Clinical manifestations of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease).
| Anatomic site . | Frequency* . | Clinical Manifestation . |
|---|---|---|
| * RDD patients from the SHML registry4 with involvement at multiple extranodal sites are included under each anatomic site. The rarity of SHML precludes accurate statistics on the incidence of SHML or the frequency of specific clinical findings in the general community. | ||
| Lymph nodes | 87% | Massive painless adenopathy: cervical most frequent, may be bilateral, occasionally accompanied by pain or tenderness |
| Skin and Soft tissue | 16% | Broad range of non-specific presentations: typically painless, maculopapular rash, yellow xanthomatous solitary or multiple, usually raised reddish or bluish lesions, scaly Subcutaneous nodules or soft tissue masses |
| Nasal cavity/Paranasal sinuses | 16% | Mucosal thickening, nasal polyps (may be recurrent) or masses causing mild or complete nasal obstruction, epistaxis, displacement of septum, infiltrative mass extending into maxillary, ethmoid, or sphenoid sinuses |
| Eye/Orbit/Ocular adnexa | 11% | Eyelid or orbital mass with proptosis; may require enucleation |
| Bone | 11% | Bone pain with osteolytic lesions in any bone of the axial or appendicular skeleton; on radiography, may be purely lucent or mixed with sclerosis or exhibit irregular borders or a broad sclerotic rim |
| Salivary gland | 7% | Bilateral parotid enlargement or submandibular mass; may be associated with dry mouth |
| Central nervous system | 7% | Intracranial epidural or dural, single or multiple masses, spinal cord masses, associated lytic bone lesions in skull or spine, cranial or spinal nerve palsies, weakness, syncope, headache |
| Oral cavity | 4% | Infiltrative mass of soft or hard palate, diffuse mucosal thickening, papillomatosis, gingival swelling |
| Kidney/Genitourinary tract | 3% | Unilateral or bilateral renal masses accompanied by obstruction: hydronephrosis, hydroureter, ureteral obstruction, glomerulonephritis, chronic pyelonephritis Testicular, scrotal or epididymal masses with intermittent penile or pubic edema |
| Respiratory tract/Larynx/Lungs | 3% | Laryngeal obstruction, thickening and fibrosis associated with hoarseness |
| Liver | 1% | Enlargement with congestion, milliary infiltrates, masses |
| Tonsil | 1% | Enlargement, may be accompanied by tonsillitis |
| Breast | < 1% | Predominantly subcutaneous involvement, rarely involves breast parenchyma, associated axillary adenopathy |
| Gastrointestinal tract | < 1% | Hyperplastic mucosal folds, single or multiple exophytic masses |
| Heart | < 1% | Subendocardial or valvular infiltrates causing arrythmias |
| Anatomic site . | Frequency* . | Clinical Manifestation . |
|---|---|---|
| * RDD patients from the SHML registry4 with involvement at multiple extranodal sites are included under each anatomic site. The rarity of SHML precludes accurate statistics on the incidence of SHML or the frequency of specific clinical findings in the general community. | ||
| Lymph nodes | 87% | Massive painless adenopathy: cervical most frequent, may be bilateral, occasionally accompanied by pain or tenderness |
| Skin and Soft tissue | 16% | Broad range of non-specific presentations: typically painless, maculopapular rash, yellow xanthomatous solitary or multiple, usually raised reddish or bluish lesions, scaly Subcutaneous nodules or soft tissue masses |
| Nasal cavity/Paranasal sinuses | 16% | Mucosal thickening, nasal polyps (may be recurrent) or masses causing mild or complete nasal obstruction, epistaxis, displacement of septum, infiltrative mass extending into maxillary, ethmoid, or sphenoid sinuses |
| Eye/Orbit/Ocular adnexa | 11% | Eyelid or orbital mass with proptosis; may require enucleation |
| Bone | 11% | Bone pain with osteolytic lesions in any bone of the axial or appendicular skeleton; on radiography, may be purely lucent or mixed with sclerosis or exhibit irregular borders or a broad sclerotic rim |
| Salivary gland | 7% | Bilateral parotid enlargement or submandibular mass; may be associated with dry mouth |
| Central nervous system | 7% | Intracranial epidural or dural, single or multiple masses, spinal cord masses, associated lytic bone lesions in skull or spine, cranial or spinal nerve palsies, weakness, syncope, headache |
| Oral cavity | 4% | Infiltrative mass of soft or hard palate, diffuse mucosal thickening, papillomatosis, gingival swelling |
| Kidney/Genitourinary tract | 3% | Unilateral or bilateral renal masses accompanied by obstruction: hydronephrosis, hydroureter, ureteral obstruction, glomerulonephritis, chronic pyelonephritis Testicular, scrotal or epididymal masses with intermittent penile or pubic edema |
| Respiratory tract/Larynx/Lungs | 3% | Laryngeal obstruction, thickening and fibrosis associated with hoarseness |
| Liver | 1% | Enlargement with congestion, milliary infiltrates, masses |
| Tonsil | 1% | Enlargement, may be accompanied by tonsillitis |
| Breast | < 1% | Predominantly subcutaneous involvement, rarely involves breast parenchyma, associated axillary adenopathy |
| Gastrointestinal tract | < 1% | Hyperplastic mucosal folds, single or multiple exophytic masses |
| Heart | < 1% | Subendocardial or valvular infiltrates causing arrythmias |