Search tips
Search criteria 


Logo of skullbasesurgInstructions for AuthorsSubscribe to Skull BaseAbout Skull BaseEditorial BoardThieme Medical PublishingSkull Base An Interdisciplinary Approach ...
Skull base surgery. 1998; 8(2): 87–92.
PMCID: PMC1656691

A New Clinical Entity Mimicking Meningioma Diagnosed Pathologically as Rosai-Dorfman Disease


Sinus histiocytosis with massive lymphadenopathy (SHML, Rosai-Dorfman disease), is a well-defined histioproliferative disorder. The extranodal type of the disease is not uncommon, but CNS involvement is extraordinary. Moreover, the isolated extranodal intracranial manifestation of the disease is extremely rare. Only a few cases of the latter form have been reported. We present a case of meningioma-like intracranial lesion, which after total resection, pathologically and immunohistochemically proved to be an isolated form of the Rosai-Dorfman disease. No concurrent nodal involvement was found, even after a detailed postoperative diagnostic work-up.

Two years follow-up disclosed full recovery from the CNS symptoms, while no other SHML signs were noted. The extranodal form of the Rosai-Dorfman Disease, nowadays has been enlisted in the differential diagnosis of the space occupying intracranial and skull base lesions. However, a number of questions, dealing with the preoperative diagnosis, the surgical strategies, as well as the natural history, the prognosis of the disease, and the possible need for adjunctive therapy, remain obscured. Additional reports are useful to better understand this rare type of disease and may lead to the establishment of a more profitable management.

Full text

Full text is available as a scanned copy of the original print version. Get a printable copy (PDF file) of the complete article (3.3M), or click on a page image below to browse page by page. Links to PubMed are also available for Selected References.

Selected References

These references are in PubMed. This may not be the complete list of references from this article.
  • Asai A, Matsutani M, Kohno T, Fujimaki T, Tanaka H, Kawaguchi K, Koike M, Takakura K. Leptomeningeal and orbital benign lymphophagocytic histiocytosis. Case report. J Neurosurg. 1988 Oct;69(4):610–612. [PubMed]
  • Foucar E, Rosai J, Dorfman R. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): review of the entity. Semin Diagn Pathol. 1990 Feb;7(1):19–73. [PubMed]
  • Kim M, Provias J, Bernstein M. Rosai-Dorfman disease mimicking multiple meningioma: case report. Neurosurgery. 1995 Jun;36(6):1185–1187. [PubMed]
  • Bhattacharjee MB, Wroe SJ, Harding BN, Powell M. Sinus histiocytosis with massive lymphadenopathy--isolated suprasellar involvement. J Neurol Neurosurg Psychiatry. 1992 Feb;55(2):156–158. [PMC free article] [PubMed]
  • Trudel M. Dural involvement in sinus histiocytosis with massive lymphadenopathy. Case report. J Neurosurg. 1984 Apr;60(4):850–852. [PubMed]
  • Shaver EG, Rebsamen SL, Yachnis AT, Sutton LN. Isolated extranodal intracranial sinus histiocytosis in a 5-year-old boy. Case report. J Neurosurg. 1993 Nov;79(5):769–773. [PubMed]
  • Mir R, Aftalion B, Kahn LB. Sinus histiocytosis with massive lymphadenopathy and unusual extranodal manifestations. Arch Pathol Lab Med. 1985 Sep;109(9):867–870. [PubMed]
  • Carey MP, Case CP. Sinus histiocytosis with massive lymphadenopathy presenting as a meningioma. Neuropathol Appl Neurobiol. 1987 Sep-Oct;13(5):391–398. [PubMed]
  • Song SK, Schwartz IS, Strauchen JA, Huang YP, Sachdev V, Daftary DR, Vas CJ. Meningeal nodules with features of extranodal sinus histiocytosis with massive lymphadenopathy. Am J Surg Pathol. 1989 May;13(5):406–412. [PubMed]

Articles from Skull Base Surgery are provided here courtesy of Thieme Medical Publishers