Type:
Educational Exhibit
Keywords:
Neoplasia, Cancer, Diagnostic procedure, CT, Oncology, Abdomen
Authors:
R. Carbone1, F. R. Setola2, C. PICONE3, O. Fabozzi3, C. Rossi 4, V. Granata5, M. L. Barretta6, E. de Lutio di Castelguidone4, A. Gallipoli D'Errico3; 1Napoli (NA)/IT, 2Naples, I/IT, 3Naples (NA)/IT, 4Naples/IT, 5Vitulazio (CE)/IT, 6Caserta (CE)/IT
DOI:
10.1594/ecr2013/C-1120
Background
Fibromatosis,
according to the classification of WHO (2002),
is a benign fibroblastic tumor which comprises a group of lesions that differ in the site of onset (see Fig.1) and for their clinical behavior.
Feature of this spectrum of disease is the discrepancy,
in particular,
between their benign histo-morphological appearance and their locally aggressive behavior,
with tendency to microscopic infiltration of the surrounding tissues and local recurrence ,
even in the absence of distant metastases.
Mesenteric fibromatosis is the most frequent form of intra-abdominal fibromatosis (desmoid tumors); it occurs in a wide ‘age-range’ of patients,
14 –75 years old (mean,
41 years),
and has no gender or race predilection.
Most cases of mesenteric fibromatoses manifest sporadically.
Thirty percent of patients with mesenteric fibromatosis have familial adenomatous polyposis (FAP),
specifically,
the Gardner-syndrome variant.
in which the disease tends to occur within 4 years from a previous surgery,
originating from its site of intervention.
Two other rare,
autosomal dominant hereditary disorders involving unique mutations of the APC (adenomatosis polyposis coli) gene are associated with mesenteric fibromatosis: familial infiltrative fibromatosis and hereditary desmoid disease.