Rosai-Dorfman disease (also known as sinus histiocytosis with massive lymphadenopathy) is a rare benign proliferative disorder of histiocytes that typically involves the lymph nodes and can also involve extranodal sites. Rosai-Dorfman disease confined to the breast is extremely rare, but important to recognize as it can mimic malignancy. We present the case of a 63-year-old woman who presented with a palpable breast lump that was highly suspicious for malignancy based on mammogram and ultrasound appearance. Biopsy revealed inflammatory tissue with lymphoplasmacytic and histiocytic predominance. The diagnosis of Rosai-Dorfman was made based on characteristic staining of histiocytes with S-100 and the presence of emperipolesis. Early recognition of this benign disease entity spared the patient further investigation and surgical intervention.
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http://dx.doi.org/10.7759/cureus.1153 | DOI Listing |
Cureus
November 2024
Hematology and Medical Oncology, Kettering Health, Kettering, USA.
Rosai-Dorfman disease (RDD) is a rare proliferative histiocytic disorder characterized by sinus histiocytosis with massive lymphadenopathy, rarely presenting with severe and life-threatening extra-nodal features. The rarity of RDD, clinically variant phenotype, limited data, and lack of a current standardized management approach make treatment decisions difficult. Herein, we present a case of life-threatening, disseminated RDD with rare clinical features of recurrent pericardial effusion, bilateral pleural effusions, and abdominal tissue fibrosis successfully treated with six cycles of cladribine, achieving clinical remission.
View Article and Find Full Text PDFInt J Surg Case Rep
December 2024
Department of Surgical Oncology, Regional Hospital of Jendouba, Jendouba, Tunisia.
Introduction: Rosai-Dorfman disease (RDD) is a rare histiocytic neoplasm. It most commonly presents with bilateral cervical lymphadenopathy. We report the fourth case in the literature of RDD presenting as isolated axillary lymphadenopathy.
View Article and Find Full Text PDFArch Dermatol Res
December 2024
Department of Dermatology, Charles Nicolle Hospital, Tunis, Tunisia.
J Clin Endocrinol Metab
December 2024
Division of Hematology- Oncology, University of Alabama at Birmingham, Birmingham, AL 35233, USA.
Endocrinopathies are frequently the initial presentation of histiocytic neoplasms, which are rare hematologic disorders affecting multiple organ systems. Langerhans cell histiocytosis and Erdheim-Chester disease are 2 such disorders known to infiltrate the hypothalamus and/or pituitary gland, leading to arginine vasopressin deficiency (AVP-D) and anterior pituitary dysfunction (APD) in 20% to 30% of cases, often as the first manifestation. Conversely, histiocytic disorders account for a notable proportion (10-15%) of all pituitary stalk lesions.
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