Inflammatory Myofibroblastic Tumor of the Lung in Children: Anaplastic Lymphoma Kinase (ALK) Expression and Clinico-Pathological Correlation
| Autor: | María T. G. de Dávila, Mónica Siminovich, Fabiana Lubieniecki, Jessica López, Laura Galluzzo |
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| Rok vydání: | 2012 |
| Předmět: |
Male
Pathology medicine.medical_specialty Lung Neoplasms Granuloma Plasma Cell Pathology and Forensic Medicine Malignant transformation medicine Humans Anaplastic lymphoma kinase Anaplastic Lymphoma Kinase Child Myofibroblasts Pathological In Situ Hybridization Fluorescence Gene Rearrangement Lung medicine.diagnostic_test Brain Neoplasms business.industry Infant Receptor Protein-Tyrosine Kinases General Medicine Gene rearrangement medicine.disease Immunohistochemistry medicine.anatomical_structure Genetic Loci Child Preschool Granuloma Pediatrics Perinatology and Child Health Female business Fluorescence in situ hybridization |
| Zdroj: | Pediatric and Developmental Pathology. 15:179-186 |
| ISSN: | 1615-5742 1093-5266 |
| DOI: | 10.2350/11-10-1105-oa.1 |
| Popis: | The inflammatory myofibroblastic tumor (IMT) is a rare neoplastic lesion with a high incidence in children and young people, and may arise in lungs, soft tissue, or viscera. It is recognized as a borderline tumor with the possibility to recur, undergo malignant transformation, and metastasize. IMT is composed of fascicles of bland myofibroblastic cells admixed with an inflammatory infiltrate consisting of lymphocytes, plasma cells, and eosinophils. We reviewed pulmonary IMT diagnosed at Garrahan Hospital in Buenos Aires, Argentina, during 12 years and examined the clinical, laboratory, and pathological features as well as molecular genetics. Eight pediatric cases were evaluated with a male-to-female ratio of 5:3 and a median age of 6 years at diagnosis. The most common lung localization was the upper lobe. All cases underwent surgical excision and no local recurrences were found. Five out of eight patients, including two cases with metastatic/multifocal lesions in the central nervous system (CNS), are alive and disease free after a median follow-up of 30 months. Anaplastic lymphoma kinase (ALK) expression was negative in all pulmonary samples by immunohistochemistry (IHC), however, rearrangement for ALK locus by fluorescence in situ hybridization was found in one lung and in two CNS samples. These findings may reflect higher sensitivity of the molecular biologic procedure compare to traditional IHC practice. In our pediatric experience, 25% of patients with lung IMT developed CNS lesions; therefore we consider that CNS screening in these patients should be considered, at diagnosis and later during follow up. |
| Databáze: | OpenAIRE |
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