Pleuropulmonary Blastoma: Outcomes of Patients Treated with Curative Intent Protocols

IF 6.4 1区医学 Q1 ONCOLOGY International Journal of Radiation Oncology Biology Physics Pub Date : 2025-02-03 DOI:10.1016/j.ijrobp.2024.11.036

S. David , N. Khanna , J. Manjali , M. Prasad , G. Chinnaswamy , B. Parambil , P. Panjwani , M. Ramadwar , S. Shah , V. Patil , S. Qureshi , S. Laskar

{"title":"Pleuropulmonary Blastoma: Outcomes of Patients Treated with Curative Intent Protocols","authors":"S. David , N. Khanna , J. Manjali , M. Prasad , G. Chinnaswamy , B. Parambil , P. Panjwani , M. Ramadwar , S. Shah , V. Patil , S. Qureshi , S. Laskar","doi":"10.1016/j.ijrobp.2024.11.036","DOIUrl":null,"url":null,"abstract":"<div><h3>Objectives</h3><div>Pleuropulmonary blastoma (PPB) is a rare and aggressive type of intrathoracic malignancy arising from pleuropulmonary mesenchyme predominantly seen in childhood. We evaluated the clinico-patho-radiological profile, treatment response, and overall outcomes for patients with PPB treated with curative intent protocols at the Tata Memorial Hospital (TMH).</div></div><div><h3>Methods</h3><div>All patients with histologically confirmed PPB were retrospectively analyzed.</div></div><div><h3>Results</h3><div>Between December 2004 to June 2023, 26 patients (15 males) with histologically proven PPB in the age group of 2 to 22 years (Median 4 years) were evaluated at TMH. Clinico-radiological and treatment details were available for 19 patients. The mean tumor size in this cohort was 11.45cm. The presence of pleural effusion and intrathoracic nodes was observed in 10 (53%) and 5 (26%) patients, respectively. All of them presented with locally extensive and type II/III disease.</div><div>Curative intent treatment was offered to 17 patients and 14 of them received upfront intensive multi-agent chemotherapy. Three patients underwent upfront surgery. Four out of fourteen (29%) patients progressed on induction chemotherapy and hence, did not undergo local treatment. Post neoadjuvant chemotherapy, out of the 9 patients, local treatment in the form of surgery was offered to 7 (64%) patients followed by adjuvant radiotherapy to 6 patients (38%), whereas 2 patients received definitive radiotherapy.</div><div>At the time of last follow up only 5 out of 17 (29%) patients were alive and disease free. All patients who had progressive disease on chemotherapy and did not receive local treatment died of the disease. Of the 12 patients receiving local treatment, 4 died of local relapse. The 2-year and 3-year overall survival of the entire cohort were 47% and 35% respectively.</div></div><div><h3>Conclusion</h3><div>PPB is associated with dismal outcomes. Patients responding to induction chemotherapy and receiving local treatment have better survival.</div></div>","PeriodicalId":14215,"journal":{"name":"International Journal of Radiation Oncology Biology Physics","volume":"121 3","pages":"Page e9"},"PeriodicalIF":6.4000,"publicationDate":"2025-02-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"International Journal of Radiation Oncology Biology Physics","FirstCategoryId":"3","ListUrlMain":"https://www.sciencedirect.com/science/article/pii/S0360301624036101","RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q1","JCRName":"ONCOLOGY","Score":null,"Total":0}

引用次数: 0

Abstract

Objectives

Pleuropulmonary blastoma (PPB) is a rare and aggressive type of intrathoracic malignancy arising from pleuropulmonary mesenchyme predominantly seen in childhood. We evaluated the clinico-patho-radiological profile, treatment response, and overall outcomes for patients with PPB treated with curative intent protocols at the Tata Memorial Hospital (TMH).

Methods

All patients with histologically confirmed PPB were retrospectively analyzed.

Results

Between December 2004 to June 2023, 26 patients (15 males) with histologically proven PPB in the age group of 2 to 22 years (Median 4 years) were evaluated at TMH. Clinico-radiological and treatment details were available for 19 patients. The mean tumor size in this cohort was 11.45cm. The presence of pleural effusion and intrathoracic nodes was observed in 10 (53%) and 5 (26%) patients, respectively. All of them presented with locally extensive and type II/III disease.

Curative intent treatment was offered to 17 patients and 14 of them received upfront intensive multi-agent chemotherapy. Three patients underwent upfront surgery. Four out of fourteen (29%) patients progressed on induction chemotherapy and hence, did not undergo local treatment. Post neoadjuvant chemotherapy, out of the 9 patients, local treatment in the form of surgery was offered to 7 (64%) patients followed by adjuvant radiotherapy to 6 patients (38%), whereas 2 patients received definitive radiotherapy.

At the time of last follow up only 5 out of 17 (29%) patients were alive and disease free. All patients who had progressive disease on chemotherapy and did not receive local treatment died of the disease. Of the 12 patients receiving local treatment, 4 died of local relapse. The 2-year and 3-year overall survival of the entire cohort were 47% and 35% respectively.

Conclusion

PPB is associated with dismal outcomes. Patients responding to induction chemotherapy and receiving local treatment have better survival.

查看原文

微信好友朋友圈 QQ好友复制链接

本刊更多论文

求助全文

约1分钟内获得全文去求助

来源期刊

International Journal of Radiation Oncology Biology Physics 医学-核医学

CiteScore

11.00

自引率

7.10%

发文量

2538

审稿时长

6.6 weeks

期刊介绍： International Journal of Radiation Oncology • Biology • Physics (IJROBP), known in the field as the Red Journal, publishes original laboratory and clinical investigations related to radiation oncology, radiation biology, medical physics, and both education and health policy as it relates to the field. This journal has a particular interest in original contributions of the following types: prospective clinical trials, outcomes research, and large database interrogation. In addition, it seeks reports of high-impact innovations in single or combined modality treatment, tumor sensitization, normal tissue protection (including both precision avoidance and pharmacologic means), brachytherapy, particle irradiation, and cancer imaging. Technical advances related to dosimetry and conformal radiation treatment planning are of interest, as are basic science studies investigating tumor physiology and the molecular biology underlying cancer and normal tissue radiation response.