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      Primary Pulmonary Tumors in Pediatric Population: Imaging Markers for Predicting Histology

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          Abstract

          Objectives:

          The objective of the study was to review the imaging features of proven pediatric primary lung tumors, with a purpose of detecting key distinguishing features among the various entities.

          Materials and Methods:

          We retrospectively reviewed multidetector computed tomography (CT) images of 17 pediatric patients with primary lung tumors. For each examination, various CT image descriptors were used to characterize the pulmonary nodules/masses; including location, size, number, morphology, cavitation, calcification, intense enhancement, airway involvement, chest wall/pleural involvement, mediastinal/vascular involvement, and nodal enlargement.

          Results:

          The age of the patients ranged from 2 to 18 years (mean age of 9.5 years). Approximately 35.3% of tumors were benign and 64.7% were aggressive/malignant. Nine distinct histopathologic tumor entities were found. Common tumor types were recurrent respiratory papillomatosis (4) and inflammatory myofibroblastic tumor (4) with two endobronchial tumors including carcinoid and mucoepidermoid carcinomas. Besides invasion and nodal enlargement, large size and central location ( P < 0.05) were predictors of aggressiveness/malignancy. Multiple lesions and cavitation ( P < 0.05), on the other hand, were frequent in benign lesions.

          Conclusion:

          On imaging, location and morphological markers can allow diagnosis in majority of the tumors.

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          Most cited references11

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          The 2015 World Health Organization Classification of Lung Tumors: Impact of Genetic, Clinical and Radiologic Advances Since the 2004 Classification.

          The 2015 World Health Organization (WHO) Classification of Tumors of the Lung, Pleura, Thymus and Heart has just been published with numerous important changes from the 2004 WHO classification. The most significant changes in this edition involve (1) use of immunohistochemistry throughout the classification, (2) a new emphasis on genetic studies, in particular, integration of molecular testing to help personalize treatment strategies for advanced lung cancer patients, (3) a new classification for small biopsies and cytology similar to that proposed in the 2011 Association for the Study of Lung Cancer/American Thoracic Society/European Respiratory Society classification, (4) a completely different approach to lung adenocarcinoma as proposed by the 2011 Association for the Study of Lung Cancer/American Thoracic Society/European Respiratory Society classification, (5) restricting the diagnosis of large cell carcinoma only to resected tumors that lack any clear morphologic or immunohistochemical differentiation with reclassification of the remaining former large cell carcinoma subtypes into different categories, (6) reclassifying squamous cell carcinomas into keratinizing, nonkeratinizing, and basaloid subtypes with the nonkeratinizing tumors requiring immunohistochemistry proof of squamous differentiation, (7) grouping of neuroendocrine tumors together in one category, (8) adding NUT carcinoma, (9) changing the term sclerosing hemangioma to sclerosing pneumocytoma, (10) changing the name hamartoma to "pulmonary hamartoma," (11) creating a group of PEComatous tumors that include (a) lymphangioleiomyomatosis, (b) PEComa, benign (with clear cell tumor as a variant) and
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            Childhood primary pulmonary neoplasms.

            Nine children (6 boys, 3 girls) were diagnosed with a primary endobronchial or pulmonary parenchymal neoplasm. The average age at diagnosis was 9 years. Presenting complaints included cough (7), fever (5), pulmonary infection (3), respiratory distress (3), weight loss (2), pain (2), and hemoptysis (1). Pulmonary x-rays showed persistent atelectasis, pneumonic infiltrates or mass lesions. A computed tomography scan was performed in 8. Five of six endobronchial tumors were diagnosed with bronchoscopy and biopsy. Treatment consisted of thoracotomy and pulmonary resection in 7 cases and laser resection in 2. The pathologic diagnoses were bronchial carcinoid (3), bronchial mucoepidermoid carcinoma (1), inflammatory pseudotumor (plasma cell granuloma) of the bronchus (2) and of the lung parenchyma (1), fibrosarcoma (1), and rhabdomyosarcoma (1). Postoperative chemotherapy was given only to the patient with pulmonary rhabdomyosarcoma; this child died. One child has developed a local recurrence while 7 children are alive and free of disease at an average of 2.4 years postresection. Pulmonary neoplasms are unusual in the pediatric age group and represent a wide spectrum of pathology. Including the present series, 383 tumors have been described. Seventy-six percent were malignant. Early investigation and surgical intervention are essential in children with persistent pulmonary symptoms or x-ray abnormalities. In most cases, the prognosis is excellent with complete surgical resection; however, malignancies other than bronchial adenoma are associated with significantly mortality.
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              Lung involvement in juvenile onset recurrent respiratory papillomatosis: a systematic review of the literature.

              Determine the exact incidence of pulmonary involvement in recurrent respiratory papillomatosis (RRP); explore available treatments and their effectiveness; determine the characteristics of cases that progress to lung cancer. MEDLINE, EMBASE, and the Cochrane Library databases between 1966 and 2007; reference lists of retrieved publication. Studies investigating recurrent respiratory papillomatosis with lung involvement. Age limited to 20 years of age to qualify for the diagnosis of juvenile-onset RRP. Data pertaining to study design, population demographics, risk factors, site of involvement, investigation including the determination of the human papillomavirus type, treatment, and outcomes including the development of cancer. No randomized control trials were retrieved. Hundred and one studies met our inclusion criteria (23 cohorts, 4 case series, 72 case reports, 2 open trials) with 161 cases of lung involvement identified. From the cohort studies we could estimate the incidence of lung involvement in RRP at 3.3%. The incidence of cancer in cases with lung involvement was 16%. We could not draw conclusions regarding treatment effectiveness in lung involvement, as that was not evaluated except in case studies. It would nevertheless appear that Interferon is not effective and the use of intravenous Cidofovir needs to be better evaluated. Well-designed, hypothesis-driven randomized control trials and prospective cohort studies are warranted to improve our understanding of the mechanisms underlying the development of lung involvement in RRP, the risks associated with different HPV types, the efficacy of potential therapeutic options as well as the risk of progression to cancer.
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                Author and article information

                Journal
                J Indian Assoc Pediatr Surg
                J Indian Assoc Pediatr Surg
                JIAPS
                Journal of Indian Association of Pediatric Surgeons
                Wolters Kluwer - Medknow (India )
                0971-9261
                1998-3891
                Nov-Dec 2020
                27 October 2020
                : 25
                : 6
                : 363-367
                Affiliations
                [1]Department of Radiodiagnosis, All India Institute of Medical Sciences, New Delhi, India
                [1 ]Department of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi, India
                Author notes
                Address for correspondence: Dr. Manisha Jana, Department of Radiodiagnosis, All India Institute of Medical Sciences, New Delhi - 110 029, India. E-mail: manishajana@ 123456gmail.com
                Article
                JIAPS-25-363
                10.4103/jiaps.JIAPS_156_19
                7815042
                1c525974-446c-4105-82b3-f05a436b98a1
                Copyright: © 2020 Journal of Indian Association of Pediatric Surgeons

                This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms.

                History
                : 11 September 2019
                : 07 November 2019
                : 11 January 2020
                Categories
                Original Article

                Surgery
                lung tumors,pediatric,primary tumors,pulmonary
                Surgery
                lung tumors, pediatric, primary tumors, pulmonary

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