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Childhood Malignancies, Cysts, and Sinuses of the Head and Neck
Published in R James A England, Eamon Shamil, Rajeev Mathew, Manohar Bance, Pavol Surda, Jemy Jose, Omar Hilmi, Adam J Donne, Scott-Brown's Essential Otorhinolaryngology, 2022
Rosai-Dorfman disease (sinus histiocytosis): inflammatory, unknown etiology. Massive bilateral cervical lymphadenopathy, fever, malaise. Older children/adolescents. Usually benign. Spontaneous regression. Biopsy excludes lymphoma, debulk if compressive symptoms.
Granulomatous Diseases
Published in Ayşe Serap Karadağ, Lawrence Charles Parish, Jordan V. Wang, Roxburgh's Common Skin Diseases, 2022
Albert Alhatem, Robert A. Schwartz, Muriel W. Lambert, W. Clark Lambert
Overview: Rosai-Dorfman disease (R-DD) is a rare, benign disorder with a good prognosis, when the disease is limited to the skin. The etiology of systemic and cutaneous Rosai-Dorfman disease is unknown, but it may be an immune dysfunction reacting to an antigen or infectious organism.
Histiocytosis and Lipid Storage Diseases
Published in Harold R. Schumacher, William A. Rock, Sanford A. Stass, Handbook of Hematologic Pathology, 2019
Salwa Shabbir Sheikh, David F. Garvin
b. Sinus Histiocytosis with Massive Lymphadenopathy (Rosai-Dorfman Disease). Rosai-Dorfman disease (RDD) was first recognized in 1969 as an uncommon proliferative histiocytic disorder (Fig. 3). It is a benign, self-limiting disorder of unknown etiology. Some cases are associated with Epstein-Barr virus, herpes virus 6 infection, and lymphoma. It usually occurs in the first two decades of life. There is a predilection for children, especially <10 years, with the clinical picture of inflammation, i.e., fever, leukocytosis, high ESR, lymphadenopathy, and systemic symptoms. Significant numbers of cases appear in children with immunologic disorders, e.g., Wiskott-Aldrich syndrome, autoimmune hemolytic disease, and glomerulonephritis. The entity was originally described in lymph nodes, but has been reported to involve almost every organ system. Almost half of patients with RDD have at least one site of extra-nodal disease. In this hemophagocytic syndrome the internalized lymphocytes are intact, suggesting that they entered the histiocytes by emperipolesis rather than phagocytosis. Usually the course is benign and requires no therapy, but steroids and chemotherapy are used in severe cases.
Isolated extranodal Rosai-Dorfman disease of the orbit in an elderly male
Published in Orbit, 2021
Charlotte L. Marous, Mohamed Kahila, Roman Shinder
A 65-year-old male on tacrolimus following liver transplant for hepatocellular carcinoma presented with 3 weeks of progressive painless right eyelid swelling. Exam disclosed periorbital fullness, proptosis, hypoglobus, mechanical blepharoptosis, and decreased ductions with diplopia (Figure 1a). There was no lymphadenopathy. Inflammatory and infectious workups were negative. CT orbits showed a diffuse enhancing superior extraconal mass (Figure 1b). Biopsy revealed a lymphohistiocytic infiltrate. Histiocytes displayed emperipolesis (containing phagocytosed lymphocytes, Figure 1c) along with CD68 (Figure 1d) and S100 immunoreactivity. Lymphocytes were polyclonal on immunohistochemistry and gene rearrangement studies. PET-CT was negative for systemic disease. The findings were consistent with isolated extranodal orbital Rosai-Dorfman Disease (RDD).
[18F]FDG-PET for evaluating pediatric Rosai–Dorfman disease
Published in Pediatric Hematology and Oncology, 2018
Nicholas S. Whipple, Laura L. Marion, David M. Dansie, Andrea T. Murina, Gail C. Megason, Vani Vijayakumar, Betty L. Herrington
Rosai–Dorfman disease is a rare, nonmalignant disease that primarily affects children and young adults. Rosai?Dorfman disease is characterized by proliferation and excess accumulation of histiocytes positive for S-100, CD68, and CD163 and negative for CD1a, in addition to inflammatory cells and emperipolesis.1,2 Bilateral cervical lymph nodes are the most frequent site of disease. Approximately 43% of cases of nodal disease include at least 1 site of systemic, extranodal disease (eg, skin, central nervous system [CNS], orbit, sinuses, thyroid, respiratory tract, liver, kidney, breast, or bone), while 23% of cases exhibit only extranodal disease, 3% manifest as cutaneous Rosai?Dorfman disease, and <1% involve the breast.2 Although Rosai?Dorfman disease is generally a benign, self-limiting disease that rarely requires treatment, morbidity due to organ compression and failure can occur with a mortality rate as high as 7%.2 Computed tomography (CT) and magnetic resonance imaging (MRI) are widely utilized imaging modalities for evaluating Rosai?Dorfman disease, while [18F]FDG-PET is less commonly used.3,4
Cranio-spinal Rosai Dorfman disease: case series and literature review
Published in British Journal of Neurosurgery, 2019
Shashank S. Joshi, Shilpa Joshi, Girish Muzumdar, Keki E. Turel, Rajan M. Shah, Indoo Ammbulkar, Muhammad Masood Hussain, Kishor A. Choudhari
We present five cases of Rosai Dorfman disease (RDD) (Table 1). One case presented as cerebral intra-axial lesion, three as extra-axial cranial lesions and the fifth presented as intra-osseous osteolytic spinal lesion. After reaching to diagnosis of craniospinal RDD, a detailed and systematic imaging was undertaken for each case to rule out any possible systemic and multiorgan involvement. In all four cranial RDD cases no systemic or multiorgan involvement was seen. Spinal RDD patient had multiple distant osteal RDD, however multiorgan involvement was not seen. No post-operative adjuvant therapy was offered in any of our cases. All of them have been followed up to five years and have shown no recurrence in three cases, stable residual disease in one and definite recurrence in one.