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Table 2 Clinical features of Rosai-Dorfman disease and other common and uncommon soft tissue tumors

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

  1. S-100, protein 100% soluble in ammonium sulfate; CD68, cluster of differentiation 68; CD1a, cluster of differentiation 1a; CD63, cluster of differentiation 63; CD54, cluster of differentiation 54; CD58, cluster of differentiation 58.