Abstract

Purpose: Amyloidosis is commonly systemic, occasionally organ-limited, and rarely a solitary localized mass. The latter, commonly referred to Tumoral Amyloidosis, is described occurring in every organ/tissue. Only a few reports of gastric amyloidosis exist today. Methods: A 72 year-old black male from Barbados presented with 3 days of diffuse abdominal pain. He described the pain severe, non-radiating, and worse with seating upright. A 60 lb weight loss over one year as well as loss of appetite for several months was noted. The patient reported early satiety for 8 months. He denied dysphagia, odynophagia, nausea, or vomiting. There was no change in bowel habits or character of stool. Colonoscopy and EGD six years ago were unremarkable. His medical history included Non-Hodgkin's Lymphoma diagnosed five years ago, status-post six rounds of CHOP chemotherapy, and currently was in remission. He gave no familial history of gastrointestinal disorders or malignancy. He had been a life-long smoker and consumed a moderate amount of alcohol daily. On examination, the patient was thin with mild pallor but no scleral icterus or jaundice. Abdominal exam revealed generalized tenderness with guarding, but no abdominal masses were felt. Complete blood count and iron panel revealed anemia of chronic disease. Electrolyte and hepatic panels were unremarkable. Results: On CT scan of the abdomen, thickening and calcification of the gastric wall was noted along with pneumatosis. A 1 cm pedunculated mass in the proximal duodenum was noted. No focal hepatic masses or biliary ductal dilatations seen. On EGD, a large circumferential friable mass was seen from the GE junction to the body. The mass was ulcerated, nodular, edematous, and bled easily on contact. A large non-bleeding 3 cm polyp was also seen in post bulbar area of duodenum. Biopsies were stained with Congo red and gave green birefringence under polarized light, consistent with tumoral amyloidosis. PET scan revealed diffuse gastric mucosa uptake compatible with gastric malignancy without metastatic foci. After medically controlling his abdominal pain, the patient was discharged with plan of action being observation. Conclusion: Tumoral amyloidosis exists as a rare disease entity in today's literature. There are no reports to support that this presentation represented a relapse of his prior disease process. Treatment for gastric amyloidomas has presently been one of observation or, at most, resection of the amyloid mass. It is not known if our patient required the same approach or if this warranted the re-institution of chemotherapy for NHL? Until more reports of tumoral amyloidosis are made known, treatment as well as prognosis remain uncertain.

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