Rare case of chondrosarcoma of the manubrium sterni: Management challenges and insights (a surgical case report)

Abstract

Introduction and Importance

Chondrosarcoma of the manubrium sterni is an exceedingly rare localization of chondrosarcoma. Its treatment poses a significant therapeutic challenge due to the tumor's proximity to the mediastinal organs and the clavicles. This challenge is magnified when the inner ends need to be resected due to tumor contact with the sternoclavicular joints and, more critically, during the reconstruction of the thoracic wall.

Case presentation

We present the case of a 71-year-old female with a 45x42x51 mm chondrosarcoma of the manubrium sterni, extending to both sternoclavicular joints. The diagnosis was confirmed cytologically and histologically after an ultrasound-guided biopsy. A surgical strategy involving en bloc resection of the manubrium sterni, the internal ends of both clavicles, and the first two ribs, followed by sternal reconstruction using a synthetic manubrial plate and titanium costal staples without clavicular bridging, was indicated and executed.

Clinical discussion

This case outlines the surgical considerations and techniques adopted for this complex procedure, emphasizing the operative planning and interdisciplinary collaboration required for a successful outcome.

Conclusion

At 18 months post-surgery, the patient demonstrated favorable clinical and radiological progress, indicating a positive response to the treatment strategy employed.

Highlights

• •Detailed presentation of a rare case of chondrosarcoma of the manubrium sterni, highlighting diagnostic and management challenges.
• •In-depth description of innovative surgical techniques used for en bloc resection and thoracic wall reconstruction.
• •Analysis of postoperative rehabilitation strategies and their impact on the patient's quality of life improvement.
• •Comprehensive literature review, placing this case in context with other similar cases of chondrosarcoma.
• •Discussion on the multidisciplinary aspects of treatment, involving oncologists, thoracic surgeons, and radiologists.
• •Significant contribution to existing literature on rare chondrosarcomas, offering practical insights for clinical diagnosis and management.

1. Introduction

Chondrosarcoma is a primary malignant bone tumor originating from cartilaginous cells. It is the most common type after osteosarcoma, accounting for 25 % of primary bone tumors and typically occurs from the fourth decade of life in both men and women, with a slight male predominance [1]. The most frequently affected sites are the pelvic bone (about one-third of cases) and the proximal femur (about one-quarter of cases) [[2], [3]]. Spinal involvement remains rare (1 to 10 % of cases); this is also true for the sacrum (5 % of cases) and the thoracic wall [4]. The sternum represents an exceptionally rare location as is the case for all primary bone tumors, with an incidence of 1 % [5]. The treatment for chondrosarcoma is exclusively surgical, as chemotherapy and radiotherapy have not shown effectiveness [6,7]; for a location such as the sternum, it poses a real therapeutic challenge given the proximity to mediastinal neurovascular structures and vital organs. The excision must be sufficiently wide to avoid the risk of local recurrence or metastasis, which may result in a significant loss of substance. Therefore, prosthetic reconstruction and its covering must be considered to restore the stability and rigidity of the thoracic cage, minimize respiratory complications, and provide a satisfactory aesthetic outcome. The materials and reconstruction techniques vary, each with theoretical pros and cons, but the choice ultimately depends on the surgeon's preference and the availability of these materials [8]. We report the case of a patient with a chondrosarcoma of the manubrium sterni in contact with both sternoclavicular joints, for whom en bloc resection of the manubrium, the internal ends of both clavicles, and the first two ribs combined with sternal reconstruction using a synthetic plate and titanium costal staples were indicated and performed.

2. Methods

This case report details the evaluation, diagnosis, and management of a patient with chondrosarcoma of the manubrium sterni, highlighting the challenges and specifics of its treatment. All medical procedures and assessments were conducted at a specialized center for orthopedic surgery.

All medical procedures, including imaging, surgical intervention, and rehabilitation, were conducted in accordance with established protocols at this specialized center. The case report was meticulously documented, and its presentation adheres to the SCARE 2023 guidelines: updating consensus Surgical Case Report (SCARE) guidelines [31], which set the standards for high-quality reporting in surgical case reports.

This methodological approach ensures a comprehensive and standardized presentation of the case, facilitating replication of the study and comparison with similar cases in the field.

3. Case presentation

A 71-year-old woman presented for consultation with a painful swelling at the antero-superior part of the thoracic wall. She had no significant personal or familial medical history, and no known hereditary conditions. The issue began 8 months prior to the consultation, with the emergence of an upper thoracic mass that progressively increased in size. The condition's progression was marked by the onset of severe, continuous thoracic pain, resistant to usual analgesics and causing insomnia, starting three months after the mass first appeared, which led to the consultation.

Furthermore, the patient did not report any difficulties with swallowing or respiratory issues, nor any episodes of fever. During the physical examination, she was found to be in good general condition. The palpebral conjunctivae were colored, and the bulbar conjunctivae were non-icteric. The neck was supple, and no enlargement of axillary or cervical lymph nodes was noted. A loco regional examination revealed a roughly round, 55 × 50 mm diameter swelling at the level of the manubrium sterni. The swelling was hard, tender upon palpation, fixed in relation to the deep plane, but mobile over the superficial plane. The overlying skin appeared normal, showing no signs of inflammation. A punctiform scar, likely from a previous biopsy, was visible at the center of the mass (Fig. 1). Additionally, pain was observed during extreme abduction of the left shoulder.

Fig. 1.

Photography of the patient's clinical presentation.

In the laboratory workup, biological analyses were conducted, including a complete blood count, biochemical profile, phosphocalcic balance, and coagulation panel. Interestingly, all these results were within normal limits, showing no significant deviations from the expected values.

Moving on to the radiological assessments, an important observation was made through frontal chest radiography (Fig. 2).

Fig. 2.

A multilocular calcified image with defined contours located over the manubrium sterni.

It showed a multilocular calcified image with defined contours located over the manubrium sterni. Notably, there were no other visible lesions, particularly in the parenchymal or parietal areas, except for calcification of the aortic knob.

Further in-depth investigation was carried out with a thoracoabdominal CT scan. At the thoracic level, the scan revealed a heterogeneous lesion on the manubrium sterni, faintly enhanced by contrast medium. This lesion measured approximately 47 × 45 mm, extending over 56 mm, and contained coarse calcifications in contact with both sternoclavicular joints, more pronounced on the left side. Interestingly, there was no bone lysis at the inner ends of both clavicles. The lesion was situated close to the initial segment of the left common carotid artery, albeit with a separation margin. The mediastinal fat appeared preserved, and there were no signs of mediastinal lymphadenopathy, pleural or pericardial effusion, pulmonary nodules, or active lesions (Fig. 3). At the abdominal level, the scan revealed no detectable lesions.

Fig. 3.

CT scan slice showing the mass at the level of the manubrium sterni.

An ultrasound-guided biopsy of the lesion was conducted, revealing hyaline cartilaginous tissue with a lobulated appearance. This tissue was partially separated by fine arciform fibrous tissue and showed increased cellularity and areas of isogenic grouping. Notably, some chondroblasts contained more than one chondrocyte, leading to the confirmation of a Grade 1 chondrosarcoma diagnosis.

Subsequently, this medical case was deliberated upon at a multidisciplinary consultation meeting. The team comprised oncologists, orthopedic trauma surgeons, thoracic surgeons, anesthetists, radiologists, and pathologists. A detailed discussion concluded that the best course of action would be an R0 resection (resection in healthy tissue) en bloc with sufficient safety margins, ensuring no tumor breach, and involving thoracic wall reconstruction. The patient and her family were thoroughly briefed about the surgical morbidities and associated risks.

Following the histological confirmation of the diagnosis and before proceeding with the surgical resection, we had to rely solely on a thoraco-abdomino-pelvic and cervical CT scan for the extension assessment, without the benefit of a PET scan or MRI for a more detailed evaluation of the soft tissues, due to the patient's low socio-economic status.

Given this diagnosis and the negative extension assessment, the decision for a surgical approach involving the resection of the mass followed by reconstruction of the defect was made by the multidisciplinary consultation meeting.

During the operation, the patient was placed in dorsal decubitus under general anesthesia and received perioperative antibiotic prophylaxis. An elliptical median skin incision, centered on the tumor and including the biopsy scar, was made. The dissection advanced to the pectoral muscles, which were not adherent to the tumor mass and were carefully detached from the thoracic cage. The infrahyoid muscles were sectioned to expose the sternal notch, revealing a grayish-white firm tumor, measuring 55 × 50 mm, in contact with both sternoclavicular joints laterally and the manubriosternal joint below (Fig. 4). Digital palpation confirmed no endo-thoracic extension. The tumor was then resected en bloc with a 2 cm margin of safety and sent for pathologic study (Fig. 5).

Fig. 4.

Perioperative aspect of the tumor.

Fig. 5.

Appearance of the tumor after resection.

The reconstruction process commenced with the placement of a polypropylene synthetic plate, shaped to fit the defect and secured to the bone with trans osseous sutures (Fig. 6). This was followed by osteosynthesis of the ribs using sliding staples and splints (Stratos®). The clavicular stumps were left free. Post-reconstruction, the thoracic wall was stable, showing no paradoxical respiratory movements. The reconstruction material was concealed by suturing the mobilized pectoral muscles along the midline, and the site was closed in layers over a suction drain. Meticulous hemostasis was achieved with an estimated blood loss of 200 ml, and no perioperative incidents were reported.

Fig. 6.

Placement of a synthetic polypropylene plate to fill the osseous defect.

The patient underwent a smooth postoperative recovery, being discharged on the fifth day after surgery and subsequently subjected to diligent clinical and radiological follow-up. This supervision was particularly rigorous during the initial three months, extending every three months for a total of 18 months. Remarkably, despite the conservative surgery and the clavicular stumps being left free, there was a notable enhancement in shoulder function, as evidenced by improvements in both the Constant Score (CS) and the Nottingham Clavicle Score (NCS). The CS ascended from 50 preoperatively to 78 one year post-surgery, while the NCS rose from 74 to 84, indicating a significant boost in the patient's quality of life. This improvement was underscored by the alleviation of pain and the lack of functional disability. Furthermore, respiratory function remained preserved, with blood gas analysis returning to normal. Follow-up CT scans provided reassurance, showing no evidence of local recurrence or distant metastases, and there were no signs of local or regional infection, fracture, or disassembly of the osteosynthesis material.

4. Discussion

We report a rare case of chondrosarcoma of the manubrium sterni, in contact with the sternoclavicular joints, where en bloc resection of both inner ends of the clavicles, the manubrium sterni, and the inner ends of the first two ribs was performed, followed by reconstruction combining a synthetic plate and titanium costal osteosynthesis material without bridging of the clavicles. Chondrosarcoma is the most common malignant bone tumor in the thoracic wall. It develops from the chondrocostal junction (60 %), the rib neck (20 %), or the sternum (20 %) [10]. Typically affecting individuals aged 30 to 50 years with a slight male predominance, it presents as a large sternocostal plastron mass; initially asymptomatic, then painful, sometimes with signs of nerve or respiratory compression [11]. It is noteworthy that our patient, at the age of 71, does not fall within the usual age range for this pathology. Radiologically, the tumor appears as a low-density, polylobed, often calcified image with adjacent bone cortex destruction. Macroscopically, the tumor is firm, grayish-white, poorly demarcated, often invading nearby structures [12]. Histologically, chondrosarcomas pose diagnostic challenges based on their degree of differentiation and histological grade. Grade 1 chondrosarcomas may mimic a chondroma; here, the diagnosis relies on radiological and clinical arguments, as thoracic wall tumors are almost always chondrosarcomas. Grades 2 and 3 chondrosarcomas present fewer diagnostic difficulties [10].

As with all well-differentiated cartilaginous tumors, treatment for chondrosarcomas is exclusively surgical. In the thoracic wall, tumor excision must adhere to standard oncological surgery practices. Resections should be in healthy tissue (R0), en bloc, with sufficient safety margins, and without tumor breach. The excision limit remains a subject of debate in the absence of high-level evidence studies [8]; 2 to 6 cm margins from the tumor boundary are recommended [[13], [14], [15]]. Although it has been shown that the quality of excision margins does not significantly influence overall survival [3,[16], [17], [18]], it remains a crucial factor for local recurrence [20]. Marruli et al. [21], in an analysis of 89 thoracic chondrosarcomas, reported that factors positively influencing overall survival included age under 55 years, tumor size under 6 cm, clean excision limits, and a histological grade of 1, although the risk of recurrence was influenced only by histological grade. Conversely, Lenze et al. [21] in an analysis of 55 thoracic chondrosarcomas, concluded that the quality of the excision margin does not affect survival or recurrence risk.

Currently, there are no definitive guidelines for the absolute indication of thoracic wall reconstruction. It remains a complex procedure with a risk of serious complications, especially if prosthetic material is used. The indication depends mainly on the extent and anatomical location of the defect. However, most studies [8,22,23] indicate that anterior and anterolateral resections over 5 cm, posterior resections over 10 cm, and sternal resections require reconstruction to restore wall rigidity, prevent respiratory complications including paradoxical breathing, prevent scapular entrapment in resections of the posterior arch of the 4th, 5th, and 6th ribs, protect underlying organs, particularly to avoid pulmonary herniation, and ensure a good aesthetic result by limiting deformation. Therefore, the minimal reduction of elasticity is balanced by the parietal strength improvement, the restoration of the physiological ventilatory mechanics, and excellent aesthetic findings.

After sternotomy, the reconstruction must be rigid, combining the use of flexible plates and osteosynthesis material to cover the large defect with good results [24], or using a complete sternal prosthesis (metal or custom-made 3D) to meet the particular technical challenge of this reconstruction. However, it remains a difficult technique, increases operative time and hospital stay, is costly, and is not available in all health facilities [[25], [26], [27]]. The treatment of clavicular stumps is not consensual. Some teams consider fixing them, while others argue that the mechanical constraints of this region often lead to material breakage, and functional sequelae are not significant when stumps are left free [8].

This case report presents the clinical outcome of a resection of a chondrosarcoma of the manubrium sterni combined with reconstruction using titanium costal osteosynthesis implants of the “StraTos” type and a polypropylene synthetic plate without bridging of the clavicular stumps. These materials remain reliable, familiar to orthopedic and thoracic surgeons, easily obtainable, cost-effective, well tolerated by patients, sufficiently flexible to not constrain respiratory dynamics, and rigid enough to fulfill the protective function of the thoracic wall and avoid paradoxical breathing [[28], [29], [30]]. Moreover, titanium, due to its low density and non-ferromagnetic nature, is conducive to postoperative radiological examinations, primarily in oncology, especially for detecting recurrences, which represents the main risk of thoracic wall chondrosarcomas [21].

5. Conclusion

The reconstruction of the thoracic wall following the resection of a chondrosarcoma of the manubrium sterni using a synthetic plate and titanium costal osteosynthesis implants without bridging of the clavicular stumps remains a reliable and readily available method. It provides sufficient rigidity to ensure the stability of the thoracic wall with good functional outcomes and also allows for easy radiological monitoring to detect local recurrences.

Consent

Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.

Provenance and peer review

Not commissioned, externally peer reviewed.

Ethical approval

At our university hospital, it is established policy that case reports focusing on non-experimental medical or surgical treatment do not require ethical consent beyond the patient's informed consent prior to publication. This policy reflects our commitment to upholding ethical standards while recognizing the educational and informational value of Author Form sharing clinical experiences. Patient confidentiality and respect for their rights remain paramount; thus, informed consent is diligently obtained before any case details are published. This approach ensures that our case reports, while contributing valuable insights to the medical community, adhere strictly to ethical guidelines and respect patient autonom

Funding

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

Author contribution

All authors have contributed equaly to all parts of the elaboration of this case series.

Guarantor

The grantors for this case series are the first author doctor Omar Chraibi and the corresponding author doctor Lamris Mohamed Amine.

Declaration of competing interest

For all the authors, there’s no financial or personal relationships with people or organosations that could inappropriately influence our work.