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DOI:10.2214/AJR.05.0460
AJR 2006; 187:430-437
© American Roentgen Ray Society


Clinical Observations

Pulmonary Sclerosing Hemangioma Presenting as Solitary Pulmonary Nodule: Dynamic CT Findings and Histopathologic Comparisons

Myung Jin Chung1, Kyung Soo Lee1, Joungho Han2, Yon Mi Sung1, Semin Chong1 and O Jung Kwon3

1 Department of Radiology and Center for Imaging Science, Samsung Medical Center, Sungkyunkwan University School of Medicine, 50, Ilwon-Dong, Kangnam-Ku, Seoul 135-710, Korea.
2 Department of Diagnostic Pathology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul 135-710, Korea.
3 Division of Pulmonary and Critical Care Medicine, Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul 135-710, Korea.

Received March 16, 2005; accepted after revision May 2, 2005.

 
Supported by grant R11-2002-103 from the Korea Science and Engineering Foundation.

Address correspondence to K. S. Lee (kyungs.lee{at}samsung.com).


Abstract
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Abstract
Introduction
Subjects and Methods
Results
Discussion
References
 
OBJECTIVE. The purpose of this study was to describe the dynamic CT findings of pulmonary sclerosing hemangioma presenting as a solitary pulmonary nodule and to compare these findings with histopathologic findings.

CONCLUSION. On dynamic CT, sclerosing hemangioma has strong and rapid enhancement attributed histopathologically to the presence of hemangiomatous or papillary components in the tumor.

Keywords: CT • hemangioma • lung disease • lung neoplasm


Introduction
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Abstract
Introduction
Subjects and Methods
Results
Discussion
References
 
Sclerosing hemangioma is a rare benign neoplasm of the lung [1], categorized as a miscellaneous tumor in the new 1999 World Health Organization/International Association for the Study of Lung Cancer classification. It is considered a distinct entity characterized by the presence of vascular proliferation with a marked tendency for sclerosis [2]. Although initially regarded as a variant of hemangioma on the basis of results of immunohistochemical and genetic studies, sclerosing hemangioma is now considered an epithelial tumor.

On chest radiographs, sclerosing hemangioma appears as a well-defined round or oval mass. According to a report on the CT findings in eight cases, this tumor presents as a well-defined juxtapleural mass with marked contrast enhancement caused by its hemangiomatous component [3]. Dynamic CT and MRI studies have shown that sclerosing hemangioma has marked homogeneous enhancement [4, 5]. Most sclerosing hemangiomas grow slowly and can be cured by wedge resection or lobectomy. However, sclerosing hemangioma can manifest as multiple tumors or as a tumor surrounded by multiple daughter nodules in the same lobe; it also can be accompanied by hilar lymph node metastasis [6, 7]. The purpose of this study was to describe the dynamic CT findings of pulmonary sclerosing hemangioma presenting as a solitary pulmonary nodule and to compare these findings with the histopathologic findings.


Subjects and Methods
Top
Abstract
Introduction
Subjects and Methods
Results
Discussion
References
 
Patients and CT
Between March 2002 and October 2003, 318 patients with a solitary pulmonary nodule at chest radiography underwent dynamic CT. Of these, 228 patients had the final diagnosis of benign tumor or malignancy, and 116 (51%) of these patients proved to have a benign nodule. Ten (9%) of the 116 patients had surgically proven sclerosing hemangioma. Surgical intervention involved wedge resection through open thoracotomy (n = 4) or video-assisted thoracoscopic surgery (n = 6). Resection was performed because percutaneous transthoracic fine-needle aspiration biopsy produced an inconclusive diagnosis with features of well-differentiated adenocarcinoma or carcinoid tumor (n = 6); the tumor showed high enhancement on enhanced dynamic CT and interval growth on serial imaging (n = 3); or percutaneous transthoracic fine-needle aspiration biopsy was technically unfeasible (n = 1). All 10 patients were women 40-73 years old (mean age, 52 years). Three of the 10 patients had no symptoms. In these patients, tumors were identified incidentally as a solitary pulmonary nodule on chest radiographs obtained at routine medical examinations. Seven patients had nonspecific symptoms such as a mild cough with sputum (n = 4), blood-tinged sputum (n = 2), or chest discomfort (n = 1).

All 10 patients prospectively underwent enhanced dynamic CT with a 4-MDCT scanner (LightSpeed QX/i, GE Healthcare) or a 16-MDCT scanner (LightSpeed 16, GE Healthcare). Our institutional review board approved the study protocol, and written informed consent was obtained from all patients. Before IV injection of contrast medium, a series of 13 images were obtained throughout each nodule, covering 30 mm along the z-axis with 2.5-mm collimation at 120 kVp, 170 mA, 0.8-second gantry rotation time, and a table speed of 3.75 mm/s over 8 seconds. Thereafter, an additional series of nine images were obtained at 20-second intervals after initiation of injection of contrast material (3 mL/s, total of 120 mL of iomeprol [Iomeron 300, Bracco]) with a power injector (MCT Plus, Medrad), according to the same parameters used for the initial unenhanced series (total of 10 series of images). Image data were reconstructed according to a standard algorithm with a thickness of 2.5 mm (13 images in each cluster; total number of dynamic images, 13 x 10 = 130 images). After the dynamic study, a helical scan (120 kVp, 50 mA, 5-mm collimation, table speed of 15 mm/s) was obtained 5 minutes after contrast injection from the lung apices to the level of the middle pole of both kidneys.


Figure 1
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Fig. 1A 47-year-old woman (patient 10) with sclerosing hemangioma with predominantly hemangiomatous and solid components. Mediastinal window of unenhanced CT scan (2.5-mm collimation) obtained at level of left upper lobar bronchus shows well-defined ovoid nodule in left upper lobe. Nodule has smooth margin and homogeneous internal attenuation.

 


Figure 2
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Fig. 1B 47-year-old woman (patient 10) with sclerosing hemangioma with predominantly hemangiomatous and solid components. Dynamic CT scan obtained 60 seconds after IV administration of contrast medium shows strong enhancement in right half of nodule (arrows) and less enhancement in left half (arrowheads).

 


Figure 3
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Fig. 1C 47-year-old woman (patient 10) with sclerosing hemangioma with predominantly hemangiomatous and solid components. CT scan 5 minutes after contrast injection shows washout (arrows) of initial strong enhancement in right half, delayed enhancement (arrowheads) in left half (solid and sclerotic area), and therefore homogeneous attenuation in entire nodule.

 


Figure 4
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Fig. 1D 47-year-old woman (patient 10) with sclerosing hemangioma with predominantly hemangiomatous and solid components. Low-magnification photomicrograph shows kidney-shaped nodule consisting of hemorrhagic hemangiomatous component (arrows) in right half of nodule and mixed hypercellular solid and sclerotic component (arrowheads) in left half. (H and E, x40)

 


Figure 5
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Fig. 1E 47-year-old woman (patient 10) with sclerosing hemangioma with predominantly hemangiomatous and solid components. Time-attenuation curve obtained at descending aorta (diamonds), strongly enhancing right component (squares), and weaker-enhancing left component (triangles) of nodule shows rapid and strong enhancement and washout in right half of hemangiomatous component and slow and weak but persistent enhancement in left half of solid and sclerotic component of nodule. In this patient, two types of nodular time-attenuation curve were plotted because nodule had two distinctive enhancement patterns.

 


Figure 6
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Fig. 2A 40-year-old woman (patient 8) with sclerosing hemangioma having mixed papillary and sclerotic components. Mediastinal window of unenhanced CT scan (2.5-mm collimation) obtained at level of distal bronchus intermedius shows well-defined ovoid nodule (arrows) in right middle lobe. Smooth margins and homogeneous internal attenuation are evident.

 


Figure 7
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Fig. 2B 40-year-old woman (patient 8) with sclerosing hemangioma having mixed papillary and sclerotic components. Dynamic CT scan 60 seconds after IV administration of contrast medium shows spotty enhancement (arrows) within nodule compared with strong enhancement in pulmonary and systemic arteries.

 


Figure 8
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Fig. 2C 40-year-old woman (patient 8) with sclerosing hemangioma having mixed papillary and sclerotic components. CT scan 90 seconds after contrast injection shows permeant enhancement (arrows) within nodule.

 


Figure 9
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Fig. 2D 40-year-old woman (patient 8) with sclerosing hemangioma having mixed papillary and sclerotic components. CT scan 5 minutes after injection shows homogeneous attenuation of entire nodule.

 


Figure 10
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Fig. 2E 40-year-old woman (patient 8) with sclerosing hemangioma having mixed papillary and sclerotic components. Low-magnification photomicrograph shows ovoid nodule with predominantly papillary component (straight arrows). Hemangiomatous (arrowheads) and sclerotic (curved arrows) components of tumor are evident. (H and E, x40)

 


Figure 11
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Fig. 3A 57-year-old woman (patient 9) with sclerosing hemangioma having predominantly solid and sclerotic components. Mediastinal window of unenhanced CT scan (2.5-mm collimation) at level of proximal left pulmonary artery shows small round nodule in superior segment of left lower lobe. Attenuation of nodule (65 H) is similar to that of chest wall muscle.

 


Figure 12
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Fig. 3B 57-year-old woman (patient 9) with sclerosing hemangioma having predominantly solid and sclerotic components. Dynamic CT scan 60 seconds after IV contrast administration shows enhancement (net enhancement, 33 H) similar to that of chest wall muscle. Curvilinear enhancement (arrows) is evident in periphery of nodule.

 


Figure 13
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Fig. 3C 57-year-old woman (patient 9) with sclerosing hemangioma having predominantly solid and sclerotic components. CT scan 5 minutes after contrast injection shows homogeneous attenuation of nodule.

 


Figure 14
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Fig. 3D 57-year-old woman (patient 9) with sclerosing hemangioma having predominantly solid and sclerotic components. Low-magnification photomicrograph shows round nodule with predominantly solid (large arrows) and some sclerotic (arrowhead) components. Hemangiomatous component (curved arrows) is evident in periphery. Cystlike features (small arrows) in nodule are caused by tissue distortion during freezing of pathologic specimen. (H and E, x40)

 
Image Analysis
All thin-section, dynamic CT, and conventional image data were directly interfaced to our PACS system (Centricity 2.0, GE Healthcare Integrated Imaging Solutions), which was used to display all image data on monitors (four monitors, 1,536 x 2,048 image matrices, 8-bit viewable gray-scale, and 60-foot-lambert luminescence). On the monitors, both mediastinal (window width, 400 H; window level, 20 H) and lung (window width, 1,500 H; window level, -700 H) window images were viewed.

The morphologic features of nodules determined by analysis of unenhanced thin-section CT scans included shape, margin characteristics, and presence of calcification. The internal texture of the nodules also was evaluated on unenhanced thin-section CT scans. The presence of hilar or mediastinal lymph node enlargement was assessed by evaluation of enhanced scans covering the entire thorax. Because a previous report suggested an intrafissural or juxtapleural tumor location [3], we also evaluated the relation between the nodule and the fissure or the pleura. Nodular size was defined as long-axis diameter on lung-window images. Nodular shapes were classified as round, ovoid, or rectangular. Nodular margins were classified as smooth, lobulated, or spiculated. Nodular texture was classified as homogeneous or heterogeneous. Calcification was classified as stippled, central nodular, laminated, or diffuse. When both sides of a nodule abutted the fissure, the nodule was defined as located within the fissure. When it abutted the chest wall at an obtuse angle, a nodule was defined as located in the pleura. Otherwise, the nodule was defined as intrapulmonary.

On dynamic studies, we evaluated whether a nodule showed homogeneous or inhomogeneous enhancement during or at the end of dynamic study. We also measured nodule attenuation values and the attenuation values of the descending thoracic aorta, when available, to ensure the adequacy of our dynamic study. We measured the great branch when the aorta was not included in the dynamic study. Nodule attenuation values, irrespective of the homogeneity of attenuation, were measured in the same areas on selected images for each series of images at each time. A circular region of interest was placed over a nodule or the descending thoracic aorta. For a given nodule, we examined a region of interest that covered approximately one half of the diameter of the nodule at the equator. On the descending thoracic aorta or great vessels, we also placed a region of interest that covered approximately one half of the diameter of the aorta or great vessels. All attenuation measurements were obtained from mediastinal-window images to ensure that partial volume averaging was minimized. Two radiologists, one with 7 and the other with 14 years of experience in chest CT, selected images together and then one radiologist measured the attenuation values. Two measurements obtained for each nodule at each imaging phase were averaged. Several dynamic characteristics of tumor enhancement were calculated from the time-attenuation curves: baseline attenuation (AVBase), peak enhancement (PE = attenuation at maximum), net enhancement (NE = PE - AVBase), time to peak enhancement (TPeak, time needed to reach peak enhancement), acceleration index (AI = NE / TPeak), attenuation value at 5-minute delay (AVDelay), and washout value (attenuation difference between PE and AVDelay, WV = PE - AVDelay).

Pathologic Comparisons
One lung pathologist with 11 years of experience in lung pathology evaluated all surgical pathologic specimens unaware of CT findings. Examinations focused on providing a histopathologic basis for the CT findings. With routine light microscopy and H and E staining, the volume extents of the four tumor components—papillary, sclerotic, solid, and hemangiomatous—were estimated to the nearest 10% level, and the predominant tumor component was determined. Extent of enhancement (peak enhancement and net enhancement) was correlated with the determined tumor component percentages.

Radiation Exposure
Radiation exposure doses to the lungs with our technique have been reported previously [8]. Radiation exposure to the breasts was measured with the same method as in the previous study [8]. Breast exposure doses were measured three times: first with the presumption that the nodule was located in the upper lung zone, second with the presumption that the nodule was located in the middle lung zone at a similar level to the breasts, and third with the presumption that the nodule was located in the lower lung zone.


Results
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Abstract
Introduction
Subjects and Methods
Results
Discussion
References
 
All sclerosing hemangiomas were located in the lungs. Seven tumors were in the right lung and three in the left. Two hemangiomas were found in the upper lobes, four in the right middle lobe or the lingular division of the left upper lobe, and four in the lower lobes.

Mean tumor long-axis diameter was 18 ± 5 mm (SD; range, 10-24 mm). Six tumors were round and four ovoid. All tumors had smooth margins (Figs. 1A, 1B, 1C, 1D, 1E, 2A, 2B, 2C, 2D, 2E, 3A, 3B, 3C, and 3D). The internal texture of the tumors before contrast enhancement was homogeneous in all cases, but during dynamic studies after contrast enhancement, four (40%) of the tumors were homogeneous and six (60%) were heterogeneous. On 5-minute images, all nodules were homogeneous. Three nodules (30%) contained an intratumoral calcification, and all were stippled. Mediastinal or hilar lymph node enlargement was not found in any case.

Enhanced dynamic CT study was feasible for all 10 patients. The dynamic CT characteristics of the 10 cases are summarized in Table 1. Mean baseline tumor attenuation was 51 ± 11 H. The mean tumor peak enhancement value was 124 ± 14 H, and the mean net enhancement value was 74 ± 18 H. The concurrently measured peak enhancement value of the thoracic aorta was 273 ± 51 H. The mean time to peak enhancement was 57 ± 12 seconds for tumors, whereas the mean time to peak enhancement for the aorta and great vessels was 39 ± 6 seconds. The time difference between time to peak enhancement of the aorta and that of the tumor was 18 ± 14 seconds. The mean washout value for tumors after the 5-minute delay was 17 ± 7 H.


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TABLE 1: Dynamic CT Characteristics Versus Histopathologic Components of 10 Sclerosing Hemangiomas

 

Five tumors had a predominantly papillary component, four were hemangiomatous, and one was solid. In one case (patient 7, Table 1) in which the hemangiomatous and papillary components were equally included, the tumor was classified as hemangiomatous. No tumor had a predominantly sclerotic component. Dynamic CT characteristics correlated with the predominant tumor components are summarized in Table 2. Except for the tumor with a predominantly solid component (Figs. 3A, 3B, 3C, and 3D.), in which peak enhancement (98 H) and net enhancement (33 H) were noticeably low, dynamic CT parameters were similar in all tumors (Table 2).


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TABLE 2: Dynamic CT Characteristics According to Histopathologic Type of Sclerosing Hemangioma

 

The measured total lung dose ranged from 186 to 192 mGy at nodule sites. Organ dose ranged from 24 to 26 mGy elsewhere in the lung. The measured total breast dose was 19-20 mGy when the nodule was in the upper lung zone, 233-241 mGy when the nodule was in the middle lung zone and on the same transaxial plane as the breast, and 24-26 mGy when the nodule was in the lower lung zone.


Discussion
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Abstract
Introduction
Subjects and Methods
Results
Discussion
References
 
Sclerosing hemangioma is composed of two types of cells: cuboidal cells that line papillary structures and round to polygonal cells that form solid sheets. The neoplasm has four possible histologic components: papillary, sclerotic, solid, and hemorrhagic. Most tumors have at least three of these components, and a minority have only two. In the large series reported to date, no tumor has shown a single component [9].

In our study, sclerosing hemangioma had several morphologic characteristics on unenhanced CT images: a round or ovoid shape (100% of cases), a smooth margin (100%), homogeneous attenuation (100%), and calcification (30%). These morphologic features are characteristic enough for differentiation of sclerosing hemangioma from malignant nodules, because malignant nodules have a lobulated and spiculated margin with occasional calcification [9]. However, these morphologic features of sclerosing hemangioma can overlap with the CT findings of other benign nodules such as hamartoma. In such situations, dynamic studies showing characteristic strong and early enhancement may allow the differential diagnosis of sclerosing hemangioma and other benign pulmonary tumors.

Strong enhancement of sclerosing hemangioma suggests the possibility that the nodule is malignant [8, 10]. According to a study by Yi et al. [8], who used the same dynamic study protocol that are used to evaluate solitary pulmonary nodules, malignant nodules had higher mean peak enhancement (98 ± 17 H vs 78 ± 30 H, p < 0.001) and mean net enhancement (53 ± 15 H vs 35 ± 26 H, p < 0.001) values and lower mean time to peak enhancement (103 ± 43 seconds vs 119 ± 45 seconds, p = 0.03) than benign nodules. In our study, however, sclerosing hemangioma had even more rapid (time to peak enhancement, 57 ± 12 seconds) and stronger (peak enhancement, 124 ± 14 H; net enhancement, 74 ± 18 H) enhancement than malignant nodules. These dynamic study results integrated with morphologic CT findings (i.e., round or ovoid shape, smooth margin, and homogeneous attenuation) allow differentiation of sclerosing hemangioma and malignant nodules. However, because lung cancer also has well-defined margins and similar strong and rapid enhancement, we cannot completely exclude the possibility of lung cancer on the basis of results of CT morphologic analysis and enhanced dynamic CT.

Benign tumors that resemble sclerosing hemangioma in terms of morphologic and dynamic features include leiomyoma and a small percentage of highly vascular hamartomas. However, differentiation of these tumors is clinically insignificant because of the common prognostic implications. Highly vascular solitary metastatic cancers, such as pulmonary metastatic renal cell carcinoma, have morphologic and dynamic CT features similar to those of sclerosing hemangioma.

Sclerosing hemangioma has been described as a benign tumor with strong enhancement after IV administration of contrast medium [3]. It has been reported that sclerosing hemangioma shows homogeneous enhancement, maximum CT values ranging from 90 to 110 H [5]. In our dynamic study, nine of 10 tumors had more than 60-H net enhancement. The mean peak enhancement value of 10 sclerosing hemangiomas was 124 H, and the mean net enhancement value was 74 H. The mean time to peak enhancement was 57 seconds, which is similar to the rapid enhancement shown by hypervascular tumors of the liver and kidneys [11]. Our pathologic study showed that the single case of a compact solid-type tumor had slow and persistent enhancement with little washout. Moreover, this slow and persistent enhancement was found in the lower-attenuation area on the time-attenuation curve when the tumors showed heterogeneous enhancement (one part strong enhancement and the other part less enhancement, as in Figs. 1A, 1B, 1C, 1D, and 1E) during the dynamic study. It therefore appears that the dynamic characteristics of sclerosing hemangioma depend on the levels of hemangiomatous or papillary (early and strong enhancement component) and solid or sclerotic (slow and persistent enhancement and little washout) components present.

Unusual manifestations of sclerosing hemangioma include mediastinal mass [12], tumor with a cystic appearance [13, 14], air trapping around the tumor [15-18], and tumor within an extralobar pulmonary sequestration. When it presents with a cystic appearance, sclerosing hemangioma should be differentiated from clear cell tumor, intrapulmonary thymoma, pleuropulmonary blastoma type 1, and alveolar adenoma [14]. Given the limited number of patients enrolled, we did not have the opportunity to see such unusual cases. When sclerosing hemangioma manifests atypical morphologic characteristics during imaging studies or at pathologic examination, a wide range of differential diagnostic techniques may be needed. Immunohistochemical analysis can help differentiate sclerosing hemangioma (epithelial origin, type 2 pneumocyte) from other tumors (mesothelial or submesothelial origin) on the basis of tumor origin. The presence of antibodies of thyroid transcription factor 1, surfactant protein B, vimentin, and cytokeratin L is helpful in differentiating sclerosing hemangioma.

In cystic tumors, the tumor is composed of a cystic wall (fibrous and partly hyalinized), tumor cells have various histologic patterns, and red blood cells accumulate within the cavity [13]. The mechanism of formation of the air-trapping zone surrounding the tumor at CT and the air meniscus sign on chest radiographs has been suggested as being peritumoral hemorrhage followed by clearance through the airway creating a peritumoral air space.

Although generally regarded as benign, sclerosing hemangioma can appear with hilar or mediastinal nodes or lung-to-lung metastasis, especially when the tumor is large [19, 20]. Sclerosing hemangioma is considered to originate from a primitive respiratory epithelium [21]. Therefore, nodal or lung-to-lung metastasis is not exceptional. In our study, however, neither nodal nor pulmonary metastasis was identified.

Our study had several limitations. First, there was selection bias because we included histopathologically proven cases only. Although the tumors were found to have mainly papillary and hemangiomatous components, we do not know precisely which components are actually predominant. Second, the high level of radiation exposure used in the dynamic study was a shortcoming. Measured lung radiation dose at the nodule location was 186-192 mGy, five times higher than the 38-40 mGy with standard MDCT [8]. Moreover, the measured breast dose was 233-241 mGy for nodules in the middle lung zone and on the same transaxial plane as the breasts. A low-dose technique with reduced radiation risk or truncation of the dynamic CT technique (30- or 60-second intervals rather than 20-second intervals) would have been more advisable for the dynamic study. Third, because we did not perform a blinded prospective study comparing the series of sclerosing hemangiomas with malignant nodules, we do not know how accurate the given morphologic and dynamic CT features are for differentiating sclerosing hemangioma from malignant nodules.

In conclusion, sclerosing hemangioma appears morphologically as a small, round, or oval homogeneous nodule with a smooth margin that on dynamic CT has strong and rapid enhancement caused primarily by hemangiomatous or papillary components.


References
Top
Abstract
Introduction
Subjects and Methods
Results
Discussion
References
 

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