DOI: 10.1148/radiol.2471042092
(Radiology 2008;247:288-290.)
© RSNA, 2008
Case 132: Lymphangiomatosis1
Prachi P. Agarwal, MD,
Frederick R. K. Matzinger, MD, FRCP(C) and
Jean M. Seely, MD
1 From the Department of Diagnostic Imaging, the Ottawa Hospital, Civic Campus, 1053 Carling Ave, Ottawa, ON, Canada K1Y 4E9. Received December 9, 2004; revision requested February 2, 2005; revision received March 6; final version accepted April 4.
Address correspondence to P.P.A. (e-mail: prachipragya{at}yahoo.com).
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HISTORY
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A 37-year-old healthy female nonsmoker presented with hemoptysis. Her medical history was remarkable for sickle cell trait and iron deficiency anemia. She had not traveled outside North America. Computed tomography (CT) and magnetic resonance (MR) imaging were performed in the chest and abdomen.
At review of the patient's history, we learned that she had been examined 5 years earlier for a "lesion in [the] right hemithorax," the cause of which remained obscure. No prior imaging studies were available for review.
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IMAGING FINDINGS
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Contrast material–enhanced CT revealed a mass that was located predominantly in the right lower hemithorax but extended across the midline to the opposite side of the body, consistent with a mediastinal location (Fig 1). This low-attenuation lesion was seen in the middle and posterior portions of the mediastinum and showed no discernible wall or central enhancement from intravenous contrast material. There was some contrast-enhanced compressed lung tissue adjacent to the lesion. The mass enveloped the right inferior pulmonary vein without displacing, narrowing, or deforming it, and thus was suggestive of a soft pliable nature (Fig 2). Another discrete nonenhancing low-attenuation lesion was noted in a right paratracheal location (Fig 3). A small right pleural effusion was seen (Fig 3), and multiple cystic lesions were identified in the spleen (Fig 4).
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Figure 1: Transverse contrast-enhanced (60 mL of nonionic iohexol [Omnipaque 300; Amersham Health, Princeton, NJ]) CT scan of the chest at the level of the cardiac ventricles reveals a low-attenuating mass located predominantly on the right side of the body but extending across the midline to the left side.
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Figure 2: Transverse contrast-enhanced CT scan of the chest at the level of the inferior pulmonary vein shows the mass in the right hemithorax enveloping the right inferior pulmonary vein (arrow) without displacing, narrowing, or altering its course.
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Figure 3: Transverse contrast-enhanced CT scan of the chest at the level of the aortic arch shows a well-circumscribed low-attenuation lesion (arrow) in the right paratracheal location and a small right pleural effusion. The attenuation of the paratracheal lesion appears to be similar to that of the effusion.
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MR imaging depicted the anatomic extension of this lesion better than did CT. This mass was isointense to muscle on images obtained with a fast low-angle shot two-dimensional MR sequence (not shown) and was hyperintense to muscle on images acquired with a half-Fourier single-shot fast spin-echo sequence (Fig 5). Hypointense linear structures likely representing septa were seen on images acquired with a half-Fourier single-shot fast spin-echo sequence (Fig 5). The signal intensity characteristics of the splenic lesions were identical to those of the thoracic lesion. No bone lesions were apparent.
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Figure 5: Coronal half-Fourier single-shot fast spin-echo MR image (repetition time msec/echo time msec, 1120/65) of the thoracoabdominal area shows the lesion in the right lower hemithorax is hyperintense. Linear hypointense structures (arrows) seen within it likely represent septa. Spleen shows multiple hyperintense lesions.
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DISCUSSION
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The differential diagnosis of a cystic mass in the mediastinum includes necrotic malignant tumors and lymph nodes, teratoma, foregut duplication cyst, hydatid cyst, mediastinal abscess or pancreatic pseudocyst, hemangioma, and lymphangioma (1). The long-standing nature of the mass, a thin barely perceptible wall without any enhancement of cyst contents, and absence of invasion of adjacent mediastinal structures are features that argue against a malignant origin (2). The lesion we observed wraps around and conforms to the shape of the right inferior pulmonary vein without any distortion, displacement, or narrowing of the vessel.
Other benign mediastinal cysts—such as teratoma, foregut duplication cyst, or hydatid cyst—are also unlikely. Although the mass was seen predominantly in the right middle and posterior portions of the mediastinum, it crossed the midline to the left side of the body and was also seen in the right paratracheal location. Teratomas are commonly located in the anterior part of the mediastinum, and only 3%–8% of teratomas occur in the posterior part of the mediastinum (3). In addition, they are seen as heterogeneous tumors on CT scans and usually contain variable amounts of fat, fluid, soft tissue, and calcium (4). According to Moeller et al (5), teratomas contain soft tissue in virtually all cases. Similarly, foregut duplication cysts are usually solitary and unilocular with a thin imperceptible wall (2), whereas the lesions in this patient were multifocal and septate. Hydatid cysts can occur in multiple locations. However, the patient did not have a history of travel to endemic areas, making this diagnosis less likely. Also, despite its large size, the lesion did not exhibit any mass effect in the form of displacement of adjacent structures. It molded and enveloped the inferior pulmonary vein and extended into the potential space of the mediastinum on the opposite side, indicating a yielding nature. The lack of mass effect and the continuous extension across the mediastinum support a diagnosis of lymphangioma (6) rather than hydatid disease.
Mediastinal abscesses and pancreatic pseudocysts occur in specific clinical scenarios. Mediastinal abscesses usually arise as a result of surgery, esophageal perforation, or spread of infection from adjacent structures (2). Pancreatic pseudocysts may manifest as cystic posterior mediastinal masses that develop after an acute episode of pancreatitis (2). These were unlikely in this case, as the patient did not have a history of prior surgery or clinical features suggestive of sepsis, esophageal perforation, or pancreatitis.
Rarely, mediastinal hemangiomas manifest as cystic masses in the mediastinum (7). The presence of vascularization and phleboliths supports a diagnosis of hemangioma (8). Unlike patients with hepatic cavernous hemangiomas, in whom phleboliths rarely are seen, 10% of patients with mediastinal hemangiomas may have phleboliths (9). None of the previously mentioned features were present in this patient; therefore, lymphangioma was the favored imaging diagnosis. The presence of multiple lymphangiomas, as in this patient, is termed lymphangiomatosis. Multiple splenic cystic lesions probably represent lymphangiomas as well.
The patient had experienced hemoptysis on more than one occasion; therefore, surgery was performed to resect the mediastinal mass. The hemoptysis was considered to originate from a focus of chronic inflammation within compressed lung tissue in the right lower lobe adjacent to the mass. The diagnosis of mediastinal lymphangioma was confirmed at surgery. At gross examination, the mass was cystic, with clear serous fluid, and had multiple dilated lymphatic channels. The lung parenchyma was congested but otherwise unremarkable. Right pleural fluid was clear and not chylous.
Lymphangiomas represent embryologic remnants of lymphatic tissues that fail to connect to efferent channels or arise from lymph sacs sequestered during development (10). These constitute 0.7%–4.5% of mediastinal tumors (11). Ninety percent of these tumors manifest as a mass in the neck or axilla in children younger than 2 years (12). On the contrary, in adults, these tumors are more commonly mediastinal (11) and are often recurrences of incompletely resected childhood tumors (13). However, in a study of 19 patients with adult thoracic lymphangiomas, up to 79% of lymphangiomas occurred without a history of prior lymphangioma (14). In this patient, there was no history of lymphangioma resection.
Patients with lymphangiomas may remain asymptomatic; however, symptoms include cough, dyspnea, stridor, hemoptysis, Horner syndrome, dysphagia, superior vena cava syndrome, constrictive pericarditis, phrenic nerve palsy, or symptoms secondary to infection (15). Lymphangiomatosis lesions can occur in any tissue where lymphatics are found (16). Lymphangiomatosis is frequently associated with other lymphatic abnormalities and may involve multiple organs in up to 75% of cases (17).
In summary, an infiltrative cystic mass with a barely perceptible wall enveloping or conforming to the shape of adjacent mediastinal structures without substantial mass effect can suggest the diagnosis of lymphangiomatosis in the appropriate clinical setting.
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FOOTNOTES
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Authors stated no financial relationship to disclose.
| Part one of this case appeared 4 months previously and may contain larger images.
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References
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- Charruau L, Parrens M, Jougon J, et al. Mediastinal lymphangioma in adults: CT and MR imaging features. Eur Radiol 2000;10:1310–1314.[CrossRef][Medline]
- Jeung MY, Gasser B, Gangi A, et al. Imaging of the cystic masses of the mediastinum. RadioGraphics 2002;22(Spec Issue):S79–S93.[Abstract/Free Full Text]
- Rosado-de-Christenson ML, Templeton PA, Moran CA. From the archives of the AFIP: mediastinal germ cell tumors—radiologic and pathologic correlation. RadioGraphics 1992;12:1013–1030.[Abstract]
- Tecce PM, Fishman EK, Kuhlman JE. CT evaluation of the anterior mediastinum: spectrum of disease. RadioGraphics 1994;14:973–990.[Abstract]
- 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]
- Oshikiri T, Morikawa T, Jinushi E, Kawakami Y, Katoh H. Five cases of the lymphangioma of the mediastinum in adult. Ann Thorac Cardiovasc Surg 2001;7:103–105.[Medline]
- Kita Y, Nogimura H, Ohi S, et al. Thoracoscopically resected cystic mediastinal hemangioma: report of a case [in Japanese]. Kyobu Geka 2004;57:497–500.[Medline]
- Agarwal PP, Seely JM, Matzinger FRK. Case 130: mediastinal hemangioma. Radiology 2008;246(2):634–637.[Free Full Text]
- Seline TH, Gross BH, Francis IR. CT and MR imaging of mediastinal hemangioma. J Comput Assist Tomogr 1990;14:766–768.[Medline]
- Laurence KM. Congenital pulmonary cystic lymphangiectasis. J Pathol Bacteriol 1955;70:325–333.[CrossRef][Medline]
- Shenoy SS, Barua NR, Patel AR, Culver GJ, Jennings EC. Mediastinal lymphangioma. J Surg Oncol 1978;10:523–528.[CrossRef][Medline]
- Nanson EM. Lymphangioma (cystic hygroma) of the mediastinum. J Cardiovasc Surg (Torino) 1968;9:447–452.[Medline]
- Brown LR, Reiman HM, Rosenow EC, Gloviczki PM, Divertie MB. Intrathoracic lymphangioma. Mayo Clin Proc 1986;61:882–892.[Medline]
- 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]
- Holden WE, Morris JF, Antonovic R, Gill TH, Kessler S. Adult intrapulmonary and mediastinal lymphangioma causing haemoptysis. Thorax 1987;42:635–636.[Free Full Text]
- Rosai J. Lymph vessels. In: Rosai J, ed. Ackerman's surgical pathology. 8th ed. St Louis, Mo: Mosby, 1995; 2221–2226.
- Faul JL, Berry GJ, Colby TV, et al. Thoracic lymphangiomas, lymphangiectasis, lymphangiomatosis, and lymphatic dysplasia syndrome. Am J Respir Crit Care Med 2000;161:1037–1046.[Free Full Text]
Congratulations to the 36 individuals and four resident groups that submitted the most likely diagnosis (lymphangiomatosis) for Diagnosis Please, Case 132. The names and locations of the individuals and resident groups, as submitted, are as follows:
Individual responses
- Harry A. Allen III, MD, Virginia Beach, Va
- Gregory J. Balmforth, MD, Spokane, Wash
- Eric L. Bressler, MD, Minnetonka, Minn
- Marco A. Cura, MD, San Antonio, Tex
- Mustafa Kemal Demir, MD, Istanbul, Turkey
- Thaworn Dendumrongsup, MD, Songkhla, Thailand
- Walter T. Depaulaneto III, MD, Rio de Janeiro, Brazil
- Seyed A. Emamian, MD, PhD, Rockville, Md
- Akira Fujikawa, MD, Setagaya, Tokyo, Japan
- Gilles Genin, Annecy, France
- Mark G. Goldshein, MD, Andover, Mass
- Bruno Miguel Graca, MD, Coimbra, Portugal
- Pramod K. Gupta, MD, Plano, Tex
- Ferris M. Hall, MD, Brookline, Mass
- Helen T. Ho, MD, Chicago, Ill
- James F. Lally, MD, Wallingford, Pa
- Xiao Xin Lan, MD, Nanchang, Jiangxi, China
- David A. Lisle, MBBS, Brisbane, Australia
- Naganathan B. Mani, MD, Miami, Fla
- Waldir H. Maymone, MD, Rio de Janeiro, Brazil
- Tammam N. Nehme, MD, East Wenatchee, Wash
- Cinthia D. Ortega, MD, São Paulo, Brazil
- Klaus Orth, Aachen, Germany
- David M. Panicek, MD, New York, NY
- Ilias Primetis, MD, Athens, Greece
- Daniel C. Rappaport, MD, Toronto, Ontario, Canada
- Matthew C. Rheinboldt, MD, Nashville, Tenn
- Tsutomu Sakamoto, MD, Tokyo, Japan
- Hideki Shima, MD, Tokyo, Japan
- Ana C. Silva, MD, Porto, Portugal
- Annemie Snoeckx, MD, Zandhoven, Belgium
- Ashmitha Srinivasan, MD, Syracuse, NY
- Anouk Stein, MD, Phoenix, Ariz
- Subramanian Subramanian, MD, New Delhi, India
- Stanko Yovichevich, MD, Sydney, Australia
- Joe Yut, Olathe, Kan
Resident group responses
- Hospital Italiano Cordoba Radiology Residents, Córdoba, Argentina
- Prince of Songkla University Radiology Residents, Songkla, Thailand
- Santa Casa da Misericórdia do Rio de Janeiro Radiology Residents, Rio de Janeiro, Brazil
- University of Pennsylvania Radiology Residents, Philadelphia, Pa
TOP
HISTORY
IMAGING FINDINGS
