Department of Medical Oncology, Institut Gustave Roussy, Villejuif, France.
Department of Pathology, Saint Joseph University, Beirut, Lebanon.
J Egypt Natl Canc Inst. 2022 Jun 27;34(1):27. doi: 10.1186/s43046-022-00128-1.
Sarcoma of unknown primary (SUP) designates an enigmatic entity with histologic confirmation of a metastatic tumor without an identifiable primary after a thorough diagnostic workup. The term "unknown primary" is heavily debatable given that sarcomas can arise from any tissue that harbors its histological structure. In this review, we discuss the validity of SUP as a distinct entity. Medline/PubMed and Google Scholar were searched from 1990 until April 2020 for publications in the English language reporting on SUP. We excluded articles reporting on cases with sarcomas from known organ sites such as lung or uterine sarcomas as well as synovial sarcomas. The Kaplan-Meier method was used to compute the median overall survival. A total of 26 patients with SUP were identified. The median age at diagnosis was 17.5 years with a similar prevalence among men and women. The tumors most commonly reported were alveolar rhabdomyosarcoma and rhabdomyosarcoma not otherwise specified. Almost two-thirds of the patients were reported to have more than one metastatic site. Among the 13 patients with survival data, the median overall survival was 10.0 months. Two patients underwent autopsy and had their primary culprit identified in the chest wall and paravertebral.
This review showed that SUP shares with sarcomas of known primary similar clinical features including an aggressive clinical course, generally poor response to chemotherapy, and dismal patient outcomes. Thus, SUP does not appear to display a different natural history and biological properties that would allude to a distinct entity.
未确定来源的肉瘤（SUP）是一种神秘的实体，其组织学特征为转移性肿瘤，经过全面的诊断检查后仍无法确定其原发部位。鉴于肉瘤可以起源于任何具有其组织学结构的组织，“未确定来源”这一术语存在很大争议。在本综述中，我们讨论了 SUP 是否为一个独立实体的有效性。从 1990 年到 2020 年 4 月，我们在 Medline/PubMed 和 Google Scholar 上搜索了以英文发表的关于 SUP 的出版物。我们排除了报告源自已知器官部位（如肺或子宫肉瘤以及滑膜肉瘤）的肉瘤病例的文章。Kaplan-Meier 法用于计算中位总生存期。共确定了 26 例 SUP 患者。诊断时的中位年龄为 17.5 岁，男女患病率相似。最常报告的肿瘤是肺泡横纹肌肉瘤和未特指的横纹肌肉瘤。几乎三分之二的患者报告有一个以上的转移部位。在有生存数据的 13 名患者中，中位总生存期为 10.0 个月。有两名患者接受了尸检，在胸壁和椎旁发现了原发性肿瘤。
本综述表明，SUP 与已知来源的肉瘤具有相似的临床特征，包括侵袭性临床病程、对化疗的普遍不良反应和较差的患者预后。因此，SUP 似乎没有表现出不同的自然病史和生物学特性，暗示其为一个独立实体。
