Erdheim-Chester disease presenting with cough, abdominal pain, and headache

Crivelli, Paola; Ledda, Roberta Eufrasia; Carboni, Marcello; Balestrieri, Antonella; Sotgiu, Maria Alessandra; Saba, Luca; Conti, Maurizio

doi:10.1016/j.radcr.2020.03.008

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“…5,6 Gastrointestinal symptoms are uncommon in ECD and may include nausea, vomiting, abdominal pain, diarrhea, and hematochezia. [5][6][7][8] Liver and biliary involvement may manifest with hepatomegaly, biliary obstruction, and liver nodules, and elevated serum levels of hepatic transaminases have been reported. 9,10 Pancreatic involvement with hypodense nodular lesions has also been reported.…”

Section: Discussionmentioning

confidence: 99%

A Rare Cause of Abdominal Pain: Erdheim-Chester Disease

et al. 2023

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A 65-year-old man presented with hematuria, night sweats, nausea, intermittent nonbloody diarrhea, and abdominal pain. Computed tomography angiogram with enterography showed retroperitoneal fibrosis surrounding both kidneys and ureters without any evidence of vascular obstruction or hydronephrosis. Laparoscopic biopsy demonstrated fibroadipose tissue involved by a subtle histiocytic infiltrate in a background of marked fibrosis, scattered lymphocytes, and plasma cells. The histiocytes strongly expressed CD163, Factor XIIIa, and BRAF V600E. He was diagnosed with Erdheim-Chester disease, a rare histiocytic neoplasm uncommonly presenting with gastroenterological manifestations.

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