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DOI: 10.1148/radiol.2462042033
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(Radiology 2008;246:634-637.)
© RSNA, 2008

Diagnosis Please

Case 130: Mediastinal Hemangioma1

Prachi P. Agarwal, MD, Jean M. Seely, MD and Frederick R. K. Matzinger, MD, FRCP(C)

1 From the Department of Diagnostic Imaging, the Ottawa Hospital, Civic Campus, 1053 Carling Ave, Ottawa, ON, Canada K1Y 4E9. Received November 29, 2004; revision requested January 28, 2005; revision received February 17; final version accepted March 15.

Correspondence: Address correspondence to J.M.S. (e-mail: jeseely{at}ottawahospital.on.ca).


   HISTORY
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 HISTORY
IMAGING FINDINGS
 DISCUSSION
 References
 
A 37-year-old healthy woman who did not smoke underwent computed tomography (CT) and magnetic resonance (MR) imaging of the abdomen for further work-up of a hepatic lesion that was incidentally detected at ultrasonography (US) and was thought to be an atypical liver hemangioma. US was performed to assess mild abdominal discomfort. The patient's medical history was unremarkable. The liver lesion had characteristics of a proteinaceous cyst on CT and MR images; however, upper sections of abdominal CT scans revealed a mass in the right cardiophrenic angle. Thus, CT and MR images of the chest were obtained.


   IMAGING FINDINGS
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 IMAGING FINDINGS
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The scout radiograph (Fig 1) showed a smoothly marginated 10-cm-diameter mass in the area of the right cardiophrenic angle that obscured the cardiac border. This mass had a broad base at the mediastinum and formed an obtuse angle superiorly in keeping with a mediastinal location. Careful inspection revealed punctate calcific opacities within the mass. This finding was confirmed with transverse unenhanced CT (Fig 2). A calcification showed central lucency and had a ringlike appearance. The mediastinal mass demonstrated heterogeneity and consisted of soft-tissue and fat attenuation with multiple scattered calcifications. When 150 mL of iohexol (Omnipaque 240; Winthrop-Breon Laboratories, New York, NY) was administered, nodular and tubular enhancing structures were identified, with a degree of enhancement comparable to that of the adjacent inferior vena cava (Fig 3).


Figure 1
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  		Figure 1: Frontal scout radiograph obtained before chest CT shows a well-marginated mass in the right cardiophrenic angle area obscuring the cardiac border. This mass contains a few punctuate calcifications (arrow).

 

Figure 2
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  		Figure 2: Transverse unenhanced CT scan of the chest at the level of the cardiac ventricles. There is a heterogenous anterior mediastinal mass in the right cardiophrenic angle. It contains discrete nodular areas of soft tissue interspersed in fat and multiple punctuate calcifications. One of the calcifications (arrow) shows central lucency characteristic of a phlebolith.

 

Figure 3
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  		Figure 3: Transverse contrast material–enhanced CT image of the chest at the level of the cardiac ventricles. Nodular and tubular enhancing structures can be seen.

 
T1-weighted MR imaging revealed a heterogenous mass of predominantly intermediate signal intensity (Fig 4). Numerous nodular intermediate-signal-intensity areas were interspersed in high-signal-intensity areas that represented fat. T2-weighted MR imaging revealed a markedly hyperintense mass lesion with a flow void that represented an enlarged vein draining into the inferior vena cava (Fig 5). After intravenous administration of contrast material, multiple tortuous serpentine enhancing structures were identified on MR images obtained with the volume-interpolated breath-hold examination sequence (Fig 6). Enhancement of the draining vein was also seen on delayed images (not shown).


Figure 4
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  		Figure 4: Transverse T1-weighted fast low-angle shot MR image (repetition time msec/echo time msec, 186/4.6; 70° flip angle) of the chest shows a heterogenous mass in the mediastinum composed of nodular intermediate-signal-intensity areas interspersed within higher-signal-intensity areas.

 

Figure 5
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  		Figure 5: Transverse T2-weighted fast spin-echo MR image (5000/92) of the chest shows a predominantly hyperintense mass. An enlarged vein (arrow) drains into the inferior vena cava.

 

Figure 6
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  		Figure 6: Transverse gadolinium-enhanced (20 mL of gadodiamide [287 mg of gadolinium per milliliter], Omniscan; GE Healthcare, Princeton, NJ]) three-dimensional volumetric interpolated breath-hold MR image (3.55/1.6, 12° flip angle) of the chest shows areas of serpentine enhancement.

 
Coronal true fast imaging with steady-state precession MR revealed the craniocaudal extent of the mass (Fig 7). The mass was closely related to the right side of the pericardium, without any definite evidence of invasion. There was no pericardial effusion.


Figure 7
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  		Figure 7: Coronal true fast imaging with steady-state precession MR image (4.44/2.22, 70° flip angle) of the chest shows the mass is closely related to the right side of the pericardium. There is no evidence of pericardial invasion or effusion.

 

   DISCUSSION
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 HISTORY
 IMAGING FINDINGS
 DISCUSSION
References
 
The differential diagnosis of a heterogenous mass in the anterior mediastinum with soft-tissue and calcific areas interspersed with fat includes mediastinal lymphoma, teratoma, and hemangioma. Morgagni hernia is a common fat-containing mass that occurs in the right cardiophrenic angle (1). However, the presence of numerous punctate calcifications and marked hyperintensity on T2-weighted MR images makes this diagnosis unlikely. In addition, patients with Morgagni hernia containing omentum have small omental vessels (1), whereas dilated vessels were seen in this case.

Mediastinal lymphoma can appear as a heterogenous mediastinal mass composed of adjacent lymph nodes in mediastinal fat. However, calcification in patients with untreated mediastinal lymphoma is exceedingly rare, and this diagnosis was considered unlikely (2).

Several key imaging features helped us make the correct diagnosis of mediastinal hemangioma. First, numerous nodular and tubular soft-tissue attenuating structures that showed vascular enhancement on CT and MR images were identified in the mass. A dilated vein draining into the inferior vena cava was also seen. This appearance was highly suggestive of mediastinal hemangioma rather than teratoma. Additionally, the mass had a characteristic appearance on MR images. It was heterogenous on T1-weighted images with nodular intermediate-signal-intensity areas interspersed with higher-signal-intensity areas that represented fat. The mass was markedly hyperintense on T2-weighted images. This appearance can be attributed to the high vascular space content of hemangiomas (3). Also, multiple rounded calcifications—one with a ringlike appearance and central lucency—were seen in the mass. These findings were consistent with a diagnosis of phleboliths. Although several patterns of calcification (ie, amorphous, peripheral curvilinear, and punctuate) and calcification resembling that of teeth or bones (4) can be seen in a patient with a mediastinal teratoma, phleboliths are virtually diagnostic of hemangioma (5). A potentially confounding feature in this case was fat in the mediastinal mass (6). This has been described in patients with hemangiomas; however, it is seen more commonly in teratomas (6). However, a confident diagnosis of hemangioma was made on the basis of multiple enhancing vascular channels, phleboliths, and a characteristic MR appearance.

Lymphangioma is another mediastinal mass that should be considered in the differential diagnosis of hemangioma. However, lymphangiomas usually are cystic lesions that typically do not enhance after contrast material administration (7). Calcification is an unusual feature of lymphangioma (7). Also, marked enhancement in a patient with lymphangioma is rare and has been reported only with associated hemangiomatous components or vascular aneurysm (7). The previously mentioned features made lymphangioma an unlikely diagnosis.

Hemangiomas are uncommon benign vascular tumors that account for less than 0.5% of all mediastinal masses (5,8). Mediastinal hemangiomas usually occur in young patients, and up to 75% of these lesions manifest before the age of 35 years (5). There is no definite sex predilection (5,9). Patients are usually asymptomatic at presentation; however, some may present with cough, stridor, hoarseness, chest pain, or dysphagia due to mass effect (9).

The cause of hemangiomas is unclear. It has been speculated that they represent either true neoplasms or developmental vascular anomalies (10,11).

At histologic analysis, these tumors consist of large interconnecting vascular spaces lined by flattened cuboidal epithelium along with a varying amount of interspersed stromal elements, such as fat, myxoid, and fibrous tissues (6). They are categorized as capillary, cavernous, or venous hemangiomas on the basis of the size of the vascular spaces. More than 90% are capillary or cavernous (12). Most mediastinal hemangiomas are found in the anterior mediastinum; however, a few arise in the posterior mediastinum (13).

It is often difficult to diagnosis hemangioma preoperatively, as the lesion usually manifests as a nonspecific mass (6). However, phleboliths are a potentially diagnostic feature, seen on up to 10% of conventional radiographs. Calcification is more readily identifiable on CT scans (6). Punctate calcification is seen more commonly and needs to be differentiated from the calcification seen in patients with teratoma or cartilaginous tumors (6).

Hemangiomas commonly appear as heterogenous lesions on unenhanced CT scans. Their appearance depends on the stromal content and degree of thrombosed vascular channels (11). Contrast enhancement is most often heterogenous and central (6). Gradually increasing and persistent enhancement has been seen on dynamic contrast-enhanced CT scans (14). Large draining veins, like those seen in this patient, have also been seen on delayed images (15).

In summary, the findings of phleboliths and multiple enhancing vessels and the characteristic MR imaging appearance made mediastinal hemangioma the most likely diagnosis.


   FOOTNOTES
 
Authors stated no financial relationship to disclose.


Part one of this case appeared 4 months previously and may contain larger images.

 


   References
TOP
 HISTORY
 IMAGING FINDINGS
 DISCUSSION
 References
 

  1. Gilkeson RC, Basile V, Sands MJ, Hsu JT. Chest case of the day: Morgagni's hernia. AJR Am J Roentgenol 1997;169:266, 268–270.[Medline]
  2. Alobeidy ST, Ilowite J, Donovan V, Selbs E, Badler R, Katz DS. Calcification in untreated mediastinal Hodgkin's lymphoma. J Thorac Imaging 2001;16:304–306.[CrossRef][Medline]
  3. Nchimi A, Ghaye B, Szapiro D, Thiry A, Dondelinger RF. A complex anterior mediastinal mass: demonstration of pericardial haemangioma by dynamic MRI (2003:10b). Eur Radiol 2004;14:160–163.[CrossRef][Medline]
  4. Moeller KH, Rosado-de-Christenson ML, Templeton PA. Mediastinal mature teratoma: imaging features. AJR Am J Roentgenol 1997;169:985–990.[Abstract/Free Full Text]
  5. Davis JM, Mark GJ, Greene R. Benign blood vascular tumors of the mediastinum: report of four cases and review of the literature. Radiology 1978;126:581–587.[Abstract]
  6. McAdams HP, Rosado-de-Christenson ML, Moran CA. Mediastinal hemangioma: radiographic and CT features in 14 patients. Radiology 1994;193:399–402.[Abstract/Free Full Text]
  7. Shaffer K, Rosado-de-Christenson ML, Patz EF Jr, Young S, Farver CF. Thoracic lymphangioma in adults: CT and MR imaging features. AJR Am J Roentgenol 1994;162:283–289.[Abstract/Free Full Text]
  8. Cohen AJ, Sbaschnig RJ, Hochholzer L, Lough FC, Albus RA. Mediastinal hemangiomas. Ann Thorac Surg 1987;43:656–659.[Abstract]
  9. Buckner S, McAllister J, D'Altorio R. Case of the season: hemangioma of the middle mediastinum. Semin Roentgenol 1994;29:98–99.[CrossRef][Medline]
  10. Pachter MR, Lattes R. Mesenchymal tumors of the mediastinum. Cancer 1963;16:95–106.[CrossRef]
  11. Gindhart TD, Tucker WY, Choy SH. Cavernous hemangioma of the superior mediastinum. Am J Surg Pathol 1979;3:353–361.[Medline]
  12. Abe K, Akata S, Ohkubo Y, et al. Venous hemangioma of the mediastinum. Eur Radiol 2001;11:73–75.[CrossRef][Medline]
  13. Klecker RJ, Sinclair DS, King MA. Case 1: mediastinal hemangioma. AJR Am J Roentgenol 2000;175:866; 868–869.
  14. Cheung YC, Ng SH, Wan YL, Tan CF, Wong HF, Ng KK. Dynamic CT features of mediastinal hemangioma: more information for evaluation. Clin Imaging 2000;24:276–278.[CrossRef][Medline]
  15. Roach H, Chowdhury P, Adams H. An incidental finding. Br J Radiol 2003;76:753–754.[Free Full Text]
Congratulations to the 31 individuals and five resident groups that submitted the most likely diagnosis (mediastinal hemangioma) for Diagnosis Please, Case 130. The names and locations of the individuals and resident groups, as submitted, are as follows:

Individual responses

Fahad Azzumeea, MBBS, Montreal, Quebec, Canada
Douglas C. Brown, MD, Virginia Beach, Va
Antonio A. Cavalcanti, MD, São Paulo, Brazil
Michael H. Childress, MD, Silver Spring, Md
Marc G. De Baets, MD, Collina d'Oro, Switzerland
Mustafa Kemal Demir, MD, Istanbul, Turkey
Thaworn Dendumrongsup, MD, Songkhla, Thailand
Gilles Genin, Annecy, France
Teeranan Intharapat, MD, Hat Yai, Songkhla, Thailand
Yasuhiro Kawahara, Nagasaki, Japan
Mario A. Laguna, MD, Milwaukee, Wis
Naganathan B. Mani, MD, Miami, Fla
Frank J. McKowne, MD, Vancouver, Wash
Sunil L. Mehta, MD, Mississauga, Ontario, Canada
Manabu Minami, MD, PhD, Yokohama, Japan
Sankar R. Mondal, MD, Nassau, Bahamas
Tetsuo Nakayama, MD, Osaka, Japan
Tammam N. Nehme, MD, East Wenatchee, Wash
Cinthia D. Ortega, MD, São Paulo, Brazil
Narendrakumar P. Patel, MD, Newburgh, NY
Rubem Pochaczevsky, MD, Bronx, NY
Ilias Primetis, MD, Athens, Greece
Daniel C. Rappaport, MD, Toronto, Ontario, Canada
Matthew C. Rheinboldt, MD, Nashville, Tenn
Taro Shimono, MD, Osaka, Sayama, Japan
Ana C. Silva, MD, Porto, Portugal
Norio Takahashi, MD, Fukui, Japan
Yukihisa Takayama, MD, Columbus, Ohio
Ayako Tamura, MD, Tokyo, Japan
Ricardo Luis Videla, MD, Córdoba, Argentina
Ivan Vollmer, MD, Barcelona, Spain

Resident group responses

Armed Forces Medical College Radiology Residents, Mumbai, Maharashtra, India
Fukushima University Radiology Residents, Fukushima, Japan
Hospital Italiano Córdoba Radiology Residents, Córdoba, Argentina
Prince of Songkla University Radiology Residents, Songkla, Thailand
University of Pennsylvania Radiology Residents, Philadelphia, Pa




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P. P. Agarwal, F. R. K. Matzinger, and J. M. Seely
Case 132: Lymphangiomatosis
Radiology, April 1, 2008; 247(1): 288 - 290.
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