Pleuropulmonary Blastoma: A Case Report and Review of the Literature

Khan, Amjad Ali; El-Borai, Ahmed Kamal; Alnoaiji, Mohammad S Mohammad

doi:10.1155/2014/509086

Cited by 21 publications

(31 citation statements)

References 4 publications

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“…There is no gender predilection and tumours of the right side are more common. [12] Type 1 PPB is predominantly cystic as in the case described above (Figs 5-8), type 2 mixed cystic and solid lesions and type 3 predominantly solid lesions. There is an increasing risk of malignant potential from types 1 to 3.…”

Section: Pleuropulmonary Blastomamentioning

confidence: 64%

“…[12] PPB type 1 lesions carry the most favourable prognosis with a long-term survival of 80%. Unfortunately, types 2 and 3 lesions are more common, are highly aggressive and carry a poorer prognosis (long-term survival of <50%).…”

Section: Pleuropulmonary Blastomamentioning

confidence: 99%

“…[13] The most common site of metastases is the brain followed by bone. [12] Radiologically, PPB can occur as a single cystic, a multicystic structure, a cyst with a polypoid mass, a mixed solid-cystic lesion or a completely solid lesion. [12] Unfortunately fine needle aspiration and cytology is inadequate to make a histological diagnosis and the entire lesion needs to be removed for the diagnosis.…”

Section: Pleuropulmonary Blastomamentioning

confidence: 99%

Section: Fig 4 Hande Section Of the Lesion In Figs 2 And 3 Demonstratmentioning

confidence: 99%

“…[12,13] The chemotherapeutic regimen suggested includes a combination of ifosfamide, vincristine, actinomycin D and doxorubicin. [12] There is much controversy in paediatric pulmonology about the indications to remove congenital pulmonary airway malformations (CPAMs). [15] Certainly in the case of symptomatic (causing compression with respiratory distress), infected CPAMs or hybrid lesions the consensus lies in removal of these lesions.…”

Section: Fig 4 Hande Section Of the Lesion In Figs 2 And 3 Demonstratmentioning

confidence: 99%

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Tumours of the chest in children: A review

Niekerk¹,

Parris²,

Campos³

et al. 2016

S Afr Res J

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Tumours of the chest in children constitute an array of pathology and clinical symptomatology. These tumours can be benign or malignant, cystic or solid, primary or as a result of secondary metastases. Collectively, tumours of the chest in children are very rare, the exact incidence of which is largely unknown globally. Non neoplastic lesions of the lung including bronchogenic cysts, sequestrations, congenital pulmonary airway malformations as well as infective and inflammatory disorders are 60 times more common than neoplastic causes.1A tumour of the chest is considerably difficult to diagnose since patients can be asymptomatic for many years before symptoms evolve. Even more so, the symptoms are non-specific and can suggest more common and less sinister pathology.Clinically patients present with a variety of symptoms that depend largely on the location of the tumour. Airway tumours can be symptomatic or can present with chronic cough, wheeze, haemoptysis, atelectasis or persistent pneumonia. Secondary malignant parenchymal tumours are likely to be symptomatic from the primary lesion. Anterior mediastinal tumours can cause compression of the large airways or superior vena caval structures. It stands to reason that the physician needs to have a very high index of suspicion when dealing with these non-specific signs and symptoms.This article provides an approach to tumours of chest and reviews the common aetiology in the different compartments of the chest. The article will focus on common tumours of the airway, lung parenchyma, mediastinum, cardiac and chest wall pathology.

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Section: Pleuropulmonary Blastomamentioning

confidence: 64%

Section: Pleuropulmonary Blastomamentioning

confidence: 99%

Section: Pleuropulmonary Blastomamentioning

confidence: 99%

Section: Fig 4 Hande Section Of the Lesion In Figs 2 And 3 Demonstratmentioning

confidence: 99%

Section: Fig 4 Hande Section Of the Lesion In Figs 2 And 3 Demonstratmentioning

confidence: 99%

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Tumours of the chest in children: A review

Niekerk¹,

Parris²,

Campos³

et al. 2016

S Afr Res J

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Intraventricular topotecan in the successful treatment of recurrent CNS pleuropulmonary blastoma

Shim

Reisner

Esiashvili

et al. 2021

Pediatric Blood & Cancer

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Pleuropulmonary blastoma (PPB) is a rare pediatric tumor of the pleura and pulmonary mesenchyme, associated with pathogenic germline DICER1 mutations. Although the most common site of metastasis is the central nervous system (CNS), patients with CNS metastasis have dismal outcome. We report a case of a patient presenting with type II PPB and intracranial and bone metastases. We describe a multimodal therapy approach and highlight the use of intraventricular topotecan for isolated CNS recurrence. In addition, a new pathogenic germline mutation heterozygous for the c.1234delT of DICER1 was identified. Patient remains in remission 3 years after recurrence.

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