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Splenic hamartoma

Splenic hamartomas are very rare with only 3 described in a series of 200 000 splenectomies. Usually solitary, mulitiple nodules present in tuberous sclerosis or Wiskott-Aldrich Syndrome4.

The only recently described entity sclerosing angiomatoid nodular transformation (SANT) of the spleen, a non-neoplastic vascular entitiy described and named in 2004 may be a fibrosing variant of splenic hamartoma 6
 

Clinical features

Hamartomas are normally an incidental finding at imaging, surgery or autopsy. They can occur in any age group. Symptoms occur from mass effect if they grow large.

Pathology

Hamartomas are solitary or multiple, round, well-circumscribed, unencapsulated bulging nodules compressing the adjacent normal splenic parenchyma. Focal fibrosis and cystic areas can be seen.

The pathological differential diagnosis includes haemangioma, Littoral cell angioma, lymphagioma, haemangioendothelioma, sclerosing angiomatoid nodular transformation (see above), angiosarcoma. A definite diagnosis can be difficult due to overlap of features, however positivity of CD8 is a key feature that differentiates hamartoma from other vascular lesions of the spleen.

Radiographic features

Ultrasound

Most splenic hamartomas are hyperechoic solid masses, with or without cystic changes in ultrasonogram, and are hypervascular in both color Doppler ultrasound and angiogram.

CT

On computed tomography, hamartomas appear as isodense or hypodense solid masses and demonstrate heterogeneous contrast enhancement relative to adjacent normal parenchyma.

MRI

MRI is the preferable imaging technique for its distinction from hemangiomas.

  • T1 - most lesions are isointense
  • T2 - most lesions are heterogeneously hyperintense
  • dynamic enhanced MRI -
    • it is typical to show faint heterogeneous enhancement on immediate postcontrast-enhanced images (key features in the differentiation between hamartomas and hemangiomas).
    • on delayed postcontrast images, hamartoma enhances in a relatively uniform and intense fashion +/- with central hypovascular areas.

See also


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