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1
The Johns Hopkins Hospital Baltimore, MD 21286
2
The Mayo Clinic Rochester, MN 55905
A 32-year-old black woman presented to the emergency department with a complaint of right flank pain of 2-weeks' duration. The patient had no significant medical history and her physical examination was unremarkable. Urinalysis revealed few RBC. CT of the abdomen with and without IV contrast material was performed. Images revealed delayed contrast excretion, a persistent right nephrogram, and a dilated right renal collecting system (Fig. 1A). Inferior to the right renal hilum images revealed an enhancing retroperitoneal mass invading the right psoas muscle to be the cause of the obstruction (Fig. 1B). Differential diagnostic considerations included lymphoma, mesenchymal sarcoma, retroperitoneal fibrosis, or possibly sarcoid, because of the patient's age and race. Tissue obtained at CT-guided biopsy revealed a mixed lymphocytic and histiocytic infiltrate without features of Langerhans' cell histiocytosis. The diagnosis of retroperitoneal Erdheim-Chester disease was made.
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Erdheim-Chester disease is a rare form of non-Langerhans histiocytosis. It is an endogenous noninherited disorder affecting men and women with equal frequency. Usually occurring in middle-age to older patients, manifestations range from a focal asymptomatic process to fatal systemic disease that may involve virtually all organ systems. When manifestations are focal, patients often present with leg pain with radiologic findings that are typical for the disease: uniform symmetric osteosclerosis with cortical and trabecular thickening in the metaphysis and, occasionally, the diaphysis of the lower extremities [1]. Approximately one half of the patients will have extraskeletal or multifocal disease. With disseminated disease, patients may have a clinical syndrome similar to the adolescent Hand-Schüller-Christian disease, with diabetes insipidus, proptosis, and histiocytic skin lesions. In addition, histiocytic infiltrates may be seen in the central nervous system, lung interstitium, surrounding major blood vessels in the thorax and abdomen, or in the retroperitoneum as seen in our patient. Once believed to be a form of histiocytosis X, Erdheim-Chester disease shows distinct radiographic, epidemiologic, and histiochemical features that define it as a distinct entity.
The diagnosis of Erdheim-Chester disease is usually derived from classic radiographic findings in the long bones of the lower extremities. Occasionally, the diagnosis will require biopsy with histologic confirmation of an extraosseous site. The histologic diagnosis of Erdheim-Chester disease is suggested in the presence of a granulomatous infiltration of lymphocytes and lipid-laden histiocytes. The absence of Birbeck granules on electron microscopy and negative findings on immunohistochemical staining for S-100 protein differentiates this condition from the Langerhans cell-type histiocytosis [2].
Retroperitoneal involvement may occur in isolation or as a component of disseminated disease. When present, histiocytic infiltrates may invade adjacent organs such as the kidney, pancreas, or adrenal gland [3]. Perivascular infiltrates may cause vessel stenosis or occlusion, whereas disease in the pancreatic head may cause biliary obstruction [4]. Renal involvement may be caused by direct invasion of the renal sinus and parenchyma or by more distal ureteral obstruction as seen in our patient. Surgical treatment of retroperitoneal disease is suboptimal because of the incidence of recurrence. Patients with renal involvement are often treated using palliative percutaneous nephrostomy to preserve renal function. Regimens including corticosteroids and chemotherapeutic agents (e.g., vincristine and methotrexate) have been used with little overall affect on progressive disease. Disseminated disease, especially involving the central nervous system, remains universally progressive and fatal.
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  V. R. Surabhi, C. Menias, S. R. Prasad, A. H. Patel, A. Nagar, and N. C. Dalrymple Neoplastic and Non-neoplastic Proliferative Disorders of the Perirenal Space: Cross-sectional Imaging Findings1 RadioGraphics, July 1, 2008; 28(4): 1005 - 1017. [Abstract] [Full Text] [PDF]
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