Rosai-Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy, is a benign histiocytic proliferative disorder of unknown etiology [
A 24-year-old Bangladeshi male, hailing from rural area of Comilla, complained of bilateral dimness of vision (more on left side), headache, nausea, and deafness of left side since early 2008. He consulted ophthalmologists, but there was no improvement. On the contrary, patient gradually became completely blind. Then he was referred to Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka. He was normotensive and nondiabetic and had no lymphadenopathy. His blood group was “O” positive. Haematological findings were as follows: haemoglobin 12.8 gm/dL, erythrocyte sedimentation rate (ESR) 14 mm after 1st hour, and total leukocyte count 8.8 × 109/liter. X-ray of chest and paranasal sinuses (occipitomental view) was normal. Audiometric examination revealed hearing sensitivity of right ear within normal limit but no response to any frequency in left ear. Only vibrotactile response was present in left ear. MRI of brain revealed hypointensity lesions alongside of the cavernous sinus and retroocular region of right orbit. Findings were suggestive of inflammatory lesion/neoplastic masses (?meningioma). An endoscopic endonasal transethmoidal approach was made to take biopsy from the lesion. Histopathology of biopsied material revealed portion of optic nerve, fragmented pieces of soft tissue, chronic inflammatory cells, and a single focus of oval cells forming whorled pattern. The lesion was suggested as an inflammatory pseudotumour with a differential diagnosis of lymphoplasmacyte-rich meningioma. Patient was released from hospital with only symptomatic treatment and was advised to come back for a follow-up after three months. Patient failed to attend in time. In the meantime, he received some homeopath treatment but there was no improvement. After one year, he developed frequent fainting, nausea, and vomiting. At the end of 2011, patient came back to Dhaka and was admitted to a private hospital in neurosurgery department. A computed tomography (CT) scan of brain revealed large, irregular, hypodense mass lesions with mild perilesional oedema in left parasellar region on the medial aspect of left temporal lobe, extending in both frontal regions and in both retrobulbar areas encircling both optic nerves. The lesion has also extended in left prepontine area causing mild compression upon the adjacent pons. The final radiological impression was left parasellar meningioma (Figure
CT scan of brain showing that the lesion has encircled both optic nerves.
Photomicrograph showing inflammatory nature of the lesion (H&E, ×200).
Photomicrograph of the lesion showing many histiocytes, some of which have engulfed intact lymphocytes (H&E, ×400).
Photomicrograph of immunohistochemistry of the lesion showing positive reactivity of the histiocytes to S-100 protein (S-100, ×400).
Rosai-Dorfman disease (RDD) was first described by Destombes in 1965 and was recognized as a distinct clinicopathological entity by Rosai and Dorfman in 1969 [
There are some hypotheses regarding the etiology of this rare condition. The histiocytes in RDD may derive from activated macrophages. This activation may be caused by an unusual response of the hematolymphoid system to an immune disorder. Some authors also have suggested a viral influence behind this disease. With in situ hybridization, Epstein-Barr virus and human herpes virus type six were identified in some cases [
Extranodal RDD occurs in about 43% of cases [
Unlike nodal disease, intracranial RDD usually affects adult males in their fourth and fifth decade (mean age 39.5 years which is little higher than nodal RDD) [
Radiologically intracranial RDD is commonly mistaken for meningioma [
The definitive diagnosis of intracranial RDD depends on histopathology and immunohistochemistry. But unfortunately the characteristic histological finding (e.g., emperipolesis) is often obscured by extensive fibrosis that occurs in intracranial RDD. Andriko et al. [
This case was initially diagnosed histologically as lymphoplasmacyte-rich meningioma. This is an important differential diagnosis of intracranial RDD. This type of meningioma produces a chronic inflammatory infiltrate which can be confused with infiltrates present in RDD. Immunostain with epithelial membrane antigen (EMA) can differentiate the two entities precisely [
Langerhans cell histiocytosis, on the other hand, is another mimicker of intracranial RDD. Eosinophils are also present in this condition. Langerhans histiocytes, when xanthomatous, may resemble histiocytes in RDD. Moreover, immunologically both RDD and Langerhans histiocytes are positive for S-100. However, Langerhans cells have folded nucleus with longitudinal grooves which is absent in RDD histiocytes. Unlike RDD, Langerhans histiocytes are also positive for CD1a [
Ophthalmic manifestations of RDD may occur due to orbital and eyelid involvement, lacrimal gland involvement, optic nerve compressive neuropathy, and uveitis. Among these, orbital manifestation is the commonest [
In the present case, the mass (RDD) extended to the retrobulbar region and has encircled both optic nerves causing compression and complete blindness. Other reported cases have shown that optic nerve compression due to RDD may be unilateral [
Cause of left sided deafness in this patient is not fully understood. Pure tone audiometry reveals sensorineural hearing loss (SNHL) of left ear. The patient showed no response to any frequency of sound wave in that ear. But the hearing sensitivity of right ear is normal.
Sensorineural hearing loss may be caused by lesions in the cochlea or the auditory nerve. It may be congenital, hereditary, or delayed onset. Delayed onset SNHL is usually caused by infection, trauma, or ototoxic medication. But it may also be caused by primary tumours in temporal bone (vestibular schwannoma, squamous cell carcinoma, and paraganglioma) or metastatic tumours to temporal bone (breast, lungs, and prostate) [
In this patient, the intracranial mass (RDD) has extended in left prepontine area and has caused mild compression upon the adjacent pons. This may cause damage to auditory nerve pathway and ultimately result in deafness of left ear.
Reported cases of RDD causing hearing loss are extremely rare. Nalini et al. [
Rosai-Dorfman disease has been treated with surgery, corticosteroids, chemotherapy, and radiotherapy [
The prognosis of RDD is not poor even if the disease is intracranial. A review of follow-up data of 43 cases of RDD with CNS involvement showed that 58% of patients were alive [
Intracranial Rosai-Dorfman disease is a very rare condition. It may lead to, although extremely rare, serious consequences like blindness and deafness [
The authors declare that there is no conflict of interests regarding the publication of this paper.