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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The response may be even better with combination of the two.

Open in a separate window. As desmoid tumors are burdened with a high rate of local recurrence, each case should be carefully assessed to evaluate the possibility of using fibromqtosis therapies. C T2-weighted sequences in the coronal plane confirming the obtuse angles in relation to the liver and aiding in the measurement of the cranio-caudal diameter of the lesion, fibromatoiss appears to be mainly localized in the abdomen.

Surgeons with expertise in sarcomas should guide the approach extraabbdominal 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.

Long-term follow-up of 28 multimodally treated patients. Tyrosine kinase inhibition has also shown response. Written informed consent was obtained from the patient for inclusion in the present study.

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Exrraabdominal information Article notes Copyright and License information Disclaimer. Among these factors, intrinsic biological characteristics of tumour cells and the host microenvironment could account for highly diverse outcomes.

Predominantly cellular with relatively less fibrosis. Four mutations in two codons of exon 3 were identified: Eur J Cardiothorac Surg.

Independent predictors for desmoids in FAP include an APC mutation 3′ of codonfamily history of desmoids, female gender, and the presence of osteomas.

A multi-disciplinary approach is highly recommended for difficult desmoids, especially those that are intra-abdominal.

Please review our privacy policy. Published online Sep 8. Fascicles of fibroblastic spindle cells.

Case Reports in Vascular Medicine

In conclusion, the present study determined that radical resection with margins free of disease still remains the optimal treatment extraabdominal for patients with extra-abdominal fibromatosis. Large, firm, white cut surface, infiltrative borders Often in muscular fascia, cuts with gritty sensation, 5 – 10 cm.

Extra-abdominal fibromatosis, also known as a desmoid tumor, is a rare, non-metastatic and locally invasive lesion that is characterized by a high percentage of local recurrences. National Center for Biotechnology InformationU. The magnetic resonance imaging of the upper abdomen confirmed the presence of a neoplasm localized between the anterior hepatic margin and the right costal plane. Chest wall reconstruction after desmoid tumor resection.

Pathology Outlines – Fibromatosis – deep (desmoid type)

World J Surg Oncol. Generally, desmoid tumors present as a mass of hard consistency that is not well-defined, and is typically infiltrative and adherent to the surrounding tissue. Aggressive fibromatosis is a rare soft tissue tumor. Desmoid tumors are not considered sarcomas; however, they have been classified as aggressive fibromatosis, and the optimal treatment plan usually requires multidisciplinary teams only found in sarcoma centers.


Fibromatosis and Desmoid Tumors

The margins often appear to be unclearly defined due to the infiltration of the surrounding tissues 27. Traditionally, patients undergo standard surgery approaches with the primary goal always being complete resection with negative margins, as indicated for sarcomas.

A report of cases.

During the last 10 years, the following concepts evolved from individual series. Desmoid tumors also called desmoid fibromatosis are benign fibrous growths that occur rarely in the general population 5 to 6 per 1 million per year but frequently in one of the familial cancer predisposition conditions known as familial adenomatous polyposis FAP or Gardner syndrome, affecting between 3.

Desmoid tumors are described as dense, collagenous lesions with intertwining bundles of spindle cells without epithelial components. Clearly, we need prospective studies to understand the molecular mechanisms behind desmoid tumorigenesis and progression, and to confirm whether some mutations are at particular risk for recurrence or progression.