A new case of cervical intramedullary sinus histiocytosis causing paraplegia and review of the literature

Abstract

Background:Rosai–Dorfman disease (RDD) is an uncommon, benign histiocytic proliferative disorder of unknown origin. It predominantly affects the lymph nodes, but can also be found extranodal in different organs. Nervous system involvement is rare, and the most cases are intracranial. Surgical treatment is indicated when the central nervous system (CNS) in compromised.Case Description:We herein describe the management of a 27-year-old woman who presented progressive spinal cord symptoms, secondary to an isolated intramedullary lesion, which had a histological confirmation of RDD. To our knowledge, this is the 6th case reported in English written manuscripts. We review these cases and analyze some of the literature concerning the disease.Conclusions:RDD shows some variability in the involvement of the entire neuraxis, and because its ability to mimic meningeal and primary brain tumors, it is essential to be aware of this entity and consider RDD in the differential diagnosis of various lesions of the CNS. The conclusive diagnosis must be obtained by histological methods, so surgical approaches have to be discussed. Although it is not considered as a malignancy, options for postoperative medical treatment are variable and include radiation, chemotherapy or maybe monoclonal antibodies for refractory or recurrent cases.

Isolated Intramedullary Spinal Rosai–Dorfman Disease in a Child: A Case Report

“…Involvement of the spine has been described in only 20% of those with CNS involvement, with the vast majority of cases being related to the Dura. 11 A retrospective analysis showed that 210 cases of CNS involvement were reported ever since the description of the disease, of which only 24 were isolated spinal RDD. Most cases of isolated spinal RDD were extramedullary duralbased lesions; only two cases were intramedullary.…”

Isolated langerhans cell histiocytosis masquerading as intradural extramedullary meningioma: Review on histiocytic disorders of spine

“…Rocha-Maguey et al [6] reported a case of intradural mass lesion at cervical level with histopathology of RDD. Their patient presented at 27 years of age contrary to the younger age of our patient.…”

Isolated intramedullary Rosai-Dorfman disease

“…entitled “A new case of cervical intramedullary sinus histiocytosis causing paraplegia and review of the literature.”[5] In their article, the authors revealed that their patient was the 6 th case that presented with an isolated intramedullary mass without any systemic manifestation. They listed information regarding all 6 reported cases.…”