A report of intracranial Rosai-Dorfman disease with literature review

doi:10.1053/adpa.2001.23027

Annals of Diagnostic Pathology

Volume 5, Issue 2, April 2001, Pages 96-102

https://doi.org/10.1053/adpa.2001.23027 Get rights and content

Abstract

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. Ann Diagn Pathol 5:96-102, 2001.

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Cited by (75)

Malignant behaviour of primary intracranial Rosai Dorfman disease: A rare presentation of a benign disease
2021, Neurochirurgie
Citation Excerpt :
S-100 positivity is consistent with the diagnosis of RDD and Langerhans cell histiocytosis, but allows to exclude Erdehim-Chester disease [23]. Unlike Langerhans cell histiocytosis, histiocytes in RDD typically do not show indented or reniform nuclei, do not contain Birbeck granules [11], and are negative for CD1a, langerhin (CD207) and mutant BRAF (V600E) protein [22]. Treatment of intracranial RDD is controversial and includes surgical resection, medical and radiation therapies [6].
The Rosai Dorfman disease (RDD) is a rare and usually benign lymphoproliferative disorder of unknown aetiology, typically characterized by head and neck lymphadenopathies. However, it may occasionally present with primary extra-nodal involvement including the central nervous system. We firstly described the potential malignant behaviour and fatal evolution of primary intracranial RDD.
A 69-year-old woman sought clinical attention for recurrent episodes of headache, fever and malaise associated with bilateral proptosis and left lower limb paresis. The brain MRI revealed bilateral retro-bulbar and fronto-parieto-occipital subdural lesions. The body CT did not show extracranial lesions. A right fronto-parietal craniotomy was performed and a subtotal resection was achieved. The histopathological diagnosis was consistent with RDD. Despite the patient was commenced on high-dose corticosteroids, she developed a worsening respiratory distress syndrome and a rapid systemic disease progression with liver and kidney failure. Given the poor general status, adjuvant medical and radiation therapies were not deemed safe and feasible and the patient died of multi-organ failure a month later.
We documented an exceptional case of primary intracranial RDD with malignant behaviour characterized by rapid systemic disease progression and poor prognosis. Although RDD with intracranial location has usually a benign and self-limiting course with good response to adjuvant treatments, it hides a malignant potential that may lead patients to death.
Craniocervical Rosai-Dorfman Disease Involving the Vertebral Artery: Case Report and Literature Review
2020, World Neurosurgery
Rosai-Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy, is a rare non–Langerhans cell reactive histiocytic disorder that rarely occurs in the central nervous system (CNS). Extranodal RDD most frequently involves the skin, upper respiratory tract, soft tissue, gastrointestinal tracts, bones, breast, and CNS.
RDD of the CNS infiltrates most commonly the dura of the sella, cavernous sinus, and the periclival regions. It is usually clinically and radiologically mistaken for meningioma because of its focal dural-based aspect. RDD is confirmed histologically by lymphoplasmacytic cells and histiocytes of varying size showing emperipolesis (lymphocytophagocytosis). To date, only 4 cases of RDD displaying spinal cord compression secondary to craniocervical junction involvement have been reported.
We report the case of a patient diagnosed with RDD localized at the foramen magnum extending to the base of the odontoid process and involving the V4 segment vertebral artery.
Intracranial Rosai-Dorfman Disease Involving the Cavernous Sinus: A Case Report and Review of the Literature
2018, World Neurosurgery
Citation Excerpt :
The immunohistochemical findings will be immunoreactive for S-100 protein, CD68 antigen, and vimentin but negative for CD1a, CD15, and CD30, without cellular atypia and mitosis. These findings make it difficult to differentiate from meningioma, Langerhans cell histiocytosis or histiocytosis X, lymphoproliferative disorders, plasma cell granulomas, Hodgkin's lymphoma, and infectious processes in the CNS.6,8,9,19,27,29,32 Although some hypotheses have been proposed, the etiology of RDD remains unclear.
Rosai-Dorfman disease (RDD), also called sinus histiocytosis with massive lymphadenopathy, is an idiopathic, non-neoplastic, lymphoproliferative disorder. Histologically, the disease is characterized by emperipolesis and S-100 immunoreactivity. RDD usually presents with massive painless cervical lymphadenopathy, with only 5% of cases affecting the central nervous system.
We report the case of a 46-year-old woman with isolated RDD of the left cavernous sinus with left facial numbness and weakness of the left masticatory muscle. The patient was successfully treated by total resection without recurrence found at the 65-month follow-up examination.
Central nervous system RDD is a diagnostic challenge preoperatively depending on the radiologic findings. Immunohistochemical confirmation is indispensable for the definite diagnosis. Total resection remains the most effective treatment to date. Adjuvant treatment such as chemotherapy, radiotherapy, and steroids can be administrated in cases of incomplete resection, recurrence, or multiple foci.
Rosai-Dorfman Disease Presenting as Peripheral Vascular Insufficiency
2018, Annals of Vascular Surgery
Rosai-Dorfman (-Destombes) disease (RDDD) is a rare idiopathic disorder of histiocyte proliferation, usually involving lymph node stations. The most common clinical finding is a bilateral cervical lymphadenopathy, fever, and weight loss. Arterial or venous structures are notably not involved. We hereby present a case of a 78-year-old Caucasian man, presenting with symptoms of progressive arterial insufficiency and right lower-limb edema, along with a nonpulsatile mass at the middle third of the thigh. Initial diagnostic hypothesis was a superficial femoral artery aneurysm thrombosis with a secondary postcompressive superficial femoral vein thrombosis. Duplex examination showed right superficial femoral arterial and venous thrombosis, along with a hypoechogenic mass causing compression of the neurovascular bundle. Suspecting a connective tissue sarcoma, computed tomography scan was performed after combined en bloc removal of the mass along with femoral artery and vein and prosthetic reconstruction of vascular continuity. Histopathology diagnosis was connective tissue RDDD. The atypical presentation of this rare syndrome induces us to include in differential diagnosis, among other more common forms of external compression of the neurovascular bundles, even rare conditions such as these, which generally only involve lymphatic stations.
Intracranial Rosai–Dorfman disease
2016, Journal of Clinical Neuroscience
Citation Excerpt :
T1-weighted MRI showed that the intracranial RDD was usually an isointense or hyperintense mass with distinct borders, and had avid homogenous contrast enhancement. On T2-weighted scanning, the lesions usually presented as isointense [19]. Moreover, an exceptional case was also reported where the lesion could be isointense on T1-weighted but hyperintense on T2-weighted MRI with minimal contrast enhancement [8].
Rosai–Dorfman disease (RDD) is a rare histioproliferative disorder that only occasionally involves the central nervous system. We present the diagnosis and treatment of five patients with intracranial RDD. The patients were preoperatively misdiagnosed as meningioma or eosinophilic granuloma. All five patients were treated by total or subtotal surgical resection and none of them experienced recurrence. Histopathological examination showed a characteristic emperipolesis, the lymphocytes were engulfed in the S-100 protein and CD68 positive histiocytes, with negative expression of CD1a. Preoperative diagnosis of intracranial RDD is still challenging because the lesion is usually a dural-based lesion that mimics a meningioma. Surgical resection is an effective treatment and radiotherapy, steroid and chemotherapy has not demonstrated reliable therapeutic efficiency.
Radiology-pathology case report: Isolated extranodal Rosai-Dorfman disease of the skull base
2013, Clinical Imaging
Citation Excerpt :
In a recent series of head and neck RD, exclusive extranodal disease occurred in 38% (5/13) of patients, dural based lesions in exclusive extranodal disease occurred in 15% (2/13) of patients, and primary lytic bone lesions occurred in only 8% (1/13) [4]. On imaging, CNS RD most commonly resembles a meningioma and definite differentiation is often not possible [3,5,6]. Radiologic and nuclear imaging can add some diagnostic value between these two entities, however.
Rosai–Dorfman disease is a rare pathologic entity characterized by massively enlarged painless cervical lymph nodes. Exclusive extranodal disease is much less common than the characteristic presentation with exclusive central nervous system and skull base involvement being quite rare. We present a case of exclusive extranodal Rosai–Dorfman disease of the skull base and briefly discuss the entity.

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Radiologic-Pathologic CorrelationsA report of intracranial Rosai-Dorfman disease with literature review

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Radiologic-Pathologic Correlations
A report of intracranial Rosai-Dorfman disease with literature review