Isolated Intracranial Rosai-Dorfman Disease

Taufiq,; Khair, Abul; Begum, Fatima; Akhter, Shaheen; Farooq, Shamim; Kamal, Mohammed

doi:10.1155/2016/1972594

Cited by 14 publications

(25 citation statements)

References 15 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Md. Tau q [25] reported a male with a huge, irregular lesion in the parasellar region. The patient was treated with incomplete removal of the space occupying lesion and steroid following the operation.…”

Section: Discussionmentioning

confidence: 99%

“…The classical symptom for patients with RDD is bilateral, massive, and painless cervical lymphadenopathy, but 43% of these patients manifest extranodal disease [5]. The central nervous system (CNS) is involved in less than 5% of cases, among which 75% occurs as intracranial and 25% as spinal lesions [23,25]. Patients with intracranial RDD always go to see a doctor when they have the onset of symptoms which include headaches, seizures, gait di culty, motor or sensory abnormalities, and cranial nerve de cits due to the mass effect.…”

Section: Introductionsmentioning

confidence: 99%

See 1 more Smart Citation

Postoperative Management of The Primary Single Intracranial Rosai-Dorfman Disease: Case Report and Literature Review

Liu

et al. 2020

Preprint

View full text Add to dashboard Cite

The isolated intracranial Rosai-Dorfman disease (RDD) is extremely rare. It remains unclear for the best management of the residual lesions. In this report, the authors describe a case of primary single intracranial RDD treated with Gamma knife radiosurgery after partial resection, review all the related literatures and summarize the postoperative management data, in order to provide useful therapeutic information.A 23-year-old male presented with a more than 10-month history of dizziness, blurred vision and weakness. Preoperative MR imaging revealed a giant lesion involving the left middle cranial fossa and extending to the left cavernous sinus. He underwent fronto-temporal craniotomy and eventually achieved partial resection of the lesion. Postoperative histopathological results suggested a diagnosis of intracranial RDD. The residual lesion was treated with Gamma knife radiosurgery about 1 month after surgery. The follow-up imaging showed an obvious decrease in the size of the residual lesion.We are the second to provide the detail parameters of gamma knife therapy, which may be a reference for clinical neurosurgeons. Compared with the firstly reported case, our patient with a longer follow-up time has different therapeutic dose. Based on our own experience and an updated literature review, adjuvant stereotactic radiosurgery may be a safe and effective treatment for primary single intracranial RDD patients with incomplete excision. Other adjuvant therapies can be as an option in refractory patients.

show abstract

Section: Discussionmentioning

confidence: 99%

Section: Introductionsmentioning

confidence: 99%

Postoperative Management of The Primary Single Intracranial Rosai-Dorfman Disease: Case Report and Literature Review

Liu

et al. 2020

Preprint

View full text Add to dashboard Cite

show abstract

“…Involvement of the central nervous system is rare [8, 9] and it occurs generally in middle-aged men (mean 39,4 years) causing headaches, seizure, visual symptoms, and focal deficits [9–12]. Involvement of the spinal cord has also been described [13, 14].…”

Section: Discussionmentioning

confidence: 99%

“…Rosai-Dorfmann disease could mimic lymphoma, histiocytic and lacrimal gland tumours [3–7]. Localisation in the central nervous system (CNS) is rare (4% of cases) [8, 9]. Various treatments have been proposed, including steroid therapy, chemotherapeutic regimens, radiotherapy, surgery, and combinations of the above but optimal treatment has yet to be established.…”

Section: Introductionmentioning

confidence: 99%

Mercaptopurine Treatment in an Adult Man with Orbital and Intracranial Rosai-Dorfman Disease

Arnao

Riolo

Savettieri

et al. 2016

Case Reports in Neurological Medicine

View full text Add to dashboard Cite

Background. Rosai-Dorfmann disease (RDD) is a rare, idiopathic non-Langerhans cell histiocytosis, affecting children and young adults, that commonly presents as painless, massive cervical lymphadenopathy with fever, weight loss, and polyclonal hypergammaglobulinemia. Cervical lymphadenopathy and extranodal involvement are the main presentations. On the contrary, ophthalmic involvement and localisation in the central nervous system are rare. Case Report. An old man was admitted to our hospital for first seizure. Brain imaging studies revealed on the left an extra-axial thickening of the dura mater with enhancement and perilesional oedema, infiltrating the sphenoorbital fissure and an isointense mass with enhancement in the orbital region with dislocation of the optic nerve. Pathological and immunohistochemistry examination of the bioptical specimen was consistent with a diagnosis of RDD. Treatment with levetiracetam and steroids was started obtaining only remission of seizures. Because of the patient refusal of the surgical debulking, therapy with mercaptopurine was started, stopping disease progression. Conclusion. So far, very few cases of extranodal RDD with multiple CNS lesions involving the orbital region have been described. Our case is significant because it is the first case in which the efficacy of mercaptopurine treatment has been documented in an adult patient with isolated ocular and intracranial RDD.

show abstract

“…Most of the cases with intracranial involvement include nodal involvement along with extra nodal component of disease including orbit, head and neck region, upper respiratory tract, skin, bone and testis. With prevalence of 43%, isolated intra cranial involvement without nodal involvement is extremely rare [1][2][3] and by now only 7 cases had been reported 4,5 . The disease usually affects young adults and can have a protracted course lasting from several months to years and commonly results in complete recovery 6 .…”

Section: Introductionmentioning

confidence: 99%

Isolated intracranial Rosai Dorfman Disease without nodal involvement.

Aziz¹,

Raja²,

Khimani³

et al. 2019

Int. j. endorsing health sci. res.

View full text Add to dashboard Cite

Background: Rosai Dorfman disease (RDD) is a benign lymphohistiocytosis that often involves lymph nodes and present as massive painless lymphadenopathy with sinus histiocytosis. Usually systemic involvement and with rare intracranial and extremely rarely intracranial involvement without the involvement of lymphadenopathy. Case Presentation: We presented a case of 60 years old female with seizures and left side weakness and no lymphadenopathy. The magnetic resonance imaging (MRI) revealed contrasting right frontal homogenously enhancing convexity mass. Management & Results: The patient was kept on antiepileptic medications but soon presented with fits and slight expansion in frontal mass. Surgery was performed (right frontal craniotomy), the mass was surgically resected and biopsy indicated RDD. Conclusion: By now only seven of such cases are reported and prognosis of the disease is not poor if surgically treated however other measures including radiotherapy, chemotherapy, steroids are not very effective for treating the disease. And due to the rarity of disease suspicion of such disease should always be kept as a key differential in homogenously enhancing lesions with dural involvement with or without lymphadenopathy.

show abstract

Isolated Intracranial Rosai-Dorfman Disease

Cited by 14 publications

References 15 publications

Postoperative Management of The Primary Single Intracranial Rosai-Dorfman Disease: Case Report and Literature Review

Postoperative Management of The Primary Single Intracranial Rosai-Dorfman Disease: Case Report and Literature Review

Mercaptopurine Treatment in an Adult Man with Orbital and Intracranial Rosai-Dorfman Disease

Isolated intracranial Rosai Dorfman Disease without nodal involvement.

Contact Info

Product

Resources

About