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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Grossly the tumor appears as a dense, hard, rubbery, grayish-white mass.
Aggressive surgical resection with safe margins 2—4 cm R0 is the prime treatment. This wide range shows the great variability of accrual, treatments and follow-up in such a rare disease which extraabvominal never been investigated in a controlled randomized study. Generally, histopathological examination is necessary for definitive diagnosis, as in our case.
Tohuku J Exp Med. Rosen Fibromatois, Ernsberger D. Desmoid fibromatosis showing fascicular arrangement of bland fibroblasts, fibromatozis are interrupted by thin-walled, compressed vascular channels resulting in an appearance akin to a hypocellular scar.
Independent predictors for desmoids in FAP include an APC mutation 3′ of codonfamily history of desmoids, female gender, and the presence of osteomas. 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.
Introduction Extra-abdominal fibromatosis, also known as fibromatosiis desmoid tumor, is a rarely observed lesion of benign biological significance, characterized as a non-metastatic lesion with local invasiveness 1. The tumors frequently recur after surgical excision, which remains the treatment of choice. Received Oct 14; Accepted Jul 7.
J Comput Assist Tomogr. Clearly, we need prospective studies to understand the molecular mechanisms behind desmoid tumorigenesis and progression, and to confirm whether some mutations are at particular estraabdominal for recurrence or progression.
Fibromatosis and Desmoid Tumors
Fibroblastic and myofibroblastic features, including intrareticular collagen fibers, thin filament bundles, cytoplasmic dense bodies. On cut section, it is hard and tan-white. In the present case, the patient was suffering from endometriosis and underwent hormonal treatment as well as surgery; in addition to this, the lesion occurred soon after the birth of the patient’s first child. Desmoids most commonly occur following surgery after an average of 4.
Contributed by Mark R. The mass was markedly inhomogeneous with the presence of hyperdense areas indistinguishable from the muscle and bone. A palpable mass was detected in the popliteal fossa of the right lower extremity in a year-old man.
This website is intended for pathologists and laboratory personnel, who understand that medical information is imperfect and must be interpreted using reasonable medical judgment. The diagnosis of desmoid tumor, or aggressive fibromatosis, should alert members of the sarcoma team to the potential underlying diagnosis of FAP.
Histologically similar desmoid tumors have different prognostic outcomes in relation extraabdominao age, being locally more aggressive in young subjects, with a higher percentage of recurrences 6. Subscribe to Table of Contents Alerts.
Pathology Outlines – Fibromatosis – deep (desmoid type)
As this mass was thought to be suspicious for malignancy, enbloc dissection of the tumor was then carried out, including dissection of its attachments to the deep popliteal region, accomplished with the assistance of orthopedic and neurological surgeons.
Chemotherapy is used in those patients in whom the tumor exhibits rapid growth or exteaabdominal those who are heavily symptomatic, and it is associated with the use of NSAIDs or COX2 inhibitors.
In a fair percentage of cases, fibromatosis is associated with familial adenomatous polyposis FAP or Gardner’s syndrome 16.
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. However, surgery, radiotherapy, or both are regarded as the treatment s of choice for lesions [ 3 ]. Overall, radical surgery with disease-free resection margins is the prime treatment option for this disease. 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.
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Primary desmoid tumor Extraabdominal fibromatosis of the breast. Click here for information on linking to our website or using our content or images. Genetic susceptibility is believed to play a role in the development of the disease, but genetic risk factors have not been identified [ 5 ]. Trisomy 20 and 8. 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, which appears to be mainly localized in the abdomen.