Department of Orthopaedics, First Medical Faculty, Charles University, University Hospital Na Bulovce, Prague, Czech Republic.
Department of Pathology, First Medical Faculty, Charles University, University Hospital Na Bulovce, Prague, Czech Republic.
Eur J Orthop Surg Traumatol. 2021 Dec;31(8):1597-1602. doi: 10.1007/s00590-021-02964-8. Epub 2021 Apr 3.
Chondrosarcomas of the small bones of the hands and feet are uncommon and account for less than 2% of all chondrosarcomas in the skeleton; a 4.2% rate of malignant degeneration of enchondromas to secondary chondrosarcomas has been reported. We performed this study to assess the outcome of the patients with chondrosarcomas of the small bones. We hypothesized that the presumed better prognosis of chondrosarcomas in these locations could be biased as the majority of these tumors tend to be of lower grades and are removed when still small sized, and that less aggressive surgery has an adverse effect on local control MATERIALS AND METHODS: We retrospectively studied the files of 44 patients with chondrosarcomas of the small bones of the hands and feet. There were 23 female and 21 male patients with a mean age of 50.9 years (range, 6-86 years). The mean follow-up was 13 years (range, 5-40 years). We recorded the patients' details including gender and age at diagnosis, type and duration of symptoms, tumor location and histology, type of surgery and complications, and outcome (local recurrences and metastases).
The most common anatomical location for chondrosarcomas of the hands was the metacarpals and proximal phalanges. The most common presenting symptom was a slowly enlarging palpable mass. Overall, 36 chondrosarcomas were secondary to a pre-existing cartilaginous tumor. Patients with syndromes were affected in younger age compared to the others. The mean age at diagnosis was higher for higher grade chondrosarcomas. Overall, 13 patients (29.5%) experienced a local recurrence; the rate of local recurrence was higher after curettage regardless the histological grade of the tumors. After wide resection of the first local recurrence, five patients experienced local re-recurrence. Five patients (11.4%) experienced lung metastases, two patients at presentation. All these patients had a high grade chondrosarcomas. At the last follow-up, one patient with lung metastases died from disease, and another patient died from unrelated cause.
The patients with chondrosarcomas of the small bones of the hands and feet may have a dismal outcome if treated improperly. A careful treatment planning is required to avoid unnecessary amputations. Curettage is associated with a high rate for local recurrence that should be treated with a more aggressive surgical resection to avoid re-recurrences. Although the risk is low, the patients may develop lung metastases, especially those with higher grade chondrosarcomas, therefore, they should be staged and followed closely.
手足小骨的软骨肉瘤少见，占骨骼中所有软骨肉瘤的不到 2%；有报道称，软骨瘤恶性转化为继发性软骨肉瘤的比例为 4.2%。我们进行这项研究是为了评估手足小骨软骨肉瘤患者的预后。我们假设这些部位的软骨肉瘤假定预后较好可能存在偏差，因为大多数肿瘤往往分级较低，并且在肿瘤还较小时就被切除，而侵袭性较小的手术对局部控制有不利影响。
我们回顾性研究了 44 例手足小骨软骨肉瘤患者的档案。患者中有 23 名女性和 21 名男性，平均年龄为 50.9 岁（范围 6-86 岁）。平均随访时间为 13 年（范围 5-40 年）。我们记录了患者的详细信息，包括性别和诊断时的年龄、症状的类型和持续时间、肿瘤位置和组织学、手术类型和并发症以及结果（局部复发和转移）。
手部软骨肉瘤最常见的解剖位置是掌骨和近节指骨。最常见的首发症状是逐渐增大的可触及肿块。总体而言，36 例软骨肉瘤继发于先前存在的软骨瘤。有综合征的患者比其他患者发病年龄更小。诊断时的平均年龄随肿瘤分级的升高而升高。总体而言，13 例（29.5%）患者发生局部复发；无论肿瘤的组织学分级如何，刮除术后局部复发率均较高。第一次局部复发后广泛切除后，5 例患者发生局部再复发。5 例（11.4%）患者发生肺转移，其中 2 例在就诊时发生。所有这些患者均患有高级别软骨肉瘤。在最后一次随访时，1 例肺转移患者死于疾病，另 1 例患者死于非相关原因。
如果治疗不当，手足小骨软骨肉瘤患者的预后可能不佳。需要仔细的治疗计划以避免不必要的截肢。刮除术与局部复发率高相关，应采用更积极的手术切除来避免再复发。尽管风险较低，但患者可能会发生肺转移，特别是那些患有高级别软骨肉瘤的患者，因此应进行分期并密切随访。
