title: High-grade soft tissue sarcoma arising in a desmoid tumor: case report and review of the literature.
abstract: Desmoid tumors are rare benign monoclonal fibroblastic tumors. Their aggressiveness is local with no potential for metastasis or dedifferentiation. Here we report on a 61-year-old patient who presented a locally advanced breast desmoid tumor diagnosed 20 years after post-operative radiotherapy for breast carcinoma. After 2 years of medical treatment, a high-grade undifferentiated pleomorphic soft tissue sarcoma arose within the desmoid tumor. Despite extensive surgery removing both tumors, the patient showed locoregional relapse by the sarcoma, followed by multimetastatic progression, then death 25 months after the surgery. The arising of a soft tissue sarcoma in a desmoid tumor is an exceptional event since our case is the fourth one reported so far in literature. It reinforces the need for timely and accurate diagnosis when a new mass develops in the region of a preexisting desmoid tumor, and more generally when a desmoid tumor modifies its clinical or radiological aspect.
authors.affiliation: Department of Medical Oncology, Centre de Recherche en Cancérologie de Marseille (CRCM), Institut Paoli-Calmettes, INSERM UMR1068, CNRS UMR725, 232 Bd de Sainte-Marguerite, 13009 Marseille, France ; Faculty of Medicine, Aix-Marseille University, Marseille, France.
authors.forename: François
authors.initials: F
authors.lastname: Bertucci
authors.affiliation: Department of Medical Oncology, Centre de Recherche en Cancérologie de Marseille (CRCM), Institut Paoli-Calmettes, INSERM UMR1068, CNRS UMR725, 232 Bd de Sainte-Marguerite, 13009 Marseille, France.
authors.forename: Marjorie
authors.initials: M
authors.lastname: Faure
authors.affiliation: Department of Pathology, Marie Lannelongue Surgical Centre, Le Plessis-Robinson, France.
authors.forename: Maria-Rosa
authors.initials: MR
authors.lastname: Ghigna
authors.affiliation: Department of Pathology, Institut Paoli-Calmettes, Marseille, France.
authors.forename: Bruno
authors.initials: B
authors.lastname: Chetaille
authors.affiliation: Department of Surgical Oncology, Institut Paoli-Calmettes, Marseille, France.
authors.forename: Jérôme
authors.initials: J
authors.lastname: Guiramand
authors.affiliation: Department of Radiotherapy, Institut Paoli-Calmettes, Marseille, France.
authors.forename: Laurence
authors.initials: L
authors.lastname: Moureau-Zabotto
authors.affiliation: Department of Radiology, Institut Paoli-Calmettes, Marseille, France.
authors.forename: Anthony
authors.initials: A
authors.lastname: Sarran
authors.affiliation: Department of Medical Oncology, Centre de Recherche en Cancérologie de Marseille (CRCM), Institut Paoli-Calmettes, INSERM UMR1068, CNRS UMR725, 232 Bd de Sainte-Marguerite, 13009 Marseille, France.
authors.forename: Delphine
authors.initials: D
authors.lastname: Perrot
keywords: Breast cancer; Desmoid tumor; Soft tissue sarcoma