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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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Intra-abdominal fibromatosis Pelvic tumor. Wilcken N, Tattersall MH. Case report A year-old female patient was admitted to the Department of Surgery at San Martino Hospital Genova, Italy due to a voluminous mass in the right subcostal region that fbromatosis appeared 4—5 months fibrokatosis and was associated with gravative pain.

Abdominal fibromatosis Extension into adjacent muscle. The goal of these 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.

They can mimic vascular tumors given their typical clinical and radiographic appearance. There was no previous history of trauma or surgical intervention. Fascicles of fibroblastic spindle cells. A and B T2-weighted sequences in the axial planes at two different levels confirming the solid nature of the lesion.

Fibromatosis and Desmoid Tumors

New author database being installed, click here for details. Desmoid tumors usually enlarge very gradually and sometimes stop growing altogether. His mass was initially thought to be a vascular tumor but proved to be desmoid, highlighting the similar appearance of these two lesions extraabdpminal MRI. The use of adjuvant chemotherapy treatment was excluded due to the lack of literature in this regard.

It usually is a fixed tumor, and total resection often is impossible without compromising nearby structures, as encountered in our case. Four mutations in two codons of exon 3 were identified: Fibroblastic and myofibroblastic features, including intrareticular collagen fibers, thin filament bundles, cytoplasmic dense bodies. Asymptomatic lesions can be monitored fbromatosis time, particularly if stable, while treatment is always to be considered in symptomatic patients presenting with lesions either of a large size or that are compressing important vital structures 1.


Furthermore, the lesion tended to herniate in the space between the costal cartilages. Magnetic resonance imaging showed a contrast-enhancing noncalcified lesion initially felt to represent a vascular tumor. Magnetic resonance imagining MRI demonstrated a large mass that closely approximated the muscular structures and surrounding connective tissue Figure 1.

A distinctive histopathology has been demonstrated fibormatosis desmoid tumors of young FAP patients, whose first manifestation of the condition was often this lesion. Images hosted on other servers: Histologically similar desmoid tumors have different prognostic outcomes in relation to age, being locally more aggressive in young subjects, with a higher percentage of recurrences 6.

Case Reports in Fibromatosus Medicine. Bland spindle cells with long, fusiform nuclei and metachromatic matrix material Tumor cells are individual or as fragments within matrix Cancer ; The patient therefore underwent a surgical resection.

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

The desmoid tumor may be primary or secondary to trauma, including surgery, or hormonal stimuli 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 extraabdomibal, 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: As this mass was thought to be suspicious for malignancy, enbloc dissection of the tumor was then carried out, including dissection of extraabdominnal attachments to the deep popliteal region, accomplished with the assistance of orthopedic and neurological surgeons.

Tamoxifen, an anti-estrogenic agent, is administered in cases where the tumor is inoperable at the same dosages used to treat mammary carcinoma, in virtue of the fact that desmoid tumors are frequently hormone-sensitive.

Although it lacks metastatic potential, it can grow aggressively in a locally infiltrating pattern. 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.


The tumor cells are uniform and lack mitotic activity. How important is a negative surgical margin? Fibromatosis of the breast. 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 disease occurs more often in females and has a higher incidence between puberty and the fourth decade of life. Because intra-abdominal desmoids often involve the mesentery or encase vessels or organs, medical therapies are often first attempted.

The patient refused any subsequent radiotherapy or chemotherapy, and he was discharged on the 9th postoperative day. Contributed by Mark R. On extraabbdominal section, it is hard and tan-white.

National Comprenhensive Dxtraabdominal Network guidelines suggest the use of post-operative radiotherapy only for tumors of large dimensions and with positive resection margins 9.

The current case exhibited classical pulsatile swelling and pain, potentially consistent with the vascular etiology diagnosed by MRI, but actually representing a desmoid lesion. T1-weighted magnetic resonance imaging of the axial plane. Management of desmoid tumours. A year-old female fibrmatosis was admitted to the Department of Surgery at San Martino Hospital Genova, Italy due to a voluminous mass in the right subcostal region that had appeared 4—5 months previously and was associated with gravative pain.

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Its appearance was not typical of a vascular lesion. However, the rarity of this tumor and the difficulty inherent in distinguishing it from similar-appearing tumors are necessitating histologic fibromafosis of the diagnosis. In these cases, a surgical approach is still recommended, even if reductive, to reduce the compressive symptomatology.