Tadala Harsha S S, Gulia Ashish, Puri Ajay, Rekhi Bharat, Laskar Siddhartha
Orthopaedic Oncology, Department of Surgical Oncology, Homi Bhabha Cancer Hospital and Research Centre, Tata Memorial Centre, Visakhapatnam, India.
Surgical Oncology, Homi Bhabha Cancer Hospital and Research Centre, Tata Memorial Centre, New Chandigarh, Punjab India.
Indian J Surg Oncol. 2025 Apr;16(2):627-632. doi: 10.1007/s13193-024-02108-4. Epub 2024 Oct 25.
Clear cell sarcoma (CCS) also called as melanoma of soft parts is a rare malignant soft-tissue tumor with melanocytic differentiation, primarily located in deep soft tissue and has preference for lymph node and pulmonary metastasis. Metastatic patients have poor oncologic prognosis and so far, no adjuvant treatment seems to be effective in these cases. All cases were retrieved from our prospectively maintained surgical database. Twenty-nine patients (14 males,15 females) with a mean age of 34 years (13-69 years) were operated between 2004 and 2020. Overall survival and recurrence free survival were evaluated. At mean follow-up of 45 months, out of 29 cases, 2 lost to follow-up, 6 patients had nodal metastasis, 3 had lung metastasis, one had both at presentation, 15 patients had died due to disease and 12 are alive. Nodal metastasis rate was 24% (7/29). Positive margin is observed in 6 (19%). 24 patients had recurrence of which 6 had both local and distant. The 5-year OS, LRFS, DRFS were 53%, 56%, 35% respectively at 5 years. Survival rates were significantly poor in patients with metastasis at presentation, 66% vs. 25% ( = 0.016). CCS is an aggressive soft tissue malignancy with high propensity for metastasis. The overall survival of CCS is poor. Metastasis at presentation negatively impacts on survival. Distant recurrences, especially nodal recurrences are not uncommon and complete surgical resection of all recurrences if possible is advisable. Research to develop new chemotherapeutic agents and targeted therapies may help improve the prognosis of CCS.
透明细胞肉瘤（CCS）也被称为软组织黑色素瘤，是一种罕见的具有黑色素细胞分化的恶性软组织肿瘤，主要位于深部软组织，易发生淋巴结和肺转移。发生转移的患者肿瘤预后较差，迄今为止，尚无辅助治疗方法对这些病例有效。所有病例均从我们前瞻性维护的手术数据库中检索获得。2004年至2020年间，对29例患者（14例男性，15例女性）进行了手术，平均年龄为34岁（13 - 69岁）。评估了总生存期和无复发生存期。平均随访45个月，29例患者中，2例失访，6例有淋巴结转移，3例有肺转移，1例初诊时两者均有，15例患者因疾病死亡，12例存活。淋巴结转移率为24%（7/29）。6例（19%）切缘阳性。24例患者出现复发，其中6例既有局部复发又有远处复发。5年时的总生存期、局部无复发生存期、远处无复发生存期分别为53%、56%、35%。初诊时发生转移的患者生存率明显较差，为66% 对 25%（P = 0.016）。CCS是一种侵袭性软组织恶性肿瘤，具有较高的转移倾向。CCS的总体生存率较差。初诊时发生转移对生存有负面影响。远处复发，尤其是淋巴结复发并不少见，建议尽可能对所有复发灶进行完整的手术切除。研发新的化疗药物和靶向治疗方法的研究可能有助于改善CCS患者的预后。
