以单一骨转移为表现的孤立性纤维性肿瘤:六例报告并文献复习。

Clinical Sarcoma Research Pub Date : 2016-09-01 eCollection Date: 2016-01-01 DOI:10.1186/s13569-016-0055-1
Vittoria Colia, Salvatore Provenzano, Carlo Morosi, Paola Collini, Salvatore Lorenzo Renne, Paolo G Dagrada, Claudia Sangalli, Angelo Paolo Dei Tos, Andrea Marrari, Paolo G Casali, Silvia Stacchiotti
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引用次数: 6

摘要

背景:孤立性纤维瘤(SFT)是一种罕见的软组织肉瘤,具有低转移潜能。在高级别/去分化的变体中观察到更高的转移率。最常见的远处扩散部位是肺和肝脏。骨受累通常被认为是疾病扩散的晚期。我们报告了一系列回顾性的SFT患者复发,首次转移事件为单一远端骨复发,没有其他器官受累的证据。病例描述:所有在本研究所观察到的伴有单一SFT远端骨转移的患者均被考虑为第一个远端事件,没有任何其他部位转移的证据。所有病例均经病理评估并经专家病理学家确认。回顾性分析了6例患者。4例患者的原发肿瘤来自脑膜,2例来自软组织。骨转移灶位于椎骨、髋关节、髋臼和肋骨。在所有病例中,骨复发是第一事件,有一名患者出现局部复发。从原发肿瘤到骨复发的中位时间为40个月(范围0-58)。2/6的骨转移患者接受放疗(RT)治疗,2/6的患者接受手术治疗,2/6的患者接受手术加RT治疗。在中位随访55个月(范围23-88)时,5/6的患者存活(2/5无疾病,3/5有多中心转移性疾病),1例死于疾病。2/6患者经骨转移治疗后未复发。结论:在一个相对罕见的组织学中,这个小的系列表明,孤立的,可能是晚期的骨转移是一种可能的情况,特别是在脑膜SFT中。值得注意的是,有SFT病史的患者的新骨病变应始终进行调查。独家局部治疗可能是一种选择,尽管需要收集这些系列来确定最佳治疗策略。
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Solitary fibrous tumour presenting with a single bone metastasis: report of six cases and literature review.

Background: Solitary fibrous tumour (SFT) is a rare soft tissue sarcoma with a low metastatic potential. A higher metastatic rate is observed in the high-grade/dedifferentiated variant. The most common expected site of distant spread are the lungs and the liver. Bone involvement is generally viewed as a late stage of disease spread. We report on a retrospective series of SFT patients relapsing with a single distant bone recurrence as first metastatic event, without evidence of other organ involvement.

Case presentation: All patients affected by a single distant bone metastasis from SFT as first distant event, without any evidence of other site of metastasis, observed at our Institution, were considered. Bone involvement from SFT was pathologically assessed in all cases and confirmed by expert pathologists. A total of six patients were retrospectively identified. Primary tumour arose from the meninges in four patients, from soft tissues in two. Bone metastases were located to the vertebrae, the hip, the acetabulum and the rib. In all cases, bone relapse was the first event, with one patient presenting a local relapse. Median time from the primary tumour and the evidence of bone relapse was 40 months (range 0-58). In 2/6 patients bone metastasis was treated with radiotherapy (RT), in 2/6 with surgery, in 2/6 with surgery plus RT. At a median follow-up of 55 months (range 23-88), 5/6 patients are alive (2/5 without disease, 3/5 with multicentric metastatic disease) and one is dead of disease. 2/6 patients did not relapse after the treatment of the bone metastasis.

Conclusions: This small series in a relatively rare histology suggests that isolated, possibly late, bone metastases are a plausible scenario, in particular in meningeal SFT. Notably, new bone lesions in a patient with a history of SFT should be always investigated. Exclusive local treatments may be an option, though collection of such series would be needed to define the best treatment strategy.

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期刊介绍: Clinical Sarcoma Research considers for publication articles related to research on sarcomas, including both soft tissue and bone. The journal publishes original articles and review articles on the diagnosis and treatment of sarcomas along with new insights in sarcoma research, which may be of immediate or future interest for diagnosis and treatment. The journal also considers negative results, especially those from studies on new agents, as it is vital for the medical community to learn whether new agents have been proven effective or ineffective within subtypes of sarcomas. The journal also aims to offer a forum for active discussion on topics of major interest for the sarcoma community, which may be related to both research results and methodological topics.
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