Pleuropulmonary Blastoma: Outcomes of Patients Treated with Curative Intent Protocols

IF 6.5 1区医学 Q1 ONCOLOGY International Journal of Radiation Oncology Biology Physics Pub Date : 2025-03-01 Epub 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

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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.

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胸膜肺母细胞瘤：治疗目的方案治疗患者的结果

目的胸膜肺母细胞瘤（PPB）是一种罕见的侵袭性胸膜肺间质恶性肿瘤，多发生于儿童。我们评估了在塔塔纪念医院（TMH）接受治疗意向方案治疗的PPB患者的临床-病理-放射学特征、治疗反应和总体结果。方法对所有经组织学证实的PPB患者进行回顾性分析。结果2004年12月至2023年6月，在TMH进行了组织学证实的PPB患者26例（男性15例），年龄2 ~ 22岁（中位4岁）。有19例患者的临床放射学和治疗细节。该队列的平均肿瘤大小为11.45cm。10例（53%）患者出现胸腔积液，5例（26%）患者出现胸内淋巴结。所有病例均表现为局部广泛的II/III型疾病。17例患者接受治疗意向治疗，其中14例患者接受前期强化多药化疗。三名患者接受了前期手术。14例患者中有4例（29%）进展为诱导化疗，因此没有接受局部治疗。新辅助化疗后，9例患者中有7例（64%）患者行手术局部治疗，6例（38%）患者行辅助放疗，2例患者行彻底放疗。在最后一次随访时，17例患者中只有5例（29%）存活且无疾病。所有接受化疗且病情进展且未接受局部治疗的患者均死于该疾病。局部治疗12例，局部复发死亡4例。整个队列的2年和3年总生存率分别为47%和35%。结论ppb与预后不良相关。对诱导化疗有反应并接受局部治疗的患者生存率较高。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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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.