Tell:400-875-6700
治疗实例

当前位置:首页 > 治疗实例 > 肉瘤> 麻省医生治疗复发的纤维肉瘤

  • 麻省医生治疗复发的纤维肉瘤

    化名:佚名 年龄:50 病症:肉瘤
    就诊医院:哈佛大学医学院教学附属麻省总医院 返回上页

    麻省总医院的专家建议手术切除阳性切缘,并考虑采取辅助放疗来清除残余瘤。另外,专家还与患者讨论了辅助化疗改善临床结果的证据有限,建议根据患者的情况做出决定。

    患者男,50多岁,2012年1月份发现右大腿根部内侧肿物,术后诊断为(右大腿肿物)符合粘液纤维肉瘤,中分化。一年后在同一部位再次出现肿物复发,再次进行扩大切除术,病例诊断为:(右大腿)去分化粘液性纤维肉瘤,去分化成分为未分化多形性肉瘤(恶性纤维组织细胞瘤),肿瘤大小8.5*6*5.5cm。

    2013年该患者寻求美国哈佛大学医学院教学附属麻省总医院会诊,麻省总医院专家介绍,肉瘤有50多种亚型,一般麻省总医院病理学家都会对病理结果再次评估,诊断发生变化的情况并不少见。恶性纤维组织细胞瘤(MFH)被病理学家称为高级未分化多形性肉瘤(HGUPS)。美国每年约有2000例这种类型的肉瘤。该疾病一般发生于50多岁人,在腿、躯干或手臂。MFH没有特殊的区别特征,除了显微镜下表现为侵袭性肿瘤,中度。该肿瘤的原始细胞不明。

    麻省总医院回复原文如下:

    As you know there are over 50 subtypes of sarcoma. Thus, for patients seen at our hospitalwe nearly always review the tumor specimen with our pathologists. It is not infrequent thatthe diagnosis changes. MFH is now termed high-grade undifferentiated pleomorphicsarcoma (HGUPS) by pathologists. There are approximately 2,000 cases a year of this type ofsarcoma in the United States. It tends to affect people over 50 years of age in the leg, trunk,or arm. MFH does not have particular distinguishing characteristics, besides appearing as a

    relatively aggressive tumor by microscopic examination, with a modest degree ofarchitecture. The cell of origin of such a tumor is unclear. Sarcomas that express no evidenceof architecture, looking to be sheets and sheets of aggressive looking sarcoma cells, are oftentimes termed "high grade sarcoma, not otherwise specified (NOS)". Thus without having thepathology evaluated here, the clinical description and pathological description does seem tofit with MFH. If your pathologist does not see much sarcoma it may be worth obtaining asecond opinion in or outside of China. Our pathologists do perform outside consultations so ifneeded the electronic consultation office can help arrange this if you like.

    对于该患者的情况,麻省总医院的专家建议手术切除,根据肿瘤大小、位置和手术切缘来决定是否进行放疗。术前或术后进行放疗取决于肿瘤的特征和外科医生的建议。鉴于患者初始表现出现的病变多灶性以及切缘不清晰,考虑放疗治疗残余瘤是合理的。

    同样的是,最近一次手术切缘是阳性,复发的可能性是非常高的。麻省总医院专家建议患者考虑手术再切除并向放射肿瘤学家咨询增加辅助放疗的可行性。治疗已知的切缘,剂量一般是60 Gy或66 Gy,但是需要向放射肿瘤科确认剂量。

    麻省总医院回复原文如下:

    For disease localized to a limb as in your patients initial presentation we would offer surgicalresection with or without radiation depending on the size, location and surgical margins. Theradiation is given before or after surgery depending on the features of the tumor and thesurgeon’s preference. At your patient’s initial presentation, given the multifocal nature andunclear margins, it would have been reasonable to consider radiation to treat any residualdisease. Likewise, given the positive margin of the most recent resection the likelihood ofrelapse is very high. I would have the patient consider repeat surgical resection and see aradiation oncologist to obtain an opinion about the feasibility of adding adjuvant radiation.Typically this would be 60 Gy or 66 Gy if treating a known margin but please confirm thedosing with radiation oncology.

    关于辅助化疗,有很多争议。仅仅根据肿瘤大小,该患者发生远处复发的可能性大于50%。不幸的是,有多个研究(>20)在切除的软组织肉瘤中评估了辅助治疗,而且,辅助化疗数据仍然有争议。2008年meta分析(Pervaiz et al Cancer 2008)提示联合异环磷酰胺(ifosfamide)和多柔比星(adriamycin)可以获得一定疗效,但是欧洲癌症治疗研究组织(EORTC)进行的大规模随机研究显示,两个对照组之间在生存期和复发方面没有差异。所以,鉴于该领域的不确定性以及化疗的疗效较小,一般是根据患者的情况做出决定。对于某些肉瘤(如滑膜肉瘤),化疗敏感性提高,所以疗效可能也提高。对于该患者,麻省总医院专家建议行再次切除阳性切缘和/或辅助放疗,然后与患者讨论辅助化疗改善结果的证据有限。在美国,至少前两年,专家会约患者每3个月行腿部影像复查和胸部CT复查。之后,则会慢慢地降低复查频率。

    编者注:以上表述仅限于截止于2013年前麻省总医院专家关于肉瘤化疗的见解,若想了解最新麻省总医院专家针对肉瘤的治疗方案,可申请麻省总医院会诊咨询。

    麻省总医院回复原文如下:

    Regarding adjuvant chemotherapy, this is an area of much controversy. Based on size aloneyour patient has greater than 50% chance of a distant recurrence. Unfortunately, there are anumber of studies (>20) that have evaluated adjuvant therapy in resected soft tissuesarcomas and, despite this, the adjuvant chemotherapy data still remains controversial. A2008 meta-analysis (Pervaiz et al Cancer 2008) suggested the combination ofifosfamide/adriamycin to have small benefit but in the largest randomized study from theEORTC there was no difference in survival or recurrence between the two groups. Thus, giventhe uncertainty in this area and marginal benefit of chemotherapy, typically decisions aremade on a patient-to-patient basis. For some histologies (e.g. synovial sarcoma) thereappears to be improved sensitivity to chemotherapy and thus perhaps improved benefit. For

    your patient, I would probably favor considering reresection of the positive margin and/oradjuvant radiation and then discuss with him the limited evidence of adjuvant chemotherapyimproving results.In the United States, at least for the first two years I would follow thepatient with serial imaging of the leg and CT of the chest every approximately 3 months.Thereafter we could consider spacing the scans out.

    关于肉瘤,麻省总医院有没有更新的药物或研究,麻省总医院专家是这样回答的:

    “在美国,帕唑帕尼,一种多重激酶抑制剂,最近被批准用于肉瘤。我会在后期使用这种疗法。我还会向我们的患者提供一些可行的临床试验。如果患者疾病发生转移并且在美国,我会建议参加SARC研究:将多柔比星与多柔比星加TH-302进行比较。”

    以上就是2013年时麻省总医院的专家关于肉瘤治疗的一些建议,如果患者想了解最新麻省总医院针对肉瘤的个性化治疗方案和效果,可以申请麻省总医院做一次会诊咨询。