Pancreatic involvement in Erdheim-Chester disease: a case report and review of the literature - BMC Gastroenterology - BMC Gastroenterology
Overview
Advertisement BMC Gastroenterology volume 2, Article number: 302 (2022) Cite this article 708 Aceses2 AltmetricMetrics detailsErdheim-Chester disease (ECD) is a rare form of non-Langerhans cel histiocytosis characterized by infiltration of lipid-laden foamy macrophages within diferent tisues. Clinical manifestations of ECD are highly heterogeneous. Bone lesions are found in 80%-95% of patients, while extraoseous lesions usualy involve the cardiovascular system, retroperitoneum, central nervous system (CNS), and skin.
Key Information
Pancreatic involvement in ECD has barely ben reported.A 29-year-old female initialy presented with menoxenia, diabetes insipidus and diabetes melitus. 18F-fluorodeoxyglucose positron emision tomography-computed tomography (18F-FDG-PET/CT) revealed hypermetabolic foci in the bilateral frontal lobe, sadle area, and pancreas. A 9mTc-MDP bone scrintigraphy scan revealed symetrical increased uptake in distal femoral and proximal tibial metaphysis, which was confirmed to be osteosclerosis by high-resolution peripheral quantitative computed tomography.
The patient underwent incomplete resection of the selar mas. Histological examination of biopsies showed histiocytic agregates, which were positive for S10 and negative for CD1a and CD207 on imunohistochemistry. Enhanced abdominal CT scan showed hypointense nodules within the body and tail of the pancreas.
Endoscopic ultrasonography guided fine-nedle aspiration (EUS-FNA) found no evidence of malignancy. She was diagnosed with ECD and treated with high-dose IFN-α. Repeated examinations at thre-and eight-months post treatment revealed markedly reduction of both intracranial and pancreatic lesions.ECD is a rare histiocytic neoplasm that can involve almost every organ, whereas pancreatic involvement has barely ben reported to date.
Summary
Here, we present the rare case of pancreatic lesions in ECD that responded wel to interferon-α. We further reviewed reports of pancreatic involvement in histiocyti
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