Symptomatic Intracranial Rosai-Dorfman Disease in the Suprasellar Region Treated with Conformal Radiotherapy - A Report of Two Cases and Literature Review
Correspondence Address: Source of Support: None, Conflict of Interest: None DOI: 10.4103/0028-3886.284371
Source of Support: None, Conflict of Interest: None
Keywords: Conformal radiotherapy, Rosai Dorfman, sellarKey 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.
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. 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.
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.
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).
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.
Central nervous Systeminvolvement by RDD occurs in <5% cases 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. Surgery is the standard treatment for both diagnostic and therapeutic purposes. 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.,, 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.
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.
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[Figure 1], [Figure 2], [Figure 3]