Case Study

Unusual case of chondroblastic osteosarcoma of the rib in an adult

Asian Cardiovascular & Thoracic Annals 2014, Vol. 22(6) 745–747 ß The Author(s) 2013 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav DOI: 10.1177/0218492313491755 aan.sagepub.com

Prakash Manoharlal Ludhani1, Radha Anathakrishnan2, Padmanabhan Chandrasekar1 and Srinivasan Muralidharan1

Abstract Primary osteosarcoma arising from the ribs is very rare. An unusual case of a primary chondroblastic osteosarcoma of the rib in a 30-year-old woman is described here. The patient underwent wide excision of the tumor along with the involved rib, followed by chemotherapy. Although clinically unsuspected in this unusual site, pathology confirmed a completely resected chondroblastic osteosarcoma.

Keywords Bone Neoplasms, Diagnosis, differential, Osteosarcoma, Ribs

Introduction Osteosarcoma (OS) is the most common primary malignant bone tumor. It principally arises in the metaphysis of long bones, particularly at the lower end of the femur, upper end of the tibia, and humerus.1 Spine, flat bones such as the ribs, scapula, pelvis and craniofacial bones are less frequently involved. Primary chondroblastic OS of the rib, especially occurring in an adult, is extremely rare. We describe one such case that posed a diagnostic dilemma, owing to its unusual location.

Case report A 30-year-old woman presented with a 3-month history of a painful and slow-growing mass over the chest wall on the right side. There was no fever, cough, breathlessness, or loss of weight. There was no history of prior radiation or a preexisting bone lesion. Physical examination revealed a bony hard mass in the right hemithorax, measuring 5  5 cm. Chest radiography revealed an opacity in the right chest wall, involving the 4th rib. Computed tomography (Figure 1) showed a lobulated, extrapleural mass involving the right 4th rib, with no lung metastasis. Fine-needle aspiration cytology indicated a diagnosis of chondrosarcoma. Via a right anterolateral thoracotomy, a 10  8-cm tumor (Figure 2) and 4th rib resection was performed.

The tumor was found to be hard, white, and bosselated, involving the rib. Microscopic examination (Figure 3) showed chondroblastic osteosarcoma composed of pleomorphic spindle-shaped cells with increased mitotic activity and focal tumor osteoid formation. Several foci of high-grade cartilage were also noted.2

Discussion Primary osteosarcomas usually originate in the metaphysis of long bones. Approximately 10% of OS are located in the flat bones, with the pelvis being the main site, and a mere 1%–2% occur in the thoracic bones inclusive of the ribs, sternum, and clavicle.1,3 OS are known to affect long bones, such as the distal femur and proximal tibia, because these are the sites of greatest bone growth, where bone cell mitotic activity is at its peak. OS arising from the ribs are infrequent and 1 Department of Cardiothoracic Surgery, G Kuppuswamy Naidu Memorial Hospital, Coimbatore, India 2 Department of Pathology, G. Kuppuswamy Naidu Memorial Hospital, Coimbatore, India

Corresponding author: Srinivasan Muralidharan, Department of Cardiothoracic Surgery, G. Kuppuswamy Naidu Memorial Hospital, Coimbatore 641037, Tamil Nadu, India. Email: [email protected]

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Figure 1. Computed tomography 3-dimensional volumerendered image showing the lytic lesion in the 4th rib.

Figure 2. The excised tumor along with rib.

Figure 3. Microscopic image of chondroblastic osteosarcoma. Hematoxylin and eosin stain, original magnification4.

have been reported mainly in the pediatric population.4–6 In a study of 228 patients admitted to St. Jude’s Cancer Hospital for the treatment of nonEwing’s sarcoma, only 2 had primary OS of the ribs.7 However, a few cases of OS arising as metastasis or a secondary malignancy in the ribs, have been reported.7 OS originating from such a rare site poses a diagnostic challenge to the radiologist, pathologist, and surgeons.

The typical sunburst radiological pattern observed in OS of the long bones may not be evident in OS of the flat bones.7 Akyu¨z and colleagues8 reported one case of OS of the rib among 129 cases of OS. There was no case of OS in a series of 49 chest wall tumors reported by Sabanathan and colleagues.9 Owing to the rare site and varying radiological images, these tumors can be confused with other bone lesions, and the differentials include chondrosarcoma, fibrosarcoma, or a metastatic tumor. Although computed tomography and magnetic resonance imaging can evaluate the exact location and extent of involvement of the bone and adjacent structures, they may not be useful in defining the exact nature of the tumor. Some authors believe that OS should be suspected if computed tomography reveals dense calcification within a mass that is centered in a rib.10 Histopathological diagnosis is imperative for instituting definite therapy. Although OS of flat bones is rarely associated with metastasis, it is prudent to include a computed tomography scan of the thorax, chest radiographs, and bone scans as part of the management protocol, to look for metastasis. Postoperative multiagent chemotherapy has improved the survival of these patients. The use of adjuvant chemotherapy reduces the risk of both local and distant relapse. Doxorubicin and cisplatin are the agents of choice.11 The overall prognosis of OS in flat bones remains poor because of the difficulty of complete excision in locations such as the skull and the pelvis. The guidelines for management and the prognosis and survival rates in primary OS of rib are not clear due to the small number of cases studied. However, there is documentation of better survival in patients who had complete resection of the tumor at the time of surgery. Our patient received 6 cycles of chemotherapy postoperatively and is doing well with no recurrence 8 months after surgery. Primary OS of the rib should be considered as a possible diagnosis in a patient presenting with a chest wall tumor. Wide local excision and careful histological examination are mandatory. With the use of adjuvant chemotherapy, the outcome in such cases is encouraging. Funding This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.

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Unusual case of chondroblastic osteosarcoma of the rib in an adult.

Primary osteosarcoma arising from the ribs is very rare. An unusual case of a primary chondroblastic osteosarcoma of the rib in a 30-year-old woman is...
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