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Year : 2006  |  Volume : 16  |  Issue : 4  |  Page : 603-606
Extraskeletal mesenchymal chondrosarcoma[Emc]


Department of Radiology & Imaging, Shri Krishna Hospital, Pramukh Swami Medical College, HM Patel Centre for Medical Care & Education, Gokal Nagar, Karamsad 388325, Anand, Guajarat, India

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Date of Submission20-Mar-2006
Date of Acceptance10-Aug-2006
 

Keywords: Chondrosarcoma, Extraskeletal

How to cite this article:
Chaudhari C R, Prajapati H, Shah H K. Extraskeletal mesenchymal chondrosarcoma[Emc]. Indian J Radiol Imaging 2006;16:603-6

How to cite this URL:
Chaudhari C R, Prajapati H, Shah H K. Extraskeletal mesenchymal chondrosarcoma[Emc]. Indian J Radiol Imaging [serial online] 2006 [cited 2019 Aug 24];16:603-6. Available from: http://www.ijri.org/text.asp?2006/16/4/603/32279

   Introduction Top


Extraskeletal chondrosarcoma is a rare malignant tumor with different biological behaviors. Only 1% of tumors occur in extraskeletal location. Mesenchymal chondrosarcoma is a variant of chondrosarcoma, resembling primitive cartilaginous mesenchyme and arising in an extraskeletal location in half of reported cases.[1]

Here, we report a case of extraskeletal mesenchymal chondrosarcoma[EMC] arising from the right gluteal region.


   Case report Top


An eighteen-year-old male patient came to hospital with chief complain of swelling [Figure - 1] and dull aching pain in the right gluteal region since last twenty days. First of all, USG of the right gluteal region was done. It revealed mixed echogenic mass lesion having calcification with strong acoustic shadowing from it. [Figure - 2] Adjacent to it whorl like mixed echogenic collection seen. [Figure - 3] Afterwards trans-axial radiograph of the right gluteal region was taken and it showed enlarged soft tissue shadow with large area of stipple calcification within it. [Figure - 4] From these findings diagnosis of the soft tissue sarcoma with calcification was kept.

MRI of the right gluteal region was performed. It revealed one heterogeneous hyperintense and hypointense large soft tissue mass lesion (Axial-13 x 7 cm, coronal 15 x 10 cm sized) in right gluteal region in T-2 and T-1 weighted images respectively. [Figure - 5],[Figure - 6] It was situated in between right gluteal maximus muscle and right gluteal medius muscle with evidence of adjacent edema. Underlying bone was normal. On T-1 and STIR image large oval hyperintese component noted within the above described lesion suggestive of presence of blood in methhaemoglobin stage. [Figure - 7] Per-operatively there were mass and haematoma. The mass was resected and sent for histopathological investigation. Grossly, specimen was tan-colored and elastic in consistency. Microscopic examination showed undifferentiated cartilaginous tissue with evidence of calcification. [Figure - 8] From these findings diagnosis of extraskeletal mesenchymal chondrosarcoma was kept. Afterwards screening of the abdomen and chest were done which revealed no evidence of metastases.


   Discussion Top


Mesenchymal chondrosarcoma, first described by Lichtenstein and Bernstein in 1959, is an uncommon, aggressive variant of chondrosarcoma. It has a strong tendency of distant metastasis and extraskeletal lesion is less common.[1]

EMC differs from skeletal and extraskeletal conventional chondrosarcomas in both sex distribution and site of origin. In EMC, there is a slight female predominance[2] whereas men predominate in skeletal and extraskeletal conventional chondrosarcoma[3]. EMC originated most frequently in the brain and meninges and in the soft tissues of the face and of the lower extremity, whereas skeletal and extraskeletal conventional chondrosarcomas are most commonly found in the pelvis and femur. [3] EMC has two peak age incidences in the adult, depending on its location: at 23.5 (range, 5-48) years with central nervous system involvement and at 43.9 (range, 21-62) years with soft-tissue and/or muscular involvement. [4]

Presenting symptoms of extraskeletal mesenchymal chondrosarcoma vary with the sites of in volvement. The common symptoms and signs are localized pain, swelling and neurological symptoms. The duration of symptoms vary. [1]

The imaging findings of calcification, enhancement, neovascularity and lobulation, although not specific for EMC, may reflect the pathologic morphology of EMC with its small, firm, blue-gray nodules of cartilage interspersed within soft, white, fleshy vascular tissue that is lobulated in two-thirds of cases. Fifty to hundred percent of EMCs demonstrate arc, ring, stippled, and highly opaque calcifications at conventional radiography and CT and histopathologic findings of EMC show calcification in cartilaginous and reactive osteoid, particularly in the central part of the tumor. [5] Synovial sarcomas and malignant fibrous histiocytomas show stippled and highly opaque calcifications more frequently than EMC, although chondroid calcifications are unusual in these tumors. The rare extraskeletal osteosarcoma may also show scattered or highly opaque calcifications [6]. A benign process, myositis ossificans, early in its development, may simulate a calcified soft-tissue sarcoma. Initially, myositis ossificans may appear at radiography as a soft tissue mass with irregular calcification corresponding to fibroblastic proliferation and immature bone at histopathologic examination. Later, as the lesion matures, the typical zonal pattern evolves with radiographic and CT findings of well-defined, peripheral calcifications surrounding a central area of low opacity (low attenuation at CT), which correlate with histopathologic findings of peripheral mature lamellar bone and osteoid around central cellular growth [7].

A recent study suggested that MR imaging may help in distinguishing among the various types of cartilaginous tumors. [8] On T2-weighted images, the signal intensity of cartilaginous tumors varies with the cellularity. Cartilaginous tumors that have a more cellular matrix, such as clear-cell chondrosarcoma or chondroblastoma, are isointense or hypointense to muscle tissue on T2-weighted images and have a more amorphous configuration. [8] Like the paucicellular cartilaginous tumors, EMC also appears as a high signal intensity, lobulated mass on T2-weighted images. In contrast to these tumors, however, EMC is composed predominantly of sheets of undifferentiated mesenchymal cells, with the differentiated cartilaginous component usually comprising only a small percentage of the lesion, although rarely it may constitute up to half the tumor area. [9] The high signal intensity in EMC, therefore, probably results from both the vascular undifferentiated mesenchyme and the foci of differentiated cartilage. In contrast, the more cellular, vascular high-grade chondrosarcomas and mesenchymal chondrosarcoma become enhanced throughout the lesions, although often inhomogeneously.[9] The enhancement at CT and MR imaging and the neovascularity at angiography also suggest the vascular nature of EMC with its "hemangiopericytoid" undifferentiated mesenchyme. [9]

In conclusion, EMC is a rare soft tissue tumor that shares imaging findings with other skeletal and soft tissue tumors. Although the imaging findings of lobulated, enhancing masses with foci of low signal intensity at MR imaging and with cartilaginous calcifications on plain radiographs and CT scans are not specific for EMC, they could reflect its unique histopathologic characteristics of vascular undifferentiated mesenchyme surrounding foci of differentiated cartilage. [9]

 
   References Top

1.Chung-Jung Chiang, Yi-Chin Fong, Horng-Chaung Hsu: Case report Extraskeletal Mesenchymal Chondrosarcoma: J Chin Med Assoc 2003; 66:307-310.   Back to cited text no. 1    
2.Harwood AR, Krajbich JI, Fornasier VL. Mesenchymal chondrosarcoma: a report of 17 cases. Clin Orthop 1981; 158:144-148. 826 #{149} Radiology March 1993  Back to cited text no. 2  [PUBMED]  [FULLTEXT]
3.Huvos AG, Rosen G, Dabska M, Marcove RC. Mesenchymal chondrosarcoma: a clinicopathologic analysis of 35 patients with emphasis on treatment. Cancer 1983; 51:1230-1237.  Back to cited text no. 3  [PUBMED]  
4.Louvet C, de Gramont A, Krulik M, et al. Extraskeletal mesenchymal chondrosarcoma: case report and review of the literahire. J Clin Oncol 1985; 3:858-863.  Back to cited text no. 4  [PUBMED]  [FULLTEXT]
5.Salvador AH, Beabout JW, Dahlin DC. Mesenchymal chondrosarcoma: observations on 30 new cases. Cancer 1971; 28:605-615.  Back to cited text no. 5  [PUBMED]  
6.Chung EB, Enzinger FM. Extraskeletal osteosarcoma. Cancer 1987; 60:1132-1142.  Back to cited text no. 6  [PUBMED]  
7.Nuovo MA, Norman A, Chumas J, Ackerman LV. "Myositis ossificans" with atypical clinical, radiographic or pathologic findings: a review of 23 cases. Skeletal Radiol 1992; 21:86-101.  Back to cited text no. 7    
8.Cohen EK, Kressel HY, Frank TS, et al. Hyaline cartilage-origin bone and soft-tissue neoplasms: MR appearance and histologic correlation. Radiology 1988; 167:477-481.  Back to cited text no. 8  [PUBMED]  
9.Lorraine G, Shapeero, Daniel Vanel, Dominique Couanet et al: Extraskeletal mesenchymal chondrosarcoma: Radiology 1993; 186:819-826.  Back to cited text no. 9    

Top
Correspondence Address:
C R Chaudhari
Department of Radiology & Imaging, Shri Krishna Hospital, Pramukh Swami Medical College, HM Patel Centre for Medical Care & Education, Gokal Nagar, Karamsad 388325, Anand, Guajarat
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0971-3026.32279

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    Figures

  [Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5], [Figure - 6], [Figure - 7], [Figure - 8]

This article has been cited by
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[Pubmed]



 

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