Atypical Case of Rosai-Dorfman Disease of the Lacrimal Gland with Adjacent Bone Erosion

Overview

Journal Ocul Oncol Pathol

Date 2019 Apr 13

PMID 30976592

Authors

Mohammad Dahrouj

Frederick A Jakobiec

Natalie Wolkow

Victoria S Starks

Nahyoung Grace Lee

Affiliations

Soon will be listed here.

Abstract

Background/aims: Rosai-Dorfman disease (RDD) is a rare, self-limited disorder of unknown etiology that affects children and young adults worldwide and typically manifests as chronic, painless cervical lymphadenopathy. Orbital involvement is very rare and may be an isolated extranodal manifestation or associated with concurrent systemic disease. Adjacent bone involvement is most exceptional, and secondary optic neuropathy has never been reported.

Methods: This is a case report with review of the literature.

Results: We present a 32-year-old man who, over a 3-month period, developed worsening vision, headache, and vertical diplopia. On examination, there was decreased vision with dyschromatopsia, proptosis, and hypotropia of the left eye. CT scan of the orbits revealed a soft tissue mass inseparable from the lacrimal gland with adjacent bone erosion. Histopathologic evaluation revealed a diffuse infiltrate of histiocytes, lymphocytes, plasma cells, and neutrophils with peripolesis and emperipolesis (tunneling of lymphocytes and plasma cells in the histiocytes' cytoplasm without destruction), consistent with RDD. Resolution of symptoms as well as of the optic neuropathy was achieved with oral corticosteroids.

Conclusion: RDD is an important diagnosis that must be considered in the differential diagnosis of an orbital mass.

References

Rocken C, Wieker K, Grote H, Muller G, Franke A, Roessner A . Rosai-Dorfman disease and generalized AA amyloidosis: a case report. Hum Pathol. 2000; 31(5):621-4. DOI: 10.1053/hp.2000.6705. View

Rumelt S, Cohen I, Rehany U . Marginal corneal infiltrates: a possible new manifestation of sinus histiocytosis with massive lymphadenopathy. Cornea. 2000; 19(6):857-8. DOI: 10.1097/00003226-200011000-00020. View

Oryschak A, Attariwala G, Ashenhurst M . Rosai-Dorfman disease presenting as bilateral lacrimal gland enlargement. Am J Ophthalmol. 2001; 131(5):677-8. DOI: 10.1016/s0002-9394(00)00864-3. View

Pulsoni A, Anghel G, Falcucci P, Matera R, Pescarmona E, Ribersani M . Treatment of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): report of a case and literature review. Am J Hematol. 2002; 69(1):67-71. DOI: 10.1002/ajh.10008. View

Beckingsale P, Sullivan T, Whitehead K . A case of Rosai-Dorfman disease involving the lacrimal gland in an elderly patient. Orbit. 2002; 21(2):169-75. DOI: 10.1076/orbi.21.2.169.7182. View

Reddy A, Beigi B, Linardos E . Rosai-Dorfman syndrome affecting the lacrimal gland. Orbit. 2002; 20(3):239-242. DOI: 10.1076/orbi.20.3.239.2625. View

Quintyn J, Ranty M, Courville P, Metayer J, Retout A . Orbital sinus histiocytosis (Rosai-Dorfman disease): a lacrimal gland involvement. Ophthalmologica. 2002; 216(4):277-80. DOI: 10.1159/000063842. View

Azoulay R, Brisse H, Freneaux P, Ferey S, Kalifa G, Adamsbaum C . Lacrimal location of sinus histiocytosis (Rosai-Dorfman-Destombes disease). AJNR Am J Neuroradiol. 2004; 25(3):498-500. PMC: 8158550. View

McClain K, Natkunam Y, Swerdlow S . Atypical cellular disorders. Hematology Am Soc Hematol Educ Program. 2004; :283-96. DOI: 10.1182/asheducation-2004.1.283. View

10.

Kaur P, Birbe R, DeHoratius R . Rosai-Dorfman disease in a patient with systemic lupus erythematosus. J Rheumatol. 2005; 32(5):951-3. View

11.

Yuen H, Cheuk W, Leung D, Tse R, Chan N . Atypical presentation of Rosai-Dorfman disease in the lacrimal gland mimicking malignancy. Ophthalmic Plast Reconstr Surg. 2006; 22(2):145-7. DOI: 10.1097/01.iop.0000203303.48983.d7. View

12.

Mohadjer Y, Holds J, Rootman J, Wilson M, Gigantelli J, Custer P . The spectrum of orbital Rosai-Dorfman disease. Ophthalmic Plast Reconstr Surg. 2006; 22(3):163-8. DOI: 10.1097/01.iop.0000217563.00975.a3. View

13.

Mehraein Y, Wagner M, Remberger K, Fuzesi L, Middel P, Kaptur S . Parvovirus B19 detected in Rosai-Dorfman disease in nodal and extranodal manifestations. J Clin Pathol. 2006; 59(12):1320-6. PMC: 1860545. DOI: 10.1136/jcp.2005.029850. View

14.

Gulwani H, Bhalla S, Srivastava A . Isolated lacrimal gland involvement in Rosai-Dorfman-Destombes disease. Indian J Ophthalmol. 2008; 56(6):507-9. PMC: 2612982. DOI: 10.4103/0301-4738.43375. View

15.

Morgan N, Morris M, Cangul H, Gleeson D, Straatman-Iwanowska A, Davies N . Mutations in SLC29A3, encoding an equilibrative nucleoside transporter ENT3, cause a familial histiocytosis syndrome (Faisalabad histiocytosis) and familial Rosai-Dorfman disease. PLoS Genet. 2010; 6(2):e1000833. PMC: 2816679. DOI: 10.1371/journal.pgen.1000833. View

16.

OMalley D, Duong A, Barry T, Chen S, Hibbard M, Ferry J . Co-occurrence of Langerhans cell histiocytosis and Rosai-Dorfman disease: possible relationship of two histiocytic disorders in rare cases. Mod Pathol. 2010; 23(12):1616-23. DOI: 10.1038/modpathol.2010.157. View

17.

Kruse A, Gengler C, Gratz K, Obwegeser J . Extranodal manifestation of Rosai-Dorfman disease without involvement of lymph nodes. J Craniofac Surg. 2010; 21(6):1733-6. DOI: 10.1097/SCS.0b013e3181f403ad. View

18.

Raslan O, Schellingerhout D, Fuller G, Ketonen L . Rosai-Dorfman disease in neuroradiology: imaging findings in a series of 10 patients. AJR Am J Roentgenol. 2011; 196(2):W187-93. DOI: 10.2214/AJR.10.4778. View

19.

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. View

20.

Li J, Ge X, Ma J, Li M . Rosai-Dorfman disease of unilateral lacrimal gland in an elderly Chinese male. Int J Ophthalmol. 2012; 5(4):541-2. PMC: 3428559. DOI: 10.3980/j.issn.2222-3959.2012.04.28. View