We present the case of a 62 year old male with no relevant medical history, who for the last two months had been complaining of dysphagia for solids and liquids, marked physical fatigue, oliguria. Clinical examination revealed palor and dehydrated skin, as well as a tumoral mass of 10/10 cm palpable in the upper abdomen. Upper digestive endoscopy was performed, showing a vegetant lesion of 6 cm on the gastric angle, with a central ulcer of 4 cm. The oesophagus and duodenum were normal, and Helicobacter pylori test was negative. Histological exam from the gastric lesion revealed diffuse large-cell malignant lymphoid tumoral proliferation, with tumoral cells infiltrating the mucosa between the glands. Immunohistochemical analysis diagnosed B cell lymphoma. CT scan of the thorax, abdomen and pelvis was performed, revealing multiple tumors involving the gastric walls, the posterior parietal peritoneum, the intra-peritoneal fat, the pancreatic tissues and the right antero-inferior mediastinum, as well as multiple adenopathies on both sides of the diaphragm and ascites. We performed paracentesis, and malignant cells were detected in the peritoneal fluid. Bone marrow biopsy was normal. The patient was referred to the hematology department for treatment. He received chemotherapy including CHOP followed by RICE with the disappearance of the gastric tumor on upper endoscopy and the disappearance of peritoneal, mediastinal and pancreatic region tumors with the persistence of a circumferential thickening of the horizontal gastric region walls on CT reevaluation.
Hereditary hemorrhagic telangiectasia (HHT) has significant morbidity due to multiorgan involvement and an unpredictable disease course. We analyzed the data of 14 patients diagnosed with HHT. The case series comprised 14 patients with a median age at presentation of 48 years old (41–74 years). In twelve patients (85.7%), the diagnosis was confirmed by using the Curacao Criteria. The most common reason for admission was epistaxis, with 9 patients (57%) presenting with nosebleed refractory to prolonged self-tamponade. The biochemical abnormalities identified were elevations in AP and gamma-GT; liver synthetic function was generally normal, even though 21% of patients had clinical or imaging findings for cirrhosis. Nosebleeds were the main reason for admission and significantly impacted quality of life through anemia and frequent hospital admissions. However, the visceral manifestations seemed to be more serious. The hepatic arteriovenous malformations (AVMs) appeared to remain asymptomatic or led to minimal changes for the majority of patients; some cases were associated with liver and biliary tract ischemia, necrosis leading to acute liver failure and even death. Hepatic AVMs can also lead to high-output heart failure due to arterio-venous shunting. The most frequent AVM was hepatic artery to hepatic vein, with secondary hepatic vein dilation and hemodynamic consequences.
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