Rosai-Dorfman disease of vertebra: Case report and literature review

Abstract

Rosai-Dorfman disease is a rare benign histiocytic disorder that arises predominantly in lymph nodes with fever and malaise, but can affect various organs, with or without lymphadenopathy. Solitary extranodal skeletal lesions are extremely rare. Herein, we describe a case of isolated disease of thoracic spine, with vertebral body fracture. A 14-year-old girl presented with cervical back pain for about one month as the only symptom. There was no lymphadenopathy. Results of laboratory tests were within reference ranges. The radiological examinations revealed collapse, osteolytic lesion and compressive fracture, which reduced vertical diameter of C4 vertebral body. The patient made a complete recovery after a limited C4 laminectomy, spondylodesis C3/C5 vertebra and biopsy of the lesion. Histologically, nonspecific cellular milieu and atypical histiocytic proliferation with emperipolesis, with CD68 and S100 positivity, confirmed a diagnosis of Rosai-Dorfman disease. Isolated extranodal skeletal Rosai-Dorfman disease should be considered among the differential diagnoses of similar changes, such as Langerhans cell histiocytosis, Erdheim-Cheister disease, eosinophilic granuloma, osteomyelitis, metastasis and lymphoma.

Rosai-Dorfman Disease Presenting with Solitary Liver Mass Without Lymphadenopathy: Case Report and Literature Review

“…The prevalence of RDD is approximately 1:200,000 in the United States, and may present with fever, neutrophilia, increased serum erythrocyte sedimentation rate, leukocytosis, lymphopenia, polyclonal hyperglobulinemia, and anemia. In most cases, however, there is no apparent or specific symptom [9][10][11][12][13] .…”

Rosai-Dorfman disease with spine involvement

“…The eyes, orbit, and bones account for 11%, the salivary glands and central nervous system account for 7%, and less frequently, the mouth, kidneys, respiratory tract, liver, tonsils, breast, gastrointestinal tract, and heart. Lesions involving the bone are usually multifocal and are associated with lymphadenopathy and other organ diseases [5,6] . Skeletal involvement is rare, and this isolated bone lesion usually occurs in adults with no other symptoms.…”

“…Lesions involving the bone are usually multifocal and are associated with lymphadenopathy and other organ diseases. [5,6] Skeletal involvement is rare, and this isolated bone lesion usually occurs in adults with no other symptoms. It is estimated that 0.6% to 1% of RDD cases have isolated or complicated spinal lesions, which may occur in the bone, dura, and spinal parenchyma, but spinal RDD has no pathologic clinical or imaging features.…”

Experience of diagnosing and managing a patient with spinal Rosai-Dorfman-Destombes disease in a tuberculosis hyperendemic region

“…Extranodal lesions have been reported in the retro-orbital tissues (11%), nasal cavity (11%), skin (10%), bone (5%–10%) and central nervous system (5%). However, about 20%–25% of patients with RDD have only extranodal disease; lymph nodes are spared 6…”