Steroid-Resistant Extranodal Rosai-Dorfman Disease of Cheek Mass and Ptosis Treated with Radiation Therapy
Background. Rosai-Dorfman Disease (RDD) is rare benign hematologic disorder of histiocytes, which usually manifests as painless lymphadenopathy, fever, leukocytosis and hypergammaglobulinemia. Extranodal RDD has been reported in 43% of cases, with skin as commonly involved site followed by head and...
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| Format: | Article |
| Language: | English |
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Wiley
2013-01-01
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| Series: | Case Reports in Hematology |
| Online Access: | http://dx.doi.org/10.1155/2013/428297 |
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| author | Ahmed Marzouk Maklad Yasser Bayoumi Mutahir Tunio Wafaa AlShakweer Mashooque A. Dahar Shomaila A. Akbar |
| author_facet | Ahmed Marzouk Maklad Yasser Bayoumi Mutahir Tunio Wafaa AlShakweer Mashooque A. Dahar Shomaila A. Akbar |
| author_sort | Ahmed Marzouk Maklad |
| collection | DOAJ |
| description | Background. Rosai-Dorfman Disease (RDD) is rare benign hematologic disorder of histiocytes, which usually manifests as painless lymphadenopathy, fever, leukocytosis and hypergammaglobulinemia. Extranodal RDD has been reported in 43% of cases, with skin as commonly involved site followed by head and neck region. However, soft tissue cheek mass is rare presentation of extra-nodal RDD. Case Presentation. A 26-year-old Saudi man presented with a six-month history of right cheek swelling and left upper eyelid swelling. Physical examination revealed right cheek mass of size cm and left upper eyelid mass of size cm and no palpable cervical lymphadenopathy. Incisional biopsy of cheek mass showed positivity for S100 and negativity for CD1a, consistent with extra-nodal RDD. Patient did not respond to systemic steroids and was given radiation therapy to left orbit with minimal response. Then, he was started on chemotherapy Rituximab, cyclophosphamide, vincristine, and prednisolone (RCVP) 8 cycles followed by reirradiation 10 Gy in 10 fractions with 6 MeV electron with complete response. After 12 months of followup, patient had recurrence in right cheek and was started on radiotherapy to the cheek mass. Conclusion. Extra-nodal RDD with involvement of cheek is a rare presentation. Incorporation of S100 and CD1a is helpful in diagnoses of RDD and differentiating it from other benign histiocytosis. Treatment consists of surgery, steroids, chemotherapy, and radiation therapy. |
| format | Article |
| id | doaj-art-6ee590083e6e476a90881040c4f2c0a7 |
| institution | Kabale University |
| issn | 2090-6560 2090-6579 |
| language | English |
| publishDate | 2013-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Hematology |
| spelling | doaj-art-6ee590083e6e476a90881040c4f2c0a72025-02-03T06:06:08ZengWileyCase Reports in Hematology2090-65602090-65792013-01-01201310.1155/2013/428297428297Steroid-Resistant Extranodal Rosai-Dorfman Disease of Cheek Mass and Ptosis Treated with Radiation TherapyAhmed Marzouk Maklad0Yasser Bayoumi1Mutahir Tunio2Wafaa AlShakweer3Mashooque A. Dahar4Shomaila A. Akbar5Clinical Oncology Department, Sohag University, Sohag, EgyptRadiation Oncology Department, NCI, Cairo University, Cairo, EgyptRadiation Oncology Department, Comprehensive Cancer Center, King Fahad Medical City, Riyadh 59046, Saudi ArabiaPathology Department, Comprehensive Cancer Center, King Fahad Medical City, Riyadh 59046, Saudi ArabiaHematooncology Department, Comprehensive Cancer Center, King Fahad Medical City, Riyadh 59046, Saudi ArabiaRadiation Oncology Department, Comprehensive Cancer Center, King Fahad Medical City, Riyadh 59046, Saudi ArabiaBackground. Rosai-Dorfman Disease (RDD) is rare benign hematologic disorder of histiocytes, which usually manifests as painless lymphadenopathy, fever, leukocytosis and hypergammaglobulinemia. Extranodal RDD has been reported in 43% of cases, with skin as commonly involved site followed by head and neck region. However, soft tissue cheek mass is rare presentation of extra-nodal RDD. Case Presentation. A 26-year-old Saudi man presented with a six-month history of right cheek swelling and left upper eyelid swelling. Physical examination revealed right cheek mass of size cm and left upper eyelid mass of size cm and no palpable cervical lymphadenopathy. Incisional biopsy of cheek mass showed positivity for S100 and negativity for CD1a, consistent with extra-nodal RDD. Patient did not respond to systemic steroids and was given radiation therapy to left orbit with minimal response. Then, he was started on chemotherapy Rituximab, cyclophosphamide, vincristine, and prednisolone (RCVP) 8 cycles followed by reirradiation 10 Gy in 10 fractions with 6 MeV electron with complete response. After 12 months of followup, patient had recurrence in right cheek and was started on radiotherapy to the cheek mass. Conclusion. Extra-nodal RDD with involvement of cheek is a rare presentation. Incorporation of S100 and CD1a is helpful in diagnoses of RDD and differentiating it from other benign histiocytosis. Treatment consists of surgery, steroids, chemotherapy, and radiation therapy.http://dx.doi.org/10.1155/2013/428297 |
| spellingShingle | Ahmed Marzouk Maklad Yasser Bayoumi Mutahir Tunio Wafaa AlShakweer Mashooque A. Dahar Shomaila A. Akbar Steroid-Resistant Extranodal Rosai-Dorfman Disease of Cheek Mass and Ptosis Treated with Radiation Therapy Case Reports in Hematology |
| title | Steroid-Resistant Extranodal Rosai-Dorfman Disease of Cheek Mass and Ptosis Treated with Radiation Therapy |
| title_full | Steroid-Resistant Extranodal Rosai-Dorfman Disease of Cheek Mass and Ptosis Treated with Radiation Therapy |
| title_fullStr | Steroid-Resistant Extranodal Rosai-Dorfman Disease of Cheek Mass and Ptosis Treated with Radiation Therapy |
| title_full_unstemmed | Steroid-Resistant Extranodal Rosai-Dorfman Disease of Cheek Mass and Ptosis Treated with Radiation Therapy |
| title_short | Steroid-Resistant Extranodal Rosai-Dorfman Disease of Cheek Mass and Ptosis Treated with Radiation Therapy |
| title_sort | steroid resistant extranodal rosai dorfman disease of cheek mass and ptosis treated with radiation therapy |
| url | http://dx.doi.org/10.1155/2013/428297 |
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