Milzhamartom

Splenic hamartomas are very rare lesions commonly found incidentally on imaging. They are most often solitary but may be present as multiple nodules in patients with tuberous sclerosis or Wiskott-Aldrich syndrome.

Terminology 

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

Epidemiology

Splenic hamartomas are very rare, with only 3 described in a series of 200,000 splenectomies.

Clinical presentation

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 and compounded by a mixture of normal splenic structures such as white and red pulp. Focal fibrosis and cystic areas can be seen.

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

Radiographic features

As hamartomas represent a focal disorganized overgrowth of splenic parenchyma, they tend to have similar echogenicity, attenuation, and signal intensity to the background normal parenchyma .

Ultrasound

Most splenic hamartomas are hypoechoic solid masses but can be heterogeneous due to hemorrhage or cystic changes .  They are hypervascular on color Doppler ultrasound and post-contrast administration.

CT

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

MRI

MRI is the preferred imaging technique for the differentiation of hamartomas from hemangiomas, showing:

• T1: most lesions are isointense
• T2: most lesions are heterogeneously hyperintense
• T1 C+ (Gd): 
  • it is typical to show vivid enhancement on immediate post-contrast 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

Differential diagnosis

Possible differential considerations include

• splenic hemangioma
• splenic lymphangioma
  • lobulated multiseptated cystic mass without solid enhancing components
  • may have walls, septa, linear calcifications
•  inflammatory myofibroblastic tumor

Siehe auch:

• Raumforderungen der Milz
• Milzhämangiom
• Tuberöse Sklerose
• sklerosierende angiomatöse noduläre Transformation der Milz
• fokale Milzläsionen und Anomalien
• Wiskott-Aldrich-Syndrom
• fokale lymphatische Malformation der Milz

und weiter:

• Hämangioperizytom der Milz
• hypodense Milzläsionen