From: Extranodal Rosai–Dorfman disease: a rare soft tissue neoplasm masquerading as a sarcoma
Rosai-Dorfman disease [[6]] | Langerhans cell histiocytosis [[25]] | Inflammatory myofibroblastic tumor [[26]] | Soft tissue sarcoma [[27]] | |
---|---|---|---|---|
Incidence (cases/million persons/year) | Rare | 0.5 to 5.4 | Not reported | 11,280/3,000,000 |
Male:female ratio | 1.33:1 | 2:1 | Not reported | 1:1 |
Racial predilection | African American | Caucasian | Not reported | None |
Most common age range at diagnosis | 20.6 years | 0 to 15 years | 6 to 10 years | <21 years |
Anatomic location | Cervical lymph nodes > skin > upper respiratory tract, bone | Lymph nodes, liver spleen, skin, bone marrow, lungs > gastrointestinal tract, central nervous system | Lung > abdomen, mesentery | Lower extremity > trunk > upper extremity, retroperitoneum > head and neck > mediastinum |
Lymph nodes | ||||
Soft tissue | ||||
Organ systems | ||||
Symptoms at presentation | Nodal disease: massive lymphadenopathy | Fever, weight loss, lethargy, bone pain, skin and scalp erythematous rash, hepatosplenomegaly, respiratory distress | Fever, weight loss, pain, malaise, night sweats, reactive lymphadenopathy, tumor compressive symptoms | Asymptomatic mass, palpable abdominal mass with symptoms such as fullness, early satiety and vague abdominal pain |
Extranodal disease: tumors in skin, upper respiratory tract, bone with or without lymphadenopathy | ||||
Both: fever, weakness, weight loss, anemia, shortness of breath, headaches, nosebleeds | ||||
Pathology | Capsular lesion with large nuclei, inflammatory infiltrate rich in lymphocytes, plasma cells and large histiocytes demonstrating emperipolesis | Noncapsulated lesion with small nuclei, eosinophils and Langerhans cells with distinct cell margins and pink granular cytoplasm with Birbeck granules | Nonencapsulated lesion containing spindle cells proliferating in a background of fibrosis, with lymphocytes, plasmacytes, histiocytes, foamy macrophages, and occasionally eosinophils and neutrophils | Highly variable location and history dependent |
Immunohistochemistry | CD1a–, S-100+, CD68+, CD63+, emperipolesis | CD1a+, S-100+, CD54+, CD58+, no emperipolesis | Smooth muscle actin+, vimentin+, factor XIIIa+, S-100– | Neurofibrosarcoma: S-100 positive |
Angiosarcoma: Factor XIIIa positive | ||||
Rhabdomyosarcoma: Myoglobin positive | ||||
Long-term prognosis | Self-limited with resection > medical therapy | Self-limited course, some variants show chronicity, patients <2 with disseminated disease are >50% likely to die | Benign, reactive, recurrent, multifocal | Malignant; course dependent on size, grade, location |