Amyloid Tumors of the Gastrointestinal Tract: A Report of Two Cases and Review of the Literature

Amyloid tumors of the gastrointestinal tract are exceedingly rare. Two cases are presented, one in the stomach of a patient who appeared to have systemic amyloidosis and one in the colon which appears to be isolated. The treatment was surgical excision. Extensive diagnostic work-up is required to evaluate the many various possible causes of amyloidosis.     

A case of arteriovenous malformation in the submucosal layer of the stomach

Arteriovenous malformation (AVM) of the stomach is extremely rare. We report a patient with asymptomatic gastric AVM detected during mass screening of the upper gastrointestinal tract. The patient, a 69-year-old female, had no history of gastro-intestinal bleeding. Endoscopy revealed a gastric submucosal tumor (3 cm in diameter) at the posterior wall below the esophago-cardiac junction. Endoscopic ultrasonography (EUS) showed a hypoechoic structure in the third layer of the stomach, suggesting gastric AVM in the submucosal layer. Complete resection of the AVM tissue was performed by strip biopsy with surgical resection. Histological examination showed AVM in the submucosal layer. EUS examination was useful for the diagnosis of gastric AVM in this case.     

Dieulafoy's lesion of the right hemicolon

Dieulafoy's lesion was described in 1896 by the French pathologist Georges D. Dieulafoy as a vascular malformation in the stomach. Although usually found in the stomach, the lesion may occur anywhere within the gastrointestinal tract and can cause severe hemorrhage. There is no sex or age predilection. The diagnosis is established endoscopically, and the current therapy of choice is endoscopic hemoclipping. Only rarely is the diagnosis confirmed histologically. We report the case of an elderly female patient who, while hospitalised for a slipped intervertebral disc, presented with lower gastrointestinal bleeding. The source of the bleeding was suspected to be in the right colon by endoscopy. Renewed massive bleeding necessitated surgical treatment with resection of the right hemicolon. The histological work-up of the resected specimen identified a 350-micron large tortuous submucosal artery that had eroded.     

A case of intrapleural rupture of pulmonary arteriovenous malformation with shock

We report a case of a 59-year-old male who suddenly developed massive right hemothorax and shock due to pulmonary arteriovenous malformation (AVM). He was admitted as an emergency case because of severe back pain, cyanosis and cold sweat. Although shock with massive right hemothorax was found, echocardiographic and X-ray computed tomographic examination showed no dilatation of the aorta, no intimal flap in the aorta and no pericardial effusion which suggested aneurysm. Pulmonary arteriography, performed subsequently, disclosed intrapleural rupture of the right pulmonary AVM. The patient recovered successfully from the state of shock. The pulmonary AVM was removed by segmentectomy of the right lung (S4). He was discharged following an uneventful postoperative course. Intrapleural rupture with shock is a very rare complication of pulmonary AVM. Pulmonary AVM should be considered as one possible cause in patients with massive hemothorax and shock.     

Pre- and intraoperative localization of small bowel arteriovenous malformation

Patients with chronic gastrointestinal bleeding with no source found after standard radiographic and endoscopic procedures are diagnostic challenges. Since angiodysplasia is a frequent cause of such bleeding, selective angiography has become an essential diagnostic tool in identifying arteriovenous malformations (AVM) of the large and small bowel. In addition to preoperative identification, some method of intraoperative localization is essential to assure removal of the involved segment. In a patient with a 7-year history of gastrointestinal bleeding from an AVM of the small bowel, a technique of preoperative angiographic catheter placement with intraoperative confirmation of catheter position proved a useful way to find such small bowel lesions and insured adequate but not excessive resection.     

Management of pancreatic arteriovenous malformation

Pancreatic arteriovenous malformations (AVM), while extremely rare, are frequently complicated by gastrointestinal bleeding. The elimination of pancreatic AVM is difficult once portal hypertension has developed. We describe herein a patient with congenital AVM of the pancreatic head presenting with recurrent episodes of melena, in whom pylorus-preserving pancreatoduodenectomy provided a means of definitive management. We also review the literature and focus on the diagnostic and therapeutic approaches. Angiography is always necessary to facilitate tactics of treatment, even if diagnosis has been established by non-invasive imaging modalities. To obtain complete regression, total extirpation of the affected organ, or at least the involved portion, should be performed before this disease leads to the lethal complications of gastrointestinal bleeding and portal hypertension. Transcatheter arterial embolization is the only alternative treatment for the control of hemorrhage.     

Massive gastrointestinal hemorrhage in a case of amyloidosis secondary to rheumatoid arthritis.

A case of a 60-year-old woman with secondary gastrointestinal amyloidosis to rheumatoid arthritis is reported. Biopsy findings in the mucosa of the stomach and lower gastrointestinal tract revealed amyloidosis. Endoscopic examination of the lower gastrointestinal tract revealed multiple nodular elevations. The patient showed massive melena. Emergency angiography was performed and an extravasation was found at branches of the jejunal artery. Embolization was performed and this lead to a good prognosis. Patients with massive hemorrhages following gastrointestinal amyloidosis generally have a poorer prognosis. Embolotherapy performed for the present case might represent an effective therapeutic method for gastrointestinal hemorrhage in gastrointestinal amyloidosis.     

Pancreatic pseudocyst with hemorrhage into the gastrointestinal tract through the duct of Santorini

Hemorrhage into a pancreatic pseudocyst frequently goes unrecognized. This catastrophic event can be heralded by intermittent bleeding, or may present as massive gastrointestinal hemorrhage. A high index of suspicion, proper diagnostic workup, and prompt surgical management afford the patient the best chance for survival. We report a patient with massive pseudocyst bleeding into the gastrointestinal tract via the duct of Santorini and discuss the current diagnostic and therapeutic approach.     

A massive left-to-right shunt due to a ruptured giant aneurysm of the sinus of Valsalva

We describe a patient with a giant ruptured sinus of Valsalva aneurysm causing a massive left-to-right shunt. The diagnosis was made by transoesophageal echocardiography and confirmed by angiography. We outline briefly a diagnostic and therapeutic work-up.     

Acute pancreatitis due to pancreatic arteriovenous malformation: 2 case reports and review of the literature

Pancreatic arteriovenous malformation (AVM) is a relatively rare disease. Based on our literature search, 51 cases of pancreatic AVM have been reported since 1968. The gastrointestinal bleeding is the most common presenting symptom (24/51 cases [47%]). There were only 6 cases of pancreatitis in these cases. We describe 2 cases of acute pancretitis with pancreatic AVM. The patients who were diagnosed with acute pancreatitis were admitted to our hospital. Pancreatitis was considered to be caused by pancreatic AVM by some modalities of diagnostic imaging. The respective pancreatic AVM lesions of patients were resected to prevent the recurrence of pancreatitis. They are asymptomatic after the surgery. Pancreatic AVM is thought to be the one of the reasons for acute pancreatitis.     

Capsule endoscopy in patients with chronic abdominal pain

BACKGROUND: Patients with chronic abdominal pain consult gastroenterologists frequently, requiring a large number of examinations. AIM: To assess the diagnostic yield of capsule endoscopy in patients with chronic abdominal pain of unknown origin and negative diagnostic work-up. PATIENTS: From January 2002 to September 2004, 16 patients (10 female; mean age 42.7 years) who complained of chronic abdominal pain were referred to our unit for capsule endoscopy. METHODS: Chronic abdominal pain was defined as continuous or almost continuous, for at least 3 months and without criteria for other gastrointestinal disorders. All patients had a previous diagnostic work-up including abdominal ultrasonography, oesophagogastroduodenoscopy, colonoscopy and small bowel follow through, performed within 2 months. RESULTS: Capsule endoscopy was normal in 12 patients (75%). Small bowel abnormalities were found in three patients, but were considered irrelevant. In one patient (6.3%), capsule endoscopy revealed ileal erosions and inflammation and was retained in a stricture undetected by radiology. This patient underwent elective surgery which revealed an ileal carcinoid neoplasm. CONCLUSIONS: Capsule endoscopy identified a specific cause of chronic abdominal pain in only one patient. Capsule endoscopy is a safe procedure but does not seem to play an important role in the evaluation of patients with chronic abdominal pain of unknown origin.     

Gangliocytic paraganglioma--a rare differential diagnosis of a submucosal duodenal tumor

We report on the case of a 36-year-old male patient who was found to have a submucosal duodenal tumour during the diagnostic work-up of gastrointestinal bleeding. After exclusion of other tumour manifestations complete endoscopic resection was performed. Histologically a gangliocytic paraganglioma was diagnosed, a very rare type of a duodenal neuroendocrine tumour. This case report discusses the epidemiology, diagnostic work-up and therapeutic options for this rare tumour type.     

Chronic Lymphocytic Leukemia Presenting with Massive Chylous Ascites

A newly diagnosed patient with chronic lymphocytic leukemia who presented with ascites is described. Paracentesis revealed the presence of massive chylous ascites. Further work-up disclosed leukemic infiltration of the liver and bone marrow. Complications leading to his death included renal failure, gastrointestinal hemorrhage, bacterial peritonitis, and Staphylococcus aureus bacteremia. Chylous ascites is a rarely reported manifestation of chronic lymphocytic leukemia. The case is presented, and a review of the literature is discussed.     

Massive Gastrointestinal Hemorrhage in an Immunosuppressed Man due to Gastric Kaposi''s Sarcoma

Kaposi's sarcoma, a neoplasm that occurs in immunocompromised patients and people of Mediterranean origin, usually presents with skin lesions. However, extracutaneous lesions are common, and the gastrointestinal tract is often involved. We report the case of an iatrogenically immunosuppressed patient who presented with a massive upper gastrointestinal hemorrhage that was caused by Kaposi's sarcoma of the stomach. Kaposi's sarcoma in the setting of immunosuppression and the gastrointestinal manifestations of this disease are reviewed.     

The viewpoint of an internist on the problem of an aortoenteric fistula

Aortoenteric fistula is an uncommon life-threatening disease; it can be divided into primary and secondary one. Primary aortoenteric fistula is the result of ongoing disease in the aorta and the intestine, secondary one is iatrogenic. Typical symptoms are abdominal pain and gastrointestinal bleeding (two-stage process). The most appropriate diagnostic method is CT aortography, treatment is only surgical. 75-year-old patient was admitted to the 5th Department of Internal Medicine in Bratislava due to progression of renal parameters, the patient had undergone an aortofemoral bypass 4 years ago. During the fourth day of his hospitalization the patient had a massive hematochezia with a shock state. After transient stabilization of the patient, 30 minutes after the first hematochezia, a massive hematemesis appeared and then the patient died. An autopsy has confirmed the secondary aortic-enteral fistula between the duodenum and the aneurysm arising from the aortofemoral bypass.     

Colonic bleeding in the elderly

In the elderly population bleeding from the colon is not unusual. In evaluating a patient with colonic bleeding, the rate of bleeding should be assessed, and the possibility of upper gastrointestinal and anorectal bleeding should be excluded. The most common cause of massive bleeding from the colon in the elderly patient is diverticulosis, and the next most common cause is angiodysplasia. A number of other less common causes of bleeding should be excluded when making the diagnosis. If angiography is employed and the bleeding site is identified, initial therapy can be started concurrently by either injecting a vasopressor drug intraarterially or by embolization. Failure to stop the bleeding leads to surgery as the next step. If the bleeding stops spontaneously, one can expect recurrence later; therefore an interval work-up is stressed to identify the anatomic area of suspected bleeding. Preoperative identification of the bleeding site is imperative so that a "blind resection" can be avoided.     

Venous thromboembolism as first manifestation of cancer.

Numerous studies, performed in recent years, have provided the convincing demonstration that the incidence of newly diagnosed malignancy is increased among patients with unexplained venous thromboembolism during the first 6 to 12 months after the thromboembolic event. Among malignancies presenting with venous thromboembolism as a first clinical manifestation, prostate and colorectal cancer are the most commonly identified. Other common sites are cancer of the lung, pancreas, stomach, ovary, brain, and bladder. Extensive screening with computer tomography scanning, gastrointestinal endoscopy, and a number of tumor markers have the potential to detect occult malignancies. However, whether an extensive diagnostic work-up in all patients presenting with unexplained venous thromboembolism is cost-effective still remains to be demonstrated.     

Malignant fibrous histiocytoma of the colon associated with diverticulitis

We report the second case of a malignant fibrous histiocytoma of the large bowel. The neoplasm constituted an incidental finding during the diagnostic work-up for diverticulitis of the sigmoid colon. There was a massive abdominal recurrence of the tumor four months after colectomy, and the patient died nine months after the initial operation. The literature on fibroblastic sarcomas of the colon is also reviewed.     

Gastric Perforation after Endoscopic Treatment of a Dieulafoy''s Lesion

Dieulafoy's vascular malformation is an underdiagnosed cause of massive, often recurrent upper gastrointestinal hemorrhage. Attempted endoscopic treatment of Dieulafoy's lesion has been recommended prior to surgery in many instances, but may occasionally be employed as primary therapy in patients that are not considered good "operative risks." Although generally considered safe and effective therapy for nonvariceal hemorrhage, combination therapy by injection and thermocoagulation techniques may result in perforation. We present a patient with a Dieulafoy's lesion of the stomach that illustrates both the efficacy and risks of combination endoscopic therapy for nonvariceal gastrointestinal hemorrhage.