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.

Author: Banos Tarisar
Country: Kenya
Language: English (Spanish)
Genre: Health and Food
Published (Last): 4 August 2010
Pages: 202
PDF File Size: 18.72 Mb
ePub File Size: 13.98 Mb
ISBN: 522-4-57645-673-3
Downloads: 57237
Price: Free* [*Free Regsitration Required]
Uploader: Tasho

In our case, the patient had no identifiable risk factors for the development of a desmoid tumor. Medical observation alone was first proposed for recurrent but stable fibromatosia.

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. Article Tools Print this article. A and B T2-weighted sequences in the axial planes at two different levels confirming the solid nature of the lesion. Main Menu for Phones Micke O, Seegenschmiedt MH.

Frequently, desmoid tumors occur in young females during or after pregnancy and can regress spontaneously in menopause or after hormonal therapy with tamoxifen 3. Poorly circumscribed with infiltration of adjacent tissue Uniform cellularity between exuberant fibrous proliferation and low grade fibrosarcoma Cells are bipolar fibroblasts and myofibroblasts reduced amphophilic cytoplasm that merges with surrounding collagen, open chromatin, well defined nuclear membrane, one distinct nucleolus Usually more collagenous and less cellular than nodular fasciitis Mucopolysaccharide matrix with thin walled, curvilinear, non-branching or ectatic vessels Stroma varies from collagenous, keloid-like to myxoid Regenerative muscle cells within lesions may resemble giant cells Perivascular lymphocytes at edge of lesion Few mitotic figures, no atypia Postradiation: The thoraco-abdominal wall reconstruction was performed and an intrabdominal-diaphragmatic prosthesis Dual-Mesh was positioned to restore the continuity.

Analysis of thirty cases followed up for ten or more years. They are slow growing and histologically benign, but tend to be locally invasive at various anatomic sites.

  G170EG01 V1 PDF

Chest wall reconstruction after desmoid tumor resection. Traditionally, patients undergo standard surgery approaches with the primary goal always being complete extdaabdominal with negative margins, as indicated for sarcomas.

There was a problem providing the content you requested

Macroscopic surgical margins were free from all aspects of the tumor mass Figure 2. Extra-abdominal desmoid tumors, as seen in our patient, very rarely originate from the extremities, and the only cases that have been previously documented in the popliteal fossa are mentioned in [ 34 ]. Presently, authors address the question whether surgery and other aggressive treatments should systematically be part of first-line treatment.

Note entrapped muscle fibers. Mass in mesentery of small bowel. In addition to the usual sarcoma team, desmoid tumor care optimally involves gastroenterologists and geneticists.

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 to the surgical treatment. The internal structure of the tumor is composed of abundant collagen material mixed with spindle cells and fibroblasts with abundant eosinophilic cytoplasm 4.

Surgeons with expertise in sarcomas should guide the approach 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. Aggressive fibromatosis is a rare soft tissue tumor. Although it lacks metastatic potential, it can grow aggressively in a locally infiltrating pattern.

Upon US the lesion appears to be hypoechogenic and homogeneous, with variable vascularization. Desmoid tumors, also known as aggressive fibromatosis, are an extremely rare entity. There was no previous history of trauma or surgical intervention.

Desmoid tumors also called desmoid extraabominal are benign fibrous growths that occur rarely in the general population 5 to 6 per 1 million per year but fibormatosis in one of the familial cancer predisposition conditions known as familial adenomatous polyposis FAP or Gardner syndrome, affecting between 3.


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

Possible risk factors for the development of desmoid tumors include female sex, or a previous history of surgery, trauma, or pregnancy. 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. How important extraabdkminal a negative surgical margin?

The patient’s remote medical history reported an endometriosis initially treated with hormonal therapy and then with surgery; in addition, the lesion occurred soon after the birth of the patient’s first child. The selection of optimal treatment is not standardized but, when possible, radical and aggressive surgical resection with margins free of disease R0 remains, to date, the preferred treatment for desmoid tumor.

Musculo-aponeurotic fibromatosis of the shoulder girdle extra-abdominal desmoid. This case report documents an extremity desmoid tumor that had been diagnosed as a vascular tumor based on its magnetic resonance imaging MRI characteristics. How to cite item. Fibroblastic and myofibroblastic features, including intrareticular collagen fibers, thin filament bundles, cytoplasmic dense bodies. The lesion appeared to be tenuously hypointense in T1-weighted sequences and unevenly hyperintense in T2-weighted sequences, which was not indicative of hematic content, nor pathognomonic of a specific nature Figs.

Sign up for our Email Newsletters.

Introduction Desmoid tumors, also known as aggressive fibromatosis, are an extremely extraaabdominal entity. The technique is also useful in the post-operative follow-up evaluation of the patient 2.

On cut section, it is hard and tan-white. Extra-abdominal and abdominal wall desmoids are best treated by surgery with adequate resection of margins.