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Germ cell tumours of the testis: 10-year survival data from a tertiary care centre in India. 睾丸生殖细胞肿瘤:印度三级保健中心的10年生存数据。
IF 1.3 Q4 ONCOLOGY Pub Date : 2025-09-30 eCollection Date: 2025-01-01 DOI: 10.3332/ecancer.2025.2003
Nidhi Gupta, Kislay Dimri, Aanchal Arora, Awadhesh Kumar Pandey, Ashok Kumar Attri

Introduction: Germ cell testicular tumours are rare tumours. The incidence is the lowest in India, leading to limited availability of published Indian data. We report here the 10-year survival data for patients with this curable malignancy.

Material and methods: Record-based analysis was done for testicular germ cell tumours presenting to a tertiary care referral centre in North India during the period from 2010 to 2019. A total of 44 patients were identified who were evaluated for the demographics, treatment modalities and 10-year disease-free survival and overall survival (OS).

Results: Forty five percent of the patients had seminoma, while 55% had nonseminomas. Stages I-III disease was seen 41%, 23%, 36% and 67%, 17%, 17% of nonseminoma and seminoma patients, respectively. Within the seminomas, 89% patients were good risk and 11% were intermediate risk. Within the nonseminoma patients, 81% were good risk, 13% were intermediate risk and 6% were poor risk. At a median follow up of 73.4 months, 5- and 10-year OS were 88% and 77% for seminoma, while 87% and 78% for nonseminomas. The 5- and 10-year progression-free survival was 88% and 76% for seminoma patients, while 83% each for nonseminoma patients. On Cox proportional univariate analysis, none of the prognostic factors were found to be associated with OS.

Conclusion: Our patients presented with a lower metastatic disease burden, minimal violation of the scrotum and upfront orchiectomy in all patients. This resulted in better survival outcomes compared to previous Indian studies. However, the outcomes are inferior as compared to the West. Raising awareness about early diagnosis, treatment safety and curability may further save lives in these young males.

生殖细胞睾丸肿瘤是一种罕见的肿瘤。印度的发病率最低,导致印度公布数据的可用性有限。我们在此报告这种可治愈的恶性肿瘤患者的10年生存数据。材料和方法:对2010年至2019年期间在印度北部三级保健转诊中心就诊的睾丸生殖细胞肿瘤进行了基于记录的分析。总共确定了44例患者,对其进行了人口统计学、治疗方式、10年无病生存期和总生存期(OS)的评估。结果:45%的患者有精原细胞瘤,55%的患者有非精原细胞瘤。I-III期疾病在非精原细胞瘤和精原细胞瘤患者中分别占41%、23%、36%和67%、17%、17%。在精原细胞瘤中,89%的患者为良好风险,11%为中等风险。在非精原细胞瘤患者中,81%为良好风险,13%为中等风险,6%为低风险。在中位73.4个月的随访中,精原细胞瘤5年和10年的OS分别为88%和77%,而非精原细胞瘤为87%和78%。精原细胞瘤患者的5年和10年无进展生存率分别为88%和76%,而非精原细胞瘤患者的无进展生存率分别为83%。在Cox比例单变量分析中,没有发现预后因素与OS相关。结论:我们的患者表现出较低的转移性疾病负担,最小的阴囊侵犯和所有患者的前期睾丸切除术。与之前的印度研究相比,这导致了更好的生存结果。然而,与西方相比,结果是低劣的。提高对早期诊断、治疗安全性和可治愈性的认识可以进一步挽救这些年轻男性的生命。
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引用次数: 0
CAR-T in relapsed refractory high-grade glioma and glioblastoma - who, what, when and how? CAR-T治疗复发难治性高级别胶质瘤和胶质母细胞瘤-谁,什么,何时以及如何?
IF 1.3 Q4 ONCOLOGY Pub Date : 2025-09-30 eCollection Date: 2025-01-01 DOI: 10.3332/ecancer.2025.2001
Deevyashali Parekh, Ansy H Patel, Areeb Khan, Eloho Olojakpoke, Ashay Karpe, Zoya Peelay, Vijay Patil

Recurrent high-grade gliomas have a dismal prognosis. This review article aimed to explore and help answer the questions about which group of patients would benefit from chimeric antigen receptor therapy (CAR-T) cell therapy in this setting, the timing of intervention and the therapeutic efficacy. CAR-T cell therapy involves the extraction of T-cells from patients, genetic modification of these cells to express chimeric antigen receptors on their cell surface, which are selectively targeted towards tumour-expressed antigens and a procedure of immune-depletion followed by re-introducing these engineered CAR-T cells into the host via infusion. Gliomas, particularly glioblastoma, present unique challenges due to their immune-evasive nature, location within the central nervous system and antigenic heterogeneity. Thus, several potential antigenic targets are being explored for CAR-T cell therapy, including B7 homolog 3, Disiloganglioside, Eph-A2, Eph-A3, IL-13Ra2, HER2, EGFRvIII and Matrix metalloproteinase-2.

复发的高级别胶质瘤预后不佳。这篇综述文章旨在探讨和帮助回答在这种情况下,哪一组患者将受益于CAR-T细胞治疗、干预时间和治疗效果的问题。CAR-T细胞疗法包括从患者体内提取t细胞,对这些细胞进行基因修饰,使其在细胞表面表达嵌合抗原受体,这些嵌合抗原受体选择性地靶向肿瘤表达的抗原,然后通过输注将这些工程化的CAR-T细胞重新引入宿主体内。胶质瘤,特别是胶质母细胞瘤,由于其免疫逃避的性质、位于中枢神经系统和抗原异质性,呈现出独特的挑战。因此,CAR-T细胞治疗的几个潜在抗原靶点正在被探索,包括B7同源物3、二脂甘脂苷、Eph-A2、Eph-A3、IL-13Ra2、HER2、EGFRvIII和基质金属蛋白酶-2。
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引用次数: 0
An evidence-based approach to pericardial synovial sarcoma: a unique case report. 以证据为基础的方法治疗心包滑膜肉瘤:一个独特的病例报告。
IF 1.3 Q4 ONCOLOGY Pub Date : 2025-09-25 eCollection Date: 2025-01-01 DOI: 10.3332/ecancer.2025.2000
Elias Zonana-Schatz, Jenniffer Ann-Swain, Jenny Naomi Shiraishi-Piña, Marcos Cherem-Kibrit, José Rodrigo Espinosa

Synovial sarcoma is a rare and aggressive mesenchymal neoplasm characterised by the presence of the SS18-SSX fusion oncogene, resulting from the chromosomal translocation t(X;18)(p11.2;q11.2). Although these tumours typically arise in the extremities, they have also been documented in atypical locations such as the pericardium, underscoring their versatile and aggressive nature. This case involves a 46-year-old male who presented with a 2-month history of neck and precordial chest pain, ultimately diagnosed with a biphasic synovial sarcoma of the pericardium. Initial imaging studies, including magnetic resonance imaging and transthoracic echocardiogram, revealed a large encapsulated intrapericardial mass with hemorrhagic and thrombotic components, severe pericardial effusion and biventricular dysfunction. Histopathological examination confirmed the diagnosis, with immunohistochemistry findings positive for CKAE1/AE3, TLE-1, EMA, BCL-2 and CD99, along with a proliferation index of 40%. The chemotherapy regimen of ifosfamide, mesna and doxorubicin proved effective for this condition, leading to a significant reduction in tumour size and metabolic activity. However, due to disease recurrence and the presence of a KDM5A-positive marker, second-line therapy with trabectedin and pazopanib became necessary.

滑膜肉瘤是一种罕见的侵袭性间充质肿瘤,其特征是存在SS18-SSX融合癌基因,由染色体易位t引起(X;18)(p11.2;q11.2)。虽然这些肿瘤通常发生在四肢,但也有文献记载在非典型部位,如心包,强调了它们的多功能性和侵袭性。该病例涉及一名46岁男性,他有2个月的颈部和心前胸痛病史,最终诊断为心包双期滑膜肉瘤。最初的影像学检查,包括磁共振成像和经胸超声心动图,显示一个大的包被心包内有出血和血栓成分,严重的心包积液和双心室功能障碍。组织病理学检查证实了诊断,免疫组化结果为CKAE1/AE3、TLE-1、EMA、BCL-2和CD99阳性,增殖指数为40%。异环磷酰胺、mesna和阿霉素的化疗方案被证明对这种情况有效,导致肿瘤大小和代谢活性显著减少。然而,由于疾病复发和kdm5a阳性标记物的存在,需要用trabectedin和pazopanib进行二线治疗。
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引用次数: 0
Radiation therapy for cervical cancer in Uganda: a practice guideline. 乌干达宫颈癌放射治疗:实践指南。
IF 1.3 Q4 ONCOLOGY Pub Date : 2025-09-25 eCollection Date: 2025-01-01 DOI: 10.3332/ecancer.2025.1999
Solomon Kibudde, Awusi Kavuma, Bonny Abal, Moses Fredrick Katumba, Cissy Bangidde Namutale, Daniel Kanyike, Israel Luutu

Introduction: Radiation therapy (RT) is crucial in the management of cervical cancer, particularly in resource-limited settings where most patients present with advanced-stage disease. Advances in external beam radiotherapy (EBRT) planning and delivery techniques, brachytherapy (BT) and systemic therapy necessitate context-adapted guidelines to standardise care. We developed a clinical practice guideline to improve and to harmonise the multidisciplinary management of cervical cancer in Uganda.

Methods: A multidisciplinary team of Radiation Oncologists, Medical Physicists and Radiation therapists developed the guideline using a modified Delphi process. The guideline was externally reviewed by experts from the International Gynaecological Radiation Oncology Consortium.

Results: All newly diagnosed patients should undergo multisciplinary evaluation prior to radiotherapy. For early-stage cervical cancer, adjuvant radiotherapy after hysterectomy is indicated in women with intermediate-risk factors, while concurrent cisplatin-based chemoradiation is indicated in women with high-risk factors. The standard EBRT dose is 45-50 Gy; women with vaginal and/or parametrial disease should receive adjuvant vaginal vault BT to achieve an equivalent dose in 2 Gy (EQD2) of 60 Gy. For locally advanced cervical cancer, the standard of care is pelvic EBRT (45-50 Gy in 25 fractions) with concurrent cisplatin (40 mg/m2 weekly for 5-6 cycles) followed by image-guided adaptive brachytherapy delivering 24-28 Gy in 3-4 fractions to achieve an EQD2 of 80-85 Gy for small tumours and 85-90 Gy for large tumours. The overall treatment time should not exceed 56 days. In recurrent disease, management depends on the location of the recurrence and the interval since the previous RT. In metastatic disease, palliative RT is directed to symptomatic sites.

Conclusion: This clinical practice guideline offers evidence-informed, context-specific recommendations for the use of EBRT and BT in cervical cancer management in Uganda. It aims to harmonise the role of RT within multidisciplinary care pathways.

简介:放射治疗(RT)在宫颈癌的治疗中至关重要,特别是在资源有限的环境中,大多数患者出现晚期疾病。外射束放疗(EBRT)计划和递送技术、近距离放疗(BT)和全身治疗的进步需要适应具体情况的指南来标准化治疗。我们制定了临床实践指南,以改善和协调乌干达宫颈癌的多学科管理。方法:一个由放射肿瘤学家、医学物理学家和放射治疗师组成的多学科团队使用改进的德尔菲过程制定了指南。该指南由国际妇科放射肿瘤学联合会的专家进行了外部审查。结果:所有新诊断的患者在放疗前均应进行多学科评估。对于早期宫颈癌,有中度危险因素的妇女应行子宫切除术后辅助放疗,有高危因素的妇女应同时行顺铂类放化疗。EBRT的标准剂量为45-50 Gy;患有阴道和/或辅助疾病的妇女应接受辅助阴道穹窿BT,以达到2 Gy (EQD2) 60 Gy的等效剂量。对于局部晚期宫颈癌,标准的治疗方案是盆腔EBRT (45-50 Gy / 25次),同时使用顺铂(每周40 mg/m2, 5-6个周期),然后进行图像引导的适应性近距离放疗(24-28 Gy, 3-4次),以实现小肿瘤80-85 Gy的EQD2和大肿瘤85-90 Gy的EQD2。总的治疗时间不应超过56天。在复发性疾病中,治疗取决于复发的位置和自上次放疗以来的间隔时间。在转移性疾病中,姑息性放疗针对症状部位。结论:该临床实践指南为乌干达宫颈癌管理中EBRT和BT的使用提供了循证的、具体情况的建议。它旨在协调RT在多学科护理途径中的作用。
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引用次数: 0
Peripheral T-cell lymphoma of the lip: a rare case unveiling key insights into diagnosis and management. 唇周围t细胞淋巴瘤:一个罕见的病例揭示了诊断和管理的关键见解。
IF 1.3 Q4 ONCOLOGY Pub Date : 2025-09-25 eCollection Date: 2025-01-01 DOI: 10.3332/ecancer.2025.1998
Tooba Ali, Laraib Khan, Bilal Mazhar Qureshi, Asim Hafiz, Maria Tariq, Khurram Minhas, Nasir Ali, Ahmed Nadeem Abbasi

Peripheral T-cell lymphomas (PTCLs) represent a rare and heterogeneous group of lymphoproliferative disorders, accounting for about 10% of non-Hodgkin lymphomas. While PTCLs typically present at nodal sites, extra nodal involvement is uncommon, particularly in the oral cavity. This case report presents a rare instance of peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS), manifesting as a persistent lesion on the lower lip in a 70-year-old male patient. The patient underwent multiple biopsies, which required immunohistochemical staining to confirm the diagnosis. Initial histopathological examinations raised suspicion of a lymphoproliferative disorder, with further testing revealing a 4.5 × 1.5 × 2.8 cm Fluorodeoxyglucose (FDG)-avid lesion on positron emission tomography (PET)/CT. The lesion was confirmed to be PTCL-NOS, characterised by positive CD3 and CD56 markers and a high Ki-67 proliferative index. Treatment involved six cycles of CHOEP chemotherapy followed by consolidative radiation therapy, delivering a total dose of 36 Gy. The patient responded well to treatment, with an interim PET scan showing a complete metabolic response (Deauville score of 3). Follow-up visits confirmed the absence of residual or recurrent disease. A teleconsultation a 6-month post-radiotherapy, along with an examination by a plastic surgeon, also showed no signs of recurrence. This case highlights the diagnostic challenges associated with PTCL at rare non-nodal sites and underscores the importance of a multidisciplinary approach in managing such cases. The patient remains in remission, with ongoing surveillance recommended for up to 5 years to monitor for potential disease recurrence. Further studies and long-term follow-up of similar cases are warranted to better understand the behaviour and optimal treatment strategies for PTCLs in rare extra nodal locations.

外周t细胞淋巴瘤(PTCLs)是一种罕见且异质性的淋巴细胞增生性疾病,约占非霍奇金淋巴瘤的10%。虽然ptcl通常出现在淋巴结部位,但淋巴结外累及并不常见,特别是在口腔。本病例报告一例罕见的外周t细胞淋巴瘤,无其他特异性(PTCL-NOS),表现为70岁男性患者下唇的持续性病变。患者接受了多次活检,需要免疫组织化学染色来确认诊断。最初的组织病理学检查提出了淋巴增生性疾病的怀疑,进一步的检查显示正电子发射断层扫描(PET)/CT显示4.5 × 1.5 × 2.8 cm的氟脱氧葡萄糖(FDG)病变。病变证实为PTCL-NOS, CD3和CD56标记物阳性,Ki-67增殖指数高。治疗包括6个周期的CHOEP化疗,随后是巩固放疗,总剂量为36 Gy。患者对治疗反应良好,中期PET扫描显示完全代谢反应(多维尔评分3分)。随访证实没有残留或复发的疾病。放射治疗后6个月的远程会诊以及整形外科医生的检查也没有显示复发的迹象。本病例强调了PTCL在罕见的非淋巴结部位的诊断挑战,并强调了多学科方法在管理此类病例中的重要性。患者仍处于缓解期,建议持续监测长达5年,以监测潜在的疾病复发。需要对类似病例进行进一步的研究和长期随访,以更好地了解罕见的额外淋巴结ptcl的行为和最佳治疗策略。
{"title":"Peripheral T-cell lymphoma of the lip: a rare case unveiling key insights into diagnosis and management.","authors":"Tooba Ali, Laraib Khan, Bilal Mazhar Qureshi, Asim Hafiz, Maria Tariq, Khurram Minhas, Nasir Ali, Ahmed Nadeem Abbasi","doi":"10.3332/ecancer.2025.1998","DOIUrl":"10.3332/ecancer.2025.1998","url":null,"abstract":"<p><p>Peripheral T-cell lymphomas (PTCLs) represent a rare and heterogeneous group of lymphoproliferative disorders, accounting for about 10% of non-Hodgkin lymphomas. While PTCLs typically present at nodal sites, extra nodal involvement is uncommon, particularly in the oral cavity. This case report presents a rare instance of peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS), manifesting as a persistent lesion on the lower lip in a 70-year-old male patient. The patient underwent multiple biopsies, which required immunohistochemical staining to confirm the diagnosis. Initial histopathological examinations raised suspicion of a lymphoproliferative disorder, with further testing revealing a 4.5 × 1.5 × 2.8 cm Fluorodeoxyglucose (FDG)-avid lesion on positron emission tomography (PET)/CT. The lesion was confirmed to be PTCL-NOS, characterised by positive CD3 and CD56 markers and a high Ki-67 proliferative index. Treatment involved six cycles of CHOEP chemotherapy followed by consolidative radiation therapy, delivering a total dose of 36 Gy. The patient responded well to treatment, with an interim PET scan showing a complete metabolic response (Deauville score of 3). Follow-up visits confirmed the absence of residual or recurrent disease. A teleconsultation a 6-month post-radiotherapy, along with an examination by a plastic surgeon, also showed no signs of recurrence. This case highlights the diagnostic challenges associated with PTCL at rare non-nodal sites and underscores the importance of a multidisciplinary approach in managing such cases. The patient remains in remission, with ongoing surveillance recommended for up to 5 years to monitor for potential disease recurrence. Further studies and long-term follow-up of similar cases are warranted to better understand the behaviour and optimal treatment strategies for PTCLs in rare extra nodal locations.</p>","PeriodicalId":11460,"journal":{"name":"ecancermedicalscience","volume":"19 ","pages":"1998"},"PeriodicalIF":1.3,"publicationDate":"2025-09-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12812815/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146009112","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Incidence and trends of multiple myeloma (MM) in Brazil - 1988-2020. 1988-2020年巴西多发性骨髓瘤(MM)的发病率和趋势
IF 1.3 Q4 ONCOLOGY Pub Date : 2025-09-24 eCollection Date: 2025-01-01 DOI: 10.3332/ecancer.2025.1997
Dandara Menezes de Araujo Oliveira, Rossana Veronica Mendonza Lopez, Lady Paola Aristizabal Arboleda, Maria Paula Curado

Background: Multiple myeloma (MM) is a chronic hematological malignancy caused by a differentiated plasma cell disorder (Pawlyn, 2019). As a consequence of population aging, there has been an increase in incidence rates (Turesson et al 2018). In 2022, there were 187,744 new cases (Bray et al 2024). The incidence of MM in Brazil has not been estimated by the National Cancer Institute (INCA).

Objective: To analyse the incidence rates and trends of MM across states in Brazil from Population-Based Cancer Registries (PBCRs).

Methodology: Information was extracted from PBCR/INCA for the 1988-2020 period. Sociodemographic data were extracted from records of the Brazilian Institute of Geography and Statistics. Age-standardised incidence rates were calculated using the Segi global standard population. Trend analysis was performed using Join point Regression, version 4.7.0.0.

Results: The highest incidence rates of MM in males were observed in the cities of Natal (Rio Grande do Norte state) and Jaú (São Paulo state) at 3.55/100,000 and 2.9/100,000, respectively. In females, rates were highest in the cities of Natal (Rio Grande do Norte state) and Aracajú (Sergipe state) at 2.66/100,000 and 2.21/100,000, respectively. Trends showed an annual increase of 10.45% in Campinas for males and 9.04% for females. Median age at diagnosis in Brazil was 65 years for both sexes, while the North region had the lowest average age at 63.2 years, and the South region the highest at 68.0 years. Porto Alegre city (Rio Grande do Sul state) had the highest average of 70.0 years for females and 67.1 for males, while Roraima had the lowest at 61.2 years for females and 54.2 for males.

Conclusion: In Brazil, the average age of incidence varies by geographic region, but is higher among males. Incidence rates are highest in the Northeast and Southeast regions, whereas the greatest upward trends are in the Southeast and Midwest regions.

背景:多发性骨髓瘤(Multiple myeloma, MM)是一种由分化浆细胞疾病引起的慢性血液恶性肿瘤(Pawlyn, 2019)。由于人口老龄化,发病率有所增加(Turesson et al . 2018)。2022年,新增病例187,744例(Bray et al 2024)。巴西国家癌症研究所(INCA)尚未估计MM在巴西的发病率。目的:从基于人口的癌症登记处(PBCRs)分析巴西各州MM的发病率和趋势。方法:从PBCR/INCA中提取1988-2020年期间的信息。社会人口统计数据取自巴西地理与统计研究所的记录。使用Segi全球标准人群计算年龄标准化发病率。趋势分析使用4.7.0.0版本的连接点回归进行。结果:男性MM发病率最高的城市是纳塔尔市(里约热内卢Grande do Norte州)和Jaú市(s o Paulo州),分别为3.55/100,000和2.9/100,000。在女性中,纳塔尔市(北大州)和Aracajú市(塞尔吉佩州)的发病率最高,分别为2.66/10万和2.21/10万。趋势显示,坎皮纳斯男性的年增长率为10.45%,女性为9.04%。巴西男女确诊时的中位年龄为65岁,而北部地区的平均年龄最低,为63.2岁,南部地区最高,为68.0岁。阿雷格里港市(巴西南格兰德州)的平均年龄最高,女性为70.0岁,男性为67.1岁,而罗莱马的平均年龄最低,女性为61.2岁,男性为54.2岁。结论:在巴西,平均发病年龄因地理区域而异,但男性较高。发病率在东北部和东南部地区最高,而东南部和中西部地区的上升趋势最大。
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引用次数: 0
Exploring the enigma within: a retrospective study of primary cardiac sarcomas from a tertiary care centre. 探索谜团:从三级保健中心的原发性心脏肉瘤的回顾性研究。
IF 1.3 Q4 ONCOLOGY Pub Date : 2025-09-24 eCollection Date: 2025-01-01 DOI: 10.3332/ecancer.2025.1995
Swasthik Upadhya, Sameer Rastogi, Adarsh Barwad, Shamim Ahmed Shamim, Dikhra Khan, Sudheer Arava, Vineeta Ojha, Akshit Kumar, Ganesan Karthikeyan, Akshya Kumar Bisoi

Background: Primary cardiac sarcomas are exceedingly rare tumours associated with a poor prognosis due to delayed diagnosis, advanced presentation and limited known chemotherapy efficacy. While surgical excision is the preferred treatment, it is often not feasible. The role of systemic therapy remains uncertain.

Methods: We analysed the medical records of patients diagnosed with primary cardiac sarcoma registered at a sarcoma clinic between January 2016 and July 2023. Clinicopathological and imaging data and treatment information were collected. Descriptive statistics were employed for clinicopathological characteristics, and Kaplan-Meier analysis was used for survival assessment.

Results: A total of 12 patients were identified with primary cardiac sarcoma, with a median age at diagnosis of 33 years (IQR 20.5; range: 17-53). At presentation, 66.7% had pericardial effusion requiring pericardiocentesis with or without pleuropericardial window. Half (6 of 12 patients) were misdiagnosed initially as either tubercular pericardial effusion or cardiac hydatid cyst. Upfront resection was done for 4 patients (33.3%), while 4 (33.3%) had locally advanced/unresectable disease and the remaining 4 (33.3%) presented with de-novo metastatic disease. Angiosarcoma constituted 50% (6 out of 12), all arising from the right atrium. Of the 12 patients included, 6 received a median two lines of therapy. Of the total 9 response assessments (both as first line and subsequent lines), 55.5% had an objective response rate with an 88.8% clinical benefit rate. The median progression-free survival for first-line systemic therapy was 5.4 months. The median overall survival for patients who received systemic therapy and the entire cohort were 19.2 and 5.1 months, respectively.

Conclusion: This study highlights the advanced presentation and poor outcomes in patients with cardiac sarcoma. Due to the rarity of the tumour, it is often misdiagnosed. Systemic chemotherapy could alleviate symptoms and prolong survival. However, sarcoma pathology is heterogenous and cannot be generalised.

Trial registration: As this is a retrospective observational study, no trial registration has been done.

背景:原发性心脏肉瘤是一种非常罕见的肿瘤,由于诊断延迟、表现较晚和已知化疗效果有限,预后较差。虽然手术切除是首选的治疗方法,但通常是不可行的。全身治疗的作用仍不确定。方法:我们分析了2016年1月至2023年7月间在一家肉瘤诊所登记的原发性心脏肉瘤患者的医疗记录。收集临床病理、影像学资料及治疗资料。临床病理特征采用描述性统计,生存评估采用Kaplan-Meier分析。结果:共有12例患者被确诊为原发性心脏肉瘤,诊断时中位年龄为33岁(IQR 20.5;范围:17-53)。在就诊时,66.7%有心包积液,需要有或没有胸膜心包窗的心包穿刺。一半(12例患者中的6例)最初被误诊为结核性心包积液或心脏包虫囊肿。4例患者(33.3%)进行了前期切除,4例(33.3%)为局部晚期/不可切除疾病,其余4例(33.3%)为新生转移性疾病。血管肉瘤占50%(6 / 12),均起源于右心房。在纳入的12例患者中,6例接受了中位数为2线的治疗。在总共9项疗效评估中(包括一线和后续一线),55.5%的患者客观有效率,88.8%的患者临床获益率。一线全身治疗的中位无进展生存期为5.4个月。接受全身治疗的患者和整个队列的中位总生存期分别为19.2个月和5.1个月。结论:本研究强调了心脏肉瘤患者的晚期表现和不良预后。由于肿瘤的罕见性,它经常被误诊。全身化疗可缓解症状,延长生存期。然而,肉瘤的病理是异质性的,不能一概而论。试验注册:由于这是一项回顾性观察性研究,没有进行试验注册。
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引用次数: 0
Targeting stromal components in pancreatic ductal adenocarcinoma: a review. 针对胰腺导管腺癌基质成分的研究进展。
IF 1.3 Q4 ONCOLOGY Pub Date : 2025-09-24 eCollection Date: 2025-01-01 DOI: 10.3332/ecancer.2025.1996
Hani Shihadeh, Ahmad Yousef, Ahmad Al-Leimon, Hussein Abu-Rumman, Laith Kreshan

Pancreatic ductal adenocarcinoma (PDAC) remains a leading cause of cancer-related mortality, largely due to a lack of highly safe and effective therapeutic options. A large proportion of the tumour's mass consists of a dense fibrous stroma, which could provide valuable therapeutic targets. This review elucidates the various possible stromal targets in PDAC, methods of targeting them and the outcomes of this targeting in in-vitro studies, studies on murine models of PDAC and clinical studies. While targeting some stromal components in PDAC yielded disappointing results in clinical studies, others have shown promise in multiple settings. More research efforts should be directed towards identifying additional stromal targets and evaluating their therapeutic potential. In addition, comprehensive clinical studies are essential to evaluate the safety and effectiveness of agents targeting stromal components of PDA of agents targeting stromal components of PDAC, both as monotherapies and in combination with standard surgical and pharmacological treatments for PDAC to improve patient's outcomes.

胰腺导管腺癌(PDAC)仍然是癌症相关死亡的主要原因,主要是由于缺乏高度安全和有效的治疗选择。肿瘤肿块的很大一部分由致密的纤维间质组成,这可能提供有价值的治疗靶点。本文综述了PDAC中各种可能的基质靶点、靶向方法及其在体外研究、PDAC小鼠模型研究和临床研究中的结果。虽然靶向PDAC中的一些基质成分在临床研究中取得了令人失望的结果,但其他成分在多种情况下显示出希望。更多的研究应致力于确定更多的基质靶点并评估其治疗潜力。此外,全面的临床研究对于评估靶向PDA基质成分的药物或靶向PDAC基质成分的药物的安全性和有效性至关重要,无论是单独治疗还是与标准的PDAC手术和药物治疗相结合,以改善患者的预后。
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引用次数: 0
Global initiative for childhood six-indexed cancers: how are we faring in Nigeria? 全球儿童六指数癌症倡议:我们在尼日利亚的进展如何?
IF 1.3 Q4 ONCOLOGY Pub Date : 2025-09-23 eCollection Date: 2025-01-01 DOI: 10.3332/ecancer.2025.1993
Motunrayo Oluwabukola Adekunle, Aisha Musa, Chioma Ginika, Chisom Nri-Ezedii, Uduak Offiong, Hauwa Yusuf, Peter Odion Ubuane, Adewunmi Oyesakin, Ijeoma Nnenna Diaku-Akinwumi, Adaorah Onyiaorah

Background: WHO's Global Initiative for Childhood Cancer (GICC) aims to increase global survival of childhood cancers to 60% by the year 2030 with a focus on six index cancers. However, there is no nationally representative epidemiologic data on these index cancers in Nigeria.

Aim: To describe the distribution, outcomes and determinants of GICC six-indexed cancer in Nigeria.

Methodology: A multi-centre ambi-directional cohort study of children was done in children <19 years diagnosed with any of acute lymphoblastic leukaemia (ALL), Wilms tumour (WT), retinoblastoma (RB), Hodgkin lymphoma (HL), Burkitt lymphoma (BL) or low-grade glioma (LGG). Seven centres in the six geopolitical zones of the country participated. A 2-year study with 18 months of retrospective data collection (January 2022-June 2023) and follow up of subjects was done for 6 months (July-December 2023).

Results: A total number of 213 subjects were enrolled and ALL (n = 72;33.8%), WT (n = 57; 26.8%), RB (n = 52; 24.4%), BL (n = 17; 8.0%), HL (n = 13; 6.1%) and LGG (n = 2; 0.9%) accounted for the disease pattern. Median age at diagnosis was 5 years (51.6%). Mortality rate was 32.4% and treatment abandonment occurred in 37.6% of subjects. Treatment-related mortalities (TRMs) were 37.7% with infection and haemorrhage the commonest specific causes of TRM (36.1% and 42.5%). Only 7/95 (7%) of subjects with WT and RB stage III and IV benefited from RT. The most common reasons for non-RT were lack of funds (29%), lack of access to RT (54%) and lack of physicians' referral (11%). Only 10 (4.3%) of subjects were enrolled in a health insurance scheme. Independent risk factor for mortality was advanced disease stage (p = <0.001). Amongst the mortalities, 36% died within the first 3 months of diagnosis.

Conclusion: Treatment abandonment, mortality and TRM are high in Nigeria. To attain the GICC goal, there is a need to educate physician on treatment protocol, ensure availability of supportive care, health insurance, RT and tackle late presentation.

背景:世卫组织全球儿童癌症倡议(GICC)旨在到2030年将全球儿童癌症存活率提高到60%,重点关注6种指标癌症。然而,尼日利亚没有关于这些指数癌症的具有全国代表性的流行病学数据。目的:描述尼日利亚GICC六指数癌症的分布、结果和决定因素。方法:在儿童中进行了一项多中心双向队列研究。结果:共纳入213名受试者,ALL (n = 72;33.8%)、WT (n = 57; 26.8%)、RB (n = 52; 24.4%)、BL (n = 17; 8.0%)、HL (n = 13; 6.1%)和LGG (n = 2; 0.9%)为疾病类型。诊断时中位年龄为5岁(51.6%)。死亡率为32.4%,治疗放弃率为37.6%。治疗相关死亡率(TRM)为37.7%,感染和出血是TRM最常见的具体原因(36.1%和42.5%)。只有7/95(7%)的WT和RB III期和IV期患者从RT中受益。非RT最常见的原因是缺乏资金(29%),无法获得RT(54%)和缺乏医生转诊(11%)。只有10人(4.3%)参加了健康保险计划。死亡的独立危险因素是疾病晚期(p =结论:尼日利亚的治疗放弃、死亡率和TRM很高。为了实现GICC的目标,需要对医生进行治疗方案教育,确保提供支持性护理、健康保险、RT和解决延迟就诊问题。
{"title":"Global initiative for childhood six-indexed cancers: how are we faring in Nigeria?","authors":"Motunrayo Oluwabukola Adekunle, Aisha Musa, Chioma Ginika, Chisom Nri-Ezedii, Uduak Offiong, Hauwa Yusuf, Peter Odion Ubuane, Adewunmi Oyesakin, Ijeoma Nnenna Diaku-Akinwumi, Adaorah Onyiaorah","doi":"10.3332/ecancer.2025.1993","DOIUrl":"10.3332/ecancer.2025.1993","url":null,"abstract":"<p><strong>Background: </strong>WHO's Global Initiative for Childhood Cancer (GICC) aims to increase global survival of childhood cancers to 60% by the year 2030 with a focus on six index cancers. However, there is no nationally representative epidemiologic data on these index cancers in Nigeria.</p><p><strong>Aim: </strong>To describe the distribution, outcomes and determinants of GICC six-indexed cancer in Nigeria.</p><p><strong>Methodology: </strong>A multi-centre ambi-directional cohort study of children was done in children <19 years diagnosed with any of acute lymphoblastic leukaemia (ALL), Wilms tumour (WT), retinoblastoma (RB), Hodgkin lymphoma (HL), Burkitt lymphoma (BL) or low-grade glioma (LGG). Seven centres in the six geopolitical zones of the country participated. A 2-year study with 18 months of retrospective data collection (January 2022-June 2023) and follow up of subjects was done for 6 months (July-December 2023).</p><p><strong>Results: </strong>A total number of 213 subjects were enrolled and ALL (<i>n</i> = 72;33.8%), WT (<i>n</i> = 57; 26.8%), RB (<i>n</i> = 52; 24.4%), BL (<i>n</i> = 17; 8.0%), HL (<i>n</i> = 13; 6.1%) and LGG (<i>n</i> = 2; 0.9%) accounted for the disease pattern. Median age at diagnosis was 5 years (51.6%). Mortality rate was 32.4% and treatment abandonment occurred in 37.6% of subjects. Treatment-related mortalities (TRMs) were 37.7% with infection and haemorrhage the commonest specific causes of TRM (36.1% and 42.5%). Only 7/95 (7%) of subjects with WT and RB stage III and IV benefited from RT. The most common reasons for non-RT were lack of funds (29%), lack of access to RT (54%) and lack of physicians' referral (11%). Only 10 (4.3%) of subjects were enrolled in a health insurance scheme. Independent risk factor for mortality was advanced disease stage (<i>p</i> = <0.001). Amongst the mortalities, 36% died within the first 3 months of diagnosis.</p><p><strong>Conclusion: </strong>Treatment abandonment, mortality and TRM are high in Nigeria. To attain the GICC goal, there is a need to educate physician on treatment protocol, ensure availability of supportive care, health insurance, RT and tackle late presentation.</p>","PeriodicalId":11460,"journal":{"name":"ecancermedicalscience","volume":"19 ","pages":"1993"},"PeriodicalIF":1.3,"publicationDate":"2025-09-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12812841/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146009389","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Is active surveillance a good treatment option for stage 1 seminoma in a developing nation? Long-term outcomes from the Indian subcontinent. 在发展中国家,主动监测是治疗1期精原细胞瘤的一个好的选择吗?印度次大陆的长期结果。
IF 1.3 Q4 ONCOLOGY Pub Date : 2025-09-23 eCollection Date: 2025-01-01 DOI: 10.3332/ecancer.2025.1994
Aditya Dhanawat, Debdeep Samaddar, Bhagyashri Jadhav, Atul Tiwari, Kunal Jobanputra, Arnav Tongaonkar, Minit Jalan Shah, Nandini Menon, Priyamvada Maitre, Mahendra Pal, Amandeep Arora, Aparna Ringe, Archi Agrawal, Santosh Menon, Gagan Prakash, Vedang Murthy, Vanita Noronha, Kumar Prabhash, Amit Joshi

Background: Stage 1 seminoma is treated with high inguinal orchiectomy (HIO) followed by either chemotherapy, radiation therapy (RT) or active surveillance (AS).

Methods: This was a retrospective analysis of a prospectively collected dataset of patients with seminoma treated at a comprehensive cancer care centre in India. Adolescent and adult males with stage 1 seminoma were included.

Results: A total of 114 patients were analysed. The median age was 39 years (IQR: 32-48 years). Stage IA was more frequently seen and 105 (92.1%) patients underwent unilateral HIO. Chemotherapy was offered to 66 (57.9%) patients. AS was offered to 32 (28.1%) patients while RT was offered to 16 (14%). Only 14 (43.8%) of the 32 patients on AS strictly adhered to the institutional follow-up guidelines for at least 2 years post treatment. Of the 114 patients, 9 (7.9%) patients had radiological relapse, while 4 (3.5%) of them were symptomatic at relapse. Bleomycin, etoposide and cisplatin were the most common regimen offered on relapse. The median follow-up of the cohort was 70.6 months (95% CI: 59.1-82 months). The mean relapse-free survival (RFS) was 107.7 months (95% CI: 102.5-112.8 months). The 1-, 2- and 5-year RFS were 97.3%, 95.5% and 92.4%, respectively. The mean overall survival (OS) was 114.9 months (95% CI: 113.2-116.6 months). The 2-, 5- and 8-year OS were 100%, 98.9% and 98.9%, respectively. There was no statistically significant benefit of 2 cycles over 1 cycle of carboplatin in terms of median RFS (96.5 versus 108.8 months, p = 0.260) or 5-year OS (95% versus 100%, p = 0.192). There was no statistically significant difference in RFS (p = 0.355) or OS (p = 0.684) based on treatment offered at baseline. There was no difference in survival between patients who strictly adhered to follow-up guidelines versus those who did not.

Conclusion: In a developing nation with constrained resources, AS remains a good treatment option for stage 1 seminoma with excellent long-term outcomes and freedom from the toxicities of chemotherapy.

背景:1期精原细胞瘤的治疗采用高腹股沟睾丸切除术(HIO),随后化疗,放疗(RT)或主动监测(AS)。方法:这是对印度一家综合癌症护理中心治疗的精原细胞瘤患者前瞻性收集数据集的回顾性分析。研究对象包括1期精原细胞瘤的青少年和成年男性。结果:共分析114例患者。中位年龄39岁(IQR: 32-48岁)。IA期多见,105例(92.1%)患者发生单侧HIO。66例(57.9%)患者接受化疗。32例(28.1%)患者接受AS治疗,16例(14%)患者接受RT治疗。32例AS患者中只有14例(43.8%)在治疗后至少2年内严格遵守机构随访指南。114例患者放射学复发9例(7.9%),复发时有症状4例(3.5%)。博莱霉素、依托泊苷和顺铂是复发时最常见的治疗方案。队列的中位随访时间为70.6个月(95% CI: 59.1-82个月)。平均无复发生存期(RFS)为107.7个月(95% CI: 102.5 ~ 112.8个月)。1、2、5年RFS分别为97.3%、95.5%、92.4%。平均总生存期(OS)为114.9个月(95% CI: 113.2 ~ 116.6个月)。2年、5年、8年生存率分别为100%、98.9%、98.9%。在中位RFS (96.5 vs 108.8个月,p = 0.260)或5年OS (95% vs 100%, p = 0.192)方面,2个周期比1个周期的卡铂没有统计学意义上的显著益处。基于基线治疗的RFS (p = 0.355)或OS (p = 0.684)无统计学差异。严格遵守随访指南的患者与没有严格遵守随访指南的患者之间的生存率没有差异。结论:在资源有限的发展中国家,AS仍然是1期精原细胞瘤的良好治疗选择,具有良好的长期预后和无化疗毒性。
{"title":"Is active surveillance a good treatment option for stage 1 seminoma in a developing nation? Long-term outcomes from the Indian subcontinent.","authors":"Aditya Dhanawat, Debdeep Samaddar, Bhagyashri Jadhav, Atul Tiwari, Kunal Jobanputra, Arnav Tongaonkar, Minit Jalan Shah, Nandini Menon, Priyamvada Maitre, Mahendra Pal, Amandeep Arora, Aparna Ringe, Archi Agrawal, Santosh Menon, Gagan Prakash, Vedang Murthy, Vanita Noronha, Kumar Prabhash, Amit Joshi","doi":"10.3332/ecancer.2025.1994","DOIUrl":"10.3332/ecancer.2025.1994","url":null,"abstract":"<p><strong>Background: </strong>Stage 1 seminoma is treated with high inguinal orchiectomy (HIO) followed by either chemotherapy, radiation therapy (RT) or active surveillance (AS).</p><p><strong>Methods: </strong>This was a retrospective analysis of a prospectively collected dataset of patients with seminoma treated at a comprehensive cancer care centre in India. Adolescent and adult males with stage 1 seminoma were included.</p><p><strong>Results: </strong>A total of 114 patients were analysed. The median age was 39 years (IQR: 32-48 years). Stage IA was more frequently seen and 105 (92.1%) patients underwent unilateral HIO. Chemotherapy was offered to 66 (57.9%) patients. AS was offered to 32 (28.1%) patients while RT was offered to 16 (14%). Only 14 (43.8%) of the 32 patients on AS strictly adhered to the institutional follow-up guidelines for at least 2 years post treatment. Of the 114 patients, 9 (7.9%) patients had radiological relapse, while 4 (3.5%) of them were symptomatic at relapse. Bleomycin, etoposide and cisplatin were the most common regimen offered on relapse. The median follow-up of the cohort was 70.6 months (95% CI: 59.1-82 months). The mean relapse-free survival (RFS) was 107.7 months (95% CI: 102.5-112.8 months). The 1-, 2- and 5-year RFS were 97.3%, 95.5% and 92.4%, respectively. The mean overall survival (OS) was 114.9 months (95% CI: 113.2-116.6 months). The 2-, 5- and 8-year OS were 100%, 98.9% and 98.9%, respectively. There was no statistically significant benefit of 2 cycles over 1 cycle of carboplatin in terms of median RFS (96.5 versus 108.8 months, <i>p</i> = 0.260) or 5-year OS (95% versus 100%, <i>p</i> = 0.192). There was no statistically significant difference in RFS (<i>p</i> = 0.355) or OS (<i>p</i> = 0.684) based on treatment offered at baseline. There was no difference in survival between patients who strictly adhered to follow-up guidelines versus those who did not.</p><p><strong>Conclusion: </strong>In a developing nation with constrained resources, AS remains a good treatment option for stage 1 seminoma with excellent long-term outcomes and freedom from the toxicities of chemotherapy.</p>","PeriodicalId":11460,"journal":{"name":"ecancermedicalscience","volume":"19 ","pages":"1994"},"PeriodicalIF":1.3,"publicationDate":"2025-09-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12812842/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146009344","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
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