J Neurol Surg A Cent Eur Neurosurg 2015; 76(03): 244-248
DOI: 10.1055/s-0034-1382780
Case Report
Georg Thieme Verlag KG Stuttgart · New York

A Singular Case of Intracranial Sinus Histiocytosis without Massive Lymphadenopathy: Isolated Rosai-Dorfman Disease of the Hypothalamus

Srinivas Chivukula
1   School of Medicine, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, United States
Kenneth Clark
2   Department of Pathology, University of Pittsburgh, Pittsburgh, Pennsylvania, United States
Geoffrey Murdoch
2   Department of Pathology, University of Pittsburgh, Pittsburgh, Pennsylvania, United States
Johnathan Engh
3   Department of Neurological Surgery, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania, United States
› Author Affiliations
Further Information

Publication History

12 August 2013

14 February 2014

Publication Date:
27 June 2014 (online)


Background Sinus histiocytosis with massive lymphadenopathy (also known as Rosai-Dorfman disease [RDD]) is a benign but chronic cervical lymphadenopathy associated with systemic inflammation. Although extranodal manifestations of RDD have been described, isolated central nervous system (CNS) involvement is exceedingly rare.

Case Description We present the case of a 66-year-old woman who presented with 3 weeks of intermittent headaches, diplopia, and increasing confusion who was found on work-up to have isolated hypothalamic RDD, evidenced by a dense admixture of large histiocytic cells admixed with numerous small mature lymphocytes and some scattered plasma cells and neutrophils on stereotactic brain biopsy. Over 19 months of follow-up, neurologic examination continues to reveal stable bilateral partial abducens nerve palsies without diplopia, and a new gradual onset short-term memory loss. Interim treatment for the histiocytic lesion consisted of 10 cycles of external-beam radiation therapy along with high-dose steroids. The patient currently experiences minimal functional loss from treatment of her intracranial sinus histiocytosis, with a Karnofsky performance status of 80, and she remains without any disease involvement outside of the CNS.

Conclusion Because misdiagnosis of a hypothalamic contrast-enhancing lesion could potentially lead to therapeutic mismanagement and poor outcomes, it is important to consider RDD in the differential diagnosis.

  • References

  • 1 Foucar E, Rosai J, Dorfman R. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): review of the entity. Semin Diagn Pathol 1990; 7 (1) 19-73
  • 2 Shuangshoti Jr S, Navalitloha Y, Sukpanichnant S , et al. Central nervous system involvement in Rosai-Dorfman disease: report of a case with a review of the literature. Neuropathology 1999; 19: 341-346
  • 3 Wu M, Anderson AE, Kahn LB. A report of intracranial Rosai-Dorfman disease with literature review. Ann Diagn Pathol 2001; 5 (2) 96-102
  • 4 Andriko JA, Morrison A, Colegial CH, Davis BJ, Jones RV. Rosai-Dorfman disease isolated to the central nervous system: a report of 11 cases. Mod Pathol 2001; 14 (3) 172-178
  • 5 Warrier R, Chauhan A, Jewan Y, Bansal S, Craver R. Rosai-Dorfman disease with CNS involvement. Clin Adv Hematol Oncol 2012; 10 (3) 196-202
  • 6 Adeleye AO, Amir G, Fraifeld S, Shoshan Y, Umansky F, Spektor S. Diagnosis and management of Rosai-Dorfman disease involving the central nervous system. Neurol Res 2010; 32 (6) 572-578
  • 7 Cooper SL, Jenrette III JM. Rosai-Dorfman disease: management of CNS and systemic involvement. Clin Adv Hematol Oncol 2012; 10 (3) 199-202
  • 8 Asai A, Matsutani M, Kohno T , et al. Leptomeningeal and orbital benign lymphophagocytic histiocytosis. Case report. J Neurosurg 1988; 69 (4) 610-612
  • 9 Rosai J. Rosai and Ackerman's Surgical Pathology. 9th ed. London: UK: Elsevier; 2004
  • 10 Shi Y, Griffin AC, Zhang PJ, Palmer JN, Gupta P. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): a case report and review of 49 cases with fine needle aspiration cytology. Cytojournal 2011; 8: 3
  • 11 Mannan AA, Karak AK. Sinus histiocytosis with massive lymphadenopathy (Rosai Dorfman disease): evidence for a dendritic cell derivation?. Indian J Pathol Microbiol 2005; 48 (3) 300-304
  • 12 Kidd DP, Revesz T, Miller NR. Rosai-Dorfman disease presenting with widespread intracranial and spinal cord involvement. Neurology 2006; 67 (9) 1551-1555