RetractionRetracted: Intramedullary Chondrosarcoma ofProximal Humerus

Case Reports in Radiology

Received 1 March 2016; Accepted 1 March 2016

Copyright © 2016 Case Reports in Radiology. This is an open access article distributed under the Creative Commons AttributionLicense, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properlycited.

The article titled “Intramedullary Chondrosarcoma of Proxi-mal Humerus” [1] has been retracted as it was found to con-tain a substantial amount of material, without referencing,from the following published article: “Review: Imaging ofChondrosarcomas,” by L. Ollivier, D. Vanel, and J. Leclere, inCancer Imaging.

References

[1] P. Yadav, D. Thakkar, and S. S. Thind, “Intramedullary chon-drosarcoma of proximal humerus,” Case Reports in Radiology,vol. 2012, Article ID 642062, 7 pages, 2012.

Hindawi Publishing CorporationCase Reports in RadiologyVolume 2016, Article ID 1208982, 1 pagehttp://dx.doi.org/10.1155/2016/1208982

Hindawi Publishing CorporationCase Reports in RadiologyVolume 2012, Article ID 642062, 7 pagesdoi:10.1155/2012/642062

Case Report

Intramedullary Chondrosarcoma of Proximal Humerus

Pratiksha Yadav, Dolly Thakkar, and S. S. Thind

Department of Radiodiagnosis, Dr. D. Y. Patil Medical College, Hospital and Research Centre, Pimpri,Maharashtra, Pune 411006, India

Correspondence should be addressed to Pratiksha Yadav, yadavpratiksha@hotmail.com

Received 13 October 2012; Accepted 30 October 2012

Academic Editors: P. Garcıa Gonzalez and A. Vade

Copyright © 2012 Pratiksha Yadav et al. This is an open access article distributed under the Creative Commons AttributionLicense, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properlycited.

Primary chondrosarcoma is the third most frequent primary malignancy of bone after myeloma and osteosarcoma. It isranging from slow growing nonmetastasising lesions to highly aggressive lesions. We report a case of primary intramedullarychondrosarcoma of proximal humerus. A 60-year-old female presented with pain and hard swelling involving the left arm for 5months. Radiograph showed a lucent expansile intramedullary lesion with matrix calcification and associated soft tissue mass. CTconfirmed the finding. MRI showed a lobulated lesion which is hyperintense on T2WI with low signal fibrous septae. Increasedtracer uptake was seen on bone scan. Histopathology confirmed the radiology diagnosis. The patient underwent wide resectionand endoprosthetic reconstruction of proximal humerus.

1. Case Report

Primary chondrosarcoma is the thirdmost common primarymalignant tumor of bone after myeloma and osteosarcoma.It is most commonly seen between 30 and 70 years of age. Wereport a case of a sixty-year-old female who presented withgradually progressive pain and swelling over the proximalpart of left arm since 5 months associated with restrictedflexion, extension, and abduction of left shoulder. Oninspection, loss of normal contour of left shoulder due to adiffuse swelling and asymmetric pectoral girdle (Figure 1).Skin over the surface was normal, with mild prominenceof the veins. On palpation, it is tender and measuringapproximately 6 cm in length and 4 cm in breadth. It wasbony hard in consistency and fixed in nature with immobileskin over the swelling. Clinical diagnosis was of a neoplasticmusculoskeletal pathology. Radiographs of left shouldershowed an ill defined, expansile, and osteolytic lesioninvolving the cortical and medullary region of neck andproximal shaft of left humerus with a wide zone of transition(Figures 2 and 3). Few specks of calcifications were seenwithin it. It shows endosteal scalloping with cortical breakand adjacent soft tissue component. In addition, a calcifiednodular opacity was seen in the peripheral left lungmid zone.As computed tomography (CT) is useful in defining the bony

anatomy, integrity of the cortex surrounding a lesion, andcalcifications within, a helical CT scan of 5mm thickness wasdone from the superior margin of left shoulder to mid armlevel. It revealed that the osteolytic expansile lesion was seenwith endosteal scalloping and cortical thinning. It containsmatrix calcification, break in cortices at multiple sites withadjacent anterolateral soft tissue component, and specks ofcalcification within it (Figure 4). The deltoid muscle in itsanterior portion was thinned out and displaced.

MRI is known to best depict the tissue character,delineate the extent of bone marrow involvement,and pinpoint the effect of soft tissue masses onsurrounding neurovascular structures. MRI of leftarm was done on 1.5 Tesla machine using T1WI,T2WI, and GRE sequences in coronal, axial, andsagittal planes. There is a well-defined lobulatedlesion which is predominantly hypointense on T1WIand hyperintense on T2WI with low signal septae(Figures 5, 6, 7, and 8). The glenohumeral jointspace was normal and neurovascular bundle wasnot affected. Subsequently bone scan was run tolook for any metastatic lesion elsewhere as 10–20%of chondrosarcomas are known to metastasise. Thelesion in humerus revealed increased uptake of tracer

2 Case Reports in Radiology

(a) (b)

Figure 1: The picture is showing a 60 years old female who came with the c/o pain and swelling in upper arm.

Figure 2: A 60- year-old female came with the c/o pain and swellingin upper arm. Radiograph of left shoulder shows lobulated, andexpansile, osteolytic lesion involving the head, neck and proximalshaft of humerus including medullary and cortical region. Fewspecks of calcification were seen. With involvement of soft tissue.

Figure 3: Axial radiograph of left shoulder confirms the findings ofthe AP radiograph and shows soft tissue swelling with few specks ofcalcification (arrow).

in 1st phase and high soft tissue pooling in 2ndphase. A focal tracer uptake seen in left mandiblewas probably related to dental pathology. Diffuseinhomogeneous increased tracer uptake was seen in

the 3rd phase (Figure 9). The nodular opacity in leftmid zone that was seen on the X-ray was presumedto be a calcified granuloma as it showed no activityon bone scan. FNAC sample showed pleomorphicnuclei with vacuolated cytoplasm in chondromyxoidbackground (Figure 10). Justification of diagnosiswas made by comparing the possible features ofchondrosarcoma with our case. As ENNEKINGSSYSTEM FOR STAGING:

STAGE- II B,

GRADE-HIGH,

SITE-EXTRACOMPARTMENTAL,

METASTASES-NONE.

After a preanaesthetic assessment, the patient was takenup for surgery under general anaesthesia for wide resectionand endoprosthetic reconstruction of proximal humerus.

2. Discussion

Chondrosarcomas account for the third most common pri-mary tumour of the bone, after myeloma and osteosarcoma[1]. This primary sarcoma of bone in adults has a malepredominance and is seen between the 3rd and 7th decadeof life, more common in male, as male : female ratio is1.5 : 1. Usual clinical presentation of chondrosarcoma is pain,tenderness, with or without a mass, and a slow growthover an average duration of 1-2 year. The characteristicfeature of chondrosarcomas is to produce coalescent cartilagelobules of varying sizes with often a necrotic or cystic centre[2]. Chondrsarcoma is graded from 1 (low) to 3 (high).Low grade chondrsarcoma is very close in appearanceto enchondroma and osteochondroma and has occasionalbinucleated cells. High grade chondrsarcoma have increasedcellularity, atypia, and mitoses [3].

Case Reports in Radiology 3

(a) (b)

Figure 4: Axial CT images show osteolytic, expansile lesion causing endosteal scalloping with thinning of cortex. Break in the bony cortexat few places (large arrow) with calcific specks seen in soft tissue (small arrow).

Figure 5: 60-year-old female with swelling in upper arm. MRIwas done on 1.5 T Siemen’s machine. Coronal MRI, STIR T2WIshowing a well-defined lobulated hyperintense lesion with fibrousseptae in the medulla and cortex. Involvement of the soft tissue onmedial and lateral aspect (arrow).

2.1. Skeletal Distribution. The commonest sites are the pelvicbones, femur, humerus, and ribs followed by other sites suchas the trunk, skull, and facial bones. Hands and feet are rarelyinvolved. Peculiar forms are known to develop on laryngealcartilage, base of the skull, or in soft tissue. Chondrosarcomascan occur on preexisting lesions.

Central chondrosarcoma predominates in long bonesand peripheral tumours in the pelvis and vertebrae.

2.2. Imaging. Plain films allow depicting the location ofthe lesion to identify the cartilaginous nature as well as itsaggressiveness. The most frequent type of lesion is centralchondrosarcoma. The tumour begins in the metaphysis andextends to the diaphysis. It is a well-defined lytic lesion,associated with endosteal scalloping, cortical thinning, orthickening. High-grade tumours show irregular margins.

Figure 6: 60-year-old female with swelling in upper arm. MRI wasdone on 1.5 T Siemen’s machine. Coronal T2WI showing hyperin-tense lobulated mass with fibrous septae (arrow).

Figure 7: 60-year-old female with swelling in upper arm. MRIwas done on 1.5 T Siemen’s machine. Coronal T1WI showing largelobulated lesion which is hypointense in signal intensity.

4 Case Reports in Radiology

(a) (b)

Figure 8: Sagittal T2W images showing hyperintense lobulated mass.

Figure 9: Bone scan: anterior and posterior images. Increased traceruptake in the lesion.

Figure 10: Histopathology shows pleomorphic nuclei with vacuo-lated cytoplasm in chondromyxoid background.

Calcifications of the tumoral matrix may be punctate,flocculent, or have a ring-like pattern they can be small, ordisseminated, dense, or subtle. Their absence is frequent inaggressive types. In the soft tissue, themass is frequently huge

and palpable when tumour has an extension. CT scan has adiagnostic role as it shows the bony destruction, the smallcalcifications, and the intra- and extraosseous extent.

In typical forms, MRI shows a lobulated lesion with alow or intermediate signal on T1-weighted images and ahigh-signal intensity on T2 [4]. MRI shows the medullaryinvolvement and the soft-tissue mass precisely. In diffusion,low-grade lesions show a lobulated pattern with enhancedseptations after intravenous injection of contrast media.High-grade tumours do not have septations and show amorediffuse, heterogeneous enhancement. Benign and low-gradetumors cannot be differentiated by the MRI appearance ofthe matrix alone.

2.3. Differential Diagnosis. The main differential diagnosisin chondromas is specially in the differentiation between abenign chondroma and a low-grade central chondrosarcoma[5]. Features suggestive of a malignant lesion are pain,proximal location or a location on the axial skeleton, sizebeing greater than 5 cm, a lobulated aspect, an ill-definedmargin, endosteal erosion, and bone destruction with anextraosseous component [6, 7]. Biopsy is necessary tomake the diagnosis. A metaphyseal lesion could suggest achondromyxoid fibroma, while an epiphyseal lesion couldsuggest a chondroblastoma or a giant cell tumour (Figures 11and 12). Fibrous dysplasia or a bone infarction can be mis-diagnosed as chondrosarcomas; the lack of cortical erosionor of soft-tissue mass would suggest something other thana chondrosarcoma. New immunohistochemistry techniquescontribute to the differentiation of malignant lesions [8, 9].More rarely, a lytic lesion can be considered with a lyticform of osteosarcoma or fibrosarcoma, a plasmocytoma ora metastasis.

2.4. Chondrosarcomas: Variants

2.4.1. Periosteal Chondrosarcomas [10]. This is a rare formrepresenting 1-2% of all cases of chondrosarcomas. The

Case Reports in Radiology 5

Figure 11: Excision of lesion was done with affected soft tissue andbone.

Figure 12: Excised mass lesion.

growth of the tumour begins at the surface of the bone (usu-ally metaphysis of the distal femur or proximal humerus)and develops in the soft tissues as a lobulated mass. Thelesion is usually well differentiated and grows slower thancentral forms. The cortex is never normal, either erodedor often thickened by the tumour, but never destroyed.Ring-like calcifications can be disseminated or localisedwithin the mass. Medullary involvement evaluated on CTor MRI is rare and limited. Uncalcified tumoral nodules arehypodense on CT and show a high signal on T2-weightedMRI. Satellite nodules can be depicted and separated fromthe principal lesion. The outcome is generally favourableafter an appropriate surgical resection.

The differentiation from an osteochondroma is generallyeasy. The diagnosis of periosteal chondroma can be made byhistology alone. Patients are younger and lesions are smaller,not painful, and are located more distally on the skeleton.The periosteal osteosarcoma is more often located on thediaphysis and has reactionary cortical spiculations.

2.4.2. Mesenchymal Chondrosarcoma. This entity represents2-3% of all chondrosarcomas and combines an undifferen-tiated cell component with well-differentiated cartilaginousareas [11]. The diagnosis is only made on this biphasicaspect. The average age of the patients is 26 years. Common

Figure 13: Prosthesis surgery.

skeletal sites are the femur, pelvic bones, ribs, and vertebrae.Extraosseous site involvement such as brain, meninges, orsoft tissues is seen in about one-third of cases. The prognosisis poor, with early pulmonary, bony, and lymph nodesmetastases. The tumours are large, destructive lesions witha purely lytic pattern. Calcified masses can be found. Thelesion appears of low signal intensity on T1-weighted MRIand heterogeneous high signal on T2 images [12]. Multidrugchemotherapy used in osteosarcomas can be combined withsurgery and radiotherapy, but the 10-year survival is only28%.

2.4.3. Clear Cell Chondrosarcoma. In rare forms (2%) ofchondrosarcoma, these lesions are distinguished by theircytology, epiphyseal location in long bones, and slow evo-lution [13]. There is a male predominance and patients arein the third to fifth decade. Clinical symptoms are painand swelling may last up to almost 23 years. Some tumoursmay be an incidental finding; pathological fractures havebeen reported in one-quarter of cases. The commonest sitesare the femur, humerus and tibia. This low-grade tumourshows a geographic lytic epiphyseal lesion with extensionto the metaphysis. The margins can be well defined, butindistinct or sclerotic margins have also been described.Calcifications of the tumoral matrix are not always present.There is no extension to the soft tissues. Periosteal reactionsare unusual. CT may be useful to depict lobulated marginsand calcified matrix. MRI shows a well-delineated low signalon T1-weighted images and heterogeneous high signal on T2images. The main differential diagnosis is the chondroblas-toma, which is a smaller lesion in younger patients. The otherdifferential diagnoses include giant cell tumours and otherepiphyseal tumours.

The treatment is radical surgery. The prognosis is goodwith a 5-year survival of 92% [2], even though metastasesare found in 15% of cases (lung, brain, and bones).

2.4.4. Dedifferentiated Chondrosarcoma. This form repre-sents 10–12% of all chondrosarcomas [2]. It is characterisedby a special histology and very poor prognosis. Pain and

6 Case Reports in Radiology

Figure 14: Postoperative radiograph.

Figure 15: Postsurgery picture.

swelling are the usual clinical symptoms as well as patholog-ical fractures [14]. The commonest locations are the femur,the acetabulum, and the proximal humerus.

These metaphyseal or diaphyseal lesions are rapidlydestructive. Osteolytic lesion is associated with calcificationsresulting in the biomorphic pattern [2]. A huge soft-tissuemass without calcifications, seen on CT or MRI, is alsoindicative for this diagnosis. Imaging helps to direct biopsy ofthe lytic area in order to improve the histological diagnosis.The treatment involves surgery and adjuvant chemotherapyor radiotherapy (Figures 13, 14, and 15). The prognosis isvery poor, with an overall 5-year survival rate of only 8–13%.The metastases appear in the lungs but also in unusual sitessuch as the adrenal gland, brain, and liver.

2.4.5. Secondary Chondrosarcoma. Twelve percent of allchondrosarcomas are developed in a preexisting lesion.It may be secondary to a solitary osteochondroma, osteo-chondromatosis, enchondromatosis (Ollier’s disease),

fibrous dysplasia, Paget’s disease, irradiated bone, or synovialchondromatosis [2]. In osteochondromatosis, the risk ofsarcomatous transformation is 5–25%; it is 25–50% inenchondromatosis and nearly 100% in Maffucci’s syndrome[14]. The increased size of an enchondroma, lytic areawith cortical destruction, associated with pain or fractureare features suggestive of malignant transformation. Anenlarging exostosis associated with pain, the appearance ofa less mineralized zone in the cartilage cap, calcifications inthe soft tissues, and the thickening of the cap (>1 cm) on CTand MRI suggest sarcomatous transformation [15].

Authors’ Contribution

P. Yadav has analysed the case by radiograph, CT, and MRIwith the assistance and useful support of D. Thakkar andhas also advised bone scan and biopsy which has confirmedthe diagnosis. S. S. Thind, the Head of the Department ofRadiodiagnosis, has given his clear concepts and critical waysof looking at different aspects of the case, encouraging therest of the authors to proceed with the case workup.

Acknowledgments

The authors are grateful to the Department of Orthopedicsand Pathology at Dr. D. Y. Patil Medical College, Hospitaland Research Centre, Pimpri, Pune, Maharashtra, India, fortheir extended support, without whom this entire case workup would have been impossible.

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[14] P. Anract, B. Tomeno, and M. Forest, “Chondrosarcomesdedifferencies. Etude de treize cas cliniques et revue dela litterature,” Revue de Chirurgie Orthopedique et Trauma-tologique, vol. 80, pp. 669–680, 1994.

[15] T. M. Hudson, D. S. Springfield, and S. S. Spanier, “Benignexostoses and exostotic chondrosarcomas: evaluation of carti-lage thickness by CT,” Radiology, vol. 152, no. 3, pp. 595–599,1984.