Late Lung Metastasis in a Patient with a Clear Cell Chondrosarcoma: An Indication for a Life-Long Follow-Up?

Background. Clear cell chondrosarcoma (CCCS) is a rare subtype of chondrosarcoma and comprises between 1.6% and 2.5% of all chondrosarcoma. They are known to be chemo- and radiotherapy resistant; surgical resection is therefore the therapy of choice. Methods. We present a 63-year-old woman with a progressive lung nodule 20 years after initial diagnosis and treatment of a clear cell chondrosarcoma of the right os naviculare. Results. On serial CT scans of the chest, an asymptomatic, slowly growing nodule in the left upper lung lobe was detected. CT-guided transthoracic biopsy of this nodule confirmed the diagnosis of a chondrosarcoma lung metastasis. Video-assisted thoracoscopic wedge resection was performed with complete removal of the nodule. The patient recovered well from surgery and remains in good health during further follow-up. Conclusion. Given the tendency of clear cell chondrosarcoma to recur and metastasize after extended periods of time, a long-term, possibly life-long follow-up and clinical surveillance is advisable in these patients.

Successful en Bloc Resection for Femoral Head Clear Cell Chondrosarcoma Without Biopsy: A Case Report

Background: Clear cell chondrosarcoma (CCCS) is a rare, low-grade, malignant chondrogenic bone tumour. This tumour commonly occurs at the epiphysis of long bones, particularly in the proximal femur. Case presentation: This report describes a 58-year-old man with right hip pain since 5 months. Plain radiography, magnetic resonance imaging (MRI), and computed tomography (CT) revealed the characteristic appearance of chondroid mineralisation in the right femoral head, suggesting typical CCCS. Although a biopsy is the gold standard for definite diagnosis before treatment, wide resection with removal of the biopsy tract is thought to affect negatively affect surgical margin and postoperative hip function. En bloc resection without a biopsy and a hip hemiarthroplasty were performed instead. The pathological diagnosis was CCCS, and an adequate surgical margin was obtained. No local recurrence or distant metastases were found, and postoperative function was excellent at the final follow-up. Conclusion: The femoral head is a typical location of CCCS. Wide resection with adequate margins is the main treatment strategy for CCCS. When radiological features are typical, performing an en bloc resection without performing a biopsy is an acceptable treatment that may improve patient outcomes.

Clear Cell Chondrosarcoma with Rib Cage Metastasis: Case Report

Clear cell chondrosarcoma is a rare histological subtype of chondrosarcoma, usually with a relatively non-aggressive clinical course. However, infrequently they may relapse and metastasize. We describe a case of a male patient, 53 years old, with rib cage metastases of a clear cell chondrosarcoma 11 years after the first surgical intervention, and review the literature.

Functional optimisation in chondrosarcoma of the subglottis: a novel surgical approach

Chondrosarcoma of the larynx is rare accounting for approximately 1% of laryngeal cancers; clear cell subtype is a rare variant. Given the low risk of occult nodal disease, they present a unique opportunity to maximise tissue preservation in order to optimise both recovery and long-term functional outcomes. We present a case of laryngeal clear cell chondrosarcoma causing critical airway obstruction. An emergency tracheostomy was performed and mapping biopsies were taken. The tumour originated from the cricoid and extended into both arytenoid superstructures precluding cricotracheal resection. A modified narrow-field laryngectomy was performed, preserving all pharyngeal mucosa and neurovascularly intact infrahyoids. Organ preservation surgery is preferred in the management of laryngeal chondrosarcoma. If laryngectomy is required, the surgeon must ensure that all uninvolved, functional tissue is preserved carefully to improve swallow and voice outcomes postoperatively. We describe a novel technique used to achieve this outcome.

Clear Cell Chondrosarcoma: Clinical Characteristics and Outcomes in 15 Patients

Background. Clear cell chondrosarcoma (CCC) represents less than 6% of all chondrosarcomas, and thus, our understanding of this rare entity is limited. Analyzing clinical characteristics and treatment patterns, thus increasing our knowledge, may improve treatment strategy. We review our institutional experience with 15 patients, including one case with dedifferentiation. Methods. A retrospective review was conducted in CCC patients treated at our institution from 1996 to 2015, with at least 2-year follow-up. Descriptive statistics and Kaplan–Meier survival analyses were performed. Results. Of 19 patients identified, 15 patients had at least 2-year follow-up and were included. The median age at diagnosis was 43 years. 80% were male. The most common presenting signs were pain (12 patients; 80%) and fracture (2 patients; 13.3%). The most common site was proximal femur (8 patients; 53%). All patients had MSTS Stage I disease. Primary treatment included wide resection in 10 patients (67%) and intralesional or marginal resection in 5 patients (33%). Three patients died of disease during the study period, 1 with dedifferentiation of recurrent CCC. The median time to death from disease was 15.3 years (95% CI: (14.2; NA)). The median time to either recurrence or death was 7.73 years for patients who had intralesional/marginal resection and 16.44 years for patients with wide resection (HR (wide vs. intralesional/marginal) = 0.21, 95% CI: (0.04; 1.18), p = 0.053 ). The median time to recurrence or death was significantly shorter for patients not initially treated at a sarcoma center ( p = 0.01 ). Conclusions. CCC is a rare entity, and our understanding of it is still evolving. We observed a higher recurrence rate for intralesional or marginal resection, and wide resection alone remains the mainstay of treatment. Better outcomes were observed in patients initially treated by trained musculoskeletal oncologists. Due to the propensity of CCC to recur decades after initial resection, lifelong surveillance is recommended.

Clear cell chondrosarcoma of proximal femur: a rare entity with diagnostic dilemma

Clear cell chondrosarcoma is an extremely rare malignant neoplasm. The rarity and slow-growing nature of this tumour often lead to prolonged symptoms and also initial misdiagnosis with benign lesions such as chondroblastoma. It can also be confused with avascular necrosis of the femoral head when the lesion is located in the femoral head, as was in the case we report. The patient was kept on observation and conservative treatment for almost 9 years before the correct diagnosis and appropriate treatment. Wide local resection with negative margins forms the mainstay of treatment since intralesional procedures predispose to high local recurrence rate. A prolonged follow-up is recommended since late local recurrences and metastases are common.