Rosai-Dorfman disease (RDD) is a rare idiopathic histioproliferative disease affecting the lymph nodes. Extranodal sites may be involved and occasionally represent the initial or sole manifestation of the disease. Central nervous system manifestations especially are exceedingly rare. We reported a case of isolated, intracranial, dural-based RDD. A 69-year-old male, admitted with a second generalized convulsion, was examined by high-resolution MR images that revealed a thickened, stratified dura in the left frontal region with associated cortical edema. The patient underwent craniotomy with subtotal resection of the lesion. Microscopically, with the cytoplasmic staining against S-100 protein the lesion was shown to consist of proliferative histiocytes exhibiting emperipolesis. The histopathological diagnosis was compatible with RDD. The diagnosis of RDD mimicking pachymeningitis is presented, and the previous reported cases of intracranial RDD are reviewed.
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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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