Pleuropulmonary blastoma in children and adolescents: The EXPeRT/PARTNER diagnostic and therapeutic recommendations

Bisogno, Gianni; Sarnacki, Sabine; Stachowicz‐Stencel, Teresa; Colin, Veronique Minard; Ferrari, Andrea; Godziński, Jan; Villars, Marion Gauthier; Bień, Ewa; Hameury, F.; Helfre, Sylvie; Schneider, Dominik T.; Réguerre, Yves; Almaraz, Ricardo López; Janić, Dragana; Cesen, Maja; Kolenová, Alexandra; Rascon, Jelena; Мартинова, Ката; Cosnarovici, Rodica; Pourtsidis, Apostolos; Ami, Tal Ben; Roganović, Jelena; Kościelniak, Ewa; Schultz, Kris Ann P.; Brecht, Ines B.; Orbach, Daniel

doi:10.1002/pbc.29045

Cited by 19 publications

(35 citation statements)

References 32 publications

(69 reference statements)

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“…There are three main strategies to treat patients with PPB: Children’s Oncology Group (COG)/International Pleuropulmonary Blastoma Registry (IPBR), Cooperative Weichteilsarkom Studiengruppe (CWS) and International Society of Pediatric Oncology (EXpERT) [ 3 , 8 – 11 ]. All these strategies reported similar survival results.…”

Section: Perioperative Managementmentioning

confidence: 99%

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International Society of Paediatric Surgical Oncology (IPSO) Surgical Practice Guidelines

Abib¹,

Chui²,

Cox³

et al. 2022

ecancer

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Most children with tumors will require one or more surgical interventions as part of the care and treatment, including making a diagnosis, obtaining adequate venous access, performing a surgical resection for solid tumors (with staging and reconstruction), performing procedures for cancer prevention and its late effects, and managing complications of treatment; all with the goal of improving survival and quality of life. It is important for surgeons to adhere to sound pediatric surgical oncology principles, as they are closely associated with improved local control and survival. Unfortunately, there is a significant disparity in survival rates in low and middle income countries, when compared to those from high income countries.The International Society of Paediatric Surgical Oncology (IPSO) is the leading organization that deals with pediatric surgical oncology worldwide. This organization allows experts in the field from around the globe to gather and address the surgical needs of children with cancer. IPSO has been invited to contribute surgical guidance as part of the World Health Organization Initiative for Childhood Cancer. One of our goals is to provide surgical guidance for different scenarios, including those experienced in High-(HICs) and Low-and Middle-Income Countries (LMICs). With this in mind, the following guidelines have been developed by authors from both HICs and LMICs. These have been further validated by experts with the aim of providing evidence-based information for surgeons who care for children with cancer.We hope that this initiative will benefit children worldwide in the best way possible.

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Section: Perioperative Managementmentioning

confidence: 99%

“…Some authors recommend to avoid leaving a chest tub in order to minimise the contamination of the pleura [ 8 ].…”

Section: Postoperative Considerationsmentioning

confidence: 99%

International Society of Paediatric Surgical Oncology (IPSO) Surgical Practice Guidelines

Abib¹,

Chui²,

Cox³

et al. 2022

ecancer

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“… 9 , 10 , 12 Since PPB is a rare tumor, the histology should be reviewed by pathologists with expertise in pediatric tumors, which was conducted in our case. 8 In general, type 2 and 3 PPBs carry a worse prognosis than type 1. Type 3 tumor, which was seen in our patient, has the poorest prognosis with a 5-year disease-free survival of 37% compared with 59% in type 2.…”

Section: Discussionmentioning

confidence: 99%

“…Radiotherapy is suggested in cases with residual viable tumors after chemotherapy and in second-look surgery. 8 …”

Section: Introductionmentioning

confidence: 99%

Unusual thoracic tumor in a teenager: A rare occurrence

Muhamad

Sayuti

Mokhtar

et al. 2022

Qatar Medical Journal

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Pleuropulmonary blastoma (PPB) is a rare malignant lung tumor in the pediatric population and occurs mainly in young children. Its clinical presentation is usually nonspecific. We report a rare occurrence of this tumor in a 15-year-old girl, who presented with symptoms mimicking respiratory tract infection and was nonresponsive to the initial treatment. Imaging investigations revealed a large solid lesion in the left hemithorax with a mass effect on the adjacent structures. Biopsy demonstrated primitive cells with blastematous appearances, and the stroma cells were positive for vimentin and desmin, consistent with PPB. Unfortunately, she died from neutropenic sepsis while undergoing chemotherapy. This report highlights the epidemiology of PPB, its imaging and histopathological features, overview of prognosis, and clinical management.

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“…At diagnosis, patients with PPB type II and III need aggressive treatment with surgery, chemotherapy (CHT) and in some cases, radiotherapy (RT). 1 According to recent data, the 5-year overall survival (OS) rates were 71% and 53% for PPB type II and III, respectively, [2][3][4] and the 5-year event-free survival (EFS) rates were 59% and 37%, respectively. [2][3][4] Therefore, a substantial part of children with PPB present treatment failure during or after treatment: progressive disease (PD) occurs in about 8% of cases and local (i.e.…”

Section: Introductionmentioning

confidence: 99%

Children with progressive and relapsed pleuropulmonary blastoma: A European collaborative analysis

Sparber‐Sauer

Tagarelli

Seitz

et al. 2021

Pediatric Blood & Cancer

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Background: Children with progressive (PD) or relapsed disease (RD) of pleuropulmonary blastoma (PPB) type II/III are known to have a very poor outcome. Methods: A retrospective review of children registered in national and European databases and trials (2000-2018) with diagnosis of PPB type II/III and PD or RD was performed. Results: A total of 35 patients with PPB were analysed: patients with PD (n = 9) and RD (n = 26). Patients experienced PD at the median age of 3.9 years [range, 0.5-17.8] despite surgery, chemotherapy (CHT, n = 9) and radiotherapy (RT, n = 1) with a median time to progression of 0.58 years [range, 0.02-1.27] from diagnosis. All of them died. Patients suffered from RD at the median age of 4.3 years [1.7-15.1], median delay to relapse 1.03 years [range, 0.03-2.95]. RD occurred locally (n = 12), combined (n = 1) and in metastatic sites (n = 13): central nervous system (n = 11) and unspecified site (n = 2). Patients were treated with salvage CHT (n = 20), surgery (n = 10) ± RT (n = 10).

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Pleuropulmonary blastoma in children and adolescents: The EXPeRT/PARTNER diagnostic and therapeutic recommendations

Cited by 19 publications

References 32 publications

International Society of Paediatric Surgical Oncology (IPSO) Surgical Practice Guidelines

International Society of Paediatric Surgical Oncology (IPSO) Surgical Practice Guidelines

Unusual thoracic tumor in a teenager: A rare occurrence

Children with progressive and relapsed pleuropulmonary blastoma: A European collaborative analysis

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