Splenic haemangiomatosis

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

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.

See also