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Isolated extradural Rosai-Dorfman disease causing the spinal cord compression: A case report.

ABSTRACT: RATIONALE:Rosai-Dorfman disease (RDD) is a rare benign histiocytic disease that is commonly characterized by massive painless cervical lymphadenopathy and systemic manifestations. Isolated extranodal involvement, especially spinal involvement, is extremely rare. PATIENT CONCERNS:A 28-year-old man presented with intermittent dorsodynia and bilateral lower-limb weakness and numbness. A magnetic resonance scan (MRI) showed an extradural lesion of the T6-T9 thoracic spine that lead to cord compression. DIAGNOSES:Histopathological findings showed distinctive emperipolesis and immunohistochemistry results that were positive for cluster of differentiation CD68 and S100. Therefore, we diagnosed the Rosai-Dorfman disease. INTERVENTIONS:we performed a nearly total surgical resection and a limited T6-T9 laminectomy. OUTCOMES:Postoperatively, the patient's symptoms were partially relieved and experienced no recurrence during the 6-month follow-up. The preoperative diagnosis of isolated spinal RDD still remains challenging. LESSONS:Thus, we should consider RDD in the differential diagnosis of the central nervous system. Besides surgical resection, the treatment also included radiation, chemotherapy or monoclonal antibodies. However, the optimal treatment remains controversial. Therefore, we should exert all our energies on the exploration of etiology and adjuvant therapy for this disease.

SUBMITTER: Li Y

PROVIDER: S-EPMC6200482 | biostudies-other | 2018 Oct

REPOSITORIES: biostudies-other

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Isolated extradural Rosai-Dorfman disease causing the spinal cord compression: A case report.

Li Yongning Y Wang Xin X Gao Jun J Yu Shuangni S Li Zhimin Z

Medicine 20181001 40

<h4>Rationale</h4>Rosai-Dorfman disease (RDD) is a rare benign histiocytic disease that is commonly characterized by massive painless cervical lymphadenopathy and systemic manifestations. Isolated extranodal involvement, especially spinal involvement, is extremely rare.<h4>Patient concerns</h4>A 28-year-old man presented with intermittent dorsodynia and bilateral lower-limb weakness and numbness. A magnetic resonance scan (MRI) showed an extradural lesion of the T6-T9 thoracic spine that lead to ...[more]

PMID: 30290681

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Project description:BackgroundRosai-Dorfman-Destombes disease (RDD) is a rare histioproliferative disease with unknown etiology. It commonly occurs in lymph nodes and can affect extra-nodal tissues and organs. Renal RDD is extremely rare, only a few cases have been reported, and its clinical symptoms and imaging findings are non-specific. To date, no literature has summarized its imaging manifestations in a large number of cases. Due to the involvement of different tissues and organs, there is no standard treatment for RDD. It has been reported that RDD patients with kidney involvement have a poor prognosis. Thus, understanding of renal RDD need to be extended.Case descriptionWe present a rare case of renal RDD in an asymptomatic 67-year-old male. The results of an ultrasound examination indicated that both kidneys were surrounded by hypoechoic soft tissue lesions, and there was a huge mass in the left kidney, which had a clear boundary with the renal capsule. The results of contrast-enhanced ultrasound (CEUS) showed hypo-enhancement in the bilateral perinephric lesions and mass. However, the computed tomography urography (CTU) findings revealed no obvious enhancement. The patient then underwent a series of laboratory tests, but no relevant information was found. To make a clear diagnosis, the urologist then removed the left perirenal mass and some perirenal tissues, and the patient was finally pathologically diagnosed with extra-nodal RDD. The patient remains asymptomatic, and no treatment has been administered to date.ConclusionsThis case may be the first reported case in which CEUS was performed and the second reported case of asymptomatic renal RDD. Based on the previous literature reports, we found that some specific characteristics of renal RDD include bilateral perirenal lesions with a "hairy kidney" appearance. CEUS and/or CTU can be used to help differentiate a solitary mass of RDD from common tumors, to avoid misdiagnosis leading to unnecessary nephrectomy.

Dataset Information

Isolated extradural Rosai-Dorfman disease causing the spinal cord compression: A case report.

Publications

Isolated extradural Rosai-Dorfman disease causing the spinal cord compression: A case report.

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