Splenic haemangiomatosis

Radiopaedia • CC-by-nc-sa 3.0 • de

Splenic haemangiomatosis involves multiple, diffuse splenic hemangiomas replacing its entire parenchyma. It is a very rare entity.

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 hemangiomas.

Associations

Reported associations include

Clinical presentation

Patients may clinically present with

splenomegaly
thrombocytopenia
anemia
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
post contrast - lesions show homogenous enhancement

MRI

Signal characteristics of individual lesions include

Typical uncomplicated lesions

T1: most are hypo-isointense while some are hyper intense
T2: hyper- intense

There is variable intensity if there is associated thrombosis, infarction and/or hemorrhage

Prognosis

In general, diffuse splenic hemangiomatosis is a benign lesion and may have an asymptomatic course. It however can present with associated complications such as some of those mentioned above.