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  • Title: Isolated Rosai-Dorfman disease presenting as peripheral mononeuropathy and clinically mimicking a neurogenic tumor: case report.
    Author: Huang HY, Liang CL, Yang BY, Sung MT, Lin JW, Chen WJ.
    Journal: Surg Neurol; 2001 Nov; 56(5):344-7. PubMed ID: 11750016.
    Abstract:
    BACKGROUND: Rosai-Dorfman disease is a rare idiopathic disorder of proliferative histiocytes affecting the lymph nodes. It usually manifests as bilateral cervical lymphadenopathy and fever with concurrent polyhyperglobulinemia. Cases involving the nervous system are quite rare; most CNS lesions are located intracranially or arise from the spinal dura or leptomeninges. To our knowledge, there has been no previous report of isolated Rosai-Dorfman disease presenting as peripheral mononeuropathy. CASE REPORT: We report a 43-year-old female with isolated extranodal Rosai-Dorfman disease in the medial aspect of the right upper arm, which presented as aberrant ulnar neuropathy caused by a mass encasing the right basilic vein and the medial anteriobrachial cutaneous branch of the right ulnar nerve. Preoperative diagnosis was a neurogenic tumor. The patient underwent excision of the mass, and pathologic examination confirmed the diagnosis of Rosai-Dorfman disease. CONCLUSION: An unusual case of extranodal isolated Rosai-Dorfman disease, presenting as peripheral mononeuropathy, is reported. Clinically, it simulated a neurogenic tumor, extending the etiologic spectrum of entrapment neuropathy of the peripheral nerve.
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