Case Report

Giant Chondrosarcoma of the Clavicle - A Case Report and Literature Review

Aftab Younus¹ and Adrian Kelly²*

¹FC Orthopedics, Orthopedic Surgeon, Helen Joseph Hospital, University of the Witwatersrand, Johannesburg, South Africa

² FC Neurosurgery, Mukhari Academic Hospital, Sefako Makgatho health sciences University, Pretoria, South Africa

Submission:June 01, 2020; Published: June 17, 2020

*Corresponding author: Dr Adrian Kelly, FC Neurosurgery (SA), Dr George Mukhari Academic Hospital, Sefako Makgatho health sciences University, Pretoria, South Africa

How to cite this article: Aftab Y, Adrian K. Giant Chondrosarcoma of the Clavicle - A Case Report and Literature Review. Ortho & Rheum Open Access J. 2020; 16(3): 555938. DOI: 10.19080/OROAJ.2020.16.555938

Abstract

Clavicular mass lesions are frequently malignant, with myeloma, Ewing’s sarcoma, and osteosarcoma comprising most cases. An infrequent diagnosis, more commonly seen in the pelvis and proximal femur, is a clavicular chondrosarcoma. These lesions are frequently chemo and radio-resistant, and for this reason complete surgical excision with clear margins offers the best chance of cure. The clavicles subcutaneous location through-out its course is favourable in this regard, and a complete claviculectomy is regarded as the surgical procedure of choice. Several papers note the minimal disability incurred by the complete removal of this unique, horizontally orientated, long bone. We report a 57-year old male patient who presented to our unit with a right-sided painless clavicular mass, of 1-years duration, that was progressively increasing in size. On examination a mass of alarming size was demonstrated, yet subsequent radiological investigations demonstrated only local, and fortunately resectable, spread. He was taken to surgery where we proceeded to successfully perform a total claviculectomy, utilizing a specific stepwise surgical technique. We recommend that the surgical technique, which we describe, is that which should be followed when a total claviculectomy is performed.

Introduction

While clavicular tumors are rare, an enlarging clavicular mass should be regarded as a primary malignancy until proven otherwise [1]. Regarding the epidemiological nuances that characterize chondrosarcomas they have a predilection for males over the age of 40-years, and occur most frequently in the pelvis, femur, and humerus [2]. Although described, their occurrence in the scapula, and even less so the clavicle, is a rarity [3]. More frequently occurring tumors of the clavicle, described in one series of 12 patients, are Ewings sarcoma and multiple myeloma [4]. Another paper, that considered 23 dedifferentiated chondrosarcomas, confirms this in that only 1 tumor in this series, was clavicular [5]. Regarding the clavicle itself, it is noted to have several osseous peculiarities. Firstly it is the only long bone in the body that is orientated horizontally, secondly it is the only long bone that lacks a consistent medullary cavity, thirdly it is the first bone in the foetus to ossify, and fourthly, which in the authors opinion is the most relevant defining feature, is the fact that it can be resected in its entirety without causing any significant disability [6]. This final point is taken advantage of in clavicular chondrosarcomas where, due to them exhibiting a poor response to both chemotherapy and radiotherapy, a total claviculectomy is the most common definitive surgical procedure performed [3,7].

Case Description

We report a 57-year old, right hand dominant, manual labourer, who presented to our unit with a progressively enlarging, painless, right clavicular mass, of 1-year duration. The medical history provided was that he was a known hypertensive patient on treatment, and the social history provided was that he was a smoker with a 25-year pack history. On systemic enquiry he confirmed associated loss of weight for the preceding year. On general examination he appeared healthy, had no stigmata of immunosuppression, and no lymphadenopathy. Examination of the right clavicle revealed a large 15cm by 10cm firm mass with its long axis orientated along the long axis of the bone. The mass was firm, non-tender, fixed to the underlying tissue, had a discreet edge, and there were no overlying skin changes (Figure 1). Neurological examination of his right upper limb was normal. Vascular examination revealed normal distal pulses and no signs of venous engorgement (Figure 1). We proceeded to do a chest Xray, which although excluding any obvious lung metastases, confirmed the right clavicle, which could not be visualized, had been completely replaced by tumour (Figure 2).

A CT chest was performed which confirmed the 15cm by 10cm soft tissue mass replacing the right clavicle. Unfortunately, right retro-pectoral lymphadenopathy, was also noted (Figure 3). On the same investigation the lung fields were confirmed clear of metastatic disease. A subsequent abdominopelvic CT scan was performed and excluded gross involvement.

An MRI of his right shoulder was subsequently performed which confirmed the hyperintense lesion to be easily discernible from the surrounding tissues. The well-circumscribed nature of the mass again eluded to a probable plane of excision that would be found intra-operatively. Due to the absence of any vascular involvement clinically, a CT angiogram was not performed. In hind-sight, due to the obvious proximity of the lesion to major vascular structures, we acknowledge that these should have been performed and recommend they be done by orthopaedic surgeons who come to face with a tumor, of this size, in this location (Figure 4).

A fine needle biopsy of the lesion confirmed the tumour to be a chondrosarcoma, and the decision taken was that the best way forward would be to perform a total claviculectomy, with subsequent referral to oncology for adjuvant therapy

Operative Procedure

The patient was taken to the operating room and post induction of anaesthesia, was placed supine with the operating table, which was adjusted to the beach chair position. His head was rotated to the left side away from the right clavicle. The acromioclavicular and sternoclavicular ends of the clavicular remnants were marked on the skin, with a cutaneous marker, and joined with a planned incision line that passed over the maximal convexity of the tumor (Figure 5).

The skin incision was made, and the skin was sharply dissected superiorly and inferiorly off from the capsule of the tumor, to which it was unattached. The dissection was continued over, and then posterior to, the superior pole of the tumor. The same procedure was performed inferiorly over, and then under, the inferior pole of the tumor. The remaining attachment directly beneath the tumor, from the lateral to the medial end of the clavicle along its length, was left intact at this point for fear of involvement of the subclavian vein (Figure 6).

Once the superior and inferior poles of the tumour had been undermined, the acromioclavicular joint was identified and disarticulated. This allowed us to use this lateral bony remnant of the clavicle to elevate the tumour to expose its remaining inferior aspect. Using a combination of diathermy and blunt dissection the bed of the tumour was freed from lateral to medial working strictly in the sub-periosteal plane on the posterior clavicular border (Figure 7).

Once reaching the medial border the sternoclavicular joint was disarticulated and the claviculectomy was completed. The area of the operative bed was noted to pulse, due to the very close proximity of the subclavian artery beneath the subclavian vein, and just lateral to the sternoclavicular joint the parietal pleura of the lung was noted to bellow behind the 1st rib. Fortunately, none of these structures were encountered or breached during the dissection. The tumor was removed in its entirety with no breach of its overlying capsule (Figure 8). Post-removal the surgical site was irrigated thoroughly, haemostasis ensured, and closed in layers with a portovac drain in situ. Post-operatively the patient had no neurological deficit with normal power and sensation in his right upper limb being demonstrated. A post-operative Xray was taken, which on the anteroposterior view, confirmed a complete claviculectomy had been performed (Figure 9).

The patient’s wound healed well, and the histology report confirmed the tumor to be a chondrosarcoma. He was referred to the general surgery department for a lymph node dissection of his involved regional nodes, as well as to the oncology department for consideration for adjuvant therapy.

Discussion

Clavicular chondrosarcomas are characteristically painless due to the clavicle being a non-weight bearing bone [8]. Radiologically clavicular chondrosarcomas are commonly radiolucent on Xray imaging, iso-dense to surrounding muscles on CT imaging, and on MRI imaging their cartilaginous matrix makes them hypo-intense on T1W, and hyper-intense on T2W, imaging [9]. Our case demonstrated all these typical radiological features (Figures 2-4). Regarding the yield from fine needle biopsy in clavicular chondrosarcomas, a considerable range is reported in the literature of between 26%-94% depending on the centre being considered [8]. Another paper recommends that needle biopsy be avoided altogether in clavicular tumours, due to an unacceptably high risk of neurovascular injury once the clavicular cortex is softened by the tumorous process. This same paper recommends incisional biopsy as a preferred, and safer, method [10]. The risk of tumour seeding along an incisional biopsy tract, and the large size of our patient’s tumour which in the authors opinion made the risk of neurovascular injury small, underpins our decision to perform a fine needle biopsy. The diagnosis obtained therefrom was useful, as pre-operatively knowing the lesion was a chondrosarcoma, allowed us to decide to perform an enbloc claviculectomy ensuring no breach of the capsule occurred.

While the poor response, to both chemotherapy and radiotherapy, makes total claviculectomy with clear margins the mainstay of definitive treatment for clavicular chondrosarcomas, in lesions that cannot be completely resected there is a role for chemotherapy which has been demonstrated to at least slow the progression of disease [7]. One paper that considered the impact of tumor size and adequacy of resection, in 45 patients with confirmed chondrosarcomas, on prognosis, concluded that both variables independently predicted survival. In this series, 32/45 (71%) subjects had tumors larger than 8cm, 31/45 (69%) subjects had clear margins post resection, and none received adjuvant chemotherapy. In terms of outcome this paper reported an overall 3-year survival rate of 73% [11]. The tumour in our case was both similar in size, with the surgery incurring similar clear margins, to most subjects in this series.

The subcutaneous nature of the clavicle along the entirety of its length underpins the reason why most clavicular tumors that expand the cortex, are immediately visible. This early visibility causes patients to present early. If entrapment of the middle suprascapular nerve occurs pain will further complicate the presentation [4]. In our case the rapid growth of the tumor, over the period of just 1-year, resulted in a tumor of alarming size at presentation. Our case is hence not in keeping with the findings of the above study and is more representative of the African situation where patients commonly present late.

Conclusion

Chondrosarcomas are rare malignant tumors that, when occurring in the clavicle, are most favorably managed as a surgical disease. Complete surgical resection, in the form of a total claviculectomy with clear margins, offers the best chance of cure. Patients with irresectable local or distal spread should be referred to oncology for consideration for adjuvant therapy. Fortunately, despite the large size of our patients tumor, it occurred in a favourable subcutaneous location. This allowed us to achieve an en bloc resection with clear margins. The confirmed local spread, only to the regional retropectoral lymph nodes, was also favourable as it allowed a subsequent local lymph node clearance to be performed. We recommend a total claviculectomy as the surgical procedure of choice for malignant clavicular tumors. We note the procedure to be largely uncomplicated, and recommend that a stepwise dissection, in the manner as described, be performed [3].

Funding

This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.

Conflicts of Interest Statement

None of the authors listed below have any financial nor personal relationships with other people, or organizations, that could inappropriately influence (bias) their work, all within 3 years of the beginning the work submitted.

References

1. Kapoor S, Tittal P, Kapoor S (2010) Tumors around shoulder. J Clin Orthop Trauma 1(1): 5-12.
2. Zheng-Ming Yang, Hui-Min Tao, Zhao-Ming Ye, Wei-Xu Li, Nong Lin, et al. (2012) Multivariate Analysis of the Prognosis of 37 Chondrosarcoma Patients. Asian Pac J Cancer Prev 13(4): 1171-1176.
3. Mhandu P, Chaubey S, Khan H (2012) Unusual presentation of a chondrosarcoma as an anterior mediastinal mass. J Surg Case Rep 2012(4): 1.
4. Kapoor S, Tiwari A, Kapoor S (2008) Primary tumors and tumorous lesions of clavicle. International Orthopaedics 32(6): 829–834.
5. Liu C, Xi Y, Li M, Jiao Q, Zhang H, et al. (2017) Dedifferentiated chondrosarcoma: Radiological features, prognostic factors and survival statistics in 23 patients. Plosone 12(3): e0173665.
6. Wood VE (1986) The results of total claviculectomy. Clin Orthop Relat Res 207: 186–190.
7. Li Z, Ye Z, Zhang M (2012) Functional and oncological outcomes after total claviculectomy for primary malignancy. Acta Orthop Belg 78(2): 170-174.
8. Widhe B, Bauer HC (2011) Diagnostic difficulties and delays with chest wall chondrosarcoma: A Swedish population based Scandinavian Sarcoma Group study of 106 patients. Acta Oncol 50(3): 435-440.
9. Efremidou E, Oikonomou AI, Pavlidou E (2013) Juxtacortical clavicular chondrosarcoma: Diagnostic Dilemmas: case Report and Review of Literature. Clin Med Insights Oncol 7: 13-19.
10. Malawer M, Wittig J (2001) Overview of resections around the shoulder girdle: anatomy, surgical considerations, and classification. Musculoskeletal cancer surgery. Treatment of sarcoma and allied diseases. Kluwer Academic, Dordrecht, pp. 179–202.
11. Puri A, Shah M, Agarwal M, Jambhekar N, Basappa P (2009) Chondrosarcoma of bone: Does the size of the tumor, the presence of a pathologic fracture, or prior intervention have an impact on local control and survival? J Cancer Res Ther 5: 14-19.