Pleuropulmonary blastoma

Baraniya, Jitendra; Desai, Sangeeta; Kane, Shubhada; Kurkure, Purna; Nair, Chandrika N.; Deshpande, Ramakant; Borges, Anita M.

doi:10.1002/(sici)1096-911x(199901)32:1<52::aid-mpo11>3.0.co;2-r

Cited by 19 publications

(5 citation statements)

References 21 publications

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“…For its development, the tumor may arise from the lung or it may be extrapulmonary, involving the mediastinum or parietal pleura. This has raised the possibility that PPB might originate from the splanchnopleural or somatopleural mesoderm (1,5). Our case showed predominant anterior mediastinal location with the partial involvement of lingular segment of the left lung.…”

Section: Discussionmentioning

confidence: 63%

Pleuropulmonary Blastoma in a Young Adult Presenting as a Ruptured Cystic Teratoma in Radiology

et al. 2003

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reported a distinctive intrathoracic pulmonary neoplasm of pleuropulmonary blastoma (PPB) in 11 children younger than 6 yrs. They suggested that PPB was a rare, distinct entity from classic pulmonary blastoma on the basis of its exclusive clinical presentation in childhood and its pathologic features of primitive blastema and sarcoma without a carcinomatous component. Since then, additional individual cases and small series of similar tumors in children have been reported under the diagnosis of PPB. Three pathological types of PPB have been described (2). Type I is exclusively cystic, type III is predominantly solid, and type II is an intermediate lesion with both solid and cystic elements. The cystic form is significant in that it may be misdiagnosed and managed as a benign cystic lesion. Generally the clinical course depends on the pathological type.We herein report a case of PPB occurring in a 21-yr-old male patient, which presented with the radiological features of mediastinal cystic teratoma. To our knowledge, the current case is the third description in the English literature of PPB diagnosed in an adult since the initial report by Hill et al. (3) in 1999. CASE REPORTA 21-yr-old male patient was referred from a hospital with a left chest pain and mild dyspnea of two weeks' duration. Laboratory findings at admission were unremarkable. Sputum culture showed no growth for acid-fast bacilli or fungal organisms. There was no history of tumors in his close relatives and his past medical history was unremarkable. A chest radiograph showed an apparent left pleural effusion, and a computed tomography (CT) scan revealed a large, multicystic tumor occupying the left lower hemithorax (Fig. 1). The radiologic impression was a ruptured cystic teratoma. On echocardiogram, a left cardiac border was deviated to the right side. A thoracentesis was performed and removed about 1 liter of non-bloody, exudative fluid with differential count of polymorphs 40% and lymphocytes 60%. Pleural fluid cytology showed the appearance of malignant small round cells with mesothelial reaction. On November 11, 2001, he underwent a left anterior thoracotomy for the removal of the tumor. On operative field, it appeared to originate from a broad pedicle attached to the infracardiac mediastinal soft tissue, and showed adhesion with the lingular segment of the left lung. The tumor was removed in large fragments, totally weighing about 140 g, together with the left lingular segment adhered.The resected tumor consisted of membranous cystic walls, associated with solid parts of fleshy, gray white masses including focal necroses and hemorrhages. The lingular segment of the left lung was submitted together and demonstrated a round, gray white, solid tumor nodule, about 2.1 cm in diameter. Histologically, the main tumor showed cystic and solid Pleuropulmonary Blastoma in a Young Adult Presenting as a Ruptured Cystic Teratoma in RadiologyPleuropulmonary blastoma (PPB) is a rare malignant dysontogenetic neoplasm primarily affecting children and is chara...

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Section: Discussionmentioning

confidence: 63%

Pleuropulmonary Blastoma in a Young Adult Presenting as a Ruptured Cystic Teratoma in Radiology

et al. 2003

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“…While there is essentially no evidence to link PPB with adult pulmonary blastoma, its relationship with other pediatric tumors is less clear [18]. In 25% of cases of PPB, a heritable predisposition to other dysplastic and neoplastic diseases has been observed in patients and their young relatives.…”

Section: Discussionmentioning

confidence: 99%

Use of Multicolor Spectral Karyotyping in Genetic Analysis of Pleuropulmonary Blastoma

et al. 2000

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Pleuropulmonary blastoma (PPB) is a rare, malignant intrathoracic pediatric tumor. It arises from the lung, pleura, or mediastinum and its pathogenesis and relationship to other pediatric solid tumors is not well understood. In this study, a case of PPB in a 3-year-old girl was studied using a combination of molecular genetic methods and cytogenetics. Molecular analysis of the commonly encountered fusion translocation gene products of pediatric solid tumors failed to detect a rearrangement. Cytogenetic analysis, supplemented by multicolor spectral karyotyping (SKY), identified an unbalanced translocation between chromosomes 1 and X, resulting in additional copies of 1q, an extra copy of Xq, and loss of part of Xp. In addition, trisomy 8 was detected. The identification of new chromosomal alterations and confirmation of previously reported ones in this rare neoplasm helps to improve our understanding of its pathogenesis and association with other pediatric tumors.

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“…Imaging studies usually show absence of chest wall invasion, presence of pleural fluid, and heterogeneous low attenuation [20]. Histologically, pleuropulmonary blastoma is also characterized by the arrangement of spindle cells in fasicles and foci of immature cartilage [21]. In some reported cases of pleuropulmonary blastoma the tumor cells were positive for vimentin, and S-100 was expressed in the chondroblastic areas.…”

Section: Discussionmentioning

confidence: 99%

Primary malignant peripheral nerve sheath tumor of the lung in a young child without neurofibromatosis type 1

Muwakkit

Rodríguez‐Galindo

Samra

et al. 2006

Pediatric Blood & Cancer

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Malignant peripheral nerve sheath tumors (MPNST) are uncommon in children and almost half of the cases occur in patients with neurofibromatosis 1 (NF1). We report a child with a primary MPNST of the lung without NF1. MPNST of the lung has similar clinical and radiologic characteristics as pleuropulmonary blastoma. We suggest to include MPNST of the lung in the differential diagnosis of intrapulmonary masses in children.

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Pleuropulmonary blastoma

Cited by 19 publications

References 21 publications

Pleuropulmonary Blastoma in a Young Adult Presenting as a Ruptured Cystic Teratoma in Radiology

Pleuropulmonary Blastoma in a Young Adult Presenting as a Ruptured Cystic Teratoma in Radiology

Use of Multicolor Spectral Karyotyping in Genetic Analysis of Pleuropulmonary Blastoma

Primary malignant peripheral nerve sheath tumor of the lung in a young child without neurofibromatosis type 1

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