A malignant neoplasm of the lung composed chiefly or entirely of immature undifferentiated cells (i.e., blast forms) with little or virtually no stroma. (From Stedman, 25th ed)
Tumors or cancer of the LUNG.
A sex cord-gonadal stromal tumor consists of LEYDIG CELLS; SERTOLI CELLS; and FIBROBLASTS in varying proportions and degree of differentiation. Most such tumors produce ANDROGENS in the Leydig cells, formerly known as androblastoma or arrhenoblastoma. Androblastomas occur in the TESTIS or the OVARY causing precocious masculinization in the males, and defeminization, or virilization (VIRILISM) in the females. In some cases, the Sertoli cells produce ESTROGENS.

Pancreatic metastasis of a pleuropulmonary blastoma in an adult. (1/32)

Pleuropulmonary blastoma (PPB) is a rare dysontogenetic tumor that usually develops in the first decade of life and has been recognized as a distinct clinico-pathological entity different from the ordinary pulmonary blastoma of adulthood. Since the tumor grows aggressively and tends to metastasize early, physicians have to be aware of late onset of symptoms and uncommon manifestations. We report a case of PPB in a young adult and its recurrence in the pancreas after primary surgical treatment and adjuvant chemotherapy. Keeping in mind the moderate prognosis of PPB in children, accurate assessment and treatment of PPB require a team approach of oncology, radiology and surgery to establish new therapeutic guidelines in the future.  (+info)

Well differentiated fetal adenocarcinoma of the lung in a 29 year old woman. (2/32)

This report describes a case of well differentiated fetal adenocarcinoma of the lung in a 29 year old female smoker. The histological pattern and immunohistochemical profile were consistent with well differentiated fetal adenocarcinoma and the patient made an uneventful postoperative recovery with no recurrence after 18 months. This neoplasm is a rare lung tumour that is composed of glycogen rich neoplastic glands and tubules that resembles fetal lung at 10 to 15 weeks of gestation. It is important to identify this rare variant of adenocarcinoma because it is a low grade malignancy with low associated mortality.  (+info)

Pleuropulmonary blastoma in a young adult presenting as a ruptured cystic teratoma in radiology. (3/32)

Pleuropulmonary blastoma (PPB) is a rare malignant dysontogenetic neoplasm primarily affecting children and is characterized histologically by a variably mixed blastematous and sarcomatous patterns. We herein report a very exceptional adult case of PPB. A 21-yr-old male patient presented with a left chest pain of two weeks' duration. A computed tomography scan revealed a large, multicystic tumor occupying the left lower hemithorax, leading to the impression of a ruptured mediastinal cystic teratoma. A thoracotomy for resection of the tumor was performed. On histologic examination, the tumor consisted of cystic walls and associated solid lesions which showed undifferentiated blastemal tissues with focal fibrosarcomatous and rhabdoid features. Immunohistochemically the tumor cells only showed diffuse strong positivity for vimentin. The histologic findings corresponded to a type II PPB. The authors suggest that PPB, especially of type I or II, should be included in the radiologic differential diagnosis of mediastinal cystic neoplasms in a young adult.  (+info)

Familial cystic nephroma in two siblings with pleuropulmonary blastoma. (4/32)

Cystic nephroma (CN) and pleuropulmonary blastoma (PPB) are rare tumors. In the cases presented here, a 13-month-old boy underwent right radical nephrectomy for CN. From the family history we learned that four years ago the patient's older sister underwent left radical nephrectomy for CN at a different center when she was 4 years old. A lung tumor was detected in the sister one year after nephrectomy. Biopsy from the lung tumor revealed PPB, and the sister died within one year after biopsy. To the knowledge of the authors, these cases represent the second reported familial occurrence of CN and the fourth of CN and PPB.  (+info)

Survival of biphasic pulmonary blastoma. (5/32)

Pulmonary blastoma is a rare malignant lung tumor with a poor prognosis. It is composed of immature mesenchymal and epithelial components that resemble fetal lung tissue. We aimed to share our treatment results in biphasic pulmonary blastoma. In Ataturk Chest Disease and Thoracic Surgery Center, five patients with biphasic pulmonary blastoma (four men, one woman, aged between 27 and 61-mean 39.4) were treated between 1987 and 2000 (0.3% of operated NSCC). Hemoptysis, cough, chest pain and dyspnea were the symptoms. Anemia and high erythrocyte sedimentation rate were determined in two patients. Radiological examinations revealed a mass in four patients and massive pleural effusion in one. None of the patients were diagnosed preoperatively and hence all patients underwent exploratory thoracotomy. Three lobectomy, one pneumonectomy and one wedge resection were performed. Histopathological examinations revealed biphasic pulmonary blastoma in all the patients. Pathological stagings were as follows: 1 patient in T1N0M0 and 1 patient in T2N0M0 (198 and 112 months survival, respectively), three patients in T2N1M0 (9,10,17 months survival). In follow up period, prostate carcinoma and rectum carcinoma were detected as second primary tumors in the patient in stage T2N0M0. In patients who have small size tumors without nodal involvement, long-term survival can be obtained with radical surgery; even in biphasic pulmonary blastomas. According to our limited experiences, N1 nodal involvement shows very poor prognosis.  (+info)

Morules and morule-like features associated with carcinomas in various organs: report with immunohistochemical and molecular studies. (6/32)

BACKGROUND: Morules have been reported in pulmonary blastoma (PB), well differentiated fetal adenocarcinoma of the lung (WDFA), and uterine endometrioid carcinoma (EC), and rarely in other carcinomas. beta Catenin gene mutation has been associated with morule formation. AIMS: To compare and clarify the cellular characteristics of morules in carcinomas in various organs and show that morules are distinct from epithelial cellular nodules. METHODS: Twenty tumours were studied: two PBs, three WDFAs, three papillary lung adenocarcinomas, 11 ECs, and one papillary thyroid carcinoma. Numerous epithelial cell, oncofetal, and neuropeptide antibodies were used for immunohistochemistry. beta Catenin gene mutation was investigated. RESULTS: Morules in PBs and ECs were uniform cell clusters distinct from squamous differentiation. All were immunonegative for epithelial cell and oncofetal antigens, but those in ECs were positive for neurone specific enolase gamma (NSEgamma). Synaptophysin, encephalin, and somatostatin were sporadically immunopositive in PB morules. Morules were not seen in the other carcinomas and WDFAs, although morule-like features closely resembling morules histopathologically were seen. These were positive for epithelial cell and oncofetal antigens, and showed squamous differentiation. Their nuclei were more atypical and slightly larger than those in morules. Morule-like features were seen in WDFAs. beta Catenin gene mutation was demonstrated in one EC and PB, and in two WDFAs. CONCLUSION: Morules were non-epithelial cell clusters showing neuronal differentiation. There were two types: endometrioid type, expressing NSEgamma, and blastoma type, expressing neuropeptides. In contrast, similar morule-like features were epithelial nodules. Although the number of cases was small, the presence of morules showed no clear prognostic correlations.  (+info)

Hidden chromosomal abnormalities in pleuropulmonary blastomas identified by multiplex FISH. (7/32)

BACKGROUND: Pleuropulmonary blastoma (PPB) is a rare childhood dysontogenetic intrathoracic neoplasm associated with an unfavourable clinical behaviour. CASES PRESENTATION: We report pathological and cytogenetic findings in two cases of PPB at initial diagnosis and recurrence. Both tumors were classified as type III pneumoblastoma and histological findings were similar at diagnosis and relapse. In both cases, conventional cytogenetic techniques revealed complex numerical and structural chromosomal abnormalities. Molecular cytogenetic analysis (interphase/metaphase FISH and multicolor FISH) identified accurately chromosomal aberrations. In one case, TP53 gene deletion was detected on metaphase FISH. To date, only few cytogenetic data have been published about PPB. CONCLUSION: The PPB genetic profile remains to be established and compared to others embryonal neoplasia. Our cytogenetic data are discussed reviewing cytogenetics PPBs published cases, illustrating the contribution of multicolor FISH in order to identify pathogenetically important recurrent aberrations in PPB.  (+info)

Type I pleuropulmonary blastoma: a report from the International Pleuropulmonary Blastoma Registry. (8/32)

PURPOSE: Type I pleuropulmonary blastoma (PPB) is a rare, cystic lung neoplasm in infants characterized by subtle malignant changes and a good prognosis. Recurrences after type I PPB are usually advanced type II or type III neoplasms with a poor prognosis. This article describes the first collection of type I PPB cases, analyzes outcome based on treatments of surgery or surgery plus chemotherapy, and presents type I PPB management recommendations. PATIENTS AND METHODS: Type I PPB cases from the International PPB Registry and literature were evaluated using standard statistical methods for outcomes based on age at diagnosis, sex, thoracic side, surgical extent, length of follow-up, constitutional/familial disease, pre-existing lung cysts, intrathoracic findings, and treatments (surgery or surgery and chemotherapy). RESULTS: Thirty-eight type I PPB cases were identified: Registry (n = 30) and literature (n = 8). Twenty children had surgery alone; eight (40%) experienced recurrence; and four died. Eighteen children had surgery and adjuvant chemotherapy; one experienced recurrence and died. All recurrences were type II or III PPB. Recurrence-free survival was higher in the surgery + chemotherapy group (P = .01); overall survival did not differ (P = .18). The improved recurrence-free survival was found only in males. Four of nine children with recurrence survived. CONCLUSION: Adjuvant chemotherapy appears to benefit type I PPB patients. Benefit limited to males requires broader substantiation. Salvage after types II and III recurrence is poor (four of nine; 44%). A rigorous surveillance schedule after type I PPB diagnosis might detect early recurrence and be an acceptable alternative to adjuvant chemotherapy.  (+info)

Pulmonary blastoma is a rare and aggressive type of lung cancer that primarily affects adults, but it can also occur in children. It's characterized by the rapid growth of primitive, undifferentiated cells that form tumors in the lungs. There are two main types of pulmonary blastomas:

1. Pleuropulmonary blastoma (PPB): This type is more common in children and adolescents. PPB can be further divided into three subtypes based on the age at diagnosis and the extent of tumor spread: Type I, Type II, and Type III. Types II and III are more aggressive and have a higher risk of metastasis compared to Type I.
2. Lung sarcomatoid carcinoma with pulmonary blastomatous components (LSC-PBC): This type is primarily found in adults and is considered a variant of lung sarcomatoid carcinoma, which is an aggressive subtype of non-small cell lung cancer. LSC-PBC contains both epithelial and mesenchymal elements, with the latter showing blastomatous features.

Both types of pulmonary blastomas have a poor prognosis due to their rapid growth and high likelihood of metastasis. Treatment typically involves surgical resection, chemotherapy, and radiation therapy. However, given the rarity of this condition, treatment options may vary depending on individual cases and access to specialized care.

Lung neoplasms refer to abnormal growths or tumors in the lung tissue. These tumors can be benign (non-cancerous) or malignant (cancerous). Malignant lung neoplasms are further classified into two main types: small cell lung carcinoma and non-small cell lung carcinoma. Lung neoplasms can cause symptoms such as cough, chest pain, shortness of breath, and weight loss. They are often caused by smoking or exposure to secondhand smoke, but can also occur due to genetic factors, radiation exposure, and other environmental carcinogens. Early detection and treatment of lung neoplasms is crucial for improving outcomes and survival rates.

A Sertoli-Leydig cell tumor is a rare type of sex cord-stromal tumor that develops in the ovaries. These tumors arise from the cells that produce hormones and help to form and maintain the ovarian tissue. Sertoli-Leydig cell tumors can occur in people of any age but are most commonly found in women between the ages of 20 and 40.

These tumors can be functional, meaning they produce hormones, or nonfunctional. Functional Sertoli-Leydig cell tumors may cause symptoms related to the production of male hormones (androgens), such as excess facial hair, a deepened voice, and irregular menstrual periods. Nonfunctional tumors typically do not cause any specific symptoms and are often found during routine pelvic examinations or imaging studies performed for other reasons.

Sertoli-Leydig cell tumors are usually slow-growing and can vary in size. Most of these tumors are benign (not cancerous), but some can be malignant (cancerous) and may spread to other parts of the body. Treatment typically involves surgical removal of the tumor, and additional therapies such as chemotherapy or radiation therapy may be recommended depending on the stage and grade of the tumor. Regular follow-up care is essential to monitor for any recurrence of the tumor.

Pleuropulmonary blastoma development occurs in children; its presentation can occur via pulmonary or pleural based neoplasms. ... Yousem SA, Wick MR, Randhawa P, Manivel JC (February 1990). "Pulmonary blastoma. An immunohistochemical analysis with ... Type 1 pleuropulmonary blastomas are composed of purely cystic tumors. Type 1 pleuropulmonary blastomas have the potential to ... Three evolutions of pleuropulmonary blastomas are known and categorized based on prognostic elements. ...
October 1988). "Pleuropulmonary blastoma. The so-called pulmonary blastoma of childhood". Cancer. 62 (8): 1516-26. doi:10.1002/ ... "Type I pleuropulmonary blastoma: pathology and biology study of 51 cases from the international pleuropulmonary blastoma ... Pleuropulmonary blastoma (PPB) is a rare cancer originating in the lung or pleural cavity. It occurs most often in infants and ... Cai S, Wang X, Zhao W, Fu L, Ma X, Peng X (2017) DICER1 mutations in twelve Chinese patients with pleuropulmonary blastoma. Sci ...
Adluri RK, Boddu SR, Martin-Ucar A, Duffy JP, Beggs FD, Morgan WE (February 2006). "Pulmonary blastoma-a rare tumor with ... In such cases, these biphasic tumors are classified as a form of pulmonary blastoma because the presence of the blastomatous ... Cutler CS, Michel RP, Yassa M, Langleben A (February 1998). "Pulmonary blastoma: case report of a patient with a 7-year ... Bodner SM, Koss MN (November 1996). "Mutations in the p53 gene in pulmonary blastomas: immunohistochemical and molecular ...
... including carcinosarcoma and pulmonary blastoma. A history of cigarette smoking is the most common cause of large cell ...
The medical examiner's report showed that he died of natural causes due to a metastatic pulmonary blastoma tumor. On December 7 ...
... which include pulmonary blastoma, fetal adenocarcinoma and pleuropulmonary blastoma. Pleuropulmonary blastomas are ... Many types of blastoma have been linked to a mutation in tumor suppressor genes. For example, pleuropulmonary blastomas have ... A blastoma is a type of cancer, more common in children, that is caused by malignancies in precursor cells, often called blasts ... The suffix -blastoma is used to imply a tumor of primitive, incompletely differentiated (or precursor) cells, e.g., ...
... pulmonary MeSH C04.588.894.797.520.734 - pancoast's syndrome MeSH C04.588.894.797.520.867 - pulmonary blastoma MeSH C04.588. ... pulmonary blastoma MeSH C04.557.435.710 - rhabdoid tumor MeSH C04.557.435.775 - sarcoma, endometrial stromal MeSH C04.557. ... pulmonary MeSH C04.557.470.035.215 - adenomatous polyps MeSH C04.557.470.035.215.100 - adenomatous polyposis coli MeSH C04.557. ... 894.797.520.933 - pulmonary sclerosing hemangioma MeSH C04.588.894.797.640 - pleural neoplasms MeSH C04.588.894.797.640.700 - ...
... the term commonly used to refer to carcinoma of the lung Pulmonary carcinoid tumor Pleuropulmonary blastoma Neuroendocrine ... These include: Primary tumors of the lung/pulmonary system: Bronchial leiomyoma, a rare, benign tumor Lung cancer, ...
... pulmonary blastoma. Abnormal duplication of the EGFR gene is a relatively infrequent phenomenon in SCL (>/= 4 copies in >/= 40 ...
Embryonal hepatoma M8971/3 Pancreatoblastoma M8972/3 Pulmonary blastoma Pneumoblastoma M8973/3 Pleuropulmonary blastoma M8974/1 ... malignant Blastoma, NOS M8000/6 Neoplasm, metastatic Neoplasm, metastatic Tumor, metastatic Tumor, secondary Tumor embolus ... Merkel cell tumor Primary cutaneous neuroendocrine carcinoma M8248/1 Apudoma M8249/3 Atypical carcinoid tumor M8250/1 Pulmonary ...
... the aorta Pulmonary artery familial dilatation Pulmonary atresia with ventricular septal defect Pulmonary blastoma Pulmonary ... of Pulmonary valve stenosis Pulmonary valves agenesis Pulmonary veins stenosis Pulmonary veno-occlusive disease Pulmonary ... Pulmonary hypertension Pulmonary hypoplasia familial primary Pulmonary sequestration Pulmonary supravalvular stenosis Pulmonary ... congenital Pulmonary alveolar proteinosis Pulmonary arterio-veinous fistula Pulmonary artery agenesis Pulmonary artery coming ...
... and pulmonary blastoma, which are (arguably) related variants. While the 4th revision ("WHO-2004") retained the same grouping ... GCCL and primary pulmonary choriocarcinoma can also be differentiated on the basis of ultrastructural features by electron ... Hagihara N, Abe T, Wakamiya T, Sugita Y, Watanabe M, Tabuchi K (2010). "A case of brain metastasis from pulmonary giant cell ... Bae HM, Min HS, Lee SH, Kim DW, Chung DH, Lee JS, Kim YW, Heo DS (October 2007). "Palliative chemotherapy for pulmonary ...
... and biphasic pulmonary blastoma (consists of embryonal type epithelial element and primitive mesenchymal stroma). Sarcomatoid ... Weissferdt A (September 2018). "Pulmonary Sarcomatoid Carcinomas: A Review". Advances in Anatomic Pathology. 25 (5): 304-313. ...
... pulmonary emphysema, and cerebral calcification Berylliosis Beta ketothiolase deficiency Beta-galactosidase-1 deficiency Beta- ... syndrome Björnstad syndrome BK virus nephritis Black piedra Bladder neoplasm Blamronesis Blaichman syndrome Blastoma ... Bromidrosiphobia Bronchiectasis Bronchiectasis oligospermia Bronchiolitis obliterans with obstructive pulmonary disease ...
ZFHX4 Pulmonary alveolar microlithiasis; 265100; SLC34A2 Pulmonary alveolar proteinosis; 300770; CSF2RA Pulmonary fibrosis, ... CD36 Pleuropulmonary blastoma; 601200; DICER1 Pneumothorax, primary spontaneous; 173600; FLCN Poikiloderma with neutropenia; ... MADH9 Pulmonary hypertension, primary, fenfluramine-associated; 178600; BMPR2 Pulmonary veno occlusive disease; 265450; BMPR2 ... pulmonary, 1; 265120; SFTPB Surfactant metabolism dysfunction, pulmonary, 2; 610913; SFTPC Surfactant metabolism dysfunction, ...
The suffix "-oma" (but not -carcinoma, -sarcoma, or -blastoma, which are generally cancers) is applied to indicate a benign ... Erasmus JJ, Connolly JE, McAdams HP, Roggli VL (2000). "Solitary pulmonary nodules: Part I. Morphologic evaluation for ... This disorder presents with many benign hamartomatous tumors including angiofibromas, renal angiomyolipomas, and pulmonary ...
Pleuropulmonary blastoma development occurs in children; its presentation can occur via pulmonary or pleural based neoplasms. ... Yousem SA, Wick MR, Randhawa P, Manivel JC (February 1990). "Pulmonary blastoma. An immunohistochemical analysis with ... Type 1 pleuropulmonary blastomas are composed of purely cystic tumors. Type 1 pleuropulmonary blastomas have the potential to ... Three evolutions of pleuropulmonary blastomas are known and categorized based on prognostic elements. ...
A Curious Case of Classic Biphasic Pulmonary Blastoma. Vinay, V.; Saini, Jitendra Kumar; Dinesh Kanna, M. N.; More ...
Congenital pulmonary airway malformations (CPAMs) are lung lesions that result from disordered development of the lower ... Pleuropulmonary Blastoma in Pediatric Lung Lesions. Pediatrics. 2021 Apr. 147 (4):[QxMD MEDLINE Link]. ... Fetal Surgery for Congenital Pulmonary Airway Malformation * Sections Fetal Surgery for Congenital Pulmonary Airway ... encoded search term (Fetal Surgery for Congenital Pulmonary Airway Malformation) and Fetal Surgery for Congenital Pulmonary ...
Pleuropulmonary blastoma.. Pulmonary Inflammatory Myofibroblastic Tumors. For more information, see Childhood Pulmonary ... Pleuropulmonary Blastoma. For more information, see Childhood Pleuropulmonary Blastoma Treatment.. Esophageal Cancer. For more ... Most pulmonary malignant neoplasms in children are due to metastatic disease, with an approximate ratio of primary malignant ... Weldon CB, Shamberger RC: Pediatric pulmonary tumors: primary and metastatic. Semin Pediatr Surg 17 (1): 17-29, 2008. [PUBMED ...
... pulmonary blastoma Subforum: Pleuropulmonary Blastoma 11 Topics. 19 Posts. Last post. by Amy2013 Mon Dec 02, 2013 11:37 am ... Including mesothelioma, neuroendocrine cancer of the lungs; including a subforum for pleuropulmonary blastoma, ppb, ...
... pulmonary blastoma Subforum: Pleuropulmonary Blastoma 11 Topics. 19 Posts. Last post. by Amy2013 Mon Dec 02, 2013 11:37 am ... Including mesothelioma, neuroendocrine cancer of the lungs; including a subforum for pleuropulmonary blastoma, ppb, ...
Primary pulmonary synovial sarcoma: a rare primary pulmonary tumor. Lung. 2014 Feb. 192 (1):211-4. [QxMD MEDLINE Link]. ... and pleuropulmonary blastoma. [94] Mesothelioma occurs in an older population and is usually diffuse or multifocal within the ... Associated pulmonary parenchymal disease is noted in some patients. [39] Chest CT scanning depicts a heterogeneous soft-tissue ... Metastatic pulmonary small cell carcinoma usually affects older adult smokers and presents as a primary lung mass. ...
... pulmonary blastoma, since the former has a much better prognosis and lacks p53 mutations in the latter. Pulmonary blastoma are ... pleuropulmonary blastoma (which occurs in children and is distinguished from pulmonary blastoma which occurs in adults); ... Pulmonary blastoma is considered as part of the spectrum of carcinomas with a pleomorphic sarcomatoid or sarcomatous element, ... Addis BJ, Corrin B. Pulmonary blastoma, carcinosarcoma, and spindle-cell carcinoma: an immunohistochemical study of keratin ...
Pulmonary blastoma Nature: Malignant Status:... ...
Pulmonary blastoma with yolk sac elements. Heena M Desai, Gayathri P Amonkar, Vikas S Kavishwar, Jaya R Deshpande. October- ...
Explore radiographic descriptions and similarities to pleuropulmonary blastoma. ... "Pleuro-Pulmonary Blastoma: A Case Report and Review of the Literature," Medical and Pediatric Oncology, Vol. 25, No. 6, 1995, ... M. Cohen and R. O. C. Kaschula, "Primary Pulmonary Tumors in Childhood: A Review of 31 Years Experience and the Literature," ... growing tumor that is indistinguishable radiographically from other large intrathoracic tumors such as pleuropulmonary blastoma ...
02/25/2011 [-] Biphagic pulmonary blastoma with a lesion of yolk sac tumor 02/25/2011 [-] Testicular teratoma with ... 02/09/2011 [-] Pleuropulmonary Blastoma a Clinicopathological Analysis 02/09/2011 [-] Diffuse postoperative cerebellar swelling ...
... and pulmonary blastoma (45%) appears to parallel that for adults. The experience with pulmonary rhabdomyosarcoma, as described ... PRIMARY PULMONARY NEOPLASMS OF CHILDHOOD - A REVIEW ANNALS OF THORACIC SURGERY Hartman, G. E., Shochat, S. J. 1983; 36 (1): 108 ... this diagnosis should be considered in young patients with solitary pulmonary masses or persistent, atypical pulmonary symptoms ... WATERSEAL GASTROSTOMY IN THE MANAGEMENT OF PREMATURE-INFANTS WITH TRACHEOESOPHAGEAL FISTULA AND PULMONARY-INSUFFICIENCY JOURNAL ...
Pleuropulmonary blastoma, pulmonary cysts, thyroid gland neoplasia, ovarian tumors, & cystic nephroma, ciliary body ... Thyroid cancer, cystic nephroma, ovarian Sertoli Leydig cell tumor, botryoid rhabdomyosarcoma, pleuropulmonary blastoma, ... pineoblastoma, pituitary blastoma, ciliary body medulloepithelioma, and/or a nasal chondromesenchymal hamartoma ... medulloepithelioma, nasal chondromesenchymal hamartoma, pineoblastoma, pituitary blastoma. GLOW syndrome (See DICER1 Tumor ...
Classic Biphasic Pulmonary Blastoma in an Adult Patient. Rajendra Kumar [11] , Vijay Raturi , Manoj Jain , Dewesh Kishan , ...
pleuropulmonary blastoma DOID:4769 * metastasis to pleura DOID:13408 * pulmonary large cell neuroendocrine carcinoma ...
Classic biphasic pulmonary blastoma in adults: an up-date review of a rare disease. Abstract. Pulmonary blastoma Pulmonary ... blastoma (BP) is a rare lung cancer that accounts for a small fraction of primary lung tumors. There are around 280 cases ...
pulmonary blastoma DOID:4765 * tumor of exocrine pancreas DOID:1795 * tubular adenocarcinoma ...
... pituitary blastoma, kidney cyst, pulmonary cyst, Sertoli-Leydig Cell Tumor. Mutation: Germline MultipleGeneVariants Variant & ... The patient originally aged 21 previously had a kidney cyst at age 14 and a pulmonary cyst at an unknown age. The patient aged ...
Pulmonary blastoma (morphologic abnormality). Code System Preferred Concept Name. Pulmonary blastoma (morphologic abnormality) ... M] Pulmonary blastoma Active Synonym false false 1229971014 [M]Pulmonary blastoma Active Synonym false false ...
N2 - Pleuropulmonary blastomas are rare malignant intrathoracic tumors of early childhood. They appear as a pulmonary- and/or ... AB - Pleuropulmonary blastomas are rare malignant intrathoracic tumors of early childhood. They appear as a pulmonary- and/or ... Pleuropulmonary blastomas are rare malignant intrathoracic tumors of early childhood. They appear as a pulmonary- and/or ... abstract = "Pleuropulmonary blastomas are rare malignant intrathoracic tumors of early childhood. They appear as a pulmonary- ...
Cystic Pleuro-Pulmonary Blastoma 2-year-old female had been diagnosed with Pleuropulmonary Blastoma. The disease extent ... Cystic Pleuro-Pulmonary Blastoma - additional opinion 2-year-old girl was diagnosed with pleuropulmonary blastoma (PPB). She ... Papillary Carcinoma of Thyroid with Multiple and Diffuse Nodular Secondarisms of the Pulmonary Parenchyma ... She thus underwent pleural drainage and thoracoscopic biopsy, revealing a diagnosis of "cystic pleuropulmonary blastoma". ...
Pulmonary blastoma with argyrophil cells and lacking sarcomatous features (pulmonary endodermal tumor resembling fetal lung). ... 7) Koss MN, Hochholzer L, O Leary T. Pulmonary blastomas. Cancer. 1991;67:2368-81. 8) Zhao YY, Liu L, Zhou T, Zhou NN, Yang YP ... ROS1 rearrangement in a case of classic biphasic pulmonary blastoma. Int J Surg Pathol. 2018;26:360-3. 13) de Kock L, Bah I, ... Somatic DICER1 mutations in adult-onset pulmonary blastoma. Eur Respir J. 2016;47:1879-82. 14) Zhao J, Xiang C, Zhao R, Guo P, ...
Rare primary malignant tumor of the lung: Pulmonary Blastoma. "Supportive and Palliative Cancer Care" Vol 1, No 3, 2005 ...
Unusual Cause of Pulmonary Mass in Childhood: Pleuropulmonary Blastoma Pages : 315-317 - Ahmet Güzel, Demet Demirkol, Agop ... The Effect of Obesity Degree on Childhood Pulmonary Function Tests Pages : 235-238 - Emel Torun, Fatma Özgüç, Erkan Cakir, ... Evaluation of the Diagnostic Role of Bedside Lung Ultrasonography in Patients with Suspected Pulmonary Embolism in the ...
Blastoma Pulmonar/genética Blastoma Pulmonar/patologia Blastoma Pulmonar/cirurgia Sarcoma/genética Sarcoma/patologia Sarcoma/ ... Children with both pulmonary and renal cysts are candidates for DICER1 mutation testing. ... Cistos RNA Helicases DEAD-box/genética Doenças Genéticas Inatas Neoplasias Renais Blastoma Pulmonar Ribonuclease III/genética ... Lung cysts resected in infancy were diagnosed retrospectively as pleuropulmonary blastoma. Both tumors had acquired somatic ...
  • For instance adult pulmonary blastoma has generated 20 or so case report PubMed citations in the past 10 years and the cumulated experience reported in them, and summarised in a handful of reviews, may help guide an uncertain clinical team - though even that function may now be superseded by online discussion forums and their more immediate feedback. (biomedcentral.com)
  • atypical adenomatous hyperplasia and diffuse idiopathic pulmonary neuroendocrine cell hyperplasia. (ersjournals.com)
  • In addition of the categories of squamous dysplasia and carcinoma in situ , two additional lesions were added to the group of pre-invasive lesions: atypical adenomatous hyperplasia (AAH) and diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH). (ersjournals.com)
  • Lung cysts resected in infancy were diagnosed retrospectively as pleuropulmonary blastoma. (bvsalud.org)
  • Children with both pulmonary and renal cysts are candidates for DICER1 mutation testing. (bvsalud.org)
  • Therefore, this article discusses bronchogenic cyst, pulmonary agenesis and hypoplasia, polyalveolar lobe, alveolocapillary dysplasia, sequestration including arteriovenous malformation (AVM) and scimitar syndrome, pulmonary lymphangiectasis, congenital lobar emphysema (CLE), and cystic adenomatoid malformation (CAM) and other lung cysts. (medscape.com)
  • [ 1 ] Most thoracic surgical procedures, such as resection of masses (eg, neurogenic tumors, bronchogenic cysts) and pulmonary lobectomy, are now accomplished with minimally invasive surgery, although the benefits of this approach for cystic adenomatoid malformations are unclear. (medscape.com)
  • DICER1 tumor predisposition (DICER1) is characterized by an increased risk for pleuropulmonary blastoma (PPB), pulmonary cysts, thyroid gland neoplasia (multinodular goiter, adenomas, and/or thyroid cancer), ovarian tumors (Sertoli-Leydig cell tumor, gynandroblastoma, and sarcoma), and cystic nephroma. (nih.gov)
  • The CPAM type 4 lesion, with its large and often thick-walled cysts lined with alveolar lining cells, may be confused with the cysts of type I pleuropulmonary blastoma, although its cyst walls contain subepithelial foci of rhabdomyosarcoma, almost exclusively underlying stretches of cuboidal-to-columnar epithelium, rather than the alveolar cell epithelium of CPAM type 4. (ersjournals.com)
  • Other cystic or pseudocystic lung lesions include post-infarction peripheral cysts resulting from intrauterine pulmonary artery thrombosis. (ersjournals.com)
  • Air-filled cysts within the interstitium are a feature of acute and persistent interstitial pulmonary emphysema, and are limited to the interlobular septa. (ersjournals.com)
  • Fluid-filled cysts of congenital pulmonary lymphangiectasia are present within the interlobular septa, and extend laterally from the septa beneath the pleura. (ersjournals.com)
  • Since 2000, advances in diagnosis include the discovery of a new gene affecting surfactant metabolism (adenosine triphosphate (ATP)-binding cassette subfamily A member 3 ( ABCA3 )) and description of two new entitities specifically affecting the infant lung (pulmonary interstitial glycogenosis and neuroendocrine cell hyperplasia of infancy). (ersjournals.com)
  • Congenital pulmonary airway malformations (CPAMs) are lung lesions that result from disordered development of the lower respiratory tract. (medscape.com)
  • Review of Early Postoperative Metrics for Children Undergoing Resection of Congenital Pulmonary Airway Malformations and Report of Pleuropulmonary Blastoma at a Single Institution. (uams.edu)
  • A new and expanded classification of congenital pulmonary airway malformations (CPAM) of the lung identifies lesions on the basis of the likely site of origin and clinical and pathological features. (ersjournals.com)
  • This is a fast growing tumor that is indistinguishable radiographically from other large intrathoracic tumors such as pleuropulmonary blastoma. (scirp.org)
  • M. Cohen and R. O. C. Kaschula, "Primary Pulmonary Tumors in Childhood: A Review of 31 Years' Experience and the Literature," Pediatric Pulmonology, Vol. 14, No. 4, 1992, pp. 222-232. (scirp.org)
  • Pulmonary blastoma Pulmonary blastoma (BP) is a rare lung cancer that accounts for a small fraction of primary lung tumors. (scientexconference.com)
  • Pleuropulmonary blastomas are rare malignant intrathoracic tumors of early childhood. (prinsesmaximacentrum.nl)
  • They appear as a pulmonary- and/or pleural-based mass and their pathogenesis and relationship to other pediatric solid tumors is not well understood. (prinsesmaximacentrum.nl)
  • Comparative genomic hybridization identified aberrations in all pleuropulmonary blastomas, including four amplifications in three tumors at chromosomes 5q33-34, 11q22.2-ter, 15q25-ter, and 19q11-13.2. (prinsesmaximacentrum.nl)
  • The identification of new genetic alterations as well as the confirmation of previously reported ones (especially 8q gains) in pleuropulmonary blastoma should help to improve our understanding of both the molecular mechanisms underlying the tumorigenesis of pleuropulmonary blastoma and the relationship of pleuropulmonary blastoma with other pediatric tumors. (prinsesmaximacentrum.nl)
  • Congenital pulmonary lymphangiectasia is also frequently associated with congenital malformations of the heart. (ersjournals.com)
  • The ability of computed tomography (CT) imaging to accurately discriminate between pleuropulmonary blastoma (PPB) and benign congenital lung malformations (CLM) is controversial. (eventscribe.net)
  • Preoperative diagnosis of MCH is reportedly difficult due to similar radiographic features as other cystic neoplasms, such as pleuropulmonary blastoma, pulmonary metastasis of endometrial stromal sarcoma, lymphangiomyomatosis (LAM), and cystic adenomatoid malformation (CAM) [ 2 ]. (biomedcentral.com)
  • An underlying malignancy, particularly pleuropulmomary blastoma that can be confused with macrocystic CPAM, is extremely rare among fetal lung lesions. (medscape.com)
  • PPB is also frequently mistaken for congenital pulmonary airway malformation (CPAM), another rare but non-tumorous cystic lung disease. (biologists.com)
  • Within the five types, recent reports have demonstrated an association of CPAM type 1 with bronchioloalveolar carcinoma (BAC), and a degree of confusion in separating CPAM type 4 from the purely cystic form of pleuropulmonary blastoma. (ersjournals.com)
  • The patient originally aged 21 previously had a kidney cyst at age 14 and a pulmonary cyst at an unknown age. (hypothes.is)
  • Some of these topics are covered in greater detail in other Medscape Reference articles (see Laryngomalacia , Pediatric Tracheomalacia , Pediatric Pulmonary Hypoplasia , Cystic Adenomatoid Malformation , and Pediatric Bronchogenic Cyst ). (medscape.com)
  • Diffuse pulmonary nodules. (radiopaedia.org)
  • Recommendations for the management of subsolid pulmonary nodules detected at CT: a statement from the Fleischner Society. (radiopaedia.org)
  • Multiple Pulmonary Nodules" is a descriptor in the National Library of Medicine's controlled vocabulary thesaurus, MeSH (Medical Subject Headings) . (wakehealth.edu)
  • This graph shows the total number of publications written about "Multiple Pulmonary Nodules" by people in this website by year, and whether "Multiple Pulmonary Nodules" was a major or minor topic of these publications. (wakehealth.edu)
  • Below are the most recent publications written about "Multiple Pulmonary Nodules" by people in Profiles. (wakehealth.edu)
  • Pulmonary blastoma (PB) is an exceedingly rare and aggressive malignant lung neoplasm that has distinct biphasic morphology. (turkjpath.org)
  • Pulmonary blastoma (PB) is an exceedingly unusual and aggressive malignant neoplasm of the lung that accounts for 0.25% to 0.5% of all resected lung cancers 1 . (turkjpath.org)
  • A decade later, Spencer termed this neoplasm as pulmonary blastema based on its histological resemblance to the fetal lung 4 . (turkjpath.org)
  • Mesenchymal cystic hamartoma is a rare pulmonary neoplasm, which occasionally presents with severe symptoms such as pneumothorax or hemothorax, and shows favorable outcomes following surgical resection. (biomedcentral.com)
  • Mesenchymal cystic hamartoma (MCH) of the lung is a rare pulmonary neoplasm that has favorable outcomes following surgical resection, although it occasionally presents with severe symptoms such as pneumothorax and hemothorax. (biomedcentral.com)
  • Kim Landry-Truchon is first author on ' Deletion of Yy1 in mouse lung epithelium unveils molecular mechanisms governing pleuropulmonary blastoma pathogenesis ', published in DMM. (biologists.com)
  • This Monograph covers the pathology of neoplastic diseases, infections, obstructive and interstitial lung diseases, and pulmonary manifestations of systemic diseases. (ersjournals.com)
  • Finally,one ofthe most common pulmonary lesions in infants and children, infantile lobar emphysema,is not cystic but simply the overinflation of a segment of lung. (ersjournals.com)
  • 4. Woolley K, Stark P. Pulmonary parenchymal manifestations of mitral valve disease. (radiopaedia.org)
  • A definite diagnosis of pulmonary blastoma was established on the left lung lobectomy specimen based on morphological and immunohistochemical findings. (turkjpath.org)
  • [ 12 ] Rare cases of primary pulmonary or pleural DSRCT have subsequently been identified. (medscape.com)
  • Sonographic images of fetal congenital pulmonary airway malformation. (medscape.com)
  • We encountered a 46-year-old woman with mesenchymal cystic hamartoma mimicking simple pulmonary aspergilloma on diagnostic imaging. (biomedcentral.com)
  • We are working on a very rare disease that is a lung cancer affecting young children, named pleuropulmonary blastoma (PPB). (biologists.com)
  • In this study, paraffin-embedded material of five cases of pleuropulmonary blastoma was analyzed for genetic alterations by comparative genomic hybridization and five genetic loci by fluorescence in situ hybridization. (prinsesmaximacentrum.nl)
  • Said SM, Marey G, Hiremath G, Aggarwal V, Kloesel B, Griselli M. Pulmonary valve replacement via left anterior minithoracotomy: Lessons learned and early experience. (uams.edu)
  • A) Microcystic congenital pulmonary airway malformation, with ultrasound showing a solid echogenic mass. (medscape.com)
  • Herein, we present a case of MCH mimicking simple pulmonary aspergilloma (SPA) on diagnostic imaging. (biomedcentral.com)