Introduction: Rosai-Dorfman disease (RDD) is a rare benign histiocytic proliferation, characterized by a group of clinical symptoms. This report presents a case of extranodal RDD manifesting as a progressively enlarging left maxillary mass in a 42-year-old woman. Surgical exploration and biopsy confirmed the diagnosis of RDD, with characteristic histopathological features including emperipolesis. Treatment involved corticotherapy, resulting in controlled maxillary pain and improvement of the disease after one year. This case underscores the potential for extra-nodal RDD presentations, posing diagnostic challenges and emphasizing the importance of considering RDD in the differential diagnosis of maxillary masses.

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A systematic literature review revealed 88 Rosai-Dorfman Disease (RDD) cases affecting the orbit. We present a review of the literature on orbital RDD in addition to two brothers with isolated bilateral extra-nodal orbital RDD cases associated with G-6PD deficiency. The disease manifested as asymmetric extensive orbital and paranasal sinus infiltration, with orbital bone destruction. Orbital mass debulking and steroids improved the condition in both patients. This is the most extensive case series of orbital RDD reported, including clinical manifestation, imaging, pathologic results, management, and outcome. Vigilant follow-up is essential to monitor for potential malignant transformation, systemic manifestations, potential vision loss, or life-threatening recurrences.

Rosai-Dorfman Disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy, is an uncommon histiocytic condition characterized by massive histopathological aggregation of CD1-a negative, CD68-positive, and S100-positive histiocytes. It was initially described by Destombes in 1965 under the term \"adenitis with lipid excess.\" However, it is named after Rosai and Dorfman who reported further histopathological features of the disease in 1969. The diagnosis of this non-Langerhans cell histiocytosis can be challenging and requires high clinical suspicion. The diagnostic process usually involves imaging, tissue biopsies, and genetic testing as needed. In this case series, we are presenting three cases of rare disease. Case 2 had both nodal and extranodal forms, which makes this case rarer than cases 1 and 3, which present with extranodal lesions.

Rosai-Dorfman disease (RDD), otherwise known as sinus histiocytosis with massive lymphadenopathy (SHML), is a very rare and typically benign disorder of unknown etiology with <10% bone involvement. The report is of a case seen at the authors\' hospital of a patient presenting with several months\' onset unspecified nontraumatic ankle pain. There was no physical mass or lymphadenopathy appreciated on examination. Plain radiographs and magnetic resonance images demonstrated an osteolytic lesion at the medial malleolus. Biopsy revealed the diagnosis of intraosseous manifestation of Rosai-Dorfman disease.

We report a case of a 49-year-old female diagnosed with extranodal multifocal Rosai-Dorfman disease (RDD) of the breast using mammography and ultrasound. RDD is a rare non-Langerhans cell benign proliferative disorder of histiocytes that usually involves the lymph nodes, but may involve extranodal sites. We review the clinical presentation as well as imaging features of this rare disease on multiple modalities and the importance of recognizing the diagnosis in order to direct treatment.

OBJECTIVE: Intra ocular Rosai-Dorfman disease (RDD) is an extremely rare disease. We are reporting the first case of RDD presenting as ciliary body mass mimicking ciliary body melanoma, and we are reviewing the English literature reporting on cases of RDD presented with intraocular disease.
METHODS: An 18-year-old lady presented with loss of vision in the right eye, and was found to have intraocular mass lesion. She was diagnosed clinically and radiologically as a case of ciliary body melanoma associated with total retinal detachment.
RESULTS: Histopathological sections and stains proved to be intraocular RDD. Review of the literature revealed three cases of intraocular RDD; two of them had choroid thickening associated with serous retinal detachment, and one presented with intraocular mass mimicking choroid melanoma. Two of the three cases were enucleated. Our case is the first case in English literature of intraocular ciliary body RDD, mimicking ciliary body melanoma.
CONCLUSIONS: RDD can present as an intraocular mass that mimics ciliary body melanoma. This case emphasizes the importance of diagnostic biopsy before considering the final therapy in unclear cases, mainly when associated with unusual systemic features like lymphadenopathy.

BACKGROUND: Lesions at the skull base warrant careful evaluation of imaging and meticulous preoperative planning. Unnecessary radical resection of these lesions could add to surgical morbidity. Occasionally, dural-based inflammatory or infectious lesions at this site radiologically mimic meningiomas, which are commonly encountered entities at this site. Not all lesions require excision, and it could be harmful in a few cases.
METHODS: Two patients had skull base lesions that resembled a meningioma on imaging, whereas frozen section in both cases differed from the preoperative diagnosis, showing an inflammatory lesion in the first case and invasive aspergillosis in the second case. Formalin-fixed, paraffin-embedded sections confirmed the frozen section diagnosis. Safe resection was performed in the first case, whereas surgery was abandoned and the patient was started on antifungal therapy in the second case. Both patients had a good outcome.
CONCLUSIONS: This report underscores the usefulness of intraoperative frozen section to decide on a further course of treatment when faced with such unexpected situations. Frozen sections and crush smears hold great potential to avoid unwarranted surgical morbidity, particularly for skull base lesions.

Sinus histiocytosis with massive lymphadenopathy (SHML) or Rosai-Dorfman disease is a non-neoplastic condition which typically presents as massive, bilateral cervical lymphadenopathy and can involve multiple extranodal organ systems such as skin, eyes, and upper respiratory tract in about 28% cases. Bone lesions in association with nodal disease are seen in less than 10% cases. Isolated bone involvement as the only manifestation of SHML is extremely rare, with less than 50 cases reported in the literature. We report a very uncommon case of Rosai-Dorfman disease with isolated multifocal osseous involvement as the only presenting feature, involving about 10 different sites with no lymphadenopathy or other organ system involvement.

Rosai-Dorfman disease (RDD), otherwise known as sinus histiocytosis with massive lymphadenopathy, is a rare disease. Cutaneous RDD (CRDD) is an extremely rare form of RDD, which is limited to the skin. The present study examined a case of purely CRDD in a 25-year-old female patient who presented with a two-month history of red plaques on her face. In addition, a review of the literature was conducted, where the etiology, pathology, clinical characteristics and treatment of the disease were discussed. From a dermatological perspective, the current study aimed to emphasize the histological features and clinical morphology of cutaneous RDD. Clinicians should have sufficient knowledge to be able to recognize and manage this rare condition. The present study found that the presence of reddish-yellow nodules on the face without any particular sensitivity may be useful in the diagnosis of CRDD. Treatment with topical steroids was found to be beneficial in alleviating CRDD.