Pancreatic metastasis of a pleuropulmonary blastoma in an adult

Walles, Thorsten; Teebken, Omke E.; Bartels, Michael; Fangmann, J.; Scheumann, G. F. W.; Pichlmaier, Maximilian; Klempnauer, J

doi:10.1023/a:1008339425495

Cited by 8 publications

(5 citation statements)

References 18 publications

(10 reference statements)

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“…Often presenting symptoms are fever, cough, hemoptysis, dyspnea and chest pain. The clinical picture of PPB in an adult resembles that in children [5]. In our case also, the patient presented with cough, dyspnea and fever.…”

Section: Discussionsupporting

confidence: 60%

Pleuropulmonary blastoma in an adult: A case report

Paliwal¹,

Gupta²,

Mullick³

et al. 2013

IJCRI

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Section: Discussionsupporting

confidence: 60%

Pleuropulmonary blastoma in an adult: A case report

Paliwal¹,

Gupta²,

Mullick³

et al. 2013

IJCRI

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“…In addition, a small number of cases have been identified on prenatal ultrasound . Two cases of type 2 PPB have been reported at ages 21 and 36 and a type 3 has been reported at age 18 . Imaging scenarios for type 1 PPB, employing alternating chest radiographs and chest CT, suggested decreased loss of life expectancy due to undetected disease .…”

Section: Discussionmentioning

confidence: 99%

DICER1 syndrome: Approach to testing and management at a large pediatric tertiary care center

Engelen

Villani

Wasserman

et al. 2017

Pediatric Blood & Cancer

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Background: To expand the current knowledge of DICER1 syndrome and to propose criteria for genetic testing based on experience at a pediatric tertiary care center.Procedure: This study involved a retrospective chart review of the 78 patients (47 probands and 31 family members) seen in the Cancer Genetics Program at The Hospital for Sick Children (SickKids) who were offered genetic testing for DICER1. Results:Of 47 probands offered genetic testing for DICER1, 46 pursued testing: 11 (23.9%) carried a pathogenic variant and one proband (2.1%) carried a missense variant of uncertain significance with evidence for pathogenicity. Thirty-one family members of variant-positive probands were offered testing: eight of the 25 who agreed to testing carried their familial variant (32.0%).Overall, 20 patients were identified to have a variant in DICER1 (eight males, 12 females). Of these, 13 (65.0%) presented with clinical manifestations associated with the syndrome. The most common lesions were pleuropulmonary blastoma (PPB) (five of 20 patients, 25.0%) and pineoblastoma (three of 20 patients, 15.0%). The average age at which individuals were diagnosed with a primary neoplasm was 5.2 years (range 0.8-20 years, median 3.0). Surveillance at our institution, with a median follow-up time of 23 months, has identified PPB in two asymptomatic individuals. These lesions were identified at early stages, thus potentially reducing treatment-related morbidity and mortality.

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“…3 It occurs in an exclusive manner in children and mostly involves local evolution with some cases presenting as metastasizing and aggressive tumors. 4 While PB in adults is more common than children's type of (PPB), PB occurs commonly in the middle-aged and is typically associated with symptoms and signs similar to lung cancer. It is divided into two groups depending on histopathological features: type (1) monophasic PB, which is called well-differentiated fetal adenocarcinoma (WDFA), and type (2), which is classic biphasic PB (CBPB) containing tissue of both fetal adenocarcinoma (type 2 is typically low grade) and primitive mesenchymal stroma.…”

Section: Introductionmentioning

confidence: 99%

A Pleuropulmonary Blastoma: A Rare Malignant Lung Disease in Children

Farhan

2023

IqNJM

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Pleuropulmonary blastoma (PPB) is Avery uncommon, mesenchymal malignant tumor in chest cavity that occurs mostly in children before preschool age (less than 6year old) . while it is the most popular primary pleural and lung tumor in children and adolescent malignancy. PPB has three macroscopically subtypes on a tumor have poor progression pathway start from cystic PPB (type I) to mixed cystic and solid (type II) and pure solid (type III). In the majority of PPB cases A gremlin mutation `need multimodal aggressive therapies including surgery and chemotherapy and/or radiotherapy. the study perform in Basra oncology center during eight years between 2012 -2019 have six cases of PPB male to female 1:1 most of cases less five years of old. most of cases were poor prognosis type II / III (mixed / pure solid) three cases type II and other three cases are type III and inspite of forceful treated by multimodal of treatment involved surgical ,chemotherapy and radiotherapy have poor destiny and the death rate 33.3%.

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Pancreatic metastasis of a pleuropulmonary blastoma in an adult

Cited by 8 publications

References 18 publications

Pleuropulmonary blastoma in an adult: A case report

Pleuropulmonary blastoma in an adult: A case report

DICER1 syndrome: Approach to testing and management at a large pediatric tertiary care center

A Pleuropulmonary Blastoma: A Rare Malignant Lung Disease in Children

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