Cancer Res Treat.  2012 Jun;44(2):146-150.

A Case of Erdheim-Chester Disease with Asymptomatic Renal Involvement

• Lee HJ
• Lee KY
• Shin DY
• Lee YG
• Choi SY
• Moon KC
• Han IK
• Kim TM

Abstract

Erdheim-Chester disease is a rare non-Langerhans-cell histiocytosis involving bones and multiple organs. Its clinical course can vary, from an asymptomatic state to a fatal disease, with renal involvement being a common cause of death. A 41-year-old man presented with a 10-month history of bilateral lower limb pain. Left perirenal soft-tissue infiltration had been found incidentally two years earlier. No progression of the lesion or deterioration of renal function was observed for a period of two years. At admission, plain radiography and magnetic resonance imaging of the patient's lower limbs showed patchy osteosclerosis. Biopsy of the tibia revealed histiocytic infiltration, which was found to be positive for CD68 and negative for CD1a. This report describes an unusual case of Erdheim-Chester disease involving a stationary course of disease with no specific treatment for a long period of time.

Keyword

Non-Langerhans-cell histiocytosis; Osteosclerosis; Retroperitoneal fibrosis

MeSH Terms

Adult
Asymptomatic Diseases
Biopsy
Cause of Death
Erdheim-Chester Disease
Histiocytosis, Non-Langerhans-Cell
Humans
Lower Extremity
Magnetic Resonance Imaging
Osteosclerosis
Retroperitoneal Fibrosis
Tibia

Figure

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  Fig. 1 Computed tomography (CT) scan of the patient's abdomen in 2008 showed tissue infiltration with low attenuation surrounding the left renal sinus and the proximal ureter (A). No significant change was observed on CT scan in 2010 (B).

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  Fig. 2 Simple radiography of the left knee showed heterogeneous patchy medullary sclerosis, coarse trabeculae, and osteopenia of the covered femur and tibia (A). Magnetic resonance imaging of the knee showed diffuse sclerotic changes combined with intramedullary low signal intensity on a T1-weighted image (B) and mixed low- and high-signal nodular lesions on a T2-weighted image (C). Involvement of mainly the meta- and diaphysis, with relative epiphyseal sparing, was observed.

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  Fig. 3 Whole-body bone scintigraphy revealed diffuse increased uptake in the bilateral clavicles, radii, ulnae, distal femora, tibiae, and fibulae.

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  Fig. 4 Results of hematoxylin and eosin staining showed sclerotic bony trabeculae containing lipid-laden histiocytes and small histiocytes with oval nuclei and pink cytoplasm. A small number of infiltrating inflammatory cells were observed; however, eosinophils were rare (A, ×200; B, ×400).

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  Fig. 5 Lipid-laden histiocytes were positive for CD68 (A, CD68 immunostain, ×100). Histiocytes were negative for CD1a (B, CD1a immunostain, ×200).

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