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Hemangiomas are benign vascular neoplasms that closely resemble
normal vasculature. They are the most common tumors of infancy and
most are medically insignificant. However, some hemangiomas can
impinge on vital structures, ulcerate, hemorrhage, incite a consumptive
coagulopathy, cause high output cardiac failure, or significant
structural abnormalities. Hemangiomas are also a common soft tissue
tumor in young adults. Hemangiomas can also occur in extra-cutaneous
sites including the liver, gastrointestinal tract, central nervous
system, pancreas, gall bladder, thymus, spleen, lymph nodes, lung,
urinary bladder, and adrenal glands. Hemangiomas cal also be found
as part of one of may of the angiomatous syndromes including: Maffucci
syndrome (multiple enchondroma and soft-tissue cavernous hemangioma),
Klippel-Trenaunay-Weber disease (cutaneous hemangioma, bone and
soft-tissue hypertrophy, and varicose veins), Osler-Weber-Rendu
syndrome (hemorrhagic teleangectasia), Kasabach-Merritt syndrome
(thrombocytopenia and hemangioma, hemangioendothelioma, or angiosarcoma),
and Gorham disease ( massive osteolysis with hemangioma or lymphangioma).
Hemangiomas are classified pathologically by the predominant type
of vascular channel and include capillary, cavernous, arteriovenous,
or venous types. Capillary hemangiomas are composed of small
vessels lined by flattened endothelium. This subtype of hemangioma
is the most common soft tissue angiomatous lesion. They are superficially
located and can be divided into juvenile, verrucous, and senile
varieties. Cavernous hemangiomas are composed of dilated,
blood-filled spaces lined by flattened endothelium. These lesions
frequently involve the deeper soft tissues, often intramuscular,
and do not spontaneously involute. Cavernous hemangiomas often present
as masses without other diagnostic features and may require surgical
resection. Arteriovenous hemangiomas are considered to be
a persistence of a fetal capillary bed, with abnormal communication
of arteries and veins. These lesions can be superficial without
arteriovenous shunting or deep lesions with arteriovenous shunting
that is usually symptomatic (e.g. limb enlargement, distended veins,
and reflex bradycardia after compression). These lesions occur in
young patients and exclusively involve the soft tissues. Arteriovenous
hemangiomas usually have high blood flow, although stenosis and
thrombosis can lead to decreased flow. Venous hemangiomas
are composed of thick-walled vessels containing muscle and are found
in adults. These are generally found in the deep soft tissues such
as the reteroperitoneum, mesentery, and muscles of the lower extremities
and often have slow blood flow.
MRI is the modality of choice for evaluating soft-tissue hemangiomas
and is most often diagnostic. Hemangiomas usually appear as heterogeneous
masses on all MR pulse sequences. On T1-weighted images, the intramuscular
hemangiomas appear as poorly marginated soft tissue masses. They
appear as a low to intermediate signal intensity mass which often
contains areas of high signal intensity. The areas of high signal
intensity almost always represent areas of fat overgrowth within
the mass. Sometimes this fat overgrowth can be so extensive, especially
in cavernous hemangiomas, such that portions of it can be indistinguishable
from lipoma. On T2-weighted MR images the vascular components of
an intramuscular hemangioma usually show marked increased signal
intensity while the associated adipose tissue appears isointense
to the subcutaneous fat.
The vascular components of a hemangioma often have a characteristic
appearance. The vascular channels can appear circular, if seen en
face, or have a linear or a serpentine appearance, if visualized
longitudinally. These vascular cannels will have either high or
low signal intensity depending on the pulse sequence used and the
velocity of the blood flow. Intramuscular hemangiomas and their
feeding vessels show marked enhancement after administration of
intravenous gadolinium contrast. Phleboliths, although more easily
recognized on CT or radiographs, appear as circular areas of low
signal intensity on all MR pulse sequences not distinguishable from
flow voids on contrast enhanced images. Fluid-fluid levels and areas
of high signal intensity on T1- and T2-weighted images can represent
hemorrhage within the hemangioma.
MR imaging can sometimes also allow differentiation of the various
histological subtypes of soft tissue hemangiomas. Cavernous lesions
are composed primarily of large cystic spaces while arteriovenous
hemangiomas demonstrate prominent serpentine vessels. Arteriovenous
hemangiomas may show rapid blood flow in these serpentine vessels
which appears as a persistent low signal intensity on all MR pulse
sequences. Venous hemangiomas also have serpentine vessels but these
vessels show slow blood flow and have a tendency to be oriented
along the long axis of extremities and neurovascular bundles. These
lesions also tend to be multifocal and have an abundant amount of
associated fat.
References:
- Berquist, TH (ed). MRI of the Musculoskeletal System, 4th Edition.
Philadelphia: Lippincott-Williams & Wilkins, c2001. pp. 875-875.
- Deutsch AL and Mink JH (eds). MRI of the Musculoskeletal System:
A Teaching File, 2nd Edition. Philadelphia: Lippincott-Raven Publishers,
c1997. pp. 641-642.
- Murphey MD Fairbairn KJ, Parman LM, et al. Musculoskeletal
Angiomatous Lesions: Radiologic-Pathologic Correlation. Radiographics.
1995; 15: 893-917.
- Antaya, RJ. Infantile Hemangioma. (2002). eMedicine.
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