodenum-preserving resection of the head of the pancreas was developed 25 years ago by Beger. This procedure is indicated in patients suffering from chronic pain in combination with inflammation of the head of the pancreas, common bile duct obstruction, pancreatic duct obstruction and/or obstruction of the retropancreatic vessels. At the Inselspital in Berne, 74 patients underwent this operation between 1993 and 1996. The median length of the operation was 380 min, with the need for transfusion in a median of 0 units (0-6). There was no postoperative mortality. Total postoperative morbidity was 13%. One patient needed relaparotomy on day 17 for small bowel obstruction. Median length of hospital stay was 11 days. Postoperatively, two patients developed diabetes. Duodenum-preserving resection of the head of the pancreas represents an organ-preserving principle of surgery. This procedure treats the complications of chronic pancreatitis and provides long-term pain relief in more than 80% of patients.
We report a case of abdominal injury secondary to child abuse in which the child had both a duodenal hematoma and contained perforations of the duodenum and proximal jejunum. These injuries were evaluated by both CT scan and upper gastrointestinal (GI) series. The child's nausea and vomiting persisted despite conservative treatment; after 3 weeks a repeat upper GI series demonstrated high-grade duodenal obstruction. An exploratory laparotomy was performed and a calcified, fibrotic mesentery and strictures in the distal duodenum and proximal jejunum were found. To our knowledge, this unusual complication of blunt abdominal trauma has not been described in association with child abuse.
Carcinoids of the stomach, duodenum, and pancreas are represented by a variety of tumors with variable histologic and clinical features. Multicentric gastric carcinoids and concomitant nonantral argyrophilic hyperplasia are common in chronic atrophic gastritis, more rarely due to a multiple endocrine neoplasia (MEN)-related Zollinger-Ellison syndrome (ZES). These tumors are infrequently associated with metastases and may generally be dealt with by repeated endoscopic fulguration. Sporadic carcinoids tend to be larger, invasive, and more often metastatic, especially in the presence of atypical histology. Small tumors may be removed by endoscopy, but larger lesions need to be surgically excised. In association with metastases a histamine-related atypical carcinoid syndrome may evolve and require treatment with a somatostatin analog. Poorly differentiated neuroendocrine carcinomas of the stomach constitute markedly aggressive tumors that rarely are suitable for radical surgery. Gastrinomas are the most prevalent duodenal carcinoids and a common cause of ZES especially in MEN-I. Despite a marked tendency for regional lymph node dissemination, liver metastases occur late and duodenal gastrinomas are often excisable, thereby offering favorable odds for cure in ZES. Unusual somatostatin-rich carcinoids in the ampulla of Vater relate to von Recklinghausen's disease and may be the cause of obstructive jaundice; depending on their size, these tumors may be removed by local excision or pancreaticoduodenectomy. Gangliocytic paragangliomas are unusual, generally benign lesions of the duodenum. Rare pancreatic tumors with serotonin immunoreactivity may be classified as carcinoids and constitute an unusual cause of the carcinoid syndrome.
We experienced a rare case of a lymphomatous polyp of mantle cell type forming a polypoid mass lesion in the duodenum bulbous together with advanced gastric cancer. A total gastrectomy was performed, and the specimen revealed atypical small- to medium-sized lymphoid cells with indented nuclei, which infiltrated the Peyer's patch and formed a nodular mass in the lamina propria and submucosa of the duodenum. The lymphoma cells also infiltrated the lymphoid follicle of the gastric mucosa, spleen, and regional lymph node with a typical mantle zone pattern. Flow cytometric analysis of the single cells of the lymph node and immunohistochemistry of a paraffin-embedded specimen revealed that the lymphoma cells expressed surface CD5, CD19, CD20, and nuclear cyclin D1. Chromosomal analysis of this single cell suspension revealed that these lymphoma cells have trisomy 3 in conjunction with t(11;14)(q13;q32), which is frequently seen in mucosa-associated lymphoid tissue lymphomas (MALToma) in the stomach and is also reported in mantle cell lymphoma as a secondary genetic alteration. Our report suggests that trisomy 3 may be a common chromosomal abnormality in lymphomatous polyps of mantle cell type.
We report the case of a 53-year-old woman with a gastrointestinal stromal tumor (GIST) of the duodenum that showed only extramural growth, mimicking a pancreatic tumor. Preoperatively, computed tomography (CT) and angiography revealed a hypervascular mass, 3.0 cm in diameter, in the pancreatic head. Hypotonic duodenography showed compression of the second and third portions of the duodenum by the pancreatic lesion. Endoscopic examination showed no specific mucosal abnormalities in the duodenal lumen. The pancreatic head tumor was diagnosed preoperatively as a nonfunctioning islet cell tumor of the pancreas, and the patient underwent pylorus-preserving pancreaticoduodenectomy. A hard mass was palpated intraoperatively in the pancreatic head region, and neither peritoneal dissemination nor metastasis was detected. Histologically, the tumor was composed of spindle-shaped cells with a fascicular growth pattern, and only a few mitotic features were seen. Immunohistochemically, most of the tumor cells were positive for c-kit oncoprotein and CD34, but negative for alpha-smooth muscle actin and S-100 protein. Therefore, this neoplasm was finally diagnosed as a duodenal GIST of the uncommitted type. This is a rare case of a duodenal GIST with exclusively extramural growth mimicking a pancreatic head tumor.
A 57 year-old man presented with abdominal discomfort and melena. Abdominal ultrasonography clearly revealed a duodenal tumor as a hypoechoic mass in the transverse segment of the duodenum. The lesion was a 4 x 4-cm oval mass with partial concavity, an irregular surface, high-level central echoes, and a hypoechoic periphery. After confirmation of the diagnosis of duodenal carcinoma, a pylorus-preserving pancreatoduodenectomy was performed curatively. Pathological examination revealed moderately differentiated tubular adenocarcinoma partially invading the pancreatic parenchyma. Ultrasonography can be used to detect lesions of the transverse segment of the duodenum as the first imaging procedure.
Primary carcinoma of the duodenum is a rare lesion. In conjunction with the widespread use of panendoscopy, reported cases of carcinoma of the duodenum have recently increased. Although benign hyperplasia of Brunner's gland is well documented, duodenal carcinoma originating in Brunner's gland is extremely rare, and, consequently, there is little data on the morphological or histochemical characteristics. We report here a case of early duodenal carcinoma arising from Brunner's gland, whose origin was proven by mucin immunohistochemistry.
We report a case of a duodenal gastrointestinal stromal tumor (GIST) necessitating urgent surgery because of a gas figure on computed tomography (CT). A 46-year-old woman, complaining chiefly of upper abdominal pain and tarry stools, consulted a local doctor. A gastrointestinal fiberscopy revealed an ulcer in the second part of the duodenum, and the patient was admitted to our hospital where a dynamic CT scan showed a hypervascular solid tumor in the pancreatic head. A repeat CT scan done 4 days later showed a gas figure in the tumor, necessitating an emergency pylorus-preserving pancreatoduodenectomy (PpPD). First, we performed a tube pancreatostomy for complete external drainage of the pancreatic juice, and planned a second-stage pancreatojejunostomy for the near future. Histopathologically, the tumor was diagnosed as a GIST originating in the duodenum. The patient was discharged on postoperative day 23 after an uneventful postoperative recovery. Her local doctor completed the second-stage pancreatojejunostomy.
A case of multiple carcinoid tumors of the duodenum accompanied by familial multiple endocrine neoplasia is reported. A 46-year-old man with duodenal polyps discovered during a mass screening was followed up for 5 years. In August 1994, a histological examination revealed carcinoid tumors, and he was thus referred to our hospital for surgery. He underwent a parathyroidectomy and cholecystectomy for primary hyperparathyroidism and cholecystolithiasis, respectively. The patient's sister had also undergone a parathyroidectomy and distal pancreatectomy for primary hyperparathyroidism and insulinoma of the pancreas. In addition, his two children were also followed up for hypercalcemia. A serum examination of the patient revealed high levels of somatostatin and pancreatic polypeptide, but normal levels of gastrin and serotonin. In November 1994, a pancreaticoduodenectomy with a D2 lymph node dissection was performed. The macroscopic findings of the resected specimen showed multiple polypoid lesions with delles on the top, measuring 3 to 15 mm in size throughout the duodenum. A microscopic examination revealed the tumor to have infiltrated into the submucosa extensively, and an immunohistochemical analysis also demonstrated the tumor cells to be positive for somatostatin, but not for pancreatic polypeptide. After surgery, the serum level of somatostatin returned to normal but the pancreatic polypeptide remained high. The post-operative course was uneventful, and the patient remains in good health.
The records of 104 patients with primary adenocarcinoma of the duodenum were reviewed. All patients were followed up to the date of this report, 91 for at least 5 years. Melena was an ominous preoperative finding. Survival was directly related to nodal status, the grade of the lesion, and the ability of the surgeon to minimize or eliminate operative mortality. Fifty percent of the lesions were resectable for cure, and the 5 year survival rate for patients with resectable lesions was 46 percent. All eight patients treated with segmental resections for lesions in the third and fourth portions of the duodenum survived at least 5 years. These data are a great improvement over those in previous reports and suggest that this disease may be much more treatable than previously believed.
The experience with 25 patients who required operation for Crohn's disease involving the duodenum is reviewed. Two distinct patterns of duodenal involvement are apparent. Intrinsic duodenal Crohn's disease has a characteristic clinical presentation that is distinct from the symptoms seen in patients with involvement of other portions of the gastrointestinal tract. Among 70 patients with duodenal Crohn's disease seen over a 30 year period, 22 required surgical intervention at the Lahey Clinic. Although hemorrhage and intractable pain were associated problems in several of these patients, unrelenting duodenal obstruction remained the primary indication for operation. Of patients who underwent operative bypass, 78 percent had a good result with a median follow-up period of 12.3 years. The presence of associated gastric Crohn's disease did not influence long-term results. A third of the patients required reoperation for duodenal disease. Marginal ulceration and recurrent gastroduodenal obstruction have been the primary reasons for reoperation. Although the addition of vagotomy to operative bypass has not helped to protect against subsequent marginal ulceration, the absence of appreciable morbidity associated with vagotomy in our series and the high incidence of marginal ulcers reported with gastroenterostomy in other clinical settings lead us to recommend gastroenterostomy with vagotomy as the procedure of choice for duodenal Crohn's disease. Proceeding with vagotomy in persons who have had previous ileocecal or extensive small bowel resection should not be undertaken without careful consideration. Similar caution should also be used in patients who are already troubled with poorly controlled diarrhea. The duodenum may also be involved by duodenoenteric fistulas which represent a complication of Crohn's disease involving other portions of the gastrointestinal tract. Most frequently this occurs in patients with Crohn's colitis who have no evidence of intrinsic duodenal disease. Management of the internal fistula requires resection of the involved colon and closure of the duodenal defect. Three patients with duodenocolic fistula have been so treated.