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Título: | FAP-related desmoid tumors: a series of 44 patients evaluated in a cancer referral center |
Fecha de publicación: | 2012 |
Editorial: | F. Hernández y Juan F. Madrid. Universidad de Murcia: Departamento de Biología Celular e Histología |
Cita bibliográfica: | Histology and histopathology, Vol. 27, nº 5 (2012) |
ISSN: | 1699-5848 0213-3911 |
Materias relacionadas: | CDU::6 - Ciencias aplicadas::61 - Medicina::616 - Patología. Medicina clínica. Oncología |
Palabras clave: | Gardner syndrome Desmoid tumors |
Resumen: | Desmoid tumors (DTs), the commonest extra-intestinal manifestation of familial adenomatosis polyposis (FAP), are monoclonal neoplasms demonstrating fibroblastic - myofibroblastic differentiation; they are locally invasive without metastatic capacity. FAP-associated DT natural history knowledge is limited; we examined patient and tumor characteristics for a FAP-DT cohort and evaluated anti-DT therapy molecular target expression levels (immunohistochemical analyses, FAP-DT tissue microarray; TMA). Forty-four patients were classified as intra-abdominal (IA; n=26), abdominal wall (AW)/extra-abdominal (EA; n=12) or concomitant IA/AW (n=6) based on DT primary diagnosis location. Positive family histories were found in 62% of FAP versus 10% of DT patients. Surgery was the mainstay therapy for AW/EW patients, whereas IA DTs received surgery, chemotherapy, radiotherapy, tamoxifen, NSAIDs, and/or imatinib. Eight of 20 completely resected DTs in the IA and AW/EA groups recurred; 12 of 38 patients in these groups (33%) developed secondary lesions elsewhere. Two intestinal mesenteric DT patients died of disease, three from other cancers, 27 are alive with disease and 12 are alive without disease. All evaluable FAP-DT exhibited nuclear ß-catenin, 65% were positive for cyclin D1, and 66% expressed nuclear p53. No ERα expression was observed, but ERß was expressed in 72%. COX2 was expressed in all evaluable FAP-DTs. KIT was rarely found in DTs but both PDGFRs and their ligands were expressed. Comparing biomarker expression (IA vs. EA DTs), only nuclear ER-ß staining was significantly higher in EA lesions (p=0.0070); no other markers were site informative. Enhanced knowledge of FAP-DT molecular underpinnings will facilitate development of novel therapeutic strategies |
Autor/es principal/es: | Colombo, Chiara Foo, Wai Chin Whiting, David Young, Eric D. Lusby, Kristelle Pollock, Raphael E. Lazar, Alexander J. Lev, Dina |
URI: | http://hdl.handle.net/10201/52460 |
Tipo de documento: | info:eu-repo/semantics/article |
Número páginas / Extensión: | 9 |
Derechos: | info:eu-repo/semantics/openAccess |
Aparece en las colecciones: | Vol.27, nº 5 (2012) |
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Fichero | Descripción | Tamaño | Formato | |
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Colombo-27-641-649-2012.pdf | 1,28 MB | Adobe PDF | Visualizar/Abrir |
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