Extra-Abdominal Fibromatosis (Desmoid Tumor): A Rare Tumor of the Lower Extremity Arising from the Popliteal Fossa. Mehmet Ali Kaygin,1. Extra-abdominal desmoids are deep-seated, benign (noncancerous) tumors. They are very rare tumors. While desmoid tumors do not spread to other parts of. Extra abdominal desmoid fibromatosis is a complex condition with many recognised treatments including active observation, hormonal therapy, chemotherapy.

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This recognition is imperative to the recruitment of gastroenterologists and geneticists who can provide a more focused approach to polyposis screening and genetic testing, which could benefit the extended family as well as the patient. Desmoid tumors may be the first manifestation of FAP in some patients and families. In these cases, a surgical approach is still recommended, even if reductive, to reduce the compressive symptomatology.

The lesion is poorly circumscribed and is centered in skeletal muscle and the adjacent fascia. Combination chemotherapy is effective and can be offered for unresectable cases that fail to respond to this therapy.

These treatments include radiotherapy, chemotherapy anthracycline, vinblastine and methotrexatehormonal therapy tamoxifennon-steroidal anti-inflammatory drugs NSAIDsinterferon and imatinib mesylate 8.

Furthermore, some families with APC mutations exhibit desmoids as their only disease manifestation. Musculo-aponeurotic fibromatosis of the shoulder girdle extra-abdominal desmoid.

Pathology Outlines – Fibromatosis – deep (desmoid type)

Oberman, Atlas of Tumor Pathology. Clearly, we need prospective studies to understand the molecular mechanisms behind desmoid fibormatosis and progression, and to confirm whether some mutations are at particular risk for recurrence or progression.

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Once the mass was isolated from the antero-lateral surface of the liver we proceeded with resection of the last four ribs, from the sternum to the posterior axillary line. World J Surg Oncol.

Grossly the tumor appears as a dense, hard, rubbery, grayish-white mass. Axial image of MRI shows a large, expansive heterogeneous soft tissue mass with contrast, closely applied to extraabdminal muscular structures, and infiltration and obliteration of adjacent structures white asteriks.

Extra-abdominal fibromatosis: Clinical and therapeutic considerations based on an illustrative case

Desmoid tumors usually enlarge very gradually and sometimes stop growing altogether. Clonal fibroblastic proliferation of deep soft tissue with infiltrative growth “Desmos” Greek means tendon-like Locally aggressive local recurrence, but no metastases See also: Open in a separate window.

The patient’s postoperative course was uneventful. To receive news and publication updates for Case Reports in Vascular Medicine, enter your email address in the box below. Extra abdominal mammary fibromatosis Desmoid tumor of breast in an elderly female. Fibromatosis desmoid tumor of the breast mimicking a case of ipsilateral metachronous breast cancer. Treatment is undertaken for symptoms, cosmetic issues, functional disruption or imminent risk to adjacent structures.

Extra-abdominal desmoid tumour of the breast: They can mimic vascular tumors given their typical clinical and radiographic appearance. As desmoid tumors are burdened with a high rate of local recurrence, each case should be carefully assessed to evaluate the possibility of using multimodal therapies.

Gross cross-sectional view of pathologyic resected specimen.

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This was localized between the anterior hepatic margin dislocated backwards and the right costal plane, with a strict anatomical association with the diaphragm, to the right rectus abdominis muscle and to the external oblique muscle without a clear cleavage plane. Furthermore, the lesion tended to herniate in the space between the costal cartilages.


Analysis of thirty cases followed up for ten or more years. The use of adjuvant chemotherapy treatment was excluded due to the lack of exrtaabdominal in this regard.

Extra-abdominal fibromatosis: Clinical and therapeutic considerations based on an illustrative case

The dimensions of the tumor, its extension and the anatomical association with the surrounding structures can be evaluated with the use of US, CT and MRI, which always must be performed prior extraabdomial the surgical treatment. Radical resection is necessary for successful excision since desmoid tumors tend to recur locally. Main Menu for Phones Postoperatively, pathologic examination demonstrated widespread proliferation of spindle-shaped cells and collagen fibers.

There was no previous history of trauma or surgical intervention. Molecular determinants of fibromatosis recurrence or progression remain obscure. It is not always possible to obtain disease-free resection margins, particularly if the tumor involves noble structures such as the spinal column, brachial plexus, major vessels or structures of the fascia of the neck 5.

Tyrosine kinase inhibition has also shown response. Generally, histopathological examination is necessary for definitive diagnosis, as in our case.