Cruz-Alvarenga Abner J, Bendaña Laínez Carlos F, Villalta Salgado Karla V
Department of Medicine, Universidad Nacional Autónoma de Honduras, Tegucigalpa, HND.
Department of Pathology, Instituto Hondureño de Seguridad Social, Tegucigalpa, HND.
Cureus. 2024 Oct 25;16(10):e72401. doi: 10.7759/cureus.72401. eCollection 2024 Oct.
Undifferentiated pleomorphic sarcoma (UPS) is a rare subtype of undifferentiated soft tissue sarcoma (USTS). UPS is a fast-growing malignant tumor with a high mitotic rate and a high risk of distant metastasis that increases proportionally to tumor size. Obtaining an early diagnosis confers a more favorable prognosis. Neoplastic fever (NF) is the refractory fever subsumed under the category of classic fever of unknown origin (FUO) caused by malignant neoplasms. NF is a paraneoplastic syndrome that rarely occurs in STS. Among UPS tumors, there is an exceptional subtype that causes refractory fever and signs of systemic inflammation. The coexistence of UPS with NF (UPS-NF) represents an unusual event. We present an exceptional case of a 58-year-old male patient with a two-month history of FUO that revealed an atypical clinical presentation of NF generated by a rare STS of the anterior thigh with subsequent sternal metastases. The patient received neoadjuvant chemotherapy and underwent a compartmentectomy with a microscopic residual tumor (R1), followed by postoperative adjuvant radiotherapy with unfavorable results. The patient also presented overlapping paraneoplastic manifestations (NF and areolar hyperpigmentation) that occur concurrently with the onset of metachronous sternal metastases, highlighting the uninvolved lung parenchyma.
未分化多形性肉瘤(UPS)是未分化软组织肉瘤(USTS)的一种罕见亚型。UPS是一种生长迅速的恶性肿瘤,有丝分裂率高,远处转移风险高,且转移风险与肿瘤大小成正比。早期诊断可带来更有利的预后。肿瘤热(NF)是由恶性肿瘤引起的经典不明原因发热(FUO)范畴内的难治性发热。NF是一种副肿瘤综合征,很少发生于软组织肉瘤。在UPS肿瘤中,有一种特殊亚型可引起难治性发热和全身炎症体征。UPS与NF(UPS-NF)并存是一种罕见情况。我们报告一例特殊病例,一名58岁男性患者,有两个月的FUO病史,其显示出由大腿前部罕见的软组织肉瘤产生的NF的非典型临床表现,随后出现胸骨转移。患者接受了新辅助化疗,并接受了间室切除术,术后有镜下残留肿瘤(R1),随后进行术后辅助放疗,但效果不佳。患者还出现了与异时性胸骨转移同时发生的重叠副肿瘤表现(NF和乳晕色素沉着),突出显示未受累的肺实质。