Surgically-challenging chondrosarcomas of the chest wall: five-year follow-up at a single institution

Chest wall primary malignant tumors are among the rarest cartilaginous tumors, accounting for 8% of cases1. Additionally, the chest wall is not the most common site for chondrosarcomas (15% of chondrosarcoma cases)2. One can reasonably conclude that this lack of cases is responsible for the scarcity of studies on thoracic chondrosarcomas. The optimal treatment for this tumor has not improved over time; radical surgery remains the only curative option. There is no effective chemotherapy, and the tumor is relatively radio-insensitive. Therefore, the pursuit of wide-margin resections often leads to the need for complex chest wall reconstruction techniques. Even though this series addresses a small sample of patients, it includes a considerable number of tumors that were surgically challenging due to their large size and/or anatomically unfavorable locations for performance of wide-margin resections. Case Reports A total of eleven patients were selected for the study. The series consisted of eight males (72.7%) and three (27.3%) females with a mean age of 51.5 years (range: 24 to 74 years). Eight (72.7%) of the tumors were located in the ribs, and three (27.3%) were in the sternum. Chest computed tomography (CT) scans were used to diagnose the tumor size, its extension to the ribs and sternum and its extension to vessels beyond pleuropulmonary dissemination. The main surgical aspects of the study are summarized in Table 1. All patients had a primary chondrosarcoma and underwent curative surgery. The mean follow-up time was 63 months (range: 15 to 167). Table 1 Characteristics of the 11 patients with chest wall chondrosarcomas. Only three (27.3%) patients noticed a palpable asymptomatic thoracic mass. Eight pre-operative biopsies were performed in 7 (63.6%) cases (cases: 1, 2, 3, 6, 7, 10 and 11). A cartilaginous malignant neoplasm was reported in 7 biopsies. Rib resection was performed in 8 of the cases (mean: 2.5 ribs, range: 1 to 4) and a subtotal sternectomy in 3. The mean tumor size was 10.66 cm (4 to 21 cm), measured by the largest diameter. Reconstruction techniques included the use of Marlex mesh for 10 cases (90.9%) and a muscle flap for 5 (45.4%) (isolated pectoralis major in 3, rectus abdominis and latissimus dorsi in 1 and rectus abdominis, pectoralis major and pectoralis minor in 1). There was one case in which an isolated pectoralis major muscle flap was used during surgery for local recurrence. The surgical margins were intralesional (defined as positive microscopic margins) in 5 patients (45.4%), wide (defined as 4 cm of healthy tissue surrounding the tumor margin) in 4 (36.4%) and marginal (defined as microscopically free from tumor but less than 4 cm from the tumor margin) in 2 (18.2%). High-grade tumors (GIII) were observed in 2 patients (18.2%), intermediate-grade tumors in 8 (72.7%) and low-grade tumors (GI) in 1 (9.1%). The average duration of postoperative hospital stay was 8.81 days (range: 2 to 32). Four patients are currently alive with no evidence of disease (NED) (cases 8 through 11). There was one perioperative death (case 3), and three patients died due to metastatic disease (cases 1, 4 and 7). It was not possible to retrieve data about the postoperative course for three patients (cases 2, 5 and 6). Regarding the perioperative death (case 3), the patient developed mediastinitis that was surgically treated, but the patient died five days later due to septic shock. Regarding the three patients who died during follow-up (cases 1, 4 and 7), the first patient had three recurrences, the first of which occurred nine years later. All of these recurrences were treated surgically. Two years after the last resection, the patient died due to metastatic disease. The second patient (case 4) was identified as lost at the 25-month follow-up. He was alive with NED at the last clinical evaluation performed. The third patient (case 7) underwent en-bloc rib resection and lung segmentectomy due to a large grade II chondrosarcoma (20×14×13 cm). One year later, she underwent lung metastasectomy and was scheduled for adjuvant chemotherapy, but she died four months later due to tumor recurrence. Four patients demonstrated postoperative complications. The complications included the following: mediastinitis followed by death, as previously described (case 3); abdomino-thoracic hernia with liver and bowel protrusion, which was treated with Marlex mesh (case 4); infected hemothorax following surgery, leading to pulmonary decortication (case 6); and an intra-operative internal mammary artery lesion and foreign body granuloma in the sternal wound that was resected 15 months later (case 9). Tumor recurrence occurred in 4 of 7 patients (cases 1, 7, 8, 10) after excluding the cases without follow-up (cases 2, 5, 6) and the case of perioperative death (case 3). The mean disease-free interval until recurrence was 43.9 months (12.6 to 115.2). All of the tumors in these patients demonstrated an intermediate degree of differentiation; the margins were intralesional in 1 patient (case 1), marginal in 2 (cases 7 and 10) and wide in 1 (case 8). All of these patients underwent a new surgery. Two of the patients died due to disease progression (cases 1 and 7), whereas two of them are currently alive without any signs of recurrence (cases 8 and 10), with a mean disease-free interval post-recurrence surgery of 51.3 months. For all four of these cases, this interval was 41.2 months (4.3 to 81.2). Survival is indicated in Table 1.