recurrent sarcoma

  • 文章类型: Case Reports
    脂肪肉瘤是一种起源于脂肪细胞的罕见软组织肿瘤。脂肪肉瘤的确切原因尚不清楚,症状因肿瘤的位置而异。一名49岁的男子出现在急诊室,抱怨上腹部疼痛辐射到背部和右上象限。横断面成像显示上腹部大肿块,最初被认为是十二指肠引起的胃肠道间质瘤(GIST)。患者接受了整块切除,并计划进行辅助化疗。随后,检查多个组织样本,最终诊断为去分化脂肪肉瘤。患者最终出现多次复发,并接受了再切除手术和三种不同的化疗方案。鉴于这种疾病的稀有性,没有标准化的治疗计划,强调需要更多的病例报告/系列和试验,以扩大我们对这种疾病的理解。
    Liposarcoma is a rare soft-tissue neoplasm originating from adipocytes. The exact cause of liposarcoma is unknown and symptoms vary depending on the tumor\'s location. A 49-year-old man presented to the emergency room complaining of epigastric pain radiating to the back and right upper quadrant. Cross-sectional imaging revealed a large upper abdominal mass that was thought to be a gastrointestinal stromal tumor (GIST) arising from the duodenum at first. The patient underwent en-bloc resection of the mass and was planned for adjuvant chemotherapy. Subsequently, multiple tissue samples were examined, leading to the final diagnosis of de-differentiated liposarcoma. The patient eventually developed multiple recurrences and was subjected to re-resection surgeries and three different chemotherapy regimens. Given the rarity of the disease, no standardized therapy plan is available, highlighting the need for more case reports/series and trials to broaden our understanding of this disease.
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  • 文章类型: Case Reports
    转子缺损的重建对重建外科医生提出了挑战。文献中描述了许多局部区域重建方案。这些包括基于旋股外侧动脉及其分支的皮瓣,例如张量筋膜,股外侧肌(VL),股前外侧(ALT)皮瓣。由于患者多次复发覆盖转子区域的肉瘤,因此本病例进一步使这一挑战复杂化。以前的手术切除,重建和放射治疗。本病例研究描述了一种在先前收获ALT的患者中收获VL瓣的方法。
    The reconstruction of trochanteric defects presents a challenge to the reconstructive surgeon. There have been a number of locoregional reconstructive options described in the literature. These include flaps based on the lateral circumflex femoral artery and its branches, such as the tensor fascia lata, vastus lateralis (VL), anterolateral thigh (ALT) flaps. The present case further complicated this challenge as the patient had multiple recurrences of a sarcoma overlying the trochanteric region, with previous surgical resections, reconstruction and radiotherapy. The present case study describes an approach to harvesting the VL flap in a patient with previously harvested ALT.
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  • 文章类型: Case Reports
    纤维肉瘤是源于恶性的肿瘤生长,成纤维细胞样间充质细胞。这种恶性肿瘤显示出增加的扩张和复发的趋势和转移的倾向,尤其是肺部。尽管它们很少,纤维肉瘤有可能在任何解剖位置出现。一名肿瘤科医生因报告的下颌不适而转诊了他们的病人,疼痛,和肿胀。活检显示纤维肉瘤,类似于根管起源的根尖周病变。及时的干预和不同但互补的医疗和牙科专业之间的合作确保患者可以享受延长的预期寿命,尽可能无症状。
    A fibrosarcoma is a neoplastic growth originating from malignant, fibroblast-like mesenchymal cells. This malignant tumor shows an increased tendency for expansion and recurrence and a propensity to metastasize, especially to the lungs. Despite their rarity, fibrosarcomas have the potential to manifest in any anatomical location. An oncologist referred their patient due to reported mandibular discomfort, ache, and swelling. The biopsy revealed a fibrosarcoma resembling a periapical lesion of endodontic origin. The timely intervention and the collaboration among different but complementary medical and dental specialties ensure that the patient may enjoy a prolonged life expectancy as symptom-free as possible.
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  • 文章类型: Journal Article
    肉瘤是由多种间充质组织引起的罕见肿瘤,在头颈部区域甚至更罕见,仅占5%肉瘤的1%。大约80%的头颈部肉瘤起源于软组织,而其余20%来自骨骼(Cormier和PollockinJClin54:94-109,2004)。我们的患者被诊断患有的头颈部常见的肉瘤变体之一是低级肌纤维母细胞肉瘤(LGMS)。即使不常见,这些也对头部和颈部区域特别是舌头有好感(Mentzel等人。在AmJSurgPathol22:1228-38,1998;Cai等人。在VirchowsArch463:827-36,2013;Meng等人。“中国医学杂志”120:363-9,2007;Demarosi等人。口服治疗中的口服治疗。108:248-54,2009)。根据2002年的WHO软组织肿瘤分类,LGMS被重新分类为一个独特的实体(Qiu等人。在OncolLett9:619-25,2015)。肿瘤切除是治疗的主要手段。在大肿瘤的情况下,切除和重建将是具有挑战性的。我们报告了一名15岁女孩的复发性LGMS,她的左颈部区域出现了巨大的软组织肿胀,这使得她的日常活动剧烈,使她无法走动,并执行了一系列治疗以让她重新站起来,健康和没有衰弱的肿瘤。
    Sarcomas are rare tumors arising from a variety of mesenchymal tissues which are even rarer in head and neck region amounting 1% only of the 5% of sarcomas. About 80% of head and neck sarcomas originate in soft tissue while the remaining 20% arise from bone (Cormier and Pollock in J Clin 54:94-109, 2004). One among the commonly presenting variants of sarcomas in head and neck that our patient was diagnosed with is Low grade myofibroblastic sarcoma (LGMS). These even though uncommon have a predilection for head and neck region particularly the tongue (Mentzel et al. in Am J Surg Pathol 22:1228-38, 1998; Cai et al. in Virchows Arch 463:827-36, 2013; Meng et al. in Chin Med J 120:363-9, 2007; Demarosi et al. in Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 108:248-54, 2009). LGMS was reclassified as a distinct entity by the WHO classification of soft tissue tumors in 2002 (Qiu et al. in Oncol Lett 9:619-25, 2015). Oncological resection is the mainstay of treatment. In case of bulky tumor, resection and reconstruction would be challenging. We report a recurrent LGMS of 15-year-old girl who presented with a ginormous soft tissue swelling in her left neck region which made her day-to-day activities strenuous and made her non ambulatory and the line of treatment executed in order to get her back on feet, healthy & free of the debilitating tumor.
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  • 文章类型: Journal Article
    背景:骨盆软组织肉瘤(STS)存在手术和肿瘤挑战。我们调查了接受盆腔肉瘤切除术的患者的预后。
    方法:回顾性分析了2014年至2021年在三级学术转诊中心接受STS手术切除的所有患者(n=172)。包括所有起源于或延伸至盆腔的原发性或复发性STS患者(n=29)。
    结果:该队列分为原发性盆腔肉瘤(n=18)和复发性盆腔肉瘤(rPS,n=11)。26例患者完成了R0/R1切除(89.6%)。术后并发症发生率为48.3%。主要并发症发生率为27.5%。手术后的中位随访时间为12.3个月(范围,0.6-60.3个月)。原发性盆腔肉瘤组的无病生存率优于rPS组(P=0.002)。然而,总生存率没有显着差异,(P=0.52)。单变量和多变量分析确定rPS组(危险比8.68,P=0.006)和切除边缘(危险比6.29,P=0.004)与无病生存率独立相关。
    结论:我们已经证明实现R0/R1切除是可行的。肿瘤结果对原发性肿瘤有利,而复发性肿瘤表现出早期复发。考虑切除复发性骨盆STS应进行仔细的多学科评估。
    Soft tissue sarcomas (STS) of the pelvis present a surgical and oncological challenge. We investigated the outcomes of patients undergoing resection of pelvic sarcomas.
    A retrospective analysis of all patients who underwent surgical resection for STS between 2014 and 2021 at a tertiary academic referral center (n = 172). Included all patients with primary or recurrent STS which originated or extended to the pelvic cavity (n = 29).
    The cohort was divided into primary pelvic sarcomas (n = 18) and recurrent pelvic sarcomas (rPS, n = 11). Complete R0/R1 resection was achieved in 26 patients (89.6%). The postoperative complication rate was 48.3%. The rate of major complications was 27.5%. The median time of follow-up from surgery was 12.3 months (range, 0.6-60.3 months). Disease-free survival was superior in the primary pelvic sarcomas group compared to the rPS group (P = 0.002). However, there was no significant difference in overall survival, (P = 0.52). Univariant and multivariant analyses identified rPS group (Hazard Ratio 8.68, P = 0.006) and resection margins (Hazard Ratio 6.29, P = 0.004) to be independently associated with disease-free survival.
    We have demonstrated that achieving R0/R1 resection is feasible. Oncological outcomes are favorable for primary tumors, whereas recurrent tumors exhibit early recurrences. Consideration of resection of recurrent pelvic STS should involve a careful multidisciplinary evaluation.
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  • 文章类型: Review
    复发性腹膜后肉瘤很少见,复发模式由组织学亚型决定。一系列患者特征和治疗概况,加上无数的复发表现和临床课程,使这种多样化的实体难以管理。尽管手术切除可以改善部分患者的生存率,肿瘤结局不如原发性腹膜后肉瘤.不可切除疾病的管理选择包括局部消融治疗,辐射和全身治疗,偶尔需要姑息性手术。疾病控制的尝试必须与潜在的发病率和对患者生活质量的影响相平衡。这篇综述旨在提供对复发性腹膜后肉瘤的当前理解的见解,并为治疗提供一些指导。
    Recurrent retroperitoneal sarcomas are rare, with patterns of recurrence determined by the histologic subtype. A range of patient characteristics and treatment profiles combined with a myriad of presentations and clinical courses of recurrences make this diverse entity challenging to manage. Although surgical resection improves survival in select patients, the oncological outcomes are inferior to that of primary retroperitoneal sarcomas. Management options for unresectable disease include local ablative therapy, radiation and systemic therapy, with palliative surgery indicated occasionally. Attempts at disease control must be balanced with potential morbidity and impact on the patient\'s quality of life. This review aims to offer insights into the current understanding of recurrent retroperitoneal sarcomas and provide some guidance on management.
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  • 文章类型: Journal Article
    以前治疗过的患者,复发性或转移性肉瘤在多行全身治疗中进展,其局部控制的选择可能有限.我们评估了四射方案姑息性质子治疗对复发性和/或转移性肉瘤患者不可切除疾病的疗效。从2014年到2018年,对28例复发或转移性肉瘤患者进行了40个总部位的姑息性质子RT治疗(每天两次14.8Gy/4)。结果包括毒性,接受进一步全身治疗的能力,和主观姑息反应。对局部无进展生存期(LPFS)和总生存期(OS)进行单变量分析。在总共40个网站中,25例(62.5%)接受≥3个周期,中位随访时间为12个月(IQR4-19)。最常见的组织学是GIST(9;22.5%)和平滑肌肉瘤(7;17.5%)。共有27个(67.5%)部位位于腹部或骨盆。17例(42.5%)治疗涉及同步全身治疗,13例(32.5%)患者在质子治疗后接受进一步全身治疗。总体主观姑息反应为70%。LPFS中位数为11个月,6个月LPFS为66.1%。在单变量分析中,接受四个周期的四射(HR0.06,p=0.02)和完成放射治疗后接受全身治疗(HR0.17,p=0.02)与改善的LPFS相关。观察到3级急性毒性。质子四射方案可作为先前治疗过的患者的可行替代方案,复发性或转移性肉瘤,整体治疗选择可能有限。
    Patients with previously treated, recurrent or metastatic sarcomas who have progressed on multiples lines of systemic therapy may have limited options for local control. We evaluated outcomes of palliative proton therapy with the quad shot regimen to unresectable disease for patients with recurrent and/or metastatic sarcoma. From 2014 to 2018, 28 patients with recurrent or metastatic sarcomas were treated to 40 total sites with palliative proton RT with quad shot (14.8 Gy/4 twice daily). Outcomes included toxicity, ability to receive further systemic therapy, and subjective palliative response. Univariate analysis was performed for local progression-free survival (LPFS) and overall survival (OS). Of the 40 total sites, 25 (62.5%) received ≥3 cycles with median follow up of 12 months (IQR 4-19). The most common histologies were GIST (9; 22.5%) and leiomyosarcoma (7; 17.5%). A total of 27 (67.5%) sites were located in the abdomen or pelvis. Seventeen (42.5%) treatments involved concurrent systemic therapy and 13 (32.5%) patients received further systemic therapy following proton therapy. Overall subjective palliative response was 70%. Median LPFS was 11 months and 6-month LPFS was 66.1%. On univariate analysis, receipt of four cycles of quad shot (HR 0.06, p = 0.02) and receipt of systemic therapy after completion of radiation therapy (HR 0.17, p = 0.02) were associated with improved LPFS. Three grade 3 acute toxicities were observed. The proton quad shot regimen serves as a feasible alternative for patients with previously treated, recurrent or metastatic sarcomas where overall treatment options may be limited.
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  • 文章类型: Journal Article
    对于患有原发性高风险骨和软组织肉瘤的选定患者,常规化疗可以对疾病的自然史产生有利影响。特别是,多药方案是治疗组织学最积极的患者不可或缺的一部分,包括尤因肉瘤,骨肉瘤,非多形性横纹肌肉瘤。适当选择高危患者,临床局部软组织肉瘤也可能受益于组织学定制的辅助或新辅助治疗.对于复发性疾病的患者,常规化疗通常是最合适的一线治疗;活性药物进行了详细讨论。目前正在开发几种新的有前途的细胞毒性化学治疗剂,包括阿霉素,TH-302和trabectedin。
    Conventional chemotherapy can have a favorable impact on the natural history of disease for selected patients with primary high-risk bone and soft-tissue sarcomas. In particular, multidrug regimens are integral to the care of patients with the most aggressive histologies, including Ewing sarcoma, osteosarcoma, and non-pleomorphic rhabdomyosarcoma. Appropriately selected patients with high-risk, clinically localized soft-tissue sarcomas may also benefit from histology-tailored adjuvant or neoadjuvant therapy. For patients with recurrent disease, conventional chemotherapy is frequently the most appropriate first-line therapy; active drugs are discussed at length. Several new promising cytotoxic chemotherapeutic agents are currently under development, including aldoxorubicin, TH-302, and trabectedin.
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  • 文章类型: Case Reports
    我们描述了我们对转移性肺动脉肉瘤患者的经历,但诊断后存活了7年。一名61岁的男子在切除双侧肺的转移性肿瘤后被诊断出患有肺动脉内膜肉瘤。肺动脉干延伸到双侧分支的原发性病变通过肿瘤动脉内膜切除术治疗,然后进行化疗。在肿瘤血管内膜切除术后4年发现复发性阻塞性肺动脉肉瘤之前,他又进行了两次肺转移切除术。包括肺动脉干在内的肿瘤整体切除,进行了瓣膜和室间隔,用无支架肺动脉瓣和马心包重建右心室流出道。三年后,他因转移性脑肿瘤手术后不久就死于这种疾病。肺动脉肉瘤预后不佳,但积极重复的手术干预可能会延长生存期.
    We describe our experience with a patient who had metastasized pulmonary artery sarcoma, but survived 7 years after diagnosis. A 61-year-old man was diagnosed with pulmonary artery intimal sarcoma after resection of metastatic tumours to the bilateral lungs. The primary lesion in the pulmonary artery trunk extending into the bilateral branches was treated by tumour endoarterectomy followed by chemotherapy. He underwent resections of lung metastases two more times before detection of recurrent obstructive pulmonary artery sarcoma 4 years after the tumour endoarterectomy. En bloc resection of the tumour including the pulmonary artery trunk, valve and interventricular septum was performed, and the right ventricular out flow tract was reconstructed with a stentless pulmonary valve and equine pericardium. He died of the disease soon after an operation for metastatic brain tumour 3 years later. Pulmonary artery sarcoma has a dismal prognosis, but aggressively repeated surgical interventions may lengthen survival.
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