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Table of Contents    
Year : 2020  |  Volume : 68  |  Issue : 2  |  Page : 489-492

Symptomatic Intracranial Rosai-Dorfman Disease in the Suprasellar Region Treated with Conformal Radiotherapy - A Report of Two Cases and Literature Review

1 Department of Radiation Oncology, Tata Memorial Centre, Homi Bhabha National Institute, Mumbai, Maharashtra, India
2 Department of Pathology, Tata Memorial Centre, Homi Bhabha National Institute, Mumbai, Maharashtra, India
3 Hemato Oncology, Tata Memorial Centre, Homi Bhabha National Institute, Mumbai, Maharashtra, India

Date of Web Publication15-May-2020

Correspondence Address:
Jayant S Goda
Department of Radiation Oncology, Tata Memorial Centre, Parel, Mumbai, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0028-3886.284371

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 ╗ Abstract 

Intracranial Rosai-Dorfman Destombes (RDD) disease is a rare entity. Lesions can lead to cranial nerve palsies and visual loss, especially in suprasellar location. Resection is considered to be definitive treatment; however, complete excision is difficult to achieve in view of the close proximity of critical structures. Radiotherapy (RT) is sometimes used for refractory or progressive disease for local tumor control and amelioration of symptoms. We report two patients with suprasellar RDD's with progressive symptoms treated with conformal RT after subtotal excision. These patients were treated with high precision conformal techniques to a dose of 45 Gy with significant and durable improvement in vision.

Keywords: Conformal radiotherapy, Rosai Dorfman, sellar
Key Message: Supra sellar Rosai Dorfman Disease(RDD) is rare. Total excision is difficult to achieve in the suprasellar location in view of proximity to critical structures. Steroid refractory RDD treated with conformal radiotherapy achieves good long term local control of the disease.

How to cite this article:
Sasidharan A, Verma A, Epari S, Gupta T, Laskar S, Khanna N, Krishnartry R, Bagel B, Jain H, Goda JS. Symptomatic Intracranial Rosai-Dorfman Disease in the Suprasellar Region Treated with Conformal Radiotherapy - A Report of Two Cases and Literature Review. Neurol India 2020;68:489-92

How to cite this URL:
Sasidharan A, Verma A, Epari S, Gupta T, Laskar S, Khanna N, Krishnartry R, Bagel B, Jain H, Goda JS. Symptomatic Intracranial Rosai-Dorfman Disease in the Suprasellar Region Treated with Conformal Radiotherapy - A Report of Two Cases and Literature Review. Neurol India [serial online] 2020 [cited 2022 May 26];68:489-92. Available from: https://www.neurologyindia.com/text.asp?2020/68/2/489/284371

Rosai Dorfman-Destombes (RDD) disease is a rare non-Langerhan cell proliferative disorder of lymphatic and extra lymphatic sites. Although the pathogenesis is not well known, immune-mediated mechanisms have been implicated. Extra-nodal sites may be involved in up to 43% cases, but intracranial or spinal involvement is rare.[1] Although there are no established guidelines for its management, various therapeutic modalities have been used, including surgery, radiotherapy, and chemotherapy. We present our experience in treating two cases of progressive intracranial RDD in suprasellar region treated with a moderate dose of conformal radiotherapy.

 ╗ Case Description Top

Case 1

A 32-year-old female presented with progressive weakness of the right arm, headache, and diminution in bilateral eyes for two years.

Magnetic resonance imaging (MRI) of the brain showed multi-focal extra-axial lesions in the brain and spine. The dominant lesion was in the suprasellar region extending laterally along with sphenoid wings with homogenous contrast enhancement compressing the pituitary gland and prechiasmatic optic nerves. Additional enhancing lesions were seen along the clivus compressing the medulla, right perisylvian convexity, and at L1 vertebral level. Metastatic workup did not show any other distant sites of disease except in the neck nodes.

The patient underwent craniotomy and subtotal excision of the cranio-sphenoidal lesion. The histomorphology [Figure 1]A mimicked a meningioma with chronic inflammation. A diagnosis of RDD was made according to immunohistochemical (IHC) studies, which were positive for S100 and CD-163 and negative for CD1a and CD20: CD3 revealed both B and T lymphocytes.
Figure 1: (A) Lesion composed of numerous histiocytes, plasma cells, and lymphocytes with few histiocytes showing emperipolesis i: 100×; ii: 400×; iii: S100 protein positive histiocytes (200×); (iv) CD 163 positive histiocytes showing emperipolesis (400×). (B) (i-iii) Fibro-connective tissue showing infiltration by plasma cells, lymphocytes, and histiocytes; few of which show emperipolesis (i: 100×; ii: 200×; iii: 400×) (iv) Histiocytes showing positivity for S100 protein

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Post-surgery her vision started deteriorating even with steroids. She was then planned for radiotherapy (RT) to the suprasellar lesion in view of the worsening vision. The patient underwent external beam RT with a 3D conformal technique [Figure 2]a to a dose of 45 Gy in 25 fractions. Twelve months post-radiotherapy patients' vision in the right eye had improved to finger counting, and MRI [Figure 3]a showed a good response in the suprasellar lesion although a residual tumor was still persisting. In view of her nodal disease, she was started on oral 6-mercaptopurine and methotrexate. The patient is free of disease progression 35 months post therapy at all the sites and has an independent activity of daily living (ADL).
Figure 2: MRI based contouring of the lesion and conformal radiotherapy planning using (a) 3-dimensional conformal radiotherapy for Patient 1 and (b) volumetric modulated arc therapy for Patient 2

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Figure 3: (a) Pre-radiotherapy MRI of Patient 1 (left) showing a well-defined lobulated lesion with homogenous post contrast enhancement in the suprasellar region extending laterally along bilateral sphenoid wings. Post radiotherapy MRI (right) shows partial response. (b) Pre radiotherapy MRI of Patient 2 (left) shows a homogenously enhancing soft tissue mass in sellar, suprasellar and parasellar region. Post radiotherapy MRI (right) shows good response. Although residual disease is still persistent.

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Case 2

A 29-year-old male presented with progressive reduction of vision in the left eye. MRI brain showed a homogenously enhancing sellar lesion involving the cavernous sinus, clivus and extending up to the pre-pontine cistern. The lesion was compressing the pons and optic chiasma. The lesion was intensely enhancing with a dural tail posteriorly along the clivus mimicking appearance of a meningioma.

The patient underwent subtotal excision of the lesion. Histomorphology [Figure 1]B was suggestive of non-Langerhans cell histiocytosis favoring extra-nodal RDD with infiltration by plasma cells, lymphocytes, and histiocytes; few of them showed emperipolesis. IHC studies showed positivity of S100 and CD163, whereas it was negative for CD1a, CD 20, and CD3.

Following partial excision, the patient had a worsening vision in spite of steroid therapy. He underwent adjuvant radiation therapy to the sellar lesion by volumetric modulated arc therapy [Figure 2]b to a dose of 45Gy in 25 fractions. Three months post-therapy, his vision had improved. MRI [Figure 3]b showed a mild reduction in size as well as the intensity of contrast enhancement. Twenty-five months post-therapy, the patient is alive with improvement in his vision.

 ╗ Discussion Top

Central nervous Systeminvolvement by RDD occurs in <5% cases[2] and the above cases exemplify the rarity of the disease. Intracranial RDD usually occurs without extracranial lymphadenopathy, and most lesions are attached to the dura without intraparenchymal extension sometimes causing pachymeninigitis like features.[3] Surgery is the standard treatment for both diagnostic and therapeutic purposes.[4] Complete resection should be attempted, where feasible, but sometimes these tumors can adhere to surrounding critical structures, precluding complete excision as observed in both of our patients. In such cases, subtotal resection of symptomatic lesions should be done to provide a pathologic diagnosis and symptomatic relief. If neurologic impairment is reversed after partial resection, a wait-and-watch approach can be implemented because most patients achieve stable disease following partial excision. If neurologic symptoms are persistent and/or the lesions are surrounding vital structures with a potential to compromise their function, adjuvant therapies such as corticosteroids or radiotherapy should be implemented.[4],[5],[6] Although RT is rarely used in RDD; its judicious use in special situations can arrest disease progression and results in durable tumor control as observed in the index cases.

Both the patients in our series had progressive symptoms post-surgery and steroid therapy that warranted RT. Radiation appears to have a moderate effect on RDD and is beneficial in refractory or progressive disease with deterioration in clinical symptoms. RT doses between 15 Gy and 45 Gy have been used for optimal local control [Table 1]. The heterogeneity in the radiation doses used by physicians for this tumor may be owing to the rarity of the tumor, infrequent use of definitive radiation, and heterogeneity in radiation response. In view of these confounding factors, understanding the mechanistics of RT in these tumors is difficult. However, the advent of modern RT delivery techniques, such as three-dimensional conformal RT (3DCRT) and intensity-modulated radiationtherapy, has minimized the risk of treatment-related toxicity but have not been formally evaluated in this situation. Both the patients in the present report were treated with high precision conformal techniques and have long-term local control post RT without any complications. Local control with surgery followed by RT as an adjuvant or as a salvage therapy has not only improved the progression of the disease [Table 1] but also reduced the morbidities associated with these lesions.
Table 1: Intracranial Rosai Dorfman Disease treated with radiotherapy - Review of literature

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 ╗ Conclusion Top

Intracranial sellar-suprasellar RDD is a rare entity and should be considered as a differential for lesions located in this region. Although surgical excision is the standard therapeutic modality, unresectable, or partially resectable, recalcitrant, progressive, and symptomatic lesions respond well to moderate doses of conformal RT with durable local control.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.[16]

 ╗ References Top

Saboo SS, Jagannathan JP, Krajewski KM, O'Regan K, Hornick JL, Fisher DC, et al. Symptomatic extranodal Rosai-Dorfman disease treated with steroids, radiation, and surgery. J Clin Oncol 2011;29:e772-5.  Back to cited text no. 1
Sandoval-Sus JD, Sandoval-Leon AC, Chapman JR, Velazquez-Vega J, Borja MJ, Rosenberg S, et al. Rosai-Dorfman disease of the central nervous system: Report of 6 cases and review of the literature. Medicine (Baltimore) 2014;93:165-75.  Back to cited text no. 2
Gupta K, Bagdi N, Sunitha P, Ghosal N. Isolated intracranial Rosai-Dorfman disease mimicking meningioma in a child: A case report and review of the literature. Br J Radiol 2011;84:e138-41.  Back to cited text no. 3
Adeleye AO, Amir G, Fraifeld S, Shoshan Y, Umansky F, Spektor S. Diagnosis and management of Rosai-Dorfman disease involving the central nervous system. Neurol Res 2010;32:572-8.  Back to cited text no. 4
Dalia S, Sagatys E, Sokol L, Kubal T. Rosai-Dorfman disease: tumor biology, clinical features, pathology, and treatment. Cancer Control 2014;21(4):322-7.  Back to cited text no. 5
Trudel M. Dural involvement in sinus histiocytosis with massive lymphadenopathy. J Neurosurg 1984;60:850-2.  Back to cited text no. 6
Petzold A, Thom M, Powell M, Plant GT. Relapsing intracranial Rosai-Dorfman disease. J Neurol Neurosurg Psychiatry 2001;71:538-41.  Back to cited text no. 7
Kitai R, Llena J, Hirano A, Ido K, Sato K, Kubota T. Meningeal Rosai-Dorfman disease: Report of three cases and literature review. Brain Tumour Pathol 2001;18:49-54.  Back to cited text no. 8
Hadjipanayis CG, Bejjani G, Wiley C, Hasegawa T, Maddock M, Kondziolka D. Intracranial Rosai-Dorfman disease treated with microsurgical resection and stereotactic radiosurgery. Case report. J Neurosurg 2003;98:165-8.  Back to cited text no. 9
El Majdoub F, Brunn A, Berthold F, Sturm V, Maarouf M. Stereotactic interstitial radiosurgery for intracranial Rosai-Dorfman disease. A novel therapeutic approach. Strahlenther Onkol 2009;185:109-12.  Back to cited text no. 10
Symss NP, Cugati G, Vasudevan MC, Ramamurthi R, Pande A. Intracranial Rosai Dorfman Disease: Report of three cases and literature review. Asian J Neurosurg 2010;5:19-30.  Back to cited text no. 11
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Das S, Biswas A, Roy S. Recurrent intracranial Rosai Dorfman disease: Management of a challenging case. Asian J Neurosurg 2017,12:537-40.  Back to cited text no. 12
[PUBMED]  [Full text]  
Kumar R, Garg K, Dash C, Sharma MC. Rosai-Dorfman disease: An unusual isolated cavernous sinus lesion. Neurol India 2016;64:1331-2.  Back to cited text no. 13
[PUBMED]  [Full text]  
Uthamalingam P, Mehta S, Duraisamy G. Intracranial Rosai-Dorfman disease: A rare entity with an uncommon presentation. Neurol India 2016;64:1064-5.  Back to cited text no. 14
[PUBMED]  [Full text]  
Sharma MS, Padua MD, Jha AN. Rosai-Dorfman disease mimicking a sphenoid wing meningioma Neurol India 2005;53:110-1.  Back to cited text no. 15
KrishnamoorthyV, Parmar CF, Panikar D. Isolated intracranial Rosai Dorfman disease. Neurol India 2011;59:443-6.  Back to cited text no. 16


  [Figure 1], [Figure 2], [Figure 3]

  [Table 1]

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