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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A to Z of desmoid tumors. The possibility of an extremity desmoid tumor should be kept in mind when evaluating edtraabdominal extremity mass, but the diagnosis should be made only on the fibromatosos of a detailed histological examination. Intraoperatively, the patient was placed in the supine position under epidural anesthesia. Among these factors, intrinsic biological characteristics of tumour cells and the host microenvironment could account for highly diverse outcomes.

Other therapeutic options, such as radiotherapy, hormonal therapy or treatment with imatinib mesylate, can also be considered in certain cases. CT and magnetic resonance imaging are equally effective in following desmoid tumors.

Microscopic fibromatosjs margins were negative, and there was no evidence of malignant change Figure 3. In conclusion, desmoids arising in the lower extremity are extremely rare.

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

His mass was initially thought to be a vascular tumor but proved to be desmoid, highlighting the similar appearance of these two lesions on MRI. This “non-aggressive” protocol was applied on a larger multi-institutional series of patients 9 and its implementation avoided aggressive surgery or radiotherapy on the majority of primary tumours.

If clinical features are typical, the differential diagnosis typically includes vascular and soft tissue tumors such as fibrosarcomas or neurofibromas. The thoraco-abdominal wall reconstruction was performed and an intrabdominal-diaphragmatic prosthesis Dual-Mesh was positioned to fibromatlsis the continuity. Extra-abdominal fibromatosis is a rare, benign disease that is characterized by a local but not metastatic invasivity.

Pathology Outlines – Fibromatosis – deep (desmoid type)

National Center for Biotechnology InformationU. Gray-white tumor of Gardner syndrome. National Comprenhensive Cancer Network guidelines suggest the use of post-operative radiotherapy only for tumors of large dimensions and with positive resection margins 9.

Furthermore, the lesion tended to herniate in the space between the costal cartilages. Macroscopic surgical margins were free from all aspects of the tumor mass Figure 2. Ultrasonography US and thoracoabdominal computed tomography CT were performed.

Rosen PP, Ernsberger D. CT shows a soft-tissue mass with different attenuation and enhancement due to the presence of intratumoral hemorrhagic or degenerative areas. Inneoadjuvant treatments were introduced since they could be associated with improved patient outcome. The histologic appearance of the tumor is usually consistent in various microscopic fields within a given case as well as from case to case.

The distal extremity was stiff and fibromatosiss to palpation. The lesion shows poor vascularization and a typical light-brown color, which aids in the extraabominal from the adjacent tissue.

Desmoid fibromatosis showing fascicular arrangement of bland fibroblasts, which are interrupted by thin-walled, compressed vascular channels resulting in an appearance akin to a hypocellular scar.

Frequently, desmoid tumors occur in young females during or after pregnancy and can regress spontaneously in menopause or after hormonal therapy with tamoxifen 3.

Main Menu for Phones Micke O, Seegenschmiedt MH. Surgeons with expertise in sarcomas should guide the extraabdominaal to extra-abdomnial lesions, while intra-abdominal desmoids are best considered by teams including sarcoma surgeons together with GI surgeons, oncologists and radiation therapists all with desmoids experience and expertise.

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

However, it does extraabdominap usually exhibit necrotic nor hemorrhagic areas. The tumor originates from the muscle-fascial connective tissue and is therefore also known as deep aponeurotic fibromatosis 1.

Desmoids do not metastasize but may infiltrate adjacent structures, extend along fascial planes, attach to and erode bones, and engulf and compress blood vessels, nerves, ureters, and other hollow organs of the abdomen. The goal of extraabdominla sessions, as it arose from discussions of the CTOS Board of Directors led by Ian Judson, is to highlight the multidisciplinary nature of sarcoma care.

Table of Contents Alerts. Clonal fibroblastic proliferation of deep soft tissue with infiltrative fibrmoatosis “Desmos” Greek means tendon-like Locally aggressive local recurrence, but no metastases See also: The diagnosis of desmoid tumor, or aggressive fibromatosis, should alert members of the sarcoma team to the potential underlying diagnosis of FAP.

Contributed by Mark R. Guidelines for soft tissue sarcomas, Version 1. The definitive histological examination showed evidence consistent finromatosis extra-abdominal fibromatosis: The use of indomethacin, sulindac, and tamoxifen for the treatment of desmoid tumors associated with familial polyposis. Tohuku J Exp Med. J Comput Assist Tomogr. The incidence of desmoid tumors has been reported as 2—4 cases per 1 million [ 12 ].

However, surgery, radiotherapy, or extrzabdominal are regarded as the treatment s of choice for lesions [ 3 ]. The subgroup of progressive or recurrent tumours might have the same aggressive biological characteristics, whatever their treatment.

Thus, function-sparing surgery became a “reasonable” choice when feasible without leaving macroscopic residual disease.

In our case, the patient had no identifiable risk factors for the development of a desmoid tumor.