a 17-year - old girl presented with complaints of a progressively increasing swelling over the front of her left chest over the previous 5 months .
the swelling was not painful , situated just adjacent to her left breast , with no associated shortness of breath .
she did not complain of an engorgement of the left breast , nipple discharge , or retraction .
there was no family history of similar complaints . on examination , her vital signs were stable .
there was a large , hard , non - tender mass , 2530 cm in size , extending from just below the left clavicle to the 6th intercostal space and from the lateral border of the sternum to the mid - axillary line ( fig .
the mass itself was adherent to the chest wall although the overlying skin was not .
the left breast had been lifted up by the mass and was not adherent to it .
chest x - ray showed a diffuse opacification over the mid - lung field extending over to the lateral chest wall .
a blood investigation revealed a raised lactate dehydrogenase and alkaline phosphatase , positive c - reactive protein , and normal total counts .
contrast - enhanced computed tomography ( ct ) of the thorax showed evidence of irregular destruction and periosteal reaction involving the left 3rd rib ( fig .
2 ) . there was a large soft tissue component extending through the skin and the subcutaneous region , anterior mediastinum , and in the left upper lobe .
this was causing compression of the left upper and lower lobe bronchus with a partial collapse of the underlying lung field .
the lesion was also abutting the arch of the aorta and its branches and was found to be displacing the left brachiocephalic vein laterally . magnetic resonance imaging ( mri )
findings were similar with the lesion appearing hyper - intense in the t2-weighted images and hypointense in the t1-weighted images .
fine - needle aspiration cytology from the mass was inconclusive , and a core - needle biopsy was taken , which was suggestive of chondrosarcoma . the patient was subsequently taken up for the exploration and excision of the mass .
the chest was opened via a median sternotomy and the tumor freed from its anterior mediastinal attachment ( fig .
the superior , inferior , and lateral extents of the tumor were accessed via a left thoracotomy , dissecting under the sub - mammary plane , and the required resection of the 2nd to the 5th ribs .
extensive involvement of the left lung parenchyma warranted a left pneumonectomy and a completion thoracoplasty to obliterate the potential space ( fig .
the biopsy report revealed features of a dedifferentiated chondrosarcoma with a highly pleomorphic sarcomatous component .
large foci of necrosis were present , and the tumor was found to infiltrate the lung parenchyma .
the patient had an uneventful postoperative course and was discharged on the 6th postoperative day .
a wide variety of benign and malignant conditions are classified under tumors of the chest wall .
the most common entities are rib metastases via a hematogenous spread and direct chest wall invasion from contiguous lung and breast carcinoma .
primary chest wall tumors , which are quite rare , may arise from any of the soft - tissue , bony , or cartilaginous constituents of the chest wall , the latter comprising only one - third of the total number of cases .
chondrosarcoma is the most common malignant primary tumor of both the bony thorax and , in fact , the entire chest wall .
secondary chondrosarcomas occur in pre - existing benign cartilaginous neoplasms , such as the complication of a pre - existing enchondroma or osteochondroma .
chondrosarcoma most commonly arises de novo within the medullary cavity of the bone ( primary or central ) but can result from a malignant transformation of the cartilage cap of a pre - existing benign cartilaginous tumor such as enchondroma or osteochondroma . a majority of the patients with thoracic chondrosarcoma present with an enlarging , painful , anterior chest wall mass arising from either the vicinity of the costochondral junction or the sternum .
asymptomatic tumors detected incidentally by thoracic imaging are more likely to be benign . in a retrospective series of patients seen at the mayo clinic over 73 years , only 6.5% of the 96 patients were without physical findings or symptoms .
a history of trauma associated with the tumor location has been implicated although its direct causation has not been established . a basic posteroanterior and lateral chest radiograph consists of the initial imaging work - up of these patients .
a classic radiographic appearance of chondrosarcoma is a lobulated , mixed lytic and sclerotic lesion .
ct is optimal for the detection and characterization of the chondroid matrix , which classically has well - formed rings and arcs of mineralization .
mri is particularly useful for defining the vascular or neural involvement and , therefore , provides complementary information to the ct scan . positron emission tomography with fluorine-18 fluorodeoxyglucose is performed to rule out extrapulmonary metastases .
percutaneous image - guided core - needle biopsy and sophisticated cytopathology techniques should yield a diagnosis in a majority of the cases .
cellularity , nuclear atypia ( including multinucleated cells ) , and increased proliferation ( > 10% ) are all indicators of an increasing grade and aggressive behavior .
the less - differentiated component of dedifferentiated chondrosarcoma usually shows the multi - lineage histological features of malignant fibrous histiocytoma and fibrosarcoma .
there is no indication of neoadjuvant radiation or chemotherapy in a suitable surgical candidate with a resectable thoracic chondrosarcoma .
a wide resection of all thoracic diseases with appropriate margins , reconstruction of the bony chest wall to ensure preservation of respiratory mechanics , and soft - tissue coverage of reconstructive prostheses with healthy , vascularized tissue is the treatment of choice .
the chondrosarcoma of the sternum should also be resected with at least a 4-cm margin .
the ipsilateral lung should be carefully palpated for occult metastases , which should be resected prior to the commencement of reconstruction . in this patient , due to the extensive involvement of the chest wall and the lung parenchyma , pneumonectomy with thoracoplasty
thoracoplasty is the surgical removal of the skeletal support of a portion of the chest .
this is accomplished by the subperiosteal removal of a varying number of rib segments to approximate the chest wall to the underlying lung or mediastinum to effect lung collapse or pleural space obliteration . in spite of mutilating surgery ,
thoracoplasty is justified in developing nations where issues such as poor patient compliance , post pneumonectomy empyema , drug resistance , poor healthcare delivery systems , and a certain amount of mismanagement at the primary and secondary levels of healthcare need to be addressed .
chondrosarcoma is relatively radioresistant , and conventional radiation therapy has demonstrated some efficacy and should be administered at the sites of positive pathologic margins ( if a wider surgical margin can not be achieved ) or in cases of unresectability . although considered a rare entity , chondrosarcoma of the chest wall is the most common malignant primary neoplasm .
its occurrence in a young female and with such a dimension as that described above is extremely rare .
a careful work - up as well as histological correlation is vital for the adequate management of these patients , who require a wide resection of the tumor supplemented with chest wall reconstruction , if possible .
overall survival is good if the tumor has been adequately resected , respiratory mechanics preserved , and the margins are found to be free of involvement during a histopathological examination .
 chondrosarcoma of the chest wall is a rare primary neoplasm found to occur in elderly men . 
 patients present with an enlarging , painful , anterior chest wall mass arising from either the vicinity of the costochondral junction or the sternum . 
 treatment includes wide resection with appropriate chest wall reconstruction . 
 we report an unusual presentation of this uncommon tumor occurring as a huge chest wall mass in a young teenage girl . 
CASE REPORT
DISCUSSION