Splenic haemangiomatosis involves multiple, diffuse splenic haemangiomas replacing its entire parenchyma and is very rare.
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Epidemiology
Associations
Reported associations include
Pathology
It can occur as a manifestation of systemic angiomatosis or, less commonly, confined to the spleen (diffuse isolated splenic haemangiomatosis). There is total or subtotal replacement of the splenic parenchyma by a vascular proliferation featuring the usual morphological findings of haemangiomas.
Clinical presentation
Patients may clinically present with
- splenomegaly
- thrombocytopenia 5
- anaemia 5
- portal hypertension
Radiographic features
Plain radiograph
Non-sensitive and non-specific but may show
- calcifications in left hypochondriac region
- features of splenomegaly
Ultrasound
May show
- solid hyperechoic masses
- complex echogenic mass with cystic areas
- splenomegaly
CT
- non-contrast - well defined iso-hypodense masses
- postcontrast - lesions show homogenous enhancement
MRI
Signal characteristics of individual lesions include
Typical uncomplicated lesions
- T1: most are hypo-isointense while some are hyperintense
- T2: hyperintense
There is variable intensity if there is associated thrombosis, infarction and/or haemorrhage.
Treatment and prognosis
In general, diffuse splenic hemangiomatosis is a benign entity and has an asymptomatic course. It may however present with associated complications (see above).
See also
- haemangioma - general
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