Imaging Assessment of Periaortic Inflammation in Erdheim-Chester Disease

Area of Science:

• Vascular Medicine
• Histiocytosis
• Diagnostic Pathology

Background:

• Retroperitoneal fibrosis diagnosis can be challenging.
• Aortic aneurysms and fibrosis are known complications.
• Erdheim-Chester disease is a rare non-Langerhans histiocytosis.

Purpose of the Study:

• To report a case of Erdheim-Chester disease presenting as retroperitoneal and thoracic aortic disease.
• To illustrate the diagnostic utility of reassessing existing histopathological samples.
• To highlight a less invasive diagnostic pathway for rare aortic conditions.

Main Methods:

• Case report of a 75-year-old male.
• Review of medical history including ruptured abdominal aortic aneurysm and retroperitoneal fibrosis.
• Multimodality imaging assessment of thoracic aortic soft tissue infiltration.
• Histological reassessment of prior abdominal periaortic tissue samples.

Main Results:

• A patient with a history of aortic aneurysm and retroperitoneal fibrosis developed thoracic aortic infiltration.
• Multimodality imaging suggested a neoplastic or inflammatory process.
• Histological reassessment of abdominal samples confirmed Erdheim-Chester disease.
• This avoided the need for an open-chest biopsy.

Conclusions:

• Erdheim-Chester disease should be considered in patients with unexplained retroperitoneal and aortic fibrosis.
• Revisiting initial histopathology can be crucial for diagnosing rare conditions.
• A multidisciplinary approach combining imaging and pathology is essential for complex cases.