Medical treatment of mammary desmoid-type fibromatosis: which benefit?

Autor: Jean-Emmanuel Kurtz, Carole Mathelin, Sébastien Molière, Louise Scheer, Massimo Lodi
Přispěvatelé: univOAK, Archive ouverte, Hôpital de Hautepierre [Strasbourg], Les Hôpitaux Universitaires de Strasbourg (HUS), Institut de Génétique et de Biologie Moléculaire et Cellulaire (IGBMC), Université de Strasbourg (UNISTRA)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Centre Hospitalier de Sarrebourg Saint-Nicolas et Hoff (CH Sarrebourg)
Jazyk: angličtina
Rok vydání: 2017
Předmět:
0301 basic medicine
Sorafenib
Oncology
Adult
medicine.medical_specialty
Wnt-beta catenin
lcsh:Surgery
Extra-abdominal fibromatosis
Breast Neoplasms
Case Report
[SDV.CAN]Life Sciences [q-bio]/Cancer
lcsh:RC254-282
03 medical and health sciences
Young Adult
0302 clinical medicine
[SDV.CAN] Life Sciences [q-bio]/Cancer
Surgical oncology
Internal medicine
Sunitinib
Tyrosine-kinase inhibitors
Medicine
Humans
Protein Kinase Inhibitors
Desmoid-type fibromatosis
business.industry
Fibromatosis
lcsh:RD1-811
Protein-Tyrosine Kinases
medicine.disease
Prognosis
lcsh:Neoplasms. Tumors. Oncology. Including cancer and carcinogens
Breast fibromatosis
Triptorelin
Surgery
Fibromatosis
Aggressive
030104 developmental biology
030220 oncology & carcinogenesis
Monoclonal
Female
business
Tamoxifen
Rare disease
medicine.drug
Zdroj: World Journal of Surgical Oncology
World Journal of Surgical Oncology, 2017, 15 (1), ⟨10.1186/s12957-017-1148-x⟩
World Journal of Surgical Oncology, Vol 15, Iss 1, Pp 1-6 (2017)
ISSN: 1477-7819
DOI: 10.1186/s12957-017-1148-x⟩
Popis: Background Breast fibromatosis is a rare disease characterized by monoclonal fibroblast proliferation. It has no ability to metastasize but has a high local recurrence rate and often infiltrates surrounding tissues. Surgical treatment is the reference, but recently, new targeted therapies have emerged. We report an original case of a patient with breast fibromatosis who received exclusive medical treatment. Our aim was to analyze these treatments based on the clinical and radiological outcome, iatrogenic effects, and pharmacological action. Case presentation We report the case of a 19-year-old woman who developed a desmoid-type fibromatosis of the lower inner quadrant of the right breast, measuring 50 × 25 mm (i.e., a volume of 27.4 cm3). Initial surgery was not possible because of potential esthetic and functional prejudice. Thus, she had an exclusive medical treatment including several lines: NSAIDs with tamoxifen and triptorelin, followed by sorafenib, then interferon α2b, and finally sunitinib. With tyrosine-kinase inhibitors (TKIs) (sunitinib), a significant partial response was observed (57% reduction of the maximal tumoral volume). For each treatment, we provided the clinical and radiological outcome in association with known pharmacological action. Conclusions TKI had been an interesting alternative option to initial surgery, providing at least a partial response and potentially allowing less mutilating surgery. However, no pharmacological mechanism can unequivocally explain TKI efficacy. In general, breast fibromatosis should be treated along with oncologist and interventional radiologists in a trans-disciplinary modality, thus offering an adapted treatment for this particular desmoid-type fibromatosis localization.
Databáze: OpenAIRE
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