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Rosai-Dorfman disease affecting the maxilla

View Article: PubMed Central - PubMed

ABSTRACT

Rosai-Dorfman disease (RDD), formerly called sinus histiocytosis with massive lymphadenopathy, is a non-neoplastic proliferative histiocytic disorder with behavior ranging from highly aggressive to spontaneous remission. Although the lymph nodes are more commonly involved, any organ can be affected. This study aimed to describe the features and the follow-up of a case of extranodal RDD. Our patient was a 39-year-old woman who was referred with an 11-month history of pain in the right maxilla. On clinical examination, some upper right teeth presented full mobility with normal appearance of the surrounding gingiva. Radiographic exams showed an extensive bone reabsorption and maxillary sinus filled with homogeneous tissue, which sometimes showed polypoid formation. An incisional biopsy demonstrated a diffuse inflammatory infiltrate rich in foamy histiocytes displaying lymphocytes emperipolesis. Immunohistochemistry showed positivity for CD68 and S-100, and negativity for CD3, CD20, and CD30. Such features were consistent with the RDD diagnosis. The patient was referred to a hematologist and corticotherapy was administrated for 6 months. RDD is an uncommon disease that rarely affects the maxilla. In the present case, the treatment was conservative, and the patient is currently asymptomatic after 5 years of follow-up.

No MeSH data available.


Photomicrography of the biopsy. A - A diffuse inflammatory infiltrate rich in xanthomatous macrophages (H&E 40X); B - Evident lymphocytes emperipolesis is also observed (arrow) (H&E 200X); C - CD3 positive for reactive cells (T lymphocyte cells); D - CD68 showed strong positivity for macrophages with lymphocytes emperipolesis (arrow); E - S-100 was positive in histiocytic cells.
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g04: Photomicrography of the biopsy. A - A diffuse inflammatory infiltrate rich in xanthomatous macrophages (H&E 40X); B - Evident lymphocytes emperipolesis is also observed (arrow) (H&E 200X); C - CD3 positive for reactive cells (T lymphocyte cells); D - CD68 showed strong positivity for macrophages with lymphocytes emperipolesis (arrow); E - S-100 was positive in histiocytic cells.

Mentions: The main clinical diagnostic hypotheses were Langerhans cell histiocytosis (LCH), NK/T-cell lymphoma, and sinus carcinoma. An incisional biopsy of the alveolar bone (upper right canine) was performed, and the histopathological analysis showed an intense diffuse mononuclear cell infiltration rich in xanthomatous cells, some of which had evident emperipolesis of the lymphocytes. Areas of necrosis were also observed (Figure 4A). The immunohistochemical reactions showed positivity for CD68 and S-100 protein in the xanthomatous cells, and negativity for CD3, CD20, CD30, and CD1a (Figure 4B-E). Considering the clinical, histopathological and immunohistochemical features, the final diagnosis was RDD involving the right maxilla and the sinus. The peripheral blood count showed mild anemia, and normal leukocyte and platelet counts.


Rosai-Dorfman disease affecting the maxilla
Photomicrography of the biopsy. A - A diffuse inflammatory infiltrate rich in xanthomatous macrophages (H&E 40X); B - Evident lymphocytes emperipolesis is also observed (arrow) (H&E 200X); C - CD3 positive for reactive cells (T lymphocyte cells); D - CD68 showed strong positivity for macrophages with lymphocytes emperipolesis (arrow); E - S-100 was positive in histiocytic cells.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC5304562&req=5

g04: Photomicrography of the biopsy. A - A diffuse inflammatory infiltrate rich in xanthomatous macrophages (H&E 40X); B - Evident lymphocytes emperipolesis is also observed (arrow) (H&E 200X); C - CD3 positive for reactive cells (T lymphocyte cells); D - CD68 showed strong positivity for macrophages with lymphocytes emperipolesis (arrow); E - S-100 was positive in histiocytic cells.
Mentions: The main clinical diagnostic hypotheses were Langerhans cell histiocytosis (LCH), NK/T-cell lymphoma, and sinus carcinoma. An incisional biopsy of the alveolar bone (upper right canine) was performed, and the histopathological analysis showed an intense diffuse mononuclear cell infiltration rich in xanthomatous cells, some of which had evident emperipolesis of the lymphocytes. Areas of necrosis were also observed (Figure 4A). The immunohistochemical reactions showed positivity for CD68 and S-100 protein in the xanthomatous cells, and negativity for CD3, CD20, CD30, and CD1a (Figure 4B-E). Considering the clinical, histopathological and immunohistochemical features, the final diagnosis was RDD involving the right maxilla and the sinus. The peripheral blood count showed mild anemia, and normal leukocyte and platelet counts.

View Article: PubMed Central - PubMed

ABSTRACT

Rosai-Dorfman disease (RDD), formerly called sinus histiocytosis with massive lymphadenopathy, is a non-neoplastic proliferative histiocytic disorder with behavior ranging from highly aggressive to spontaneous remission. Although the lymph nodes are more commonly involved, any organ can be affected. This study aimed to describe the features and the follow-up of a case of extranodal RDD. Our patient was a 39-year-old woman who was referred with an 11-month history of pain in the right maxilla. On clinical examination, some upper right teeth presented full mobility with normal appearance of the surrounding gingiva. Radiographic exams showed an extensive bone reabsorption and maxillary sinus filled with homogeneous tissue, which sometimes showed polypoid formation. An incisional biopsy demonstrated a diffuse inflammatory infiltrate rich in foamy histiocytes displaying lymphocytes emperipolesis. Immunohistochemistry showed positivity for CD68 and S-100, and negativity for CD3, CD20, and CD30. Such features were consistent with the RDD diagnosis. The patient was referred to a hematologist and corticotherapy was administrated for 6 months. RDD is an uncommon disease that rarely affects the maxilla. In the present case, the treatment was conservative, and the patient is currently asymptomatic after 5 years of follow-up.

No MeSH data available.