1/7. Fine-needle aspiration cytology of Rosai-Dorfman disease of the breast: A case report.We report the cytologic findings of a case of Rosai-Dorfman disease of the breast in a 52-year-old diabetic woman, initially sampled by fine-needle aspiration biopsy (FNA). The patient presented with a 2-week history of a 3 x 2 cm nodule in the mid-upper area of the left breast. A mammogram taken 6 months prior was negative. FNA smears demonstrated lymphocytes, plasma cells, and large pale cells, with enlarged irregular nuclei, admixed with fragments of fibrous tissue and calcific debris. Lymphophagocytosis (emperipolesis) was scarce. Our diagnosis was atypical lymphohistiocytic proliferation. flow cytometry was negative, but in the face of a strong clinical suspicion of a lymphoid malignancy, excision was performed. The final diagnosis was Rosai-Dorfman disease (RDD). The differential diagnosis of FNA of breast inflammatory lesions with atypical cytology is discussed, with a review of the literature. The early recognition on FNA of the hallmarks of this rare disease should prevent unnecessary radical surgery. Diagn. Cytopathol. 1999;21:287-291.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
2/7. Rosai-Dorman disease causing cervical myelopathy.STUDY DESIGN: A case report of a rare disease entity and review of the literature. OBJECTIVES: To illustrate the occurrence of an unusual disease affecting the spine and spinal canal. SUMMARY OF BACKGROUND DATA: Rosai-Dorman disease is considered an idiopathic benign lymphoproliferative disease that typically occurs in the earlier decades of life. The usual manifestation is painless massive cervical lymphadenopathy, although involvement of many extra nodal sites is common. This case illustrates Rosai-Dorman disease occurring in an elderly man with myelopathy and mass lesions of the cervical, thoracic, and lumbar spinal canal without typical lymphadenopathy.- methods: Case report illustrating clinical presentation as well as radiographic and pathologic findings, including comparisons to cases previously reported. RESULTS: Surgical decompression with incomplete resection of the lesion was performed, providing diagnosis and treatment guidance. The patient experienced significant neurologic improvement of myelopathy. CONCLUSIONS: Recognizing clinical and laboratory features of this disease may permit earlier diagnosis and limit or avoid surgical intervention in some cases.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
3/7. Renal involvement by Rosai-Dorfman disease: CT findings.Rosai-Dorfman disease is a rare disease characterized histologically by proliferation of histiocytes and has clinical features suggestive of a lymphomalike disease. lymph nodes and extranodal sites might be involved, but renal involvement is rare. We present computed tomographic findings in three cases of renal involvement by Rosai-Dorfman disease. Two cases showed renal hilar masses and one case showed subcapsular hypodense infiltration. Renal involvement by Rosai-Dorfman disease has a characteristic appearance and should be included in the differential diagnosis of renal hilar masses or subcapsular hypodense infiltration.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
4/7. A case of Rosai-Dorfman disease involving the lacrimal gland in an elderly patient.Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy) is a rare disease characterised by benign proliferation of histiocytes, with painless lymph node enlargement and frequent extranodal involvement. Orbital involvement occurs in 11% of cases and onset of the disease is typically in the first two decades of life. A benign course is usual, but in some cases blindness or fatality may result. We present a case with late onset at age 78 years and lacrimal gland involvement, and review the literature on this disease.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
5/7. PET imaging of Rosai-Dorfman disease: correlation with histopathology and ex-vivo beta-imaging.We report the case of a woman who developed an early relapse of a squamous cell carcinoma (SCC) and was thus restaged twice within a year using [(18)F]fluorodeoxyglucose (FDG) positron emission tomography (PET). While there was no evidence of metastatic tumor outspread, focally increased FDG uptake was visible in numerous nodes but showed no change during the period between the two PET scans. These nodes, predominantly located at the proximal extremities, ranged in size from about 1 cm to over 6 cm. They were located subcutaneously, showed a red/bluish livid color and were of stout consistency. These nodes occurred first after radiochemotherapy for a non-Hodgkin's lymphoma (NHL) about 6 years earlier and slowly increased in size and number. One node of the right forearm was resected and ex-vivo beta-imaging, directly measuring the positron emission of the intranodal FDG distribution, was done and showed an overall increased glucose utilization with distinct spots of high metabolism. Histopathological work-up of the tumor showed widespread granulomatous tissue with lymphocyte follicles. Immunostaining showed the tumor to be positive for S100, CD68 and vimentin. Rosai-Dorfman disease (RDD) was diagnosed and no evidence of a potential relapse of the previous NHL was detected. RDD is a rare disease that is associated with the multifocal growth of benign tumors. The lesions are metabolically highly active. The correlation of the beta-imaging and histopathological results showed a high metabolism within granulomatous tissue with more intense metabolism within lymphocyte follicles.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
6/7. Sinus histocytosis with massive lymphadenopathy (Rosai-Dorfman disease) in a patient with primary sjogren's syndrome.Sinus histocytosis with massive lymphadenopathy is a rare disease that has been described by Rosai and Dorfman. It is characterized by massive, cervical lymphadenopathy, with extranodal manifestations in about 40% of patients. It occurs as a distinct entity, never associated with other diseases, and in most cases the prognosis is good. Lymphadenopathy is also a frequent sign of patients with primary sjogren's syndrome (SS), usually associated with disease activity or concurrent infection. However, excessive lymphadenopathy in SS patients is a sign of lymphoproliferative disorder development. In this report, we describe a patient with primary SS, and excessive lymphadenopathy and splenomegaly who developed Rosai-Dorfman disease, and we discuss the possible aetiopathophysiological mechanism linking these two entities.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
7/7. Rosai-Dorfman disease presenting as extranodal renal mass.We present a unique case of a man presenting with an abnormal prostate and large renal mass who was ultimately found to have prostate cancer and renal Rosai-Dorfman disease. We discuss the method of diagnosis and treatment in this patient, as well as review the presentation, diagnosis, and treatment of this rare disease.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |