Splenic haemangiomas (also known as splenic venous malformations) while being rare lesions, are considered the second commonest focal lesion involving the spleen after simple splenic cysts 5,12.
Most splenic venous malformations are so called cavernous malformations, which are found throughout the body. This article focuses on splenic haemangiomas. For a general discussion please refer to the general article on cavernous venous malformation.
It is important to note that according to newer nomenclature (ISSVA classification of vascular anomalies) these lesions are merely known as slow flow venous malformations. Having said that it is probably helpful in reports to include the word 'hemangioma' as this term is ubiquitous in the literature and most familiar to many clinicians. The remainder of this article uses the term 'splenic hemangioma' for consistency with the majority of the existing literature.
Their autopsy prevalence rate is thought to range around 0.1-14% 7-8. Most haemangiomas tend to be discovered in adults from mid-30s to mid-50s years of age 8.
The vast majority are asymptomatic and are incidentally discovered. Occasionally they may be associated with splenomegaly, abdominal pain, dyspnoea, diarrhoea, or constipation [ref needed].
Splenic hemangiomas are comprised of non-encapsulated non-neoplastic vascular channels of varying size ranging from capillary to cavernous, containing slow flowing blood. These vessels are lined with a single layer endothelium 2,8.
- splenic haemangiomatosis - the presence of multiple splenic haemangiomas
- a splenic manifestations of a systemic angiomatosis syndrome such as
Most lesions tend to be small in size (< 2cm 12). Calcification if present, either central punctate or peripheral curvilinear, can be detected on radiographs or CT scans.
Unenhanced CT scans can show a low-attenuation mass; following IV contrast, the vascular channels show centripetal fill( from the periphery inward). Larger lesions can fill more slowly and may do so incompletely and inhomogeneously 2.
Haemangiomas can have a variety of sonographic appearances depending on their exact histological composition (i.e. hypo, iso or hyper echoic).
The dominant pattern however is considered to be a homogeneous echotexture that is predominantly hyperechoic 11.
Colour Doppler imaging
Usually does not demonstrate any intrinsic colour flow (~ 80% of cases) 11
Contrast enhanced sonography
Isoechogenicity to splenic parenchyma in all phases is the most frequent typical enhancement pattern of splenic haemangiomas observed on contrast-enhanced sonography 11.
Reported signal characteristics of most haemangiomas are similar to hepatic haemangiomas and are 6,10
- T1 - iso to hypo-intense to normal splenic parenchyma
- T2 - hyper-intense to splenic parenchyma
- most demonstrate early nodular centripetal enhancement and uniform enhancement at delayed imaging
- smaller lesions may however show homogeneous enhancement on immediate post-contrast acquisitions remaining enhanced on delayed images 12.
Angiography - DSA
Angiographic findings are non-specific (more content required).
Nuclear imaging with 99mTc labelIed red cells shows slow accumulation of activity in the lesion followed by slow washout. Nuclear scans with 99mTc IabelIed sulphur colloid usually shows a photopenic defect due to the radionuclide accumulation being restricted to functional splenic tissue.
- spontaneous rupture with haemorrhage is a risk with larger lesions
- in some patients, they may cause the Kasabach-Merritt syndrome 8
Treatment and prognosis
They are benign lesions and carry no malignant potential. Their natural course is very slow growth over time 8. Haemangiomas are generally not treated unless they are symptomatic or very large, with increased risk of haemorrhage; treatment then is usually a splenectomy.
- normal appearance of the spleen
- pseudolesion of the spleen: inhomogeneous splenic enhancement
splenic lesions and anomalies
- congenital anomalies
- mass lesions
- infiltrative processes
- incidental splenic lesion (approach)
Vascular tumours and malformations
vascular malformations and tumours
- vascular tumours
- tufted angioma (with or without Kasabach Merritt syndrome)
- Kaposiform hemangioendothelioma (with or without Kasabach Merritt syndrome)
- spindle cell hemangioendothelioma
- other, rare hemangioendotheliomas
- dermatologic acquired vascular tumors
- slow flow vascular malformations
- capillary malformation (CM)
- venous malformation (VM)
- common sporadic venous malformation
cavernous venous malformation (cavernoma or cavernous haemangioma)
- cerebral cavernous venous malformation
- orbital cavernous venous malformation
- hepatic cavernous venous malformation (hepatic haemangioma)
- splenic cavernous venous malformation (splenic haemangioma)
- breast venous malformation (breast haemangioma)
- retroperitoneal venous malformation
- soft tissue venous malformation
- primary intraosseous venous malformation
- cardiac venous malformations
- cavernous venous malformation (cavernoma or cavernous haemangioma)
- Bean syndrome
- familial cutaneous and mucosal venous malformation
- glomuvenous malformation (glomangioma)
- Maffucci syndrome
- common sporadic venous malformation
- lymphatic malformation (LM)
- fast flow vascular malformations
- arterial malformation
- arterial malformation
- arteriovenous fistulae (with one or more shunts)
- arteriovenous malformations (with a nidus of multiple shunts)
- 1. Mayne KM, Brown DC. Splenic haemangioma associated with splenomegaly and raised erythrocyte sedimentation rate. J. Clin. Pathol. 1989;42 (12): 1312. J. Clin. Pathol. (link) - Free text at pubmed - Pubmed citation
- 2. Disler DG, Chew FS. Splenic hemangioma. AJR Am J Roentgenol. 1991;157 (1): 44. AJR Am J Roentgenol (citation) - Pubmed citation
- 3. Katz DS, Wojtowycz AR, Markarian B. Splenic hemangioma. Detection on a technetium-99M MDP bone scan. Clin Imaging. 18 (4): 279-82. - Pubmed citation
- 4. Ros PR, Moser RP, Dachman AH et-al. Hemangioma of the spleen: radiologic-pathologic correlation in ten cases. Radiology. 1987;162 (1): 73-7. Radiology (abstract) - Pubmed citation
- 5. Duddy MJ, Calder CJ. Cystic haemangioma of the spleen: findings on ultrasound and computed tomography. Br J Radiol. 1989;62 (734): 180-2. doi:10.1259/0007-1285-62-734-180 - Pubmed citation
- 6. Elsayes KM, Narra VR, Mukundan G et-al. MR imaging of the spleen: spectrum of abnormalities. Radiographics. 25 (4): 967-82. doi:10.1148/rg.254045154 - Pubmed citation
- 7. Cooper SG, Strauss EB, Levine AH. Detection of noncalcified splenic hemangioma by radionuclide bone scan. J. Nucl. Med. 1989;30 (6): 1111-2. J. Nucl. Med. (link) - Pubmed citation
- 8. Hoeger PH, Helmke K, Winkler K. Chronic consumption coagulopathy due to an occult splenic haemangioma: Kasabach-Merritt syndrome. Eur. J. Pediatr. 1995;154 (5): 365-8. - Pubmed citation
- 9. Abbott RM, Levy AD, Aguilera NS et-al. From the archives of the AFIP: primary vascular neoplasms of the spleen: radiologic-pathologic correlation. Radiographics. 24 (4): 1137-63. doi:10.1148/rg.244045006 - Pubmed citation
- 10. Ramani M, Reinhold C, Semelka RC et-al. Splenic hemangiomas and hamartomas: MR imaging characteristics of 28 lesions. Radiology. 1997;202 (1): 166-72. Radiology (abstract) - Pubmed citation
- 11. Taibbi A, Bartolotta TV, Matranga D et-al. Splenic hemangiomas: contrast-enhanced sonographic findings. J Ultrasound Med. 2012;31 (4): 543-53. J Ultrasound Med (full text) - Pubmed citation
- 12. Luna A, Ribes R, Caro P et-al. MRI of focal splenic lesions without and with dynamic gadolinium enhancement. AJR Am J Roentgenol. 2006;186 (6): 1533-47. doi:10.2214/AJR.04.1249 - Pubmed citation