Pleuropulmonary Blastoma and its association with other childhood neoplasms
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8542 Background: Pleuropulmonary Blastoma (PPB) is a rare childhood tumor of the chest wall and lung with three distinct pathology subtypes: type I—entirely cystic; type II—cystic and solid; type III—entirely solid. Poor outcomes have been observed in patients with types II and III PPB. Common sites of metastatic involvement are the brain and bones. In numerous cases, other dysplastic and/or neoplastic conditions occur in the PPB patient or in young, close relatives. Methods: The PPB Registry, established in 1988, collects diagnostic, treatment, and outcome data about this disease for patients worldwide. Results: Registry and literature cases reveal the following conditions associated with PPB in 30 families: Familial cases: -9 patients with relatives having childhood or young adult cancers -3 families with 2 PPB patients in each; an additional family with a child with PPB and another child with a brain sarcoma undistinguishable from PPB -6 patients with relatives having dysplasia in childhood or young adulthood Metachronous or synchronous conditions: -4 patients with additional cancers -5 patients with bilateral PPB -12 patients with additional dysplastic conditions Common associated dysplastic conditions are lung cysts and cystic nephroma. Other associated neoplastic conditions include germ-cell tumors, brain tumors, lymphoma and leukemia, sarcomas, histiocytosis, Wilms' tumor, thyroid tumors, neuroblastoma, and Sertoli-Leydig tumor. Conclusion: 19 of 82 registry patients (23%) with central PPB pathology review have a constitutional or familial association with other neoplasias/dysplasias. At least 3 PPB patients have relatives with germ cell tumors, an association that needs further exploration. Similarly, the preponderance of renal malformations/tumors implies a possible common genetic pathway. PPB is an extraordinary marker for familial childhood dysplasia and neoplasia which deserves intensive molecular study. No significant financial relationships to disclose.Keywords:
Pulmonary Blastoma
Disease registry
Abstract. Dercum's disease, or adiposis dolorosa, is a rare condition characterized by painful lipomas, usually on the extremities of obese postmenopausal women. At present, there are no treatments known to alter the progressive course of this often incapacitating disease. Surgical intervention, while not preventive, is useful in ameliorating local symptoms of pain. We report a characteristic 15‐year course of Dercum's disease.
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Pulmonary Blastoma
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Fetal lung interstitial tumor, a newly recognized lung lesion in infants, was first reported in 2010. Here, we report the first J apanese case of fetal lung interstitial tumor which was originally diagnosed as atypical congenital cystic adenomatoid malformation/congenital pulmonary airway malformation type 3. A 7‐day‐old girl was referred to our hospital with respiratory distress and a left lung mass and she subsequently underwent left lower lobectomy. The specimen showed a 5 cm solid mass with a fibrous capsule. Histological examination revealed immature airspaces and interstitium, containing bronchioles and cartilage. The epithelial and interstitial cells contained abundant glycogen granules. Immunohistochemistry showed nuclear/cytoplasmic expression of β‐catenin in the epithelial and interstitial cells. β‐catenin gene mutations and trisomy 8 were not detected, so a neoplastic origin could not be confirmed. The histological findings were partly consistent with normal fetal lung at the canalicular stage, pulmonary interstitial glycogenosis, and congenital cystic adenomatoid malformation/congenital pulmonary airway malformation type 3. In this report, we compare the above conditions and discuss the pathogenesis of fetal lung interstitial tumor.
Pulmonary Blastoma
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A case of blastomatous tumor with teratoid features is presented. The polypoid mass was observed in the left nasal cavity of a 72‐year‐old man. Histologically, the lesion was composed of neuroepithelial cells with blastomatous appearance, cystic squamous nests filled with keratin materials, many mucous glands, complex tubular and glandular structures with edematous fibroblastic stroma. Sinonasal neoplasms including teratoid components and immature neuroepithelium are exceedingly rare. We suggest that the term ‘immature teratoma’ is more suitable than blastoma or blastomatous tumor when there is no carcinomatous or sarcomatous component besides the immature neuroepithelium and teratoid elements.
Neuroepithelial cell
Pulmonary Blastoma
Immature teratoma
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8542 Background: Pleuropulmonary Blastoma (PPB) is a rare childhood tumor of the chest wall and lung with three distinct pathology subtypes: type I—entirely cystic; type II—cystic and solid; type III—entirely solid. Poor outcomes have been observed in patients with types II and III PPB. Common sites of metastatic involvement are the brain and bones. In numerous cases, other dysplastic and/or neoplastic conditions occur in the PPB patient or in young, close relatives. Methods: The PPB Registry, established in 1988, collects diagnostic, treatment, and outcome data about this disease for patients worldwide. Results: Registry and literature cases reveal the following conditions associated with PPB in 30 families: Familial cases: -9 patients with relatives having childhood or young adult cancers -3 families with 2 PPB patients in each; an additional family with a child with PPB and another child with a brain sarcoma undistinguishable from PPB -6 patients with relatives having dysplasia in childhood or young adulthood Metachronous or synchronous conditions: -4 patients with additional cancers -5 patients with bilateral PPB -12 patients with additional dysplastic conditions Common associated dysplastic conditions are lung cysts and cystic nephroma. Other associated neoplastic conditions include germ-cell tumors, brain tumors, lymphoma and leukemia, sarcomas, histiocytosis, Wilms' tumor, thyroid tumors, neuroblastoma, and Sertoli-Leydig tumor. Conclusion: 19 of 82 registry patients (23%) with central PPB pathology review have a constitutional or familial association with other neoplasias/dysplasias. At least 3 PPB patients have relatives with germ cell tumors, an association that needs further exploration. Similarly, the preponderance of renal malformations/tumors implies a possible common genetic pathway. PPB is an extraordinary marker for familial childhood dysplasia and neoplasia which deserves intensive molecular study. No significant financial relationships to disclose.
Pulmonary Blastoma
Disease registry
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Carcinosarcoma
Pulmonary Blastoma
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A tumor of the lung in a 35-year-old woman contained numerous complex, branching tubules resembling the developing fetal lung in its canalicular stage. Some of the epithelial cells within the tubules were argyrophilic, and electron microscopy demonstrated the dense-core neurosecretory-type cytoplasmic granules. These characteristics support an endodermal origin for this neoplasm. Morphologically the tubules are similar to the epithelial component seen in pulmonary blastoma, but the tumor lacks the sarcomatous areas integral to the concept of blastoma. The designation pulmonary endodermal tumor resembling fetal lung may better describe this unusual neoplasm.
Pulmonary Blastoma
Neoplasm
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Objective To investigate the clinicopathological features,the diagnosis and differential diagnosis of pulmonary blastoma(PB). Methods 8 cases of PB were analyzed by light microscopy,immunohistochemistry and their follow-up data.Results 8 cases aged from 5 to 63 years(mean 41.6).Most masses were located in the upper lobes of left lung or right lung,Most patients were assessed at stage T(2～3),N(2～3) and M(0).Average survival period was about 11 months.Histologically,these tumors included 6 cases of biphasic type PB with embryonic malignant glandular showing CK positive,and the spindle stromal component positive for vimentin,nodules of cartilage positive for S-100;1 case was apithelial type PB,It was characterized by structure with positive for NSE and AE1.The morula may be the primordium comopnent with adeno-epithelia and neuroepithelial differentiation in NB;1 case pleuropulmonary blastoma were composed of maligant mesenchymal embryonic stroma with non-neoplastic epithelium expressing pulmonary surface protein B.The tumor cells were negative for CgA,Syn,desmin,actin,CD34 and TTF-1 in all cases.Conclusion The pulmonary blastoma is a high-grade primary malignant tumor with poor-prognosis,and It should be distinguished from carcinosarcoma,pulmonary adenocarcinoma,metastatic endometrial cancer and embryonal rhabdomyosarcoma of lung.
Pulmonary Blastoma
Carcinosarcoma
Desmin
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