Marquina Gloria, Sanchez-Ramon Silvia, Sarnago Ana, Ortega Luis, Bustos Ana, Hernando Florentino, Cebrian Juan Luis, Casado Antonio
Department of Medical Oncology, Hospital Universitario Clinico San Carlos, Madrid, Spain.
Department of Immunology, Hospital Universitario Clinico San Carlos, Madrid, Spain.
Case Rep Oncol. 2021 Mar 1;14(1):152-159. doi: 10.1159/000512276. eCollection 2021 Jan-Apr.
The vast majority of patients with soft tissue sarcomas (STS) of the trunk and bilateral lung metastases at diagnosis are considered incurable. These tumors have poor prognosis as only a palliative therapeutic approach can be offered to patients. We report on an extremely rare case in which bilateral lung metastases disappeared spontaneously following surgical resection of the primary CIC-rearranged sarcoma with no addition of chemotherapy or any other systemic therapy. A 53-year-old female presented with a rapidly swelling mass on her back. A magnetic resonance imaging scan of the chest revealed a large soft tissue mass on the posterior chest wall and bilateral lung metastases. Soon after stereotactic core-needle biopsy confirmation of round-cell sarcoma, the patient underwent surgery of the primary tumor as it started to be increasingly symptomatic. The resected specimen was pathologically diagnosed a poorly differentiated grade 3 sarcoma. Approximately 1 month later, a new CT scan revealed that the lung metastases were smaller and some of them had completely disappeared. Shortly afterward, the patient started adjuvant external beam radiotherapy of the tumor bed for 14 months. During the last follow-up visit, the patient confirmed no evidence of disease for 35 months postoperatively. In parallel, a histological study of pulmonary nodules, molecular analyses of the tumor, and a comprehensive study of the patient's immunophenotype were performed to gain some additional insights in the potential causes of this rare phenomenon.
绝大多数在诊断时患有躯干软组织肉瘤（STS）并伴有双侧肺转移的患者被认为无法治愈。这些肿瘤预后较差，因为只能为患者提供姑息性治疗方法。我们报告了一例极为罕见的病例，一名原发性CIC重排肉瘤患者在接受手术切除后，未进行化疗或任何其他全身治疗，其双侧肺转移灶自发消失。一名53岁女性因背部肿块迅速肿大就诊。胸部磁共振成像扫描显示后胸壁有一个大的软组织肿块及双侧肺转移灶。在立体定向粗针活检确诊为圆细胞肉瘤后不久，由于原发性肿瘤症状日益加重，患者接受了手术。切除标本经病理诊断为3级低分化肉瘤。大约1个月后，新的CT扫描显示肺转移灶变小，部分已完全消失。此后不久，患者开始对瘤床进行为期14个月的辅助外照射放疗。在最后一次随访时，患者术后35个月无疾病证据。同时，对肺结节进行了组织学研究、对肿瘤进行了分子分析，并对患者的免疫表型进行了全面研究，以进一步了解这一罕见现象的潜在原因。
