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Year : 2011  |  Volume : 54  |  Issue : 1  |  Page : 144-146
Extraskeletal mesenchymal chondrosarcoma of the pleura: Report of a rare case

1 Department of Pathology, All India Institute of Medical Sciences (AIIMS), New Delhi, India
2 Department of Medicine, All India Institute of Medical Sciences (AIIMS), New Delhi, India

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Date of Web Publication7-Mar-2011


Mesenchymal chondrosarcoma is a rare aggressive variant of chondrosarcoma that frequently occurs in extraskeletal location. A 28-year-old female presented with a history of dyspnea and fever and succumbed to her illness before a conclusive diagnosis was established. An autopsy performed revealed the presence of an extraskeletal mesenchymal chondrosarcoma (ESMC) involving the pleura. Only one case of ESMC of the pleura has been reported previously. Herein, we report the second case of ESMC of the pleura.

Keywords: Chondrosarcoma, mesenchymal chondrosarcoma, pleura

How to cite this article:
Jain A, Safaya R, Jagan C, Sharma S K. Extraskeletal mesenchymal chondrosarcoma of the pleura: Report of a rare case. Indian J Pathol Microbiol 2011;54:144-6

How to cite this URL:
Jain A, Safaya R, Jagan C, Sharma S K. Extraskeletal mesenchymal chondrosarcoma of the pleura: Report of a rare case. Indian J Pathol Microbiol [serial online] 2011 [cited 2022 May 29];54:144-6. Available from: https://www.ijpmonline.org/text.asp?2011/54/1/144/77374

   Introduction Top

Mesenchymal chondrosarcoma (MC) is a specific variant comprising about 1% of all chondrosarcomas, and is characterized microscopically by a dimorphic pattern. About a third of MCs are extraskeletal. [1] Most common age of presentation is second to third decade, and a slight female preponderance has been noted. The commonly described sites for the extraskeletal origin are lower extremities, orbit, central nervous system, etc. [2] Rarely, it has also been described in lungs, rib and kidney. [3],[4],[5] Till date, there is only one previous report of MC involving the pleura. [2] We report here the second case of pleural extraskeletal mesenchymal chondrosarcoma (ESMC) in a 28-year-old female.

   Case Report Top

A 28-year-old unmarried female presented to the emergency room (ER) with complaints of fever and shortness of breath. She was apparently well 8 months prior to presentation when she developed low grade fever without chills and rigors. Gradually, she noted progressive shortness of breath and developed a dry cough. On investigation in another hospital 6 months back, she was thought to have a right-sided pleural effusion. A provisional diagnosis of tuberculosis was made and the patient was started on anti-tubercular treatment; however, her condition worsened 2 weeks prior to presentation.

At the age of 18 years, the patient had hirsuitism, and on investigation, was found to have a right-sided ovarian mass measuring 30 × 18 cm for which she was operated. Histological examination had revealed a diagnosis of a poorly differentiated Sertoli-Leydig cell tumor with areas of necrosis and mitosis. No metastatic tumor deposits were found.

On examination, at the present admission, she was dyspneic, febrile and cyanosed with a respiratory rate of 35/minute. Her trachea was deviated to the left and chest movements were reduced on the right side. There was a dull note on percussion, decreased vocal resonance and decreased air entry over the right side. Extensive polyphonic rhonchi and coarse inspiratory rales were heard in all areas of right side of chest. Examination of other systems did not reveal any abnormality.

On investigation, chest X-ray showed opaque right hemithorax with mediastinal shift to the left and was interpreted as a right-sided pleural effusion. An ultrasound examination demonstrated thickening of right side pleura and multi-loculated pleural effusion. A computerized tomography (CT) examination of the chest showed features indicative of a right-sided pleural effusion with soft tissue densities and dense amorphous calcification [Figure 1]. Multiple attempts at aspiration of pleural fluid yielded only scant hemorrhagic fluid which was negative for malignant cells on cytology. A pleural biopsy attempted showed fibrocollagenous tissue only.
Figure 1: (a-c) Contrast-enhanced computed tomography (CECT) thorax shows a large low attenuation mass lesion with enhancing soft tissue strands within the mass and scattered areas of punctuate to coarse calcification, occupying almost the entire right thoracic cavity, leading to passive collapse of right lung and contralateral mediastinal displacement. The thoracic cage is not involved

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The patient was treated with oxygen, bronchodilators, steroids and antibiotics. However, she continued to deteriorate, remained bed bound, required oxygen and repeated nebulizations. She required mechanical ventilation on the 5th day and succumbed to her illness on the 7th day of admission. A thorax-limited autopsy was requested.

Autopsy Findings

At autopsy, the trachea was found to be shifted to the left, and the right pleural cavity showed 100 ml of amber colored clear fluid. The left lung weighed 390 g, while the right lung weighed 150 g and was compressed by a large, well-defined, solid mass measuring 20.5 × 16.5 × 7.5 cm arising from the pleura. The mass was bosselated, encapsulated, with a fleshy cut surface and was easily separable from the lung and chest wall [Figure 2]a. On microscopic examination, sections from the right lung revealed areas of compression and collapse, while sections form the left lung showed features of aspiration pneumonia. Sections from the mass showed a tumor with a dimorphic pattern [Figure 2]b-d. There were large islands of immature cartilage surrounded by sheets of small round blue cells, focally arranged in a hemangiopericytomatous pattern, having scant cytoplasm and hyperchromatic nuclei. On immunohistochemistry, the cartilaginous areas were immunopositive for S-100 and the round cell component for Mic-2 (CD99) [Figure 3]a, b. Other positive immunostains included vimentin and p53, while cytokeratin, epithelial membrane antigen (EMA), calretinin, carcinoembryonic antigen, and desmin were negative. MIB-1 labeling index was approximately 20%. Sections from mediastinal lymph nodes and bone marrow did not reveal any metastatic deposits. Based on these features, a final anatomical diagnosis of MC arising from the pleura was made.
Figure 2: (a) Gross photograph of en bloc excised autopsy specimen shows a large, well-circumscribed, encapsulated, bosselated tumor on the right side. Right lung and other mediastinal structures appear compressed by the tumor. (b) Low power view of the tumor showing islands of cartilage, surrounded by sheets of undifferentiated mesenchymal cells (H and E; ×40). (c) Undifferentiated mesenchymal cells arranged in hemangiopericytomatous pattern (H and E; ×100). Inset: mesenchymal cells showing scant cytoplasm and hyperchromatic nuclei (H and E; ×400). (d) Island of cartilage surrounded by small round cells (H and E; ×200)

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Figure 3: (a) Immunohistochemically, the cartilaginous areas display immunopositivity for S-100 protein. The mesenchymal cells appear negative (S-100; ×200). (b) The mesenchymal cells show immunopositivity for Mic-2 (CD99) which is absent in the cartilaginous areas (CD99; ×200)

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   Discussion Top

MC is a rare, highly aggressive variant of chondrosarcoma and has a poor prognosis. [6] Compared to conventional chondrosarcomas, which are very rarely extraskeletal and occur later in life, MCs frequently have an extraskeletal location and an earlier age at presentation. [7] The most common extraskeletal sites are the brain and meninges and soft tissues of the lower extremity. [6]

ESMCs arising in the lung and chest wall have been reported previously. [3],[4],[7],[8] Till date, there is only one case report of ESMC involving the pleura in an elderly male, diagnosed by imaging techniques. [2] The present case was radiologically a diagnostic challenge. All imaging modalities failed to alert the clinician to the presence of a malignancy. Due to inadequacy of the initial pleural biopsy and multiple pleural taps, and inability to obtain a representative adequate biopsy due to the poor condition of the patient, the diagnosis remained elusive ante-mortem to the pathologist also. The past history of an ovarian mass also led to a delay in the conclusive decision making. In this case, autopsy showed that the tumor was present in the pleural cavity, easily separable from the lungs and chest wall, conclusively establishing its origin in the pleura. To the best of our knowledge, this is only the second such case of ESMC arising from the pleura.

Retrospective analysis of radiological images revealed a large predominantly hypodense mass filling the right thoracic cavity, leading to passive collapse of the lung parenchyma. Multiple enhancing soft tissue strands and punctate to coarse calcified foci were noted scattered irregularly within the mass. No extrathoracic extension of the mass was seen. The overlying thoracic cage including ribs and sternum were unremarkable, suggesting intrathoracic and extraskeletal origin of mass, which was confirmed at autopsy. The visualized lung window was normal.

Histologically, ESMC characteristically display a bimorphic pattern, comprising islands of mature cartilage and cellular undifferentiated mesenchymal cells. The main differential diagnoses of ESMC include synovial sarcoma, Ewings sarcoma, hemangiopericytoma or a dedifferentiated chondrosarcoma, particularly in biopsy specimens. [6] The identification of cartilage in the biopsy helps in differentiating from the first three and an intermixing of the cartilaginous elements with the undifferentiated small round cell component helps in differentiating from a dedifferentiated chondrosarcoma in which both the components are sharply delineated. Immunohistochemistry too plays a role, particularly in small biopsies. Immunopositivity for vimentin, S-100 and Mic-2 (CD99) and negativity for cytokeratin, EMA and CD34 is seen in ESMC. Synovial sarcomas usually display focal positivity for cytokeratin and EMA, and hemangiopericytomas display immunopositivity for CD34 and EMA.

In the present case, classical clinical presentation of the tumor in a young female, presence of characteristic bimorphic histological appearance and immunohistochemical findings helped in confirming a diagnosis of ESMC. In ESMC involving the pleura, another differential diagnosis that can be considered is a sarcomatoid mesothelioma with cartilaginous differentiation. However, in our case, immunohistochemical negativity for calretinin and cytokeratin helped in excluding that diagnosis.

Our patient had a past history of being operated for a poorly differentiated Sertoli-Leydig cell tumor of the ovary 10 years back. On careful review of the slides from previous surgery, the original diagnosis was confirmed. No cartilaginous areas were identified in that tumor.

Not much is known about the pathogenesis and molecular events resulting in the development of this tumor. Various etiological factors have been suggested for sarcomas arising in the pleura, for example, secondary sarcomas in patients having received radiotherapy for another primary malignancy, most commonly resulting in malignant fibrous histiocytomas, osteosarcomas or angiosaromas. [9] However, in the present case, the patient had not received any postoperative radiotherapy or chemotherapy after the initial surgery, indicating a very rare unfortunate occurrence of a second primary malignancy in the same patient, at a young age, within a span of 10 years. To the best of our knowledge, there is no previously published report of the concurrent occurrence of an ovarian malignancy with ESMC. Other possibilities suggested include asbestos exposure or prior episodes of empyema and pleuritis. [2] In this case, no such etiological associations could be identified. Only one study by Park et al. has explored the genetic origins of MCs. They demonstrated that a substantial fraction of MCs display overexpression of p53 protein though they found genetic alteration of p53 gene to be a relatively rare event, indicating there may be unidentified epigenetic mechanisms involved in the genesis of such tumors. [10] In our case also, the tumor cells showed strong nuclear immunopositivity for p53, signifying its overexpression.

In conclusion, this rare case highlights the importance of an autopsy in establishing diagnosis in challenging cases, describes the rare occurrence of a second primary malignancy in a young patient without any known etiological factors and is only the second case of ESMC of the pleura in literature.

   References Top

1.Taori K, Patil P, Attarde V, Chandanshive S, Rangankar V, Rewatkar N. Primary retroperitoneal extraskeletal mesenchymal chondrosarcoma: A computed tomography diagnosis. Br J Radiol 2007;80:e268-70.  Back to cited text no. 1
2.Luppi G, Cesinaro AM, Zoboli A, Morandi U, Piccinini L. Mesenchymal chondrosarcoma of the pleura. Eur Respir J 1996;9:840-3.  Back to cited text no. 2
3.Huang HY, Hsieh MJ, Chen WJ, Ko SF, Yang BY, Huang SC. Primary mesenchymal chondrosarcoma of the lung. Ann Thorac Surg 2002;73:1960-2.  Back to cited text no. 3
4.Aoki T, Watanabe M, Takagi K, Tanaka S, Aida S. Mesenchymal chondrosarcoma of the rib: Report of a case. Surg Today 1996;26:1020-3.  Back to cited text no. 4
5.Malhotra CM, Doolittle CH, Rodil JV, Vezeridis MP. Mesenchymal chondrosarcoma of the kidney. Cancer 1984;54:2495-9.  Back to cited text no. 5
6.Shapeero LG, Vanel D, Couanet D, Contesso G, Ackerman LV. Extraskeletal mesenchymal chondrosarcoma. Radiology 1993;186:819-26.  Back to cited text no. 6
7.Nakashima Y, Unni KK, Shives TC, Swee RG, Dahlin DC. Mesenchymal chondrosarcoma of bone and soft tissue. A review of 111 cases. Cancer 1986;57:2444-53.  Back to cited text no. 7
8.Rocca M, Vanel D, Couanet D, Caillaud JM, Brugière L. Pulmonary mesenchymatous chondrosarcoma in children. Report of 2 cases and review of the literature. J Radiol 1988;69:329-32.  Back to cited text no. 8
9.Lagrange JL, Ramaioli A, Chateau MC, Marchal C, Resbeut M, Richaud, et al. Sarcoma after radiation therapy: Retrospective multiinstitutional study of 80 histologically confirmed cases. Radiation Therapist and Pathologist Groups of the Fédération Nationale des Centres de Lutte Contre le Cancer. Radiology 2000;216:197-205.   Back to cited text no. 9
10.Park YK, Park HR, Chi SG, Kim CJ, Sohn KR, Koh JS, et al. Overexpression of p53 and rare genetic mutation in mesenchymal chondrosarcoma. Oncol Rep. 2000;7:1041-7  Back to cited text no. 10

Correspondence Address:
Rajni Safaya
Department of Pathology, All India Institute of Medical Sciences, New Delhi - 110 029
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0377-4929.77374

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