A rare case of intracranial rosai-dorfman disease mimicking multiple meningiomas. A case report and review of the literature (original) (raw)

A Rare Case of Intracranial Rosai-Dorfman Disease Mimicking Multiple Meningiomas

The Neuroradiology Journal, 2012

Rosai-Dorfman disease (RDD) was firstly described in 1969 as a benign proliferative disorder of histiocytes with systemic symptoms and lymphadenopathy. This disease is of uncertain pathogenesis and mostly occurs in children and young adults. The typical clinical features of RDD include bilateral painless cervical lymphadenopathy, but extranodal involvement may also be present. The most common extranodal sites include organs such as the respiratory tract, skin, nasal cavity, orbit and bone. Isolated central nervous system (CNS) manifestations are extremely rare. In case of CNS involvement, the commonest imaging findings are dural-based extra-axial enhancing masses. We describe a case of intracranial RDD mimicking multiple meningiomas both clinically and radiologically in a 57-year-old man presenting with a six-year history of progressive right visual and hearing loss and tinnitus. In cases of multiple extra-axial lesions it is worth bearing in mind the possible differential diagnosis...

Rosai-Dorfman disease mimicking images of meningiomas: Two case reports and literature review

Surgical Neurology International, 2021

Background: Rosai-Dorfman disease (RDD) is a rare non-Langerhans cell histiocytic proliferative disorder classically as a massive cervical lymphadenopathy. However, over the years, extranodal locations were confirmed with the central nervous system involvement in less than 5% of cases, which is marked as a significant differential diagnosis of meningiomas, with which they are widely confused due to the similarity of their radiological images. Case Description: We report a 37-year-old man and 45-year-old man who were diagnosed with intracranial RDD but whose radiological images mimic meningiomas, requiring anatomopathological and tumor’s immunohistochemistry for definitive diagnosis. Moreover, a review of 184 publications with 285 cases of intracranial involvement of this disease was also performed, comparing these findings with those brought in the previous studies. Conclusion: Intracranial Rosai-Dorfman tumors should always be remembered as differential diagnosis of meningiomas sin...

Isolated intracranial Rosai–Dorfman disease: A case report with CT and MR findings

European Journal of Radiology Extra, 2007

Rosai-Dorfman disease (RDD) is an idiopathic histiocytic proliferation affecting lymph nodes. Isolated intracranial RDD is rare and usually appears as a well-defined, dura-based lesion without lymphadenopathy. The clinical and radiological features of the intracranial RDD are similar with meningioma. Histological examination is the gold standard for a definitive diagnosis. We present the CT, MR and pathological findings of a 17-year-old girl with isolated intracranial RDD, which manifested as an extraaxial avascular mass with adjacent bone erosion.

Isolated intracranial Rosai-Dorfman disease: Case report and literature review

Pathology International, 1998

Rosai-Dorfman disease, first described in 1969, is a rare idiopathic histioproliferative disease affecting the lymph nodes. Typical clinical features include bilateral painless lymphadenopathy, fever and polyclonal hypergammaglobulinemia. In approximately 43% of cases, extranodal sites may be involved and occasionally represent the initial or sole manifestation of the disease. Central nervous system manifestations are exceedingly rare, and only 17 cases have been recorded, among which there are merely seven isolated intracranial cases without concurrent nodal or other extranodal involvement. Herein, we report a 38-year-old male presenting with generalized tonic-clonic seizure and radiological findings indicative of meningioma. Complete physical examinations and laboratory surveys demonstrate the absence of involvement at other body sites. Microscopically, the lesion consists of proliferative histiocytes exhibiting emperipolesis coupled with the characteristic cytoplasmic staining against S-100 protein. The differential diagnosis of polymorphic inflammatory meningioma-mimicking masses is discussed, and a review of previously reported intracranial Rosai-Dorfman disease is presented.

Isolated intracranial Rosai?-?Dorfman disease mimicking meningioma in a child

British Journal of Neurosurgery, 2004

Rationale: Rosai-Dorfman disease (RDD) is a rare, idiopathic, and non-neoplastic histioproliferative disease with distinctive entity of unknown etiology. Central nervous system (CNS) RDD is uncommon, hence, isolated intracranial RDD is extremely rare. So far only 6 cases of CNS RDD with the lesions originating from petroclival region have been reported. We present a case of isolated intracranial RDD mimicking petroclival meningioma. Patient concerns: A 14-year-old girl was admitted at our hospital with a 3-month history of dizziness, slowly progressing headache, and 2-month history of instability in walking. Cranial nerve deficits, including left facial paralysis, left facial numbness and left hearing loss, were evident on examination. Diagnoses: Initial diagnosis of petroclival meningioma was made according to preoperative magnetic resonance imaging. Interventions: The lesion was resected subtotally and pathology confirmed RDD. The patient received gamma-knife treatment for the residual lesion. Outcomes: The patient recovered well and the residual lesion significantly retrogressed on follow-up images. Lessons: Preoperative diagnosis of petroclival RDD is full of challenges. Although surgical resection of lesions is an effective treatment option, total resection is not highly recommended because the surgery-related defect must be minimal. Patient with residual lesion can be put on steroid therapy and/or radiotherapy, especially for IgG4 positive subset of RDD. Abbreviations: CNS = central nervous system, EMA = epithelial membrane antigen, GFAP = glial fibrillary acidic protein, H-B = House-Brackmann, MRI = magnetic resonance imaging, RDD = Rosai-Dorfman disease.

Intracranial Rosai Dorfman Disease: report of three cases and literature review

Asian journal of neurosurgery, 2010

Rosai-Dorfman Disease (RDD) is a rare idiopathic non-neoplastic histioproliferative disease characterized clinically by massive painless cervical lymphadenopathy, fever and weight loss. Extranodal involvement has also been recognized. Central nervous system (CNS) manifestations are extremely rare and patients with intracranial involvement usually present with clinical and radiological findings suggestive of a meningioma. We report our experience in the management of three patients with RDD. Two patients had dural based lesions, radiologically in favour of a meningioma, and one patient had a parenchymal lesion suggestive of a tuberculous granuloma. Treatment consisted of total excision in one case, and subtotal excision followed by conventional radiotherapy in two cases. The diagnosis was confirmed by histopathology and immunochemistry which is essential for a definite diagnosis of RDD.

A report of intracranial Rosai-Dorfman disease with literature review

Annals of Diagnostic Pathology, 2001

Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy), is a rare benign histiocytic proliferative disorder. Over 650 cases have been reported since 1969. To the best of our knowledge, there have been only 31 cases with central nervous system involvement reported in the literature. Intracranial disease usually presents clinically and radiologically as a "meningioma". It can be misdiagnosed as a nonspecific inflammatory process because of the atypical histologic features of Rosai-Dorfman disease occurring in a non-nodal location. Familiarity with such atypical histologic features and appropriate use of immunohistochemical stains is required for a definitive diagnosis of central nervous system Rosai-Dorfman disease. We report such an intracranial lesion with other extranodal sites of involvement with a 5-year follow up and a review of previously reported cases.

Isolated Intracranial Rosai Dorfman Disease A Rare Presentation with Diagnostic Mimickers

Annals of Pathology and Laboratory Medicine, 2020

Rosai-Dorfman Disease (RDD) is an idiopathic lymphohisticyocytic disorder characterized clinically by massive painless cervical lymphadenopathy, fever and weight loss. Extranodal involvement has been recognized. Central nervous system (CNS) manifestations are rare. Intracranial localization of the disease without involvement of other sites is very rare and needs to be differentiated from various mimicking lesions especially meningioma. A radiological and histological confirmation followed by complete resection of the lesion is the treatment of choice.

Extranodal Rosai-Dorfman disease with multiple spinal lesions: a rare presentation

Surgical Neurology, 2006

Background: Rosai-Dorfman disease is an idiopathic histiocytic proliferative disorder characterized by sinus histiocytosis and massive lymphadenopathy. Extranodal involvement is rare and central nervous system (CNS) involvement is unusual. We present a case of a multiple-level spinal involvement by Rosai-Dorfman disease without cervical lymphadenopathy, which is distinctly rare. Very few cases have been reported in literature. Its resemblance with meningiomas both clinically and radiologically makes its diagnosis all the more difficult. Case Description: A 23-year-old woman presented with quadriparesis and incontinence of urine and stool with a history of being operated on twice for dorsal spine tumor.On examination she had hypoesthesia below the C6 vertebra with anesthesia below D3, and absent joint position and vibration sensations. The patient had spasticity in lower limbs with normal tone in upper limbs. Deep tendon reflexes were hyporeflexic in upper limbs and hyperreflexic in lower limbs with bilateral ankle clonus with bilateral Babinski sign. Magnetic resonance imaging of the cervicodorsal spine was suggestive of multiple intradural extramedullary space-occupying lesion with probability of meningiomas. Surgical resection of 3 tumors was done and sent for histopathology, which was suggestive of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease). The patient had good postoperative recovery and was followed up for 5 months with no new complaints. Conclusion: Rosai-Dorfman disease is a rare entity having clinical and radiological similarity to meningioma. It is similar to lymph node histiocytosis and rarely involves the CNS. It is a benign lymphohistiocytic proliferative condition of unknown etiology. Histopathology showing emperipolesis (lymphophagocytosis) and immunohistochemistry showing S-100 positivity is diagnostic. Surgical resection is the treatment of choice.

Rosai-Dorfman disease manifesting as intracranial and intraorbital lesion

Journal of Neuro-Oncology, 2009

Rosai-Dorfman disease is a rare benign idiopathic histioproliferative disorder usually manifesting as massive painless adenopathy. Extranodal involvement of the Central Nervous System (CNS) mimicking a skull base meningioma is rare. A 42-year-old male presented with painless, progressive left visual loss of 4 months duration. Clinically, he had a left ptosis, proptosis and ophthalmoplegia. Magnetic Resonance Imaging (MRI) of the brain with gadolinium revealed a destructive lesion of the left orbital apex, middle cranial fossa and cavernous sinus. He was treated with corticosteroids and underwent debulking. Pathology showed inflammatory infiltrate in the absence of an infectious agent, emperipolesis and a positive S100 stain was consistent with Rosai-Dorfman disease. As there was no improvement following steroids and debulking, he underwent radiation therapy with significant improvement of his symptoms. Although a rare entity, Rosai-Dorfman disease should be considered in the differential of a skull base lesion. Fig. 3 Touch preparation: H&E 60 9 (orig. mag.): Large histiocytes with emperipolesis and inflammatory cells J Neurooncol