Type III pleuropulmonary blastoma in a <i>dicer1</i> germline mutation carrier: The management of residual lung cystic lesions

Fita, Ana; Llinares-Riestra, Esther; Doménech-Abellán, Ernesto; Bermúdez-Cortés, Mar; Galera, Ana María; Bas-Bernal, Agueda; Ruiz, Antonio José Ortiz

doi:10.1002/pbc.26438

Cited by 6 publications

(4 citation statements)

References 9 publications

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“…Type I PPB should be suspected when a cystic pulmonary lesion is evident (especially if there is a known familial history of DICER1 disease). [25][26][27] In this case, total tumor resection with a conservative intent should be planned (Level IV, Grade A). Therefore, a total pneumonectomy is not recommended for Type I PPB (Level IV, Grade E).…”

Section: Type I Ppb Surgerymentioning

confidence: 99%

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

Bisogno

Sarnacki

Stachowicz‐Stencel

et al. 2021

Pediatric Blood & Cancer

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Pleuropulmonary blastoma (PPB) is a rare cancer occurring mainly during early childhood and often associated with germline DICER1 mutations. It is classified by the macroscopic appearance into three interrelated clinico‐pathologic entities on a developmental continuum. Complete tumor resection is a main prognostic factor and can be performed at diagnosis or after neoadjuvant treatment that includes chemotherapy and in some cases radiotherapy. Optimal modalities of neo‐ or adjuvant treatments can be challenging taking into account potential long‐term toxicities in this young population. This paper presents the recommendations for diagnosis and treatment of children and adolescents with PPB elaborated by the European Cooperative Study Group for Pediatric Rare Tumors (EXPeRT) within the European Union‐funded project PARTNER (Paediatric Rare Tumours Network ‐ European Registry).

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Section: Type I Ppb Surgerymentioning

confidence: 99%

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

Bisogno

Sarnacki

Stachowicz‐Stencel

et al. 2021

Pediatric Blood & Cancer

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“…For example, in the present series, of the 26 type II/III PPB patients, 12 had large tumors invading vital structures that resulted in incomplete resection. Therefore, some specialists suggest that patients with unresectable tumors be initially treated with core needle biopsy and neoadjuvant chemotherapy to reduce the tumor size and make it resectable 7–10 . Following the protocol, the 5‐year overall survival was elevated from 44% to 68% 11 .…”

Section: Discussionmentioning

confidence: 99%

“…Therefore, some specialists suggest that patients with unresectable tumors be initially treated with core needle biopsy and neoadjuvant chemotherapy to reduce the tumor size and make it resectable. [7][8][9][10] Following the protocol, the 5-year overall survival was elevated from 44% to 68%. 11 However, a core needle biopsy cannot always yield the correct diagnosis because it may penetrate necrotic tissue.…”

Section: Outcomementioning

confidence: 99%

Treatment and prognosis of pleuropulmonary blastoma: A single‐center report of 31 cases

Wang

Zhuang

Liang

et al. 2022

Pediatric Pulmonology

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Objectives: Pleuropulmonary blastoma (PPB) is a very rare and highly aggressive neoplasm occurring in children, mostly under 6 years of age. We assessed the clinical characteristics, treatment modalities, treatment outcomes, and prognostic factors affecting survival in patients with PPB treated at our institution over a 10-year period to improve the prognosis.Methods: From November 2008 to November 2019, 31 children (21 boys and 10 girls) with a median age of 30 months (ranging, 22 days to 54 months) were treated at our institution. Here we describe the patient characteristics, treatment modalities, and treatment outcomes. The Kaplan-Meier method was used to estimate the progression-free survival (PFS) and overall survival (OS). Log-rank test was performed for comparison between groups.Results: Three children were lost to follow-up and two were dead due to postoperative complications. Of the 26 patients included in the follow-up, 16 PPB patients displayed tumor-free survival. The 5-year PFS and OS were 60.4% and 60.1% respectively. By stratified statistical analysis, the 5-year PFS and OS of type I PPB were 100%, while those of type III PPB were 43.7% and 43%, respectively. The 5-year PFS and OS of complete tumor resection were 76.5% and 75.6%, respectively, while those with tumor residue were 31.3%. The 5-year PFS and OS combined with chemotherapy were 62.2% and 61.6%, respectively, while those without chemotherapy were 0%.Conclusions: PPB is an aggressive neoplasm. The main factors related to the prognosis of PPB are pathological type, tumor resection degree, and postoperative adjuvant therapy.

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“…Therefore, some specialists suggest that patients with unresectable tumors be initially treated with core needle biopsy and neoadjuvant chemotherapy to reduce the tumor size and make it resectable. [7][8][9][10] Following the protocol, the five-year overall survival was elevated from 44% to 68%. 11 However, core needle biopsy cannot always yield the correct diagnosis because it may penetrate necrotic tissue.…”

Section: Discussionmentioning

confidence: 99%

Treatment and prognosis of pleuropulmonary blastoma: A Single-Center Report of 31 Cases

Wang

Lin

Liang

et al. 2021

Preprint

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Objectives: Pleuropulmonary blastoma (PPB) is a very rare, characteristic and highly aggressive neoplasm occurring in children, most under 6 years of age. We assessed the clinical characteristics, treatment modalities, treatment outcomes, and prognostic factors affecting survival in patients with PPB treated at our institution over a 10-year period to improve the prognosis of PPB. Methods: From November 2008 to November 2019, 31 children (21 boys and 10 girls) with a median age of 30 months (range, 22 days-54 months) were treated at our institution. Here, we describe the patient characteristics, treatment modalities and treatment outcomes. The Kaplan-Meier method was used to estimate the progression free survival probability (PFS) and overall survival (OS). Log-rank test was performed for comparison between groups. Results: 3 children were lost to follow-up and 2 were dead of postoperative complications. Of the 26 patients included in the follow-up, 16 PPB patients displayed tumor-free survival. The 6-month, 1-, 3-, and 5-year PFS were 80.8%, 69.0%, 60.4% and 60.4%, respectively. Accordingly, the 6-month, 1-, 3-, and 5-year OS were 84.6%, 72.7%, 60.1% and 60.1%, respectively. Sex, extent of surgery and chemotherapy/irradiation appeared to affect the survival, while age and pathology type appeared not to do. Conclusions: PPB is an aggressive neoplasm. To improve the prognosis of PPB, we should promote radical resection and improve the auxiliary treatment measures.

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Type III pleuropulmonary blastoma in a dicer1 germline mutation carrier: The management of residual lung cystic lesions

Cited by 6 publications

References 9 publications

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

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

Treatment and prognosis of pleuropulmonary blastoma: A single‐center report of 31 cases

Treatment and prognosis of pleuropulmonary blastoma: A Single-Center Report of 31 Cases

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