Isolated Rosai–Dorfman disease of intracranial meninges

Z'Graggen, Werner Josef; Sturzenegger, Matthias; Mariani, Luigi; Keserue, Borbala; Каппелер, Андреас; Vajtai, István

doi:10.1016/j.prp.2005.11.004

Cited by 32 publications

(18 citation statements)

References 14 publications

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“…It typically occurs in young adults (50% cases between ages 20 and 40 years) and is frequently associated with massive, bilateral, painless cervical lymphadenopathy, fever and weight loss 12 . RDD of the CNS is very uncommon (approximately 5% of cases) and almost invariably presents as an isolated dural‐based mass mimicking a meningioma 13–15 . In our patient, a diagnosis of RDD was ruled‐out based on the lack of classic emperipolesis on histology, absence of S100 immunoreactivity, and uncharacteristic distribution of intracranial lesions.…”

Section: Discussionmentioning

confidence: 80%

Non-Langerhans cell histiocytosis with isolated CNS involvement: An unusual variant of Erdheim-Chester disease

Conley¹,

Manjila²,

Guan

et al. 2010

Neuropathology

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Benign histiocytic proliferations are identified by their component cells and classified as either Langerhans cell histiocytosis or non-Langerhans cell histiocytosis. We report a 58-year-old Caucasian woman who presented with diabetes insipidus and was found to harbor a large suprasellar mass. Histopathological analysis was consistent with non-LCH. The differential diagnoses included juvenile xanthogranuloma, adult-onset xanthogranuloma, xanthoma disseminatum, Rosai-Dorfman disease, and Erdheim-Chester disease. Immunohistochemical examination demonstrated a proliferation of large lipid-laden histiocytic cells which were positive for CD68, negative for S100 protein, and showed only faint, background staining for CD1a. We present a case of an autopsy-confirmed non-Langerhans cell histiocytosis limited to the central nervous system and evaluated with both immunohistochemical and ultrastructural studies. Based on the multifocality, anatomic distribution, and immunostaining features, a diagnosis of Erdheim-Chester disease was made. This is only the second reported case of Erdheim-Chester disease with intracranial involvement but absence of extracerebral manifestations. Given the overlapping clinicopathologic, radiographic, and immunohistochemical profiles, differentiating between these rare histiocytic disorders can often present a significant diagnostic challenge. A systematic approach using all available clinical, laboratory, radiographic, histologic, immunohistochemical and ultrastructural data is essential for proper discrimination between the numerous histiocytoses.

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Section: Discussionmentioning

confidence: 80%

Non-Langerhans cell histiocytosis with isolated CNS involvement: An unusual variant of Erdheim-Chester disease

Conley¹,

Manjila²,

Guan

et al. 2010

Neuropathology

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“…Both large and medium size histiocytes are positive for CD68 and negative for CD1a. 16,25 The differential diagnosis includes meningioma, histiocytosis X (i.e. eosinophilic granuloma), lymphoproliferative disorders, plasma cell granulomas and infectious disease.…”

Section: Discussionmentioning

confidence: 99%

Isolated Intracranial Rosai-Dorfman Disease Mimicking Suprasellar Meningioma: Case Report with Review of the Literature

et al. 2008

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Rosai-Dorfman disease (RDD) is an idiopathic histiocytic proliferation affecting the lymph nodes. Isolated intracranial RDD is rare and usually appears as a well-defined, dural-based lesion without lymphadenopathy. The clinical and radiological features of intracranial RDD are similar to meningioma. Histopathology and immunohistochemistry are essential for a definitive diagnosis. This is a report of a 43-year old male with isolated intracranial RDD, which manifested as a suprasellar meningioma. The clinical, radiological and pathological aspects of the disease are discussed within the context of a review of previously reported cases.

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“…However, the steroid doses reported for RDD have been 1–2 mg/kg per day of prednisolone, 2–4 70 mg/day of prednisolone, 5 60 mg/kg of prednisolone (oral pulse therapy), 6 8–20 mg/day of dexamethasone, 7,8 and 500 mg/day of i.v. methylprednisolone, 9 all of which are considerably higher than the dose applied in this patient. Although cases in which oral steroid therapy was ineffective for RDD have been reported, 10,11 the present case not only strengthens the case for the effectiveness of oral steroid therapy, but also indicates that low‐dose oral corticosteroid therapy should be considered as the first therapeutic option before aggressive surgery and high‐dose steroid.…”

Section: Discussionmentioning

confidence: 65%

Successful treatment of Rosai–Dorfman disease with low‐dose oral corticosteroid

Oka¹,

Kamo²,

Goto³

et al. 2009

The Journal of Dermatology

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We present herein a Japanese case of Rosai-Dorfman disease (RDD) in which cutaneous manifestations completely remitted after treatment with low-dose oral corticosteroid. A 69-year-old Japanese man presented with a 1-year history of enlarged submandibular lymph nodes and subsequent nasal and pharyngeal bleeding. RDD was diagnosed based on biopsy results from a lymph node in the left parotid region. The patient had also noted several skin eruptions that repeatedly appeared and disappeared on the face and arms. Biopsies were taken from skin eruptions on the face and cuboidal fossa. Both biopsy specimens showed dense, well-demarcated infiltration of histiocytes, lymphocytes and multinucleated giant cells from just under the epidermis to the subcutaneous tissue. These histiocytes were positive for CD68 and S-100, but negative for CD1a, and some displayed emperipolesis. Given the histopathological findings and the fact that the patient was suffering from RDD, skin lesions were diagnosed as cutaneous manifestations of RDD. Cutaneous lesions gradually began to persist concomitant with enlargement of extranodal lymphadenopathy in the nasopharyngeal area. Increasing respiratory obstruction prompted a trial with oral prednisolone commencing at 0.4 mg/kg per day. Both the lymphadenopathy and skin lesions responded quickly. Within 3 months, all his skin lesions disappeared completely with almost complete resolution of lymphadenopathy. Twelve months after the beginning of oral prednisolone therapy, slight recurrence of mucosal and cutaneous lesions appeared, but disappeared quickly with an increase in prednisolone to 0.3 mg/kg per day. Low-dose prednisolone appeared very effective in the case of RDD.

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Isolated Rosai–Dorfman disease of intracranial meninges

Cited by 32 publications

References 14 publications

Non-Langerhans cell histiocytosis with isolated CNS involvement: An unusual variant of Erdheim-Chester disease

Non-Langerhans cell histiocytosis with isolated CNS involvement: An unusual variant of Erdheim-Chester disease

Isolated Intracranial Rosai-Dorfman Disease Mimicking Suprasellar Meningioma: Case Report with Review of the Literature

Successful treatment of Rosai–Dorfman disease with low‐dose oral corticosteroid

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