Diagnosis and treatment of Rosai-Dorfman disease of the spine: a systematic literature review

Abstract

Purpose To review and summarize the clinical features, diagnosis, treatment strategies, and prognosis of spinal Rosai-Dorfman disease (RDD). Methods RDD is also termed as sinus histiocytosis with massive lymphadenopathy. We searched the databases of PubMed, Elsevier ScienceDirect, SpringerLink, and OVID. The keywords were Rosai-Dorfman disease and spine/central nervous system. Research articles and case reports with accessibility to full texts regarding spinal RDD were eligible for the inclusion. A total of 62 articles were included, and they contained 69 cases. We extracted the information of interest and analyzed them using SPSS statistics package. Results The average age was 33.1 ± 18.3 years. The ratio of males to females was 1.9/1. Overall, 63 cases presented with spine-related symptoms. A total of 27 cases (39.1%) had multi-organ lesions, and 12 cases had records of massive lymphadenopathy. Among 47 cases who first manifested spine-related symptoms, 93.6% were preoperatively misdiagnosed. The disease had a predilection for cervical spine (38.8%) and thoracic spine (40.3%). 62.9% of lesions were dura-based. Surgery remained the mainstream treatment option (78.8%), with or without adjuvant therapies. Total lesion resection was achieved in 34.8% of cases. The rate of lesion recurrence/progression was 19.5%, which was marginally lower for total resection than for non-total resection. Conclusion Spinal RDD has no pathognomonic clinical and imaging features. Most cases first present with spine-relevant symptoms. Massive lymphadenopathy is not common, but a tendency for multi-organ involvement should be considered. Spinal RDD has a high recurrence rate; thus, total resection is the treatment of choice. Adjuvant therapies are indicated for multi-organ lesions and residual lesions. A wait and watch strategy is recommended for asymptomatic patients. Herein, a workflow of diagnosis and treatment of the spinal RDD is established.

Spinal extranodal Rosai-Dorfman disease: A case report and literature review

“…But, it is challenging to make a differential diagnosis with other spinal conditions, such as meningioma, lymphoma, metastatic tumors, extradural hematoma, and spinal infections [10] . In the Mayo Clinic histiocytosis working group consensus statement for diagnosing and evaluating adult patients with histiocytic neoplasms, positron emission tomography/computed tomography (PET/CT) is recommended to exclude systemic lesions, especially the involvement of superior lymph nodes [7] , [13] .…”

“…Surgical resection remained the mainstay of treatment (78.8%), with or without adjuvant therapies [13] . The surgery varies from total tumor removal to biopsy only.…”

“…The surgery varies from total tumor removal to biopsy only. In 44.2% of cases, complete resection was achieved, while decompressive surgery with or without subtotal resection occurred in 55.8% [13] .…”

“…Radiotherapy and chemotherapy are usually used as adjuvant treatments of surgery. Radiotherapy was proved to provide reasonable locoregional control in recurrence and progressive diseases, while chemotherapy was preserved for life-threatening systemic cases [10] , [13] . The standard radiation dose for spinal RDD has not been reported in the literature.…”

“…The standard radiation dose for spinal RDD has not been reported in the literature. Similarly, chemotherapy regimens were usually prescribed based on personal experience rather than high-level evidence, and its outcome was varied in most patients [13] . Vinblastine, cladribine, etoposide, 6-mercaptopurine plus low-dose methotrexate are the reported chemotherapy agents with mixed results.…”