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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For this reason, tumors should be removed as soon as possible after identification in order to achieve the most optimal resection possible. T2-weighted magetic resonance imaging MRI showing a non-homogeneous high signal intensity tumor. The tumor originates from the connective muscle-fascial tissue. Extraabrominal is the Initiative? Musculo-aponeurotic fibromatosis of the shoulder girdle extra-abdominal desmoid.

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

A to Z of desmoid tumors. Discussion Extra-abdominal fibromatosis, also known as a desmoid tumor, is a rare, non-metastatic and locally invasive lesion extraabddominal is characterized by a high percentage of local recurrences. Aggressive surgical resection with safe margins 2—4 cm R0 is the prime treatment.

Note entrapped muscle fibers. Axial image of MRI shows a large, expansive heterogeneous soft tissue mass with contrast, closely applied to the muscular structures, and infiltration and obliteration of adjacent structures white asteriks.

The selection of the optimal treatment is not standardized, above all due to the small number of patients suffering from this disease and due to the few clinical trials that have been conducted. Fewer than half of tumors so treated recur, and further therapy with radiation and repeat excision is usually successful.


Extra-abdominal fibromatosis Fibroblasts, spindled, dense, wavy nuclei and minimal cytoplasm. Genetic susceptibility is believed to play a role in the development of the disease, but genetic risk factors have not been identified [ 5 ]. The lesion appeared to be tenuously hypointense in T1-weighted sequences and unevenly hyperintense in T2-weighted sequences, which was not indicative of extraabvominal content, nor pathognomonic of a specific nature Figs.

Firm, white lobulated fusiform mass.

Subscribe to Table of Contents Fibrpmatosis. It typically presents as a mass of a hard consistency, with a poorly demarcated margin and poor vascularization, and is commonly infiltrating and adherent to the surrounding tissue.

Radical resection is necessary for successful excision since desmoid tumors tend to recur locally. The tumors frequently recur after surgical excision, which remains the treatment of choice. No necrosis or pleomorphism is present [ 7 ]. Huge intrathoracic desmoid tumor.

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Results of surgical intervention. Desmoids most commonly occur following surgery after an average of 4. The intraoperative histological examination evidenced the mesenchymal nature of the lesion and showed that the resection margins were free from disease R0.

Predominantly fibrotic with less cellularity. Through a right subcostal laparotomy, the voluminous neoplasm of the right subdiaphragmatic thoracic wall was excised. Mass in mesentery of small bowel.

Fibromatosis and Desmoid Tumors

Page views in Email this article Login required. Images hosted on other servers: Large, firm, white cut surface, infiltrative borders Often in muscular fascia, cuts with gritty sensation, 5 – 10 cm. This was extraabdomijal 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.


The technique is also useful in the post-operative follow-up evaluation of the patient 2.

The patient refused any subsequent radiotherapy or chemotherapy, and he was discharged on the 9th postoperative day. National Center for Biotechnology InformationU. This is an open access article distributed under the Creative Commons Attribution Licensewhich permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

They are slow growing and histologically benign, but tend to be locally invasive at various anatomic sites. Tumor infiltrates thoracic wall.

Extra-abdominal fibromatosis, also known as a desmoid extraavdominal, is a rare, non-metastatic and locally invasive lesion that is characterized by a high percentage of local recurrences. The definitive histological examination showed evidence consistent with extra-abdominal fibromatosis: User Username Password Remember me.