Kaposi sarcoma
Updates to Article Attributes
Kaposi sarcoma is a low-to-intermediate grade mesenchymal tumour that involves the lymphovascular system. The tumour can involve the pulmonary, gastrointestinal, cutaneous and musculoskeletal systems.
Pathology
There are four recognised variants 1:
- classic (chronic): multiple distal lower extremity predominant purple skin plaques and rarely involve visceral organs
- endemic (lymphadenopathic): common in equatorial Africa
- iatrogenic (organ transplant-related)
- AIDS-related (epidemic): usually requires the CD4 count to drop <200 cells/mm3; may develop in up to 35% of patients with AIDS 2 and when developed it is considered an AIDS-defining illness
The latter two variants are much more common.
Aetiology
An aetiological association with human herpes virus type 8 (HHV8) has been postulated. The AIDS-related and post-transplant variants are also associated with immunosuppressive states states.
Histology
Histologically can comprise of sheets of plump spindle-shaped cells surrounding and lining slit-like vascular spaces.
Associations
- lymphoproliferative disorders (particularly with the classic form)
Radiographic features
There is a wide spectrum of imaging findings depending on which organ is involved. However, most features are non-specific 1,2,4 but may assist in diagnosis if relevant clinical risk factors (e.g. background AIDS history) are evident. In 30% of cases, there is no concurrent cutaneous involvement 1.
Plain radiograph
The following features may be seen on chest radiographs:
- parenchymal nodular or reticular opacities with a predilection towards perihilar mid to lower zones; has two major patterns
- linear interstitial nodules
- fluffy ill-defined nodules
- pleural effusion(s)
- mediastinal and/or hilar lymphadenopathy
CT
On HRCT of the chest:
- ill-defined (flame-shaped) nodular opacities with usually bilateral and roughly symmetrical perilymphatic and peribronchovascular distribution (1-2 mm)1
- may have surrounding patchy ground glass changes
- interlobular septal thickening
- lymphadenopathy (may be present in up to 50%) 5
- lymphadenopathy is typically of high attenuation 6
On liver CT:
- ill-defined multifocal peripheral portal nodules with variable attenuation (commonest hepatic neoplasm in AIDS patients) 1,4
Nuclear medicine
Scintigraphy may be useful if concurrent opportunistic infection or lymphoma is suspected:
- thallium-201: usually positive in both lymphoma and Kaposi sarcoma
- gallium-67: usually negative in Kaposi sarcoma but positive in lymphoma and infection
History and etymology
This condition was first described by Moritz Kaposi (1837-1902), an Austro-Hungarian dermatologist, in 1872.
Differential diagnosis
For thoracic involvement consider:
- lymphoma (AIDS-related lymphoma: ARL): appear more well defined
- atypical pneumonia (e.g. Pneumocystis jiroveci)
- fungal (e.g. angioinvasive aspergillosis) or mycobacterial infection
- bacillary angiomatosis: also has skeletal lesions
-</ul><p>The latter two variants are much more common.</p><h5>Aetiology</h5><p>An aetiological association with human herpes virus type 8 (HHV8) has been postulated. The AIDS-related and post-transplant variants are also associated with immunosuppressive states.</p><h5>Histology</h5><p>Histologically can comprise of sheets of plump spindle-shaped cells surrounding and lining slit-like vascular spaces.</p><h5>Associations</h5><ul><li>lymphoproliferative disorders (particularly with the classic form)</li></ul><h4>Radiographic features</h4><p>There is a wide spectrum of imaging findings depending on which organ is involved. However, most features are non-specific <sup>1,2,4</sup> but may assist in diagnosis if relevant clinical risk factors (e.g. background AIDS history) are evident. In 30% of cases, there is no concurrent cutaneous involvement <sup>1</sup>.</p><h5>Plain radiograph</h5><p>The following features may be seen on chest radiographs:</p><ul>- +</ul><p>The latter two variants are much more common.</p><h5>Aetiology</h5><p>An aetiological association with human herpes virus type 8 (HHV8) has been postulated. The AIDS-related and post-transplant variants are also associated with <a href="/articles/immunosuppression">immunosuppressive</a> states.</p><h5>Histology</h5><p>Histologically can comprise of sheets of plump spindle-shaped cells surrounding and lining slit-like vascular spaces.</p><h5>Associations</h5><ul><li>lymphoproliferative disorders (particularly with the classic form)</li></ul><h4>Radiographic features</h4><p>There is a wide spectrum of imaging findings depending on which organ is involved. However, most features are non-specific <sup>1,2,4</sup> but may assist in diagnosis if relevant clinical risk factors (e.g. background AIDS history) are evident. In 30% of cases, there is no concurrent cutaneous involvement <sup>1</sup>.</p><h5>Plain radiograph</h5><p>The following features may be seen on chest radiographs:</p><ul>