Pleuropulmonary blastoma in an adolescent

Bownes, Laura V.; Hutchins, Sara C.; Cardenas, Agustin M.; Kelly, David R.; Beierle, Elizabeth A.

doi:10.1016/j.epsc.2020.101482

Cited by 7 publications

(20 citation statements)

References 5 publications

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“…On IHC, the hyperchromatic blue cells were positive for vimentin and occasional large cells showed immunoreactivity for Pan CK. Our immunohistochemical results also ruled out the diagnosis of this tumor [12,13]. ( 5) Some other rare tumors such as soft tissue clear cell sarcoma, extraosseous Ewing sarcoma, non-Hodgkin lymphoma, and malignant melanoma, etc, resembled acinar RMS, but immunohistochemical markers of those tumors such as HMB45, S-100, CD99, Fli-1, CD20, CD3 and Pax5 were all negative.…”

Section: Discussionmentioning

confidence: 60%

Clinicopathological Features And Prognosis of Primary Pulmonary Rhabdomyosarcoma In The Middle-Aged And Elderly: A Case Report And Literature Review

Wei¹,

Jin²,

Jiatai³

et al. 2021

Preprint

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Background: Primary pulmonary rhabdomyosarcoma（PPRMS）in the middle-aged and elderly is a very rare event with only a few cases published. To date, only thirty-five cases of PPRMS in the middle-aged and elderly have been published. However, the analyses on clinicopathological characteristics and prognosis of PPRMS in the middle-aged and elderly have not been performed.Case presentation: We report an additional case of PPRMS in the middle-aged and elderly.Conclusions: PPRMS in the middle-aged and elderly was a highly malignant soft tissue tumor with obvious gender characteristics and significant age distribution. Pleomorphic rhabdomyosarcoma (RMS) was the most common subtype, with poor prognosis and significantly prolonged survival time after surgical resection.

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Section: Discussionmentioning

confidence: 60%

Clinicopathological Features And Prognosis of Primary Pulmonary Rhabdomyosarcoma In The Middle-Aged And Elderly: A Case Report And Literature Review

Wei¹,

Jin²,

Jiatai³

et al. 2021

Preprint

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“…By contrast, most of the previously reported PPB cases among adolescents were types 1 and 2. 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.…”

Section: Discussionmentioning

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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“…PPB is the most common primary pediatric pulmonary neoplasm and in most cases it occurs before 4 years of age [6] . It has a rare occurrence beyond the first decade of life and only a few isolated cases of PPB have been reported in literature in adult population [7 , 8 , 9] .…”

Section: Discussionmentioning

confidence: 99%

Type II pleuropulmonary blastoma in a 3-years-old female with dyspnea: a case report and review of literature

Ferrara

Esposito

Rossi

et al. 2021

Radiology Case Reports

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Pleuropulmonary blastoma (PPB) is a rare but aggressive pediatric tumor originates from either lung or pleura. It was recently linked to the DICER I mutation as a part of predisposition syndrome for different type of tumor. It is characterized histologically by a primitive, variably mixed blastomatous and sarcomatous tissue. PPB is classified into four subtypes: cystic (type I and type Ir); cystic and solid (type II); solid (type III). PPB has no characteristic imaging findings. Integrated imaging can help to make a differential diagnosis and to recognize the subtypes in order to set up therapy. An early recognition and differentiation from congenital airway malformations and other benign cysts are very important. The treatment consists in a multimodal therapy including surgery and chemoterapy. We report a case of 3 years old female admitted at our hospital with fever, non productive cough and dyspnea, who was diagnosed with type II PPB.

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Pleuropulmonary blastoma in an adolescent

Cited by 7 publications

References 5 publications

Clinicopathological Features And Prognosis of Primary Pulmonary Rhabdomyosarcoma In The Middle-Aged And Elderly: A Case Report And Literature Review

Clinicopathological Features And Prognosis of Primary Pulmonary Rhabdomyosarcoma In The Middle-Aged And Elderly: A Case Report And Literature Review

Unusual thoracic tumor in a teenager: A rare occurrence

Type II pleuropulmonary blastoma in a 3-years-old female with dyspnea: a case report and review of literature

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