Dey Treshita, Khosla Divya, Kumar Divyesh, Chatterjee Debajyoti, Madan Renu, Singh Harjeet, Singh Harkant, Kapoor Rakesh
Department of Radiotherapy and Oncology, Postgraduate Institute of Medical Education and Research, Regional Cancer Centre, Chandigarh, India.
Department of Histopathology, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
Ochsner J. 2021 Winter;21(4):431-435. doi: 10.31486/toj.20.0164.
Pleomorphic liposarcoma (PLS) is a very rare type of primary pulmonary sarcoma. Clinical data about these tumors are limited, and optimal treatment has not yet been defined. A 32-year-old male presented with pain and heaviness over the right chest. Contrast-enhanced computed tomography (CECT) of the chest showed a heterogeneous hypodense pleural-based mass and a mediastinal mass. The patient was treated with chemoradiotherapy, followed by excision and adjuvant chemotherapy. Seven months after completion of treatment, he presented with an abdominopelvic mass and soft tissue peritoneal deposits. The mass was resected, and second line chemotherapy resulted in a partial response. The patient was routinely followed. Six months after completion of the second round of chemotherapy, CECT showed multiple soft tissue deposits in the right lumbar region, right hemipelvis, and presacral region with no evidence of pulmonary disease. Chemotherapy elicited a partial response. Three years from the date of diagnosis, the patient was alive with stable disease. This case is unique because of the rare primary site of PLS presentation and the rare presentation of peritoneal metastasis. Citing such cases would help us to define adequate treatment protocols for this aggressive tumor.
多形性脂肪肉瘤(PLS)是一种非常罕见的原发性肺肉瘤。关于这些肿瘤的临床数据有限,尚未确定最佳治疗方案。一名32岁男性出现右胸部疼痛和沉重感。胸部增强计算机断层扫描(CECT)显示一个不均匀的低密度胸膜下肿块和一个纵隔肿块。患者接受了放化疗,随后进行了切除和辅助化疗。治疗完成七个月后,他出现了腹盆腔肿块和软组织腹膜转移。肿块被切除,二线化疗产生了部分缓解。对患者进行常规随访。第二轮化疗完成六个月后,CECT显示右腰部、右半骨盆和骶前区域有多处软组织转移,无肺部疾病迹象。化疗产生了部分缓解。自诊断之日起三年,患者存活且病情稳定。该病例独特之处在于PLS的原发部位罕见以及腹膜转移的表现罕见。引用此类病例将有助于我们为这种侵袭性肿瘤确定适当的治疗方案。