Expression of FAP, ADAM12, WISP1, and SOX11 is heterogeneous in aggressive fibromatosis and spatially relates to the histologic features of tumor activity.

Aggressive fibromatosis (AF) represents a group of tumors with a variable and unpredictable clinical course, characterized by a monoclonal proliferation of myofibroblastic cells. Identification and validation of genes whose expression patterns are associated with AF may elucidate biological mechanisms in AF, and aid treatment selection. This study was designed to examine the protein expression by immunohistochemistry (IHC) of four genes, ADAM12, FAP, SOX11, and WISP1, that were found in an earlier study to be uniquely overexpressed in AF compared with normal tissues.

A rare case of aggressive desmoplastic fibromatosis in a 12-year-old girl is presented in this article with emphasis on the need and challenges for diagnosing such lesions as they have to be differentiated from other soft tissue tumors which display borderline pathological features regarding benign or malignant behaviour.

Rao, R.N.

This study demonstrates that tumor size and a history of recurrence are independent predictors of EFS. Surgery is warranted if it can be R0 and function sparing. Nonsurgical modalities or a policy of watchful waiting may be a better choice for unresectable disease.

Although rarely located in the abdomen, pregnancy-associated desmoid tumours should be included in the differential diagnosis of intra-abdominal tumours detected during or shortly after pregnancy. Based on this case and a few others reported in the literature, subsequent pregnancy does not necessarily seem to be a risk factor for recurrent or new disease.

Insulin-like growth factor II messenger RNA-binding protein 3 (IMP3) is a recently identified biomarker demonstrated to be useful in diagnosis and prognostic prediction for several kinds of malignant tumors. In this study, we examined the immunohistochemical expression of IMP3 in gastrointestinal stromal tumor (GIST) (n = 150), malignant melanoma (n = 17), malignant mesothelioma (n = 6), leiomyosarcoma (n = 6), inflammatory myofibroblastic tumor (IMT) (n = 12), desmoid fibromatosis (n = 8), leiomyoma (n = 20), and schwannoma (n = 20). GIST, desmoid fibromatosis, leiomyoma and schwannoma, were essentially negative for IMP3.