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.
{"title":"Germ cell tumours of the testis: 10-year survival data from a tertiary care centre in India.","authors":"Nidhi Gupta, Kislay Dimri, Aanchal Arora, Awadhesh Kumar Pandey, Ashok Kumar Attri","doi":"10.3332/ecancer.2025.2003","DOIUrl":"10.3332/ecancer.2025.2003","url":null,"abstract":"<p><strong>Introduction: </strong>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.</p><p><strong>Material and methods: </strong>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).</p><p><strong>Results: </strong>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.</p><p><strong>Conclusion: </strong>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.</p>","PeriodicalId":11460,"journal":{"name":"ecancermedicalscience","volume":"19 ","pages":"2003"},"PeriodicalIF":1.3,"publicationDate":"2025-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12812845/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146009318","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}
Pub Date : 2025-09-30eCollection Date: 2025-01-01DOI: 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.
{"title":"CAR-T in relapsed refractory high-grade glioma and glioblastoma - who, what, when and how?","authors":"Deevyashali Parekh, Ansy H Patel, Areeb Khan, Eloho Olojakpoke, Ashay Karpe, Zoya Peelay, Vijay Patil","doi":"10.3332/ecancer.2025.2001","DOIUrl":"10.3332/ecancer.2025.2001","url":null,"abstract":"<p><p>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.</p>","PeriodicalId":11460,"journal":{"name":"ecancermedicalscience","volume":"19 ","pages":"2001"},"PeriodicalIF":1.3,"publicationDate":"2025-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12812813/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146009405","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}
Pub Date : 2025-09-25eCollection Date: 2025-01-01DOI: 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.
{"title":"An evidence-based approach to pericardial synovial sarcoma: a unique case report.","authors":"Elias Zonana-Schatz, Jenniffer Ann-Swain, Jenny Naomi Shiraishi-Piña, Marcos Cherem-Kibrit, José Rodrigo Espinosa","doi":"10.3332/ecancer.2025.2000","DOIUrl":"10.3332/ecancer.2025.2000","url":null,"abstract":"<p><p>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.</p>","PeriodicalId":11460,"journal":{"name":"ecancermedicalscience","volume":"19 ","pages":"2000"},"PeriodicalIF":1.3,"publicationDate":"2025-09-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12812826/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146009184","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}
Pub Date : 2025-09-25eCollection Date: 2025-01-01DOI: 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.
{"title":"Radiation therapy for cervical cancer in Uganda: a practice guideline.","authors":"Solomon Kibudde, Awusi Kavuma, Bonny Abal, Moses Fredrick Katumba, Cissy Bangidde Namutale, Daniel Kanyike, Israel Luutu","doi":"10.3332/ecancer.2025.1999","DOIUrl":"10.3332/ecancer.2025.1999","url":null,"abstract":"<p><strong>Introduction: </strong>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.</p><p><strong>Methods: </strong>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.</p><p><strong>Results: </strong>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/m<sup>2</sup> 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.</p><p><strong>Conclusion: </strong>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.</p>","PeriodicalId":11460,"journal":{"name":"ecancermedicalscience","volume":"19 ","pages":"1999"},"PeriodicalIF":1.3,"publicationDate":"2025-09-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12812846/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146009052","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}
Pub Date : 2025-09-25eCollection Date: 2025-01-01DOI: 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.
{"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}
Pub Date : 2025-09-24eCollection Date: 2025-01-01DOI: 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。
{"title":"Incidence and trends of multiple myeloma (MM) in Brazil - 1988-2020.","authors":"Dandara Menezes de Araujo Oliveira, Rossana Veronica Mendonza Lopez, Lady Paola Aristizabal Arboleda, Maria Paula Curado","doi":"10.3332/ecancer.2025.1997","DOIUrl":"10.3332/ecancer.2025.1997","url":null,"abstract":"<p><strong>Background: </strong>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 <i>et al</i> 2018). In 2022, there were 187,744 new cases (Bray <i>et al</i> 2024). The incidence of MM in Brazil has not been estimated by the National Cancer Institute (INCA).</p><p><strong>Objective: </strong>To analyse the incidence rates and trends of MM across states in Brazil from Population-Based Cancer Registries (PBCRs).</p><p><strong>Methodology: </strong>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.</p><p><strong>Results: </strong>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.</p><p><strong>Conclusion: </strong>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.</p>","PeriodicalId":11460,"journal":{"name":"ecancermedicalscience","volume":"19 ","pages":"1997"},"PeriodicalIF":1.3,"publicationDate":"2025-09-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12812833/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146009397","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}
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.
{"title":"Exploring the enigma within: a retrospective study of primary cardiac sarcomas from a tertiary care centre.","authors":"Swasthik Upadhya, Sameer Rastogi, Adarsh Barwad, Shamim Ahmed Shamim, Dikhra Khan, Sudheer Arava, Vineeta Ojha, Akshit Kumar, Ganesan Karthikeyan, Akshya Kumar Bisoi","doi":"10.3332/ecancer.2025.1995","DOIUrl":"10.3332/ecancer.2025.1995","url":null,"abstract":"<p><strong>Background: </strong>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.</p><p><strong>Methods: </strong>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.</p><p><strong>Results: </strong>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.</p><p><strong>Conclusion: </strong>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.</p><p><strong>Trial registration: </strong>As this is a retrospective observational study, no trial registration has been done.</p>","PeriodicalId":11460,"journal":{"name":"ecancermedicalscience","volume":"19 ","pages":"1995"},"PeriodicalIF":1.3,"publicationDate":"2025-09-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12812843/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146009364","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}
Pub Date : 2025-09-24eCollection Date: 2025-01-01DOI: 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.
{"title":"Targeting stromal components in pancreatic ductal adenocarcinoma: a review.","authors":"Hani Shihadeh, Ahmad Yousef, Ahmad Al-Leimon, Hussein Abu-Rumman, Laith Kreshan","doi":"10.3332/ecancer.2025.1996","DOIUrl":"10.3332/ecancer.2025.1996","url":null,"abstract":"<p><p>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 <i>in-vitro</i> 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.</p>","PeriodicalId":11460,"journal":{"name":"ecancermedicalscience","volume":"19 ","pages":"1996"},"PeriodicalIF":1.3,"publicationDate":"2025-09-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12812828/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146009060","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}
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.
{"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}
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}