title: Extra-abdominal fibromatosis: Clinical and therapeutic considerations based on an illustrative case.
abstract: Extra-abdominal fibromatosis is a rare, benign disease that is characterized by a local but not metastatic invasivity. In particular, desmoid tumors of the chest wall represent only 10-20% of all deep fibromatoses. The disease occurs more often in females and has a higher incidence between puberty and the fourth decade of life. The present study reports the case of a 34-year-old female who came to our attention due to a voluminous mass in the right subcostal region. The magnetic resonance imaging of the upper abdomen confirmed the presence of a neoplasm localized between the anterior hepatic margin and the right costal plane. Through a right subcostal laparotomy, the voluminous 95×45×94-mm neoplasm was excised. Histological examination showed evidence consistent with extra-abdominal fibromatosis. The patient has not shown recurrence of the disease for 4 years since the surgery. Overall, radical surgery with disease-free resection margins is the prime treatment option for this disease. Other therapeutic options, such as radiotherapy, hormonal therapy or treatment with imatinib mesylate, can also be considered in certain cases.
authors.affiliation: Department of Surgery, IRCCS San Martino Hospital, National Cancer Research Institute, University of Genoa, Genova 16132, Italy.
authors.forename: Rosario
authors.initials: R
authors.lastname: Fornaro
authors.affiliation: Department of Surgery, IRCCS San Martino Hospital, National Cancer Research Institute, University of Genoa, Genova 16132, Italy.
authors.forename: Elisa
authors.initials: E
authors.lastname: Caratto
authors.affiliation: Department of Surgery, IRCCS San Martino Hospital, National Cancer Research Institute, University of Genoa, Genova 16132, Italy.
authors.forename: Michela
authors.initials: M
authors.lastname: Caratto
authors.affiliation: Department of Surgery, IRCCS San Martino Hospital, National Cancer Research Institute, University of Genoa, Genova 16132, Italy.
authors.forename: Alexander
authors.initials: A
authors.lastname: Salerno
authors.affiliation: Department of Anatomical Pathology, IRCCS San Martino Hospital, National Cancer Research Institute, University of Genoa, Genova 16132, Italy.
authors.forename: Francesca
authors.initials: F
authors.lastname: Sarocchi
authors.affiliation: Department of Radiology, IRCCS San Martino Hospital, National Cancer Research Institute, University of Genoa, Genova 16132, Italy.
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Minetti
authors.affiliation: Department of Surgery, IRCCS San Martino Hospital, National Cancer Research Institute, University of Genoa, Genova 16132, Italy.
authors.forename: Marco
authors.initials: M
authors.lastname: Frascio
authors.affiliation: Department of Oncology, IRCCS San Martino Hospital, National Cancer Research Institute, University of Genoa, Genova 16132, Italy.
authors.forename: Roberto
authors.initials: R
authors.lastname: Murialdo
authors.affiliation: Department of Surgery, IRCCS San Martino Hospital, National Cancer Research Institute, University of Genoa, Genova 16132, Italy.
authors.forename: Mario
authors.initials: M
authors.lastname: Taviani
keywords: chest wall; desmoid tumor; extra-abdominal fibromatosis; histology; surgery