Rodenko Peter N, Elefteratos Josh S, Herrell Colton A, Rodenko Emily L, Townsend Timothy
Medicine, St. George's University School of Medicine, St. George, GRD.
Internal Medicine, St. George's University School of Medicine, St. George, GRD.
Cureus. 2025 Jun 14;17(6):e86018. doi: 10.7759/cureus.86018. eCollection 2025 Jun.
Liposarcoma (LPS) is a malignant mesenchymal neoplasm originating from adipocytes. LPS comprises several histological variants, among which pleomorphic liposarcoma (PL) constitutes a rare and highly aggressive subtype. PL is most commonly found in the extremities and presents primarily in individuals aged 50 and above. Primary thoracic presentations of PL are exceedingly rare, particularly in young adults, and are often associated with metastases to the lungs. This case reports a 28-year-old white male with no significant past medical history presenting with shortness of breath, left posterior thoracic pain, and a dry cough. Imaging revealed a large intrathoracic, extrapulmonary mass arising in the left pleura with compression of the heart and ipsilateral lung, resulting in atelectasis, but without evidence of chest wall or pulmonary invasion. Hemothorax, empyema, or pleural effusion were among the differentials initially suspected due to the location and density of the abnormality; however, drainage only yielded scant fluid. Thoracotomy for pleural decortication later revealed a solid mass with local pleural adherence. Histopathology of tissue biopsies confirmed a high-grade pleomorphic liposarcoma with extensive necrosis and pleomorphic lipoblasts. The patient was started on the AIM (adriamycin/doxorubicin, ifosfamide, mesna) regimen, and care was transferred for further oncologic management. Many features of this rare liposarcoma subtype were highly unusual, including the age of presentation, tumor location, lack of extensive lung invasion or metastasis, and absence of known genetic or environmental risk factors. Given the poor prognosis associated with PL, as well as its highly aggressive nature, treatment generally requires multimodal management that often challenges existing treatment frameworks. This is in part due to the relative deficit of literature regarding PL compared to other LPS subtypes. This case not only demonstrates an unlikely sarcoma presentation but also emphasizes the therapeutic limitations in managing pleomorphic liposarcoma and the necessity for further research into subtype-specific treatment strategies.
脂肪肉瘤（LPS）是一种起源于脂肪细胞的恶性间叶性肿瘤。脂肪肉瘤包括几种组织学亚型，其中多形性脂肪肉瘤（PL）是一种罕见且侵袭性很强的亚型。PL最常见于四肢，主要发生在50岁及以上的人群中。PL原发于胸部的情况极为罕见，尤其是在年轻成年人中，且常伴有肺转移。本病例报告了一名28岁无重大既往病史的白人男性，出现呼吸急促、左后胸痛和干咳症状。影像学检查显示左胸膜出现一个巨大的胸内、肺外肿块，压迫心脏和同侧肺，导致肺不张，但无胸壁或肺部侵犯的证据。由于异常部位和密度，最初怀疑的鉴别诊断包括血胸、脓胸或胸腔积液；然而，引流仅引出少量液体。后来进行的胸膜剥脱胸廓切开术显示一个实性肿块，伴有局部胸膜粘连。组织活检的组织病理学证实为高级别多形性脂肪肉瘤，伴有广泛坏死和多形性脂肪母细胞。患者开始接受AIM（阿霉素/多柔比星、异环磷酰胺、美司钠）方案治疗，并转诊进行进一步的肿瘤管理。这种罕见的脂肪肉瘤亚型的许多特征都非常不寻常，包括发病年龄、肿瘤位置、缺乏广泛的肺侵犯或转移，以及不存在已知的遗传或环境危险因素。鉴于PL预后不良及其高度侵袭性，治疗通常需要多模式管理，这往往对现有的治疗框架构成挑战。部分原因是与其他脂肪肉瘤亚型相比，关于PL的文献相对较少。本病例不仅展示了一种不太可能出现的肉瘤表现，还强调了多形性脂肪肉瘤治疗的局限性以及对亚型特异性治疗策略进行进一步研究的必要性。
