Patient: Male, 76 Final Diagnosis: Aorto-enteric fistula Symptoms: Abdominal pain ? bloody vomiting ? shock Medication: Clinical Process: CT abdomen with contrast Specialty: Gastroenterology ? vascular surgery Objective: Rare disease Background: Main Aorto-Enteric Fistula (PAEF) is usually a unique and rare life threatening communication between the Aorta and Gastrointestinal Tract with an unusually high mortality rate and delayed diagnosis. Aneurysm is usually implicated in vast majority of cases and malignancy constitutes only 1% of all causes of main aorto-enteric fistulae. The most common cancers associated with PAEF are esophageal and pancreatic malignancies but case reports of metastatic testicular and recurrent cervical cancers have also been reported. We present the first case statement of main aorto-enteric fistula secondary to Large B-Cell Lymphoma, followed by a review of literature in regards to malignant aorto-enteric fistulae. Case Statement A 76 12 order GSK2126458 months old Hispanic Male with a past medical history of thoraco-abdominal aneurysm and B-Cell Lymphoma presented with abdominal pain and hematemesis. Two-and-a-half years ago he was diagnosed with a thoraco-abdominal aneurysm with maximum dilation of 4.7 cm and which ran from 4 cm past the left subclavian artery to just below the level of the SMA. The aneurysm was under active surveillance but did not meet criteria for surgical repair. Three months prior to admission, the patient experienced 25-pound excess weight loss, associated with anorexia, weakness and pallor. A recent routine surveillance Abdominal CT followed by MRI (Physique 1) exhibited a 9.28.07.9 cm retroperitoneal mass encasing the aorta and intimately associated with the 3rd and 4th portions of the duodenum as well as to parts of the jejunum and ileum. Open in a separate window Physique 1 Abdominal CT Angiography showing abdominal aortic aneurysm and large retroperitoneal mass encasing abdominal aorta with close proximity to duodenum (star) and ileum (star). CT-guided biopsy of the mass revealed an invasive, poorly differentiated, EBV positive, diffuse large B-cell lymphoma (Physique 2). Labs were notable for any hemoglobin level of 8.3 and hemoccult-positive stool. A gastroenterological evaluation found no massive gastrointestinal bleeding and occult bleeding was attributed to intraluminal lymphoma. The patient deferred surgery SUGT1L1 but agreed to chemotherapy to try and control the disease locally. Open in a separate window Physique 2 H&E and CD20 Antibody staining showing poorly differentiated lymphoid cells that are strongly CD20 positive. Five days after receiving his first cycle of CHOP chemotherapy regimen the patient was admitted with a7/10 abdominal pain radiating to the back with multiple episodes of bloody emesis which lasted approximately 4 hours. Initial blood pressure was 81/45 with a pulse of 125. After two order GSK2126458 hours of resuscitation with IV saline, his blood pressure decreased to 64/29 while his abdominal pain increased to 10/10, and he developed shortness of breath, increased hematemesis, and profuse, dark red hematochezia. His hemoglobin was 7.5, platelet count 125,000 and INR 1.3. A CT aortogram (Physique order GSK2126458 3) showed an Aorto-Enteric Fistula at the duodenum, with extravasation of contrast mixed with bowel gas. A femoral collection was placed for order GSK2126458 aggressive resuscitation with fluids and blood transfusions. The patient was intubated, and an NG tube consistently suctioned 500 cc per-hour of blood. Levophed and vasopressin drips were initiated, but regrettably with limited effect. Vascular and General Surgery were consulted, as well as Gastroenterology. The patient was considered a poor surgical candidate in light of his moribund hemodynamic instability. After a conversation of his grave prognosis was held with the patients family, aggressive steps were withdrawn. The patient expired approximately 3 hours later. Open in a separate window Physique 3 CT Angiography showing bowel gas and adjacent extravasations of contrast material with in the mass pathognomonic of a main aortoenteric fistula. Conversation Main Aorto-Enteric Fistula (PAEF) C a life-threatening, exsanguinating illness C was first reported by Sir Astley Cooper in 1829 [1,2]. He observed a lethal communication between the aorta and gastrointestinal tract causing massive upper gastrointestinal bleeding. The reported incidence is only 0.04C0.007%, however the true incidence of a PAEF is probably underreported [2,3]. More than 85% of PAEF are due to Aortic Abdominal Aneurysms [3] while malignancy associated with main aorto-enteric fistula is usually rare [1C3]. During the 1990s, A. Gad explained four cases of squamous esophageal malignancy and two cases of pancreatic malignancy associated with PAEF in the first autopsy case series [4]. Unusual malignant etiologies of AEF such as recurrent cervical malignancy, colorectal malignancy and testicular germ cell tumor were also reported on a.