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AJR 2000; 175:1340
© American Roentgen Ray Society


Radiologic-Pathologic Conferences of The
University of Texas M. D. Anderson Cancer Center

Primary Liposarcoma of the Mediastinum

Reginald F. Munden1, Jonathan C. Nesbitt2, Bonnie L. Kemp3,4, Marvin H. Chasen1 and Gary J. Whitman1

1 Division of Diagnostic Imaging, The University of Texas M. D. Anderson Cancer Center, Box 57, 1515 Holcombe Blvd., Houston, TX 77030.
2 Cardiovascular Surgery Associates, 4230 Harding Rd., Nashville, TN 37205.
3 Division of Pathology, The University of Texas M. D. Anderson Cancer Center, 1515 Holcombe Blvd., Houston, TX 77030.
4 Present address: Department of Pathology, Mayo Clinic, 200 First St. S.W., Rochester, MN 55905.

Received February 9, 2000; accepted after revision May 17, 2000.

 
Address correspondence to R. F. Munden.


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A 56-year-old man with a history of melanoma of the left cheek was referred for evaluation of mediastinal widening noted on chest radiography (Fig. 1A). CT showed a large low-density (-70 H) mediastinal mass surrounding and displacing the aorta and trachea (Fig. 1B). At surgery, an encapsulated mass was removed. The final pathologic diagnosis was well-differentiated low-grade liposarcoma (atypical lipomatous tumor) (Fig. 1C).



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Fig. 1A. Primary liposarcoma of mediastinum in 56-year-old man. Posteroanterior chest radiograph shows mediastinal widening with tracheal deviation (arrows) to right.

 


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Fig. 1B. Primary liposarcoma of mediastinum in 56-year-old man. CT scan shows large fatty mediastinal mass impinging on left innominate vein (arrows) and causing deviation of trachea (arrowhead) to right.

 


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Fig. 1C. Primary liposarcoma of mediastinum in 56-year-old man. Photomicrograph shows fibrous bands (arrows) containing atypical cells intermixed with fatty areas. (H and E, x200)

 

Primary mediastinal liposarcomas are extremely rare, and they usually occur in adults, with most cases occurring in patients more than 40 years old. Most patients present with dyspnea, vague chest discomfort, cough, or constitutional symptoms. Patients may present with signs of superior vena caval obstruction, and as many as 15% of patients may be asymptomatic [1].

Four main types of liposarcomas have been described: myxoid, well differentiated, dedifferentiated, and pleomorphic. Evans [2] reported that survival in patients with dedifferentiated or pleomorphic liposarcomas was significantly shorter than in patients with myxoid or well-differentiated liposarcomas. Well-differentiated low-grade liposarcomas, also known as atypical lipomatous tumors, have histologic features in many areas resembling mature adipose tissue. Interspersed areas contain lipocytes that exhibit greater variation in size and shape than the lipocytes observed in lipomas. Scattered cells in these areas and in fibrous bands show atypical cytologic features. The cytoplasm of the atypical cells is usually indistinct or amorphous, and occasional cytoplasmic vacuoles are noted. Rarely, lipoblasts exhibit enlarged hyperchromatic nuclei with peripheral indentations due to cytoplasmic vacuoles [2].

The predominant finding of mediastinal liposarcoma on conventional chest radiography is a widened mediastinum. Deviation of the trachea and vessels may be apparent. On CT and MR imaging, mediastinal liposarcomas appear as inhomogeneous fatty masses that vary in appearance depending on the amount of soft tissue and fibrous bands in the tumor. The more solid components may enhance with contrast material administration, and surrounding structures may be infiltrated or displaced. These morphologic features along with a rapid rate of growth and tracheal or vascular displacement should raise the suspiction for liposarcoma [3].

The recommended treatment for mediastinal liposarcoma is surgical excision [4]. If the entire tumor cannot be resected, surgical debulking often results in symptomatic relief. Radiation therapy and chemotherapy may be added as adjuncts to surgical excision. Approximately 40% of mediastinal liposarcomas recur after surgery [1]. Multiple successive recurrences are common [2]. These local recurrences are likely related to the inability to obtain adequate complete excisions. Evans [2] reported that atypical lipomatous tumors may transform into dedifferentiated liposarcomas. Atypical lipomatous tumors usually do not metastasize [2].


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Introduction
References
 

  1. Plukker JTM, Joosten HJM, Rensing JBM, Van Haelst UJGM. Primary liposarcoma of the mediastinum in a child. J Surg Oncol 1988;37:257 -263[Medline]
  2. Evans HL. Liposarcomas and atypical lipomatous tumors: a study of 66 cases followed for a minimum of 10 years. Surg Pathol 1988;1:41 -54
  3. Munk PL, Lee MJ, Janzen DL, et al. Lipoma and liposarcoma: evaluation using CT and MR imaging. AJR 1997;169:589 -594[Free Full Text]
  4. Grewal RG, Prager K, Austin JHM, Rotterdam H. Long term survival in non-encapsulated primary liposarcoma of the mediastinum. Thorax 1993;48 : 1276-1277[Abstract/Free Full Text]

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B. Coulibaly, C. Bouvier, M. Jose Payan, and P. Thomas
Recurrent dedifferentiated liposarcoma of mediastinum involving lung and pleura
Interactive CardioVascular and Thoracic Surgery, October 1, 2009; 9(4): 741 - 742.
[Abstract] [Full Text] [PDF]


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