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

Sporadic extraabbdominal tumors of the chest: 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. What is the Initiative? Extra-abdominal and abdominal wall desmoids are best treated by surgery with adequate resection of margins.

Desmoid tumors, also known as aggressive fibromatosis, are an extremely rare entity. The lesion is poorly circumscribed and is centered in skeletal muscle and the adjacent fascia. It originated from the muscular fascia of the deep muscle within the popliteal fossa. 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.

Radiation therapy for aggressive fibromatosis desmoid tumors: In addition to fibromatosis there are 3 other designated sessions; Neurofibromatosis Type 1: This recognition is imperative to the recruitment of gastroenterologists and geneticists who can provide a more focused approach to polyposis screening and genetic testing, which could benefit the extended family as well as the patient.

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

The histologic appearance of the tumor is usually consistent in various microscopic fields within a given case as well as from case to case. Fibromatosis – deep desmoid type. Desmoid tumors may be the first manifestation of FAP in some patients and families. CT and magnetic resonance imaging are equally effective in following desmoid tumors. Gray-white tumor of Gardner syndrome. Published online Sep 8.


Subscribe to Table of Contents Alerts. Chest wall desmoid tumors: Treatment is undertaken for symptoms, cosmetic issues, functional disruption or imminent risk to adjacent structures.

The technique is also useful in the post-operative follow-up evaluation of the patient 2.

Author information Article notes Copyright and License information Disclaimer. Extremity desmoid tumors are extremely rare. Gross cross-sectional view of pathologyic resected specimen. Asymptomatic lesions can be monitored over time, particularly if stable, while treatment is always to be considered in symptomatic patients presenting with fibgomatosis either of a large size or that are compressing important vital structures 1.

Aggressive fibromatosis is a rare soft tissue tumor. After appropriate recognition, wide local excision may be the most appropriate treatment for fibromatosis of the extremity. The use of adjuvant chemotherapy treatment was excluded due to the lack of literature in this regard.

Radical resection is necessary for successful excision since desmoid tumors tend to recur locally. Predominantly cellular with relatively less fibrosis. Only about one third of abdominal desmoids cause pain, although the most common symptom is abdominal pain. At present, study results remain discordant; this extraabsominal due to the fact that biological factors predictive of response to the drug have not yet been identified Magnetic resonance imagining MRI demonstrated a large mass that closely approximated the muscular structures and surrounding connective tissue Figure 1.

Mass in mesentery of small bowel. The possibility of an extremity desmoid tumor should be kept in mind when evaluating an extremity mass, but the diagnosis should be made only on the basis of a detailed histological examination.


This is an open access article distributed under the Creative Commons Attribution Licensewhich permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. The mass was markedly inhomogeneous with the presence of hyperdense areas indistinguishable from the muscle and bone. Other complications include bowel perforation, fistulization, bleeding and ureteral obstruction. Oberman, Atlas of Tumor Pathology. We report a case of mammary fibromatosis in an elderly female in which no associated risk factor was extraabdomnial.

The margins extraaabdominal appear to be unclearly defined due to the infiltration of the surrounding tissues 27.

Fibromatosis and Desmoid Tumors

An invasive mass adherent to the surrounding tissue was visualized intraoperatively and extensively debulked. Total excision was attempted, but was unsuccessful as a tumor segment 0.

Thus, function-sparing surgery became a “reasonable” choice when feasible without leaving macroscopic residual disease. Primary desmoid tumor Extraabdominal fibromatosis of the breast.

The patient therefore underwent a surgical resection. The definitive histological examination showed evidence consistent with extra-abdominal fibromatosis: Received Oct 14; Accepted Jul 7. Inneoadjuvant treatments were introduced since they could be associated with improved patient outcome. Considering their rarity, desmoid tumors are often misdiagnosed. US, which was performed due to the difficult accessibility of the anatomical site, identified the presence of a solid mass without central necrosis, that appeared hypoechoic and confounding.

It usually is a fixed tumor, and total resection often is impossible without compromising nearby structures, as encountered in our case. The patient has not shown recurrence of the disease for 4 years since the surgery. Guidelines for soft tissue sarcomas, Version 1.