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>3.3.co;2-i

Cited by 3 publications

(5 citation statements)

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“…For this reason, bronchial biopsy is diagnostic and must be performed (11). In three out of our MEC cases PPB is a rare malignant mesenchymal tumor found in children which is quite distinct from the pulmonary blastoma of adults (12). It consists of polyphenotypic mesenchymal elements and embryonic stroma without a neoplastic epithelial component in contrast to the pulmonary blastoma ( Fig.…”

Section: Resultsmentioning

confidence: 96%

Primary Neoplasms of the Lung in Children and Adolescents: 22 Cases from a Single Institute

Han

Ahn

et al. 2009

J Lung Cancer

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Purpose: Prim ary lung tum ors in children and adolescents are uncom m on. W e report here on the clinical presentations, the m ethods of treatm ent, the results and the outcom es of a series of children with these rare tum ors. Materials and Methods:We conducted a retrospective review on all the patients less than 19 years of age with prim ary lung tum ors and who were treated at Samsung Medical Center between 1995 and 2009. Twenty two cases of prim ary lung tumors were reviewed and the clinical-pathological inform ation concerning the tum or characteristics, the therapy and the follow-up was collected. Results: The average age of the 10 m ale patients and 12 fem ale patients was 11.5 years (range: 3 m onths to 18 years). Of the 22 prim ary lung tum ors, 18.2% (4/22) were benign, 36.4% (8/22) were of borderline m alignancy and 45.4% (10/22) were m alignant. The pathologic types were inflam m atory m yofibroblastic tum or (6/22), m ucoepiderm oid carcinom a (4), pleuropulmonary blastoma (2), adenocarcinom a (1), alveolar soft part sarcoma (1), m alignant lym phom a (1), leiom yom a (1), pulm onary leiom yom atous ham artom a (1), congenital pulm onary m yofibroblastic tum or (1), Langerhans' cell histiocytosis (1), neurilem m oma (1), sclerosing pneum ocytoma (so-called sclerosing hem angiom a) (1) and atypical carcinoid tum or (1). After surgery for the benign tumors, no postoperative recurrence was been observed during long-term follow-up. All the patients with the benign tum ors were alive with no evidence of disease. For the patients with the tum ors of borderline malignancy, one patient experienced recurrence, but there was no death from disease. For the group with m alignant tum ors, two patients died from untreatable m etastatic disease and 8 patients survived their disease on the long-term follow-up. Conclusion: The prim ary lung tum ors during childhood and adolescence had different characteristics com pared with their adult counterparts with respect to the incidence and prognosis. These facts m ust be considered when m aking the diagnosis and deciding on proper therapy. (J Lung Cancer 2009;8(2):103 110)

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Section: Resultsmentioning

confidence: 96%

Primary Neoplasms of the Lung in Children and Adolescents: 22 Cases from a Single Institute

Han

Ahn

et al. 2009

J Lung Cancer

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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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“…PPB is usually large, it often occupies the entire hemithorax and may involve both lung parenchyma and pleura, whereas adult PB lesions are usually small, solitary and peripheral. In contrast to adult blastomas, no epithelial differentiation is seen in PPB although glandular structures or mesothelial islands may be found entrapped in the mesenchymal stroma (1,2). Differential diagnosis of PPB includes other pulmonary sarcomas of childhood such as rhabdomyosarcoma and leiomyosarcoma.…”

Section: Discussionmentioning

confidence: 99%

“…However, only myogenous differentiation is present in these neoplasms, whereas PPB is characterised by a multidirectional differentiation. Nevertheless, some reported cases of pleuropulmonary rhabdomyosarcoma may indeed represent variants of PPB (1,2). PPB has been classified by Dehner et al in three subtypes according to histopathological features (3).…”

Section: Discussionmentioning

confidence: 99%

“…Pleuropulmonary blastoma (PPB) is an uncommon neoplasm which arises in the lung, mediastinum or pleura of children and is characterised by a primitive blastema and malignant mesenchymal stroma that may show multidirectional differentiation (2). Initially classified together with the ordinary pulmonary blastoma of adulthood, PPB was first recognised as a distinctive intrathoracic lung tumour of childhood by Manivel in 1988 (4).…”

Section: Introductionmentioning

confidence: 99%

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Pleuropulmonary Blastoma

Granata¹,

Gambini²,

Carlini³

et al. 2001

Eur J Pediatr Surg

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We report a rare case of pleuropulmonary blastoma arising in a 3-year-old boy and involving the middle and upper lobes of the right lung. Radical resection of the mass was achieved by a bilobectomy. Microscopic examination of the histologic sections showed the typical findings of blastemal and mesenchymal areas with focal zones of rhabdomyoblastic and liposarcomatous differentiation. Monthly cycles of chemotherapy consisting of ifosfamide, vincristine and actinomycin D were given for 10 months after surgical resection. Our patient is presently alive and disease-free two years after diagnosis. As complete tumour ablation is essential to prevent local recurrence and allow any chance of survival, the favourable outcome of our patient is probably due to the radical resection of the neoplasm.

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Pleuropulmonary blastoma

Cited by 3 publications

References 0 publications

Primary Neoplasms of the Lung in Children and Adolescents: 22 Cases from a Single Institute

Primary Neoplasms of the Lung in Children and Adolescents: 22 Cases from a Single Institute

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

Pleuropulmonary Blastoma

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