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DOI: 10.1148/rg.243035165
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RadioGraphics 2004;24:849-854
© RSNA, 2004

AFIP ARCHIVES

Soft-Tissue Cavernous Hemangioma1

Kristina I. Olsen, MD, G. Scott Stacy, MD and Anthony Montag, MD

1 From the Departments of Radiology (K.I.O., G.S.S.) and Pathology (A.M.), University of Chicago, 5841 S Maryland Ave, Chicago, IL 60637. Received July 15, 2003; revision requested September 12 and received October 23; accepted October 28. Address correspondence to K.I.O. (e-mail: kolsen25@hotmail.com).

Index Terms: Angioma, muscular, 444.362 • Angioma, soft tissues, 444.362 • Muscles, neoplasms, 444.362 • Soft tissues, neoplasms, 444.362


   History
Top
 History
Imaging Findings
 Pathologic Evaluation
 Discussion
 References
 
A 29-year-old policewoman with no medical or surgical history presented to the orthopedic clinic with a 10-year history of intermittent chronic pain in the right distal thigh. The pain was exacerbated by physical exertion. She could point to a discrete region on the anteromedial aspect of the right thigh where the pain was located. However, no palpable mass, overlying skin discoloration, or soft-tissue swelling was noted at clinical examination. Given the long-standing nature of the symptoms, the clinical suspicion for malignancy was low.


   Imaging Findings
Top
 History
 Imaging Findings
Pathologic Evaluation
 Discussion
 References
 
Initially, computed tomography (CT) of the right thigh was performed, with and without intravenous administration of contrast material. The precontrast images revealed a soft-tissue mass, of relative isoattenuation to muscle, in the distal vastus medialis (Fig 1a). This measured approximately 1 x 2 cm and did not appreciably distort the surrounding musculature. It was circumscribed by a rim of low attenuation. A nonspecific punctate focus of calcification along the periphery of the soft-tissue mass was noted.



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  		Figure 1a.  (a) Nonenhanced axial CT scan of the right thigh shows an intramuscular soft-tissue mass circumscribed by fat attenuation. The central soft-tissue component is isoattenuating relative to muscle. A small focus of calcification (arrow) is noted close to the periphery of the mass. (b) Contrast material-enhanced axial CT scan shows marked enhancement of the lesion, which is composed of a cluster of tubular structures that represent discrete vessels. The fatty rim does not enhance (arrow). The calcified focus is unchanged.

 


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  		Figure 1b.  (a) Nonenhanced axial CT scan of the right thigh shows an intramuscular soft-tissue mass circumscribed by fat attenuation. The central soft-tissue component is isoattenuating relative to muscle. A small focus of calcification (arrow) is noted close to the periphery of the mass. (b) Contrast material-enhanced axial CT scan shows marked enhancement of the lesion, which is composed of a cluster of tubular structures that represent discrete vessels. The fatty rim does not enhance (arrow). The calcified focus is unchanged.

 
After intravenous administration of contrast material, the composition of this soft-tissue mass was clarified. It was primarily composed of multiple prominently enhancing circular structures, compatible with vessels seen in cross section (Fig 1b). The adjacent cortex of the distal femoral diaphysis was normal, without evidence of bone erosion or overgrowth.

Ultrasound (US) evaluation of this region revealed a small ill-defined mass (Fig 2). An irregular hyperechoic margin separated the lesion from adjacent musculature and blended imperceptibly with the intermuscular fat planes. No acoustic shadowing was noted. Color Doppler images demonstrated a few punctate foci of internal flow. Pulsed Doppler evaluation was not performed.



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  		Figure 2.  Gray-scale US image shows the mass (arrow), which is isoechoic and intramuscular. It is delineated by a hyperechoic margin, which appears to arise from the surrounding intermuscular fat planes.

 
Subsequently, magnetic resonance (MR) imaging was performed. A heterogeneous mass in the vastus medialis was identified on both T1- and T2-weighted images. The mass was delineated on T1-weighted images by a rim of high signal intensity (Fig 3), which was subsequently low in signal intensity on fat-saturated T2-weighted images (Fig 4a), characteristic of fat. This rim partially extended into the surrounding muscular septa. Axial fat-saturated T2-weighted images more clearly defined the circular structures constituting the lesion, as previously noted at CT. The fat-saturated T2-weighted coronal and sagittal images (Fig 4b, 4c) revealed the serpentine nature of these high-signal-intensity structures. One sagittal T2-weighted image clearly demonstrated a linear high-signal-intensity abnormality connecting the intramuscular lesion and the adjacent femoral vein (Fig 5). Gadolinium contrast material was not administered.



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  		Figure 3a.  Axial (a), coronal (b), and sagittal (c) T1-weighted MR images show the fatty rim (arrow), which has high signal intensity. The mass is isointense with a diameter of 2 cm.

 


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  		Figure 3b.  Axial (a), coronal (b), and sagittal (c) T1-weighted MR images show the fatty rim (arrow), which has high signal intensity. The mass is isointense with a diameter of 2 cm.

 


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  		Figure 3c.  Axial (a), coronal (b), and sagittal (c) T1-weighted MR images show the fatty rim (arrow), which has high signal intensity. The mass is isointense with a diameter of 2 cm.

 


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  		Figure 4a.  Axial (a), coronal (b), and sagittal (c) fat-saturated T2-weighted MR images show that the mass is made up of enhancing tubular structures (arrow in b and c), which are compatible with vessels. The surrounding fat has been successfully saturated. A feeding serpentine vessel is seen on the axial image (arrow in a).

 


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  		Figure 4b.  Axial (a), coronal (b), and sagittal (c) fat-saturated T2-weighted MR images show that the mass is made up of enhancing tubular structures (arrow in b and c), which are compatible with vessels. The surrounding fat has been successfully saturated. A feeding serpentine vessel is seen on the axial image (arrow in a).

 


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  		Figure 4c.  Axial (a), coronal (b), and sagittal (c) fat-saturated T2-weighted MR images show that the mass is made up of enhancing tubular structures (arrow in b and c), which are compatible with vessels. The surrounding fat has been successfully saturated. A feeding serpentine vessel is seen on the axial image (arrow in a).

 


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  		Figure 5.  Sagittal fat-saturated T2-weighted MR image shows a relatively large vessel (arrow) connecting the angiomatous cluster and the femoral vein.

 
These imaging characteristics were pathognomonic of an angiomatous lesion, specifically a soft-tissue hemangioma.


   Pathologic Evaluation
Top
 History
 Imaging Findings
 Pathologic Evaluation
Discussion
 References
 
The soft-tissue lesion and surrounding musculature were surgically resected. The total tissue removed measured 8 x 5.5 x 2.5 cm. In the center of this resected section of striated muscle, a discrete hemorrhagic lesion was found, measuring 0.8 cm in maximal diameter (Fig 6). The lesion was circumscribed by adipose tissue.



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  		Figure 6.  Photograph of the gross specimen shows the lesion, which is hemorrhagic and surrounded by adipose tissue.

 
Further histologic evaluation revealed a mix of dilated blood-filled spaces and discrete vessels. The cystic spaces were lined by flattened endothelium. One cystic space was filled by organizing thrombus and dystrophic calcification, compatible with a phlebolith (Fig 7). These microscopic findings allowed classification of the lesion as a mixed (primarily cavernous) soft-tissue hemangioma.



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  		Figure 7.  Photomicrograph (original magnification, x100; hematoxylin-eosin stain) shows a calcified thrombus in the cavernous hemangioma.

 

   Discussion
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 History
 Imaging Findings
 Pathologic Evaluation
 Discussion
References
 
Soft-tissue hemangiomas are common neoplasms of benign histologic origin. They are the most common of the angiomatous lesions and represent up to 7% of all benign soft-tissue tumors in the general population (1). Women are more often affected than men. Hemangiomas are the most frequently diagnosed soft-tissue neoplasm in children.

Histologically, soft-tissue hemangiomas are subdivided into five categories, depending on the predominant type of vascular channel identified. These subdivisions include capillary, cavernous, arteriovenous, venous, and mixed variations (2,3).

Of these categories, capillary hemangiomas are the most common (4). They are usually diagnosed during the first few years of life and are found in the skin, subcutaneous tissue, or vertebral bodies. Microscopically, capillary hemangiomas are composed of a disordered array of capillary-sized vessels. Most of these hemangiomas spontaneously involute (5).

Cavernous hemangiomas are larger and deeper and occur later in life. As in our patient, they are often intramuscular. Cavernous hemangiomas are composed of dilated, blood-filled spaces lined by flattened endothelium. Calcification is common. They do not spontaneously involute and therefore may require surgical intervention (3).

Arteriovenous hemangiomas may be deep or superficial. They represent an abnormal communication between arteries and veins and can cause a variable degree of shunting. The composition of these lesions reflects a persistent fetal capillary bed.

Venous soft-tissue hemangiomas typically involve deep structures and can classically be found in the retroperitoneum, mesentery, and extremities. Histologically, they are composed of thicker-walled vessels containing smooth muscle cells.

In addition to their vascular components, angiomatous tumors can contain thrombus, calcification, hemosiderin, fat, smooth muscle, and fibrous tissue (4). This is particularly true of cavernous hemangiomas. The most common association is that of reactive fat overgrowth. In fact, overgrowth of adipose tissue can be so prominent that in the past, these lesions were classified as angiolipomas.

Soft-tissue hemangiomas can arise from various anatomic locations, including striated muscle, skin, subcutaneous tissue, and synovial tissue, depending on the histologic subtype. If large enough, they may manifest as a smooth, palpable soft-tissue mass, which in women can increase in size during pregnancy (1). As in our patient, up to 60% of these patients experience chronic pain (4). This is particularly true with physical exertion, which can result in retrograde flow in the arterial segment distal to the hemangioma. This reversal of blood flow, which favors the vascular tumor, has been referred to as the steal phenomenon and results in ischemia of the surrounding tissues. In asymptomatic patients, direct palpation of the mass can induce pain. Rarely, one may note a characteristic bluish discoloration of the overlying skin. Occasionally, a bruit may be discovered in the region. A soft-tissue hemangioma may cause osseous overgrowth secondary to chronic hyperemia, depending on its location.

Although radiographs of soft-tissue hemangiomas are usually normal, phleboliths are associated with cavernous hemangiomas in approximately 50% of cases (1,6). A nonspecific soft-tissue mass may also be noted (6). If the mass is large enough and in close proximity to adjacent bone, osseous changes including periosteal reaction and cortical thickening can occur (1,3,4,6). In some cases, pressure erosion from the adjacent mass can result in a pathologic fracture.

At nonenhanced CT, an ill-defined mass of similar attenuation to muscle may be identified. Phleboliths too small to identify on radiographs can be revealed. After administration of contrast material, significant enhancement is typical. As seen in the presented case, serpentine vascular structures may be depicted as well as surrounding adipose overgrowth.

US can demonstrate a complex mass. If phleboliths are abundant, acoustic shadowing may also be documented (1,5). Doppler evaluation may show low-resistance arterial flow with forward flow during both systole and diastole (1,5,7).

Currently, the standard for imaging evaluation of soft-tissue hemangiomas is MR imaging (1,4). Typically, all sequences show a heterogeneous mass (although lesions measuring under 2 cm tend to be homogeneous), reflecting the mix of tissues present. T1-weighted images best reveal areas of high-signal-intensity adipose tissue, most prominent along the circumference of the vascular complex. This fatty tissue may reflect muscle atrophy secondary to chronic vascular insufficiency caused by the steal phenomenon. In some patients, the fat overgrowth is so prominent that these lesions are mistaken for lipomas (1). The central angiomatous core of the neoplasm shows high signal intensity on T2-weighted images. As on contrast-enhanced CT images, the serpentine nature of the hemangioma may be depicted. If blood flow through these vascular channels is rapid enough, the signal may remain low in intensity with all MR imaging sequences. If gadolinium contrast material is administered, prominent enhancement of the angiomatous tumor is expected (1).

Arteriography of capillary and cavernous soft-tissue hemangiomas shows pooling of contrast material with arteriovenous shunting and enlarged feeding vessels. An arteriovenous soft-tissue hemangioma is typified by large tortuous feeding vessels, early draining veins, and tumor staining. Delayed imaging is necessary to detect the stain of venous hemangiomas because they are not usually identified in the arterial phase (1). Otherwise, direct venous puncture can more clearly depict venous hemangiomas. At most medical centers, angiography has probably been supplanted by MR or CT angiography in providing a surgical road map. Unless embolization is indicated prior to resection, angiography can usually be avoided.

Nuclear medicine can also help diagnose a soft-tissue hemangioma. Increased focal radiotracer activity is expected in tagged red blood cell studies with technetium-99m methylene diphosphonate. As in hepatic hemangiomas, the uptake of radiotracer is progressive over time, and delayed blood pool imaging is necessary. Delayed static bone scan images may demonstrate focal uptake of radiotracer of lesser intensity (1,4).

Biopsy attempts in cavernous tumors primarily yield blood products and are unlikely to provide sufficient solid tissue for histologic analysis. Furthermore, biopsy of soft-tissue hemangiomas may lead to bleeding complications, particularly if the tumor is intramuscular. Given that imaging is often diagnostic for soft-tissue hemangiomas, biopsy can generally be avoided.

Asymptomatic soft-tissue hemangiomas typically do not warrant treatment. Therapy for symptomatic lesions most often includes surgical resection or laser treatment. Many orthopedic surgeons promote wide excisions to prevent local recurrence (4). To avoid significant blood loss, surgery may be preceded by embolotherapy or sclerotherapy. If the angiomatous tumor is too large and thus not amenable to surgery, embolotherapy and sclerotherapy may relieve symptoms after multiple sessions. These individuals may also benefit from radiation therapy.


   References
Top
 History
 Imaging Findings
 Pathologic Evaluation
 Discussion
 References
 

  1. Murphey MD, Fairbairn KJ, Parman LM, Kirkman GB, Parsa MB, Smith WS. Musculoskeletal angiomatous lesions: radiologic-pathologic correlation. RadioGraphics 1995; 15:893-917.[Abstract]
  2. Greenspan A, McGahan JP, Vogelsang P, Szabo RM. Imaging strategies in the evaluation of soft-tissue hemangiomas of the extremities: correlation of the findings of plain radiography, angiography, CT, MRI, and ultrasonography in 12 histologically proven cases. Skeletal Radiol 1992; 21:11-18.[Medline]
  3. Resnick D. Vascular and lymphatic tumors. In: Draud LA, Fix CF, eds. Diagnosis of bone and joint disorders. 4th ed. Philadelphia, Pa: Saunders, 2002; 4186-4195.
  4. Wild AT, Raab P, Krauspe R. Hemangioma of skeletal muscle. Arch Orthop Trauma Surg 2000; 120:139-143.
  5. Paltiel HJ, Burrows PE, Kosakewich HP, Zurakowski D, Mulliken JB. Soft-tissue vascular anomalies: utility of US for diagnosis. Radiology 2000; 214:747-754.[Abstract/Free Full Text]
  6. Ly JQ, Sanders TG, Mulloy JP, et al. Osseous change adjacent to soft-tissue hemangiomas of the extremities: correlation with lesion size and proximity to bone. AJR Am J Roentgenol 2003; 180:1695-1700.[Abstract/Free Full Text]
  7. Stark DD, Bradley WG, Jr. Hemangioma of soft tissue. Magnetic resonance imaging. 3rd ed. St Louis, Mo: Mosby, 1999; 1007-1011.



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