Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019625 (Rosai-Dorfman disease)
763 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In an eleven part series published in Pathologica, we have presented various tumoral, quasitumoral and pseudotumoral lesions of the superficial and somatic soft tissue (ST), which emerged as new entities or as variants of established entities during the last quarter of a century. Detailed clinicomorphological and differential diagnostic features of approximately sixty entities were chosen on the basis of their clinical significance and morphologic distinctiveness. The series included fibrous and myofibroblastic tumors (e.g. solitary fibrous tumor, high grade classic and pigmented dermatofibrosarcoma protuberans, inflammatory myofibroblastic tumor and myofibrosarcomas), fibromyxoid and fibrohistiocytic neoplasms (e.g., Evans' tumor, phosphaturic mesenchymal tumor, inflammatory myxohyaline tumor), special adipocytic/vascular/and smooth muscle lesions (e.g., chondroid lipoma, Dabska's tumor, ST hemangioblastoma, lipoleiomyosarcoma), epithelioid mesenchymal malignancies of diverse lineages (e.g., epithelioid liposarcoma, proximal-type epithelioid sarcoma, neuroendocrine extraskeletal chondromyxoid sarcoma), ST Ewing's tumor and peripheral nerve sheath tumors (perineuriomas and pigmented and rosetting tumors of the schwannoma/neurofibroma group), extranodal dendritic or histiocytic proliferative processes (follicular dendritic cell sarcoma, Rosai-Dorfman disease, Castleman's disease, and plexiform xanthomatous tumor), and tumors with myoepithelial differentiation. The section devoted to selected pseudotumoral entities considered representatives of the hamartoma group (neural fibrolipomatous hamartoma, ectopic hamartomatous thymoma, rudimentary meningocele), metabolic diseases (amyloid tumor, nephrogenic fibrosing dermopathy, tophaceous pseudogout, pseudoinfiltrative parathyromatosis), stromal tissue reactions to trauma (fibroosseous pseudotumors of digits) and infections (bacillary angiomatosis), and normal organs (glomus coccygeum). To conclude the descriptive phase, supplementary material has now been collected and appended in an attempt to provide a quick digest of essential knowledge both for comparison and differential diagnosis. The data have been tailored to synthesize diverse sources, integrating clinical elements and references to articles that previously appeared in Part I ("Introduction"), Part II ("The List and Review of New Entities") and Parts III to XI ("Excerpta"). At the very least we hope this final part ("Appendix") will provide the reader with a useful tabular organization of ST lesions and a reference resource.
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PMID:Tumoral, quasitumoral and pseudotumoral lesions of the superficial and somatic soft tissue: new entities and new variants of old entities recorded during the last 25 years. Part XII: appendix. 1717 94

A 38 years-old man, who had olfactive schwannoma totally removed, with favorable clinical evolution and no radiological recurrence, suffered from headache and frontal syndrome, seven years after surgery. MRI of the brain showed multiple extra-axial lesions, disseminated, mimicking multiple meningiomas. The surgical strategy consisted in primary removal of the most symptomatic lesion. Histopathological examination after immunohistochemical tests permitted the definitive diagnosis of Rosai-Dorfman disease of the central nervous system. Evolution was still favorable 6 months after surgical removal of the whole lesions.
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PMID:[Intracranial Rosai-Dorfman disease mimicking multiple meningiomas]. 2375 18

Rosai-Dorfman disease of the central nervous system is extremely rare and difficult to diagnose also for pathologists. We describe three unusual cases of meningeal Rosai-Dorfman disease and illustrate the difficulties of preoperative and pathological diagnosis. We retrospectively analyzed three patients who underwent surgery for a suspected meningioma for whom the final diagnosis was Rosai-Dorfman disease of the central nervous system. Pathological initial diagnosis was schwannoma, lymphoplasmacyte-rich meningioma, or inflammatory tumor, but final diagnosis in all cases was Rosai-Dorfman disease. These cases underline the preoperative and pathological difficulties of such diagnosis. Pathologists and physicians should be aware of the occurrence of such rare localization of this disease and should think about this differential diagnosis in lymphocyte-rich meningeal tumors mimicking, clinically and radiologically, a meningioma. Communication of significant previous medical history to pathologists and careful examination of slides with appropriate medical history and the use of S100 antibody in the diagnosis of meningeal tumors mimicking Rosai-Dorfman disease could lower the rate of misdiagnosis.
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PMID:Meningeal Rosai-Dorfman disease mimicking meningioma. 2436 36

Kidney neoplasms are common diseases with varying prognoses depending on the subtype of the tumor. The most common solid lesion of the kidney is renal cell carcinoma, and the treatment is typically surgical removal. With increasing use of cross-sectional imaging in the last two decades, the detection of renal lesions has significantly increased, especially in asymptomatic patients who are scanned for other reasons. In this article, we present the imaging findings of rare solid benign primary kidney neoplasms including renal leiomyoma, reninoma, carcinoid tumor, metanephric adenoma, solitary fibrous tumor of the kidney, lipomatous hemangiopericytoma of the kidney, renal schwannoma, inflammatory myofibroblastic tumor of the kidney, extramedullary hematopoiesis in the kidney, and extranodal renal Rosai-Dorfman disease. Accurate preoperative or prebiopsy diagnoses of these lesions are unusual; however, informed radiologists may sometimes be able to favorably change the patient management and treatment.
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PMID:Unusual benign solid neoplasms of the kidney: cross-sectional imaging findings. 2620 Apr 82

The demographics, clinical features, and histopathological classification of orbital space-occupying lesions in adults have not been widely described in our part of the world except for the pediatric population. In this retrospective study, we collected 110 consecutive adult patients (18 years and older) with orbital lesions (excluding lacrimal gland lesions) that were diagnosed histopathologically in two tertiary eye centers in Riyadh, Saudi Arabia (January 2000 to July 2017). Patients with thyroid-related orbitopathy, infectious, and inflammatory/pseudo-inflammatory lesions were excluded. We had 60 males (54.5%) and 50 females (45.5%). The mean age at presentation was 51.4 years (range 19-99). Proptosis was the most common clinical presentation (mean duration 15.4 months). The orbital lesions in order of increasing prevalence were: lymphoproliferative lesions in 26.4%; vascular in 21.8%; secondary tumors in 14.6%; neurogenic in 13.6%; structural in 10.0%; soft tissue tumors 8.2%; then metastatic tumors (2.7%) and others (extramedullary leukemia, fibrous dysplasia, and histiocytic lesion: Rosai-Dorfman disease): one case each. Gender distribution was varied in lymphoproliferative disorders compared to vascular lesions. Cavernous hemangioma was the most common vascular lesion (83.3%) and schwannoma was the most common neurogenic tumor (60%). Secondary lesions extended to the orbit mostly from eyelids in nine out of 16 or conjunctiva in four out of 16 cases. A favorable outcome was observed in about 80% of patients who underwent excisional biopsy. The rest encountered local recurrence of the tumors, growing of residual lesions, and recurrence with further invasion to nearby structures. We concluded having a similar demographic pattern of orbital lesions in adults as has been universally reported. We have fewer secondary tumors. We have summarized the pathological profile of adult orbital lesions according to patients' age, gender, symptoms, and location of the lesion as a baseline guide for proper diagnosis of any orbital mass prior to surgical management planning and for future prognostic studies.
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PMID:Adult Orbital Lesions in Saudi Arabia: A Multi-centered Demographic Study with Clinicopathological Correlation. 3295 8