An angioasarcoma of the spleen is a rare malignent splenic neoplasm. The term is usually given to describe a primary angiosarcoma of the spleen although angiosarcoma elsewhere can also rarely metastasise to the spleen. Despite its absolute rarity, a splenic angiosarcoma is considered the most common primary splenic malignancy 2.
The general consensus is that there is no recognised gender predilection (occurs almost equally in females and males)1. Occasional publication however suggest a slight male predilection 4 .The peak incidence is thought be around the 6th decade.
Clinical symptomatology can be highly variable, often posing difficult diagnostic problems.
Macroscopic examination often shows splenomegaly with cut sections revealing discrete lesions in a majority of cases. These can range from well-circumscribed firm nodules to poorly delineated foci of necrosis and haemorrhage associated with cystic spaces. Microscopically, the tumours are often heterogenous. The lesions typically demonstrate focal vasoformative component lined by atypical endothelial cells. Solid sarcomatous, papillary, and epithelioid growth patterns can be observed.
Unlike with primary hepatic angiosarcoma, there is no known association between splenic angiosarcoma and occupational exposure to chemicals, such as vinyl chloride or arsenic, or prior injection with the contrast agent thorium dioxide 4.
Reported sonographic features are non specific and include splenomegaly with cystic and solid masses with mixed echogenicity 4.
CT may show solitary or multiple nodular masses of heterogeneous low attenuation in an enlarged spleen. There is generally irregular and poorly defined contours, is often detected in a globally enlarged spleen 16.
Occasional large subcapsular intrasplenic or perisplenic extracapsular blood collections (haemoperitoneum) may be present.
Some of these masses may show peripheral enhancement with the margins of the lesions are often irregular or poorly marginated.
- pre-contrast CT - the tumours may appear hyperattenuating due to components of acute hemorrhage.
- dynamic contrast-enhanced CT scans - the lesions may exhibit substantial peripheral contrast enhancement similar to that of hepatic haemangiomas 4.
On occasion, CT scans may show a moderate splenomegaly with micronodular involvement of the organ 16.
Reported MRI features include
T1 and T2 :
- nodular hypointense (relative to the normal adjacent splenic parenchyma) masses on both T1- and T2- weighted images -
- large masses with increased signal intensity on both T1- and T2-weighted images that are likely related to areas of subacute haemorrhage, as well as tumour necrosis
- areas of decreased signal intensity within the tumour, owing to chronic haemorrhage with haemosiderin deposition.
- C+ (Gd) : usually shows intense and multinodular (heterogenous 15) enhancement with focal areas of non-enhancement - likely represeting intratumoral haemorrhage and necrosis
- spontaneous splenic rupture 2,4 : can occur in upto 30% of cases 4
Treatment and prognosis
It is an extremely aggresive fatal neoplasm at is almost universally fatal (median survival at aprroximately 24- 36 months at time of inital writing11) desptie treatment 3,5. Distant metastases occur most frequently in the liver (approximately 70% of cases), lung, pleural lymph nodes, bone, and brain. Prompt splenectomy prior to splenic rupture may improve survival 5.
It was first described in 1879 by T Langhans 1
General imaging differential considerations include vascular splenic lesions such as
- splenic haemangioma
- Littoral cell angioma of spleen
- lymphangioma of spleen
- haemangiopericytoma of spleen
- 1. Chung YF, Busmanis I, Hong GS et-al. Splenic angiosarcoma--an unusual cause of bleeding gastrointestinal tract. Singapore Med J. 1999;40 (2): 106-8. - Pubmed citation
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- 10. Thompson WM, Levy AD, Aguilera NS et-al. Angiosarcoma of the spleen: imaging characteristics in 12 patients. Radiology. 2005;235 (1): 106-15. doi:10.1148/radiol.2351040308 - Pubmed citation
- 11. Hsu JT, Chen HM, Lin CY et-al. Primary angiosarcoma of the spleen. J Surg Oncol. 2005;92 (4): 312-6. doi:10.1002/jso.20419 - Pubmed citation
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- 14. Kinoshita T, Ishii K, Yajima Y et-al. Splenic hemangiosarcoma with massive calcification. Abdom Imaging. 24 (2): 185-7. Abdom Imaging (link) - Pubmed citation
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Synonyms & Alternative Spellings
|Synonyms or Alternative Spelling||Include in Listings?|
|Angioasarcoma of the spleen||✗|
|Primary angiosarcoma of the spleen.||✗|
|Primary angiosarcoma of spleen||✗|
|Primary splenic angiosarcoma||✗|
|Primary splenic haemangiosarcoma||✗|