Extranodal Rosai-Dorfman disease involving paranasal sinuses, orbits and anterior cranial fossa
Rosai-Dorfman disease (RDD) is a rare, benign pseudolymphatous condition, predominantly involving lymph nodes. Although several cases of extra-nodal involvement have been reported previously, central nervous system involvement, particularly in the absence of nodal disease is extremely rare. Extranod...
Saved in:
| Main Authors: | , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Thieme Medical and Scientific Publishers Pvt. Ltd.
2014-05-01
|
| Series: | Indian Journal of Neurosurgery |
| Subjects: | |
| Online Access: | http://www.thieme-connect.de/DOI/DOI?10.4103/2277-9167.138920 |
| Tags: |
Add Tag
No Tags, Be the first to tag this record!
|
| _version_ | 1850182521391677440 |
|---|---|
| author | Sudhansu Sekhar Mishra Souvagya Panigrahi Kalpalata Tripathy Niranjan Mishra Aparna Dwibedi |
| author_facet | Sudhansu Sekhar Mishra Souvagya Panigrahi Kalpalata Tripathy Niranjan Mishra Aparna Dwibedi |
| author_sort | Sudhansu Sekhar Mishra |
| collection | DOAJ |
| description | Rosai-Dorfman disease (RDD) is a rare, benign pseudolymphatous condition, predominantly involving lymph nodes. Although several cases of extra-nodal involvement have been reported previously, central nervous system involvement, particularly in the absence of nodal disease is extremely rare. Extranodal large RDD presenting as a single lesion involving sino-orbital and anterior cranial fossa has rarely been described previously. We report a case of incisional biopsy proved RDD in a young lady who presented with nasal obstruction and subsequent proptosis with visual diminution. Radiography of head and paranasal sinus demonstrated a strongly enhanced, diffuse polypoid lesion filling the bilateral sinonasal cavity and orbit with extension to the anterior cranial fossa by way of splaying the bony foramina. Pre-operative low dose steroid therapy had resulted in decreased size of the mass which facilitate gross-total surgical resection. RDD was confirmed by histopathology (emperipolesis) and immuno-histochemistry (S-100 positivity). The follow-up computed tomography 3 months later showed minimal tumor residue in left parasellar region with complete sinonasal decompression. |
| format | Article |
| id | doaj-art-444386bc6d70410eb931ec2f1c9c4a96 |
| institution | OA Journals |
| issn | 2277-954X 2277-9167 |
| language | English |
| publishDate | 2014-05-01 |
| publisher | Thieme Medical and Scientific Publishers Pvt. Ltd. |
| record_format | Article |
| series | Indian Journal of Neurosurgery |
| spelling | doaj-art-444386bc6d70410eb931ec2f1c9c4a962025-08-20T02:17:37ZengThieme Medical and Scientific Publishers Pvt. Ltd.Indian Journal of Neurosurgery2277-954X2277-91672014-05-01030211011410.4103/2277-9167.138920Extranodal Rosai-Dorfman disease involving paranasal sinuses, orbits and anterior cranial fossaSudhansu Sekhar MishraSouvagya PanigrahiKalpalata Tripathy0Niranjan Mishra1Aparna Dwibedi2Pathology, S.C.B. Medical College and HospitalOromaxillofacial Surgery, S.C.B. Medical College and HospitalOphthalmology, S.C.B. Medical College and HospitalRosai-Dorfman disease (RDD) is a rare, benign pseudolymphatous condition, predominantly involving lymph nodes. Although several cases of extra-nodal involvement have been reported previously, central nervous system involvement, particularly in the absence of nodal disease is extremely rare. Extranodal large RDD presenting as a single lesion involving sino-orbital and anterior cranial fossa has rarely been described previously. We report a case of incisional biopsy proved RDD in a young lady who presented with nasal obstruction and subsequent proptosis with visual diminution. Radiography of head and paranasal sinus demonstrated a strongly enhanced, diffuse polypoid lesion filling the bilateral sinonasal cavity and orbit with extension to the anterior cranial fossa by way of splaying the bony foramina. Pre-operative low dose steroid therapy had resulted in decreased size of the mass which facilitate gross-total surgical resection. RDD was confirmed by histopathology (emperipolesis) and immuno-histochemistry (S-100 positivity). The follow-up computed tomography 3 months later showed minimal tumor residue in left parasellar region with complete sinonasal decompression.http://www.thieme-connect.de/DOI/DOI?10.4103/2277-9167.138920emperipolesisextranodalintracranialrosai-dorfman diseasesino-orbital |
| spellingShingle | Sudhansu Sekhar Mishra Souvagya Panigrahi Kalpalata Tripathy Niranjan Mishra Aparna Dwibedi Extranodal Rosai-Dorfman disease involving paranasal sinuses, orbits and anterior cranial fossa Indian Journal of Neurosurgery emperipolesis extranodal intracranial rosai-dorfman disease sino-orbital |
| title | Extranodal Rosai-Dorfman disease involving paranasal sinuses, orbits and anterior cranial fossa |
| title_full | Extranodal Rosai-Dorfman disease involving paranasal sinuses, orbits and anterior cranial fossa |
| title_fullStr | Extranodal Rosai-Dorfman disease involving paranasal sinuses, orbits and anterior cranial fossa |
| title_full_unstemmed | Extranodal Rosai-Dorfman disease involving paranasal sinuses, orbits and anterior cranial fossa |
| title_short | Extranodal Rosai-Dorfman disease involving paranasal sinuses, orbits and anterior cranial fossa |
| title_sort | extranodal rosai dorfman disease involving paranasal sinuses orbits and anterior cranial fossa |
| topic | emperipolesis extranodal intracranial rosai-dorfman disease sino-orbital |
| url | http://www.thieme-connect.de/DOI/DOI?10.4103/2277-9167.138920 |
| work_keys_str_mv | AT sudhansusekharmishra extranodalrosaidorfmandiseaseinvolvingparanasalsinusesorbitsandanteriorcranialfossa AT souvagyapanigrahi extranodalrosaidorfmandiseaseinvolvingparanasalsinusesorbitsandanteriorcranialfossa AT kalpalatatripathy extranodalrosaidorfmandiseaseinvolvingparanasalsinusesorbitsandanteriorcranialfossa AT niranjanmishra extranodalrosaidorfmandiseaseinvolvingparanasalsinusesorbitsandanteriorcranialfossa AT aparnadwibedi extranodalrosaidorfmandiseaseinvolvingparanasalsinusesorbitsandanteriorcranialfossa |