Intrathoracic Extension of a Giant Cell Tumour of the Medial End of Clavicle: A Case Report with Review of Literature

Abstract

Introduction

The clavicle is a very rare site for primary bone tumours, and Giant cell tumours of the clavicle are even rarer. Very few cases have been reported in the literature.

Method

The authors report a rare presentation of intrathoracic extension of a giant cell tumour of the medial end of the clavicle, in an 18-year-old female. The patient had painful swelling at the sternal end of the clavicle associated with a painful shoulder range of motion. Complete resection of the mass was done, preserving the lateral half of the clavicle.

Result and Discussion

At a 3-year follow-up, the patient has a good clinical outcome with no signs of recurrence. Being a very rare entity, no clear guidelines are available for the management of GCT of Clavicle. Adequate resection of the tumour mass remains the mainstay of treatment and has shown promising results. Denosumab has been proved to be effective in some studies, but the high cost of treatment is a major limitation in the Indian scenario.

Conclusion

Resection of the tumour mass with partial cleidectomy provided good results in this patient.

Introduction

Giant Cell Tumour (GCT) is a benign but locally aggressive tumour that occurs commonly in the bones of young adults. In the latest classification of bone tumours by the World Health Organization, GCTs are classified as “intermediate locally aggressive, rarely metastasizing” tumours [1]. GCT accounts for over 20% of all benign bone tumours [1] and about 4–9.5% of all primary bone tumours [2]. Common sites of GCT are the distal femur, proximal tibia, and distal radius; GCTs rarely occur in the clavicle [3].

The clavicle is a rare site for bone tumours [1], its incidence has been reported to be from 0.45 to 1.01% of all bone tumours [4, 5]. Metastatic tumours are more common than primary tumours in this region. Amongst primary lesions, malignant tumours are more common than benign in clavicle [6]. GCT of the clavicle is a very rare entity. In this Case Report, we present a case of Medial End Clavicle GCT with Intrathoracic Extension and its surgical management with a 3-year follow-up.

Written informed consent was taken from the patient. The patient was informed that data concerning the case would be submitted for publication, and she provided consent. The study was approved by the Medical Ethics Committee.

Case Report

An 18-year-old right-hand dominant female, student, presented with complaints of insidious onset of progressive swelling over her left upper chest region over the preceding 4 years, associated with pain which was aggravated with shoulder movements. There was no history of trauma or associated constitutional symptoms or any other systemic involvement.

At presentation, the patient had a well-defined mass of approximately 4 cm × 5 cm × 5 cm over the medial end of the left clavicle (Fig. 1). The mass was tender, firm in consistency with crackling felt on pressing the swelling. It was firmly attached to the bone underneath. No local warmth or pulsation was felt over the swelling. The ipsilateral shoulder had a normal range of motion but was painful throughout. Axillary, brachial, and radial pulsations were well felt. There was no neuro-deficit in the extremity. Examination of the cardiovascular and respiratory systems was within normal limits. She had a Constant score [7] of 68 and a Japanese Orthopedic Association Score [8] of 74 at presentation.

Fig. 1.

Clinical picture at presentation showing mass at medial end of left clavicle

Radiographs (Fig. 2) showed an eccentric, expansile, lytic lesion in the epiphysio-metaphyseal region of the medial end of the left clavicle. The margins of the lesion were not clearly demarcated and there was no evidence of sclerosis. The lateral end of the clavicle was apparently normal and so was the shoulder joint.

Fig. 2.

Anteroposterior view of both clavicle at presentation

Magnetic resonance imaging (MRI) of the clavicle (Fig. 3) revealed an expansile lesion of the Medial End of Clavicle, the epicentre being metaphysis. It showed multi-loculated cystic lesions with multiple fluid levels within. The lesion was extending medially up to the subchondral bone with compression and medial displacement of the brachiocephalic vein and was in close proximity of the sternocleidomastoid muscle. Inferiorly it was abutting the apical lobe of the left lungs. Intrathoracic extension of 2×2 cm behind the manubrium sterni was noted. Posteriorly, it was in close proximity to the left subclavian artery, left common carotid artery, and internal jugular vein. A provisional diagnosis of GCT with secondary aneurysmal changes was given on MRI.

Fig. 3.

MRI T1 Proton density weighted images (left to right columns—sagittal, coronal and axial sections)

Fine Needle Aspiration Cytology was done and cyto-morphological features confirmed the diagnosis of GCT (Fig. 4).

Fig. 4.

FNAC slide microscope image at 40× magnification (left) showing multiple giant cells indicated by black arrows and 100× (right) magnification showing one giant cell

Surgery was planned for the patient in the form of en masse excision of the tumour with partial cleidectomy. Blood investigations of the patient including thyroid profile, serum calcium, serum phosphorous, liver, and renal function tests were within normal limits. Considering the proximity of the lesion with the major vessels, a contrast enhanced computed tomography pulmonary angiography was done (Fig. 5) for better preoperative planning.

Fig. 5.

3D reconstruction of contrast enhanced computed tomography pulmonary angiography of tumour showing proximity to vital structures

A team of orthopaedic and cardiothoracic surgeons operated on the patient. After exposure, tumour mass was separated from the surrounding structures with fine dissection (Fig. 6). Corticotomy was done with a 2 cm margin of normal bone laterally. Mass was then gradually lifted from the lateral to the medial side to visualise the underlying structures. Blunt dissection was carried out to separate the entire mass off the soft tissue bed. The Intrathoracic Extension was removed at the end going behind the manubrium sterni. Myodesis of sub-clavious muscle was done to the free lateral end of the clavicle (Fig. 7). Postoperatively, the patient was allowed shoulder movements as tolerated. Physiotherapy in the form of shoulder range of motion exercises and muscle strengthening exercises was ensured for 6 months. She is under follow-up for 3 years now with good clinical outcome, no signs of recurrence, and is carrying out her personal and professional life with no limitations (Figs. 8, 9).

Fig. 6.

Intraoperative images showing tumour mass

Fig. 7.

Postoperative radiographs

Fig. 8.

Radiographs at 3-year follow-up

Fig. 9.

Clinical picture at 3-year follow-up

An extensive literature search was performed with the PubMed search engine of the National Library of Medicine of the Institutes of Health (http://www.ncbi.nlm.nih.gov/pubmed) using the following combinations of keywords: “Giant cell tumour”; or “GCT”; and “Clavicle”. The search was limited to English language publications with no date limitations (Table 1).

Table 1.

Summary of clinical data of 7 cases of clavicle GCT published in English

Sl no	Reference article	Year of publication	Chief complaints and duration	Treatment	Result
1	Friedman et al. [22]	1989	Case 1 42-year-old female, experiencing pain and swelling over left clavicle since past 2 years	Cystic mass measuring 3 cm in diameter was found at distal end of the clavicle and resected en bloc	The patient was followed up for 1 year and there was no sign of recurrence. The patient regained full range of motion and the cosmetic result was good
			Case 2 Swelling over right clavicle since 2 and half years	Fusiform enlargement of distal clavicle was found and resected en bloc	The patient made an uneventful recovery and could resume work after 2 months
2	Beg et al. [23]	1989	Painless swelling over upper part of left anterior chest wall	A wide excision of the mass, including 3 cm of healthy tissue of the clavicle, was performed	The postoperative period was uneventful. At follow-up 1 year later, the patient was doing well and did not have any local recurrence or distant metastasis
3	Bajpai et al. [24]	2013	1-year history of progressively increasing swelling and pain over the left lateral end of the clavicle	A wide excision of the mass, including 3 cm of healthy tissue of the clavicle, was performed	At the follow-up 1 year later, the patient was doing well and did not have any local recurrence or distant metastasis
4	Nagano et al. [25]	2015	Swelling over proximal aspect of right clavicle	The tumour was resected en bloc with the proximal half of the clavicle	No postoperative shoulder disproportion was observed, and full range of motion of the right shoulder was maintained. The patient was satisfied with the surgical outcome (Musculoskeletal Tumour Society score of 96%) at 1 year follow-up
5	Khatri et al. [26]	2016	Pain and swelling over lateral end of left clavicle, gradually increasing in size since past 4 months	Partial claviculectomy	At 1-year follow-up, there was no shoulder disability or any incidence of recurrence
6	Akinsulire et al. [27]	2017	Painless progressively increasing left upper chest swelling since 6 months	The tumour was excised with a wide margin and reconstruction done with an autogenous fibula graft	Full functional ability was restored in limb with the radiological union. After 5 years of regular follow‑up, there was no evidence of recurrence and shoulder function remained excellent

Discussion

Sir Astley Cooper first reported GCT in the eighteenth century [9]. In 1940, Jaffe and Lichtenstein defined GCT more rigorously to distinguish it from other tumours [6]. Campanacci classified these tumours based on plain radiographs [10]. It is most commonly seen in early adulthood, with a peak incidence in the third decade and with a slight female preponderance; it is usually seen in the skeletally mature patient [11]. Most GCTs occur in the bones around the knee (50–65% of all cases); the most common site is the distal femur (23–30%) followed by the proximal tibia (20–25%), distal radius (10–12%), sacrum (4–9%), and proximal humerus (4–8%) [12–14]. Very few cases of clavicle GCT have been reported in the literature. Hence, no definite treatment guidelines are available for the management of clavicle GCT. For sites, such as distal femur or proximal tibia, curettage remains the mainstay of treatment with the use of adjuvants e.g. cementation with poly-methyl methacrylate, alcohol, phenol, hydrogen peroxide, zinc chloride, cryo-ablation with liquid nitrogen, speed burr drilling, local application of zoledronic acid, and combinations [15]. If the tumour has advanced to the extent that reconstruction is not possible, or in cases of recurrence beyond salvage, replacement is used as the last option. In cases of GCT occurring in expendable bones like the iliac wing, distal ulna, proximal fibula, etc. en masses resection is performed with no need for reconstruction.

The patient we have described had a Campanacci Grade 3 GCT [6] of the Medial End of the Clavicle with Intrathoracic Extension. Since the tumour had breached the cortices of the bone with no clear margins left, curettage was not an option. Furthermore, the clavicle is an expendable bone, and studies have shown long term outcomes of cleidectomy are good [16, 17]. However, Rockwood and Wirth [18] have shown unsatisfactory outcomes in most of their patients following total cleidectomy secondary to pain, loss of muscle strength, drooping of shoulder or shoulder joint instability; they, therefore, recommended to preserve as much of clavicle as possible. The described patient in our study was not involved in heavy physical labour or sports. Hence, en masse resection of the tumour with partial cleidectomy was done. We preserved 6–7 cm of lateral end of the clavicle, thus preserving the acromio-clavicular joint and normal shoulder anatomy. Postoperative physiotherapy in the form of active range of motion exercises and muscle strengthening exercises ensured that the patient had good shoulder function. At 3-year follow-up, she had a Constant Score of 89 and a Japanese Orthopedics Association score of 93, with excellent clinical outcome, no signs of recurrence and good patient satisfaction.

Preoperative denosumab [19] would have been very helpful in this case considering the atypical location of the tumour and its extent. Despite studies showing chances of recurrence of GCT following denosumab injections [20] and incidence of sarcomatous transformation [21], the risk–benefit ratio, in this case, was clearly in favour of using denosumab. But the high cost of denosumab injection (INR 23,000 or USD 300 approximately per dose, where the total annual income of the family was INR 60,000 or USD 770 per year), was a major limitation and thus could not be provided to the patient.

Conclusion

To the best of our knowledge, this is perhaps the only case of clavicle GCT with an Intrathoracic Extension, which was successfully managed with en masse resection of the tumour with partial cleidectomy. This report highlights the need for a careful and thorough assessment of clavicle tumours to rule out malignancy. Early detection and timely intervention can save the patient from high-risk surgeries. The need for complete removal of the GCT mass to prevent recurrence cannot be emphasised more. And lastly, partial cleidectomy alone, even in advanced cases, is an effective and low-cost alternative to preoperative denosumab and subsequent resection of the clavicle, especially for economically weaker sections of our society who can’t afford the high cost of treatment.

Declarations

Conflict of interest

On behalf of all authors, the corresponding author states that there is no conflict of interest.

Ethical Standard Statement

This article does not contain any studies with human or animal subjects performed by the any of the authors.

Informed Consent

Written informed consent was taken from the patient.