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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On cut section, it is hard and tan-white. Asymptomatic lesions can be monitored over 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. Although it lacks metastatic potential, it can grow aggressively in a locally infiltrating pattern. 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 had appeared 4—5 months previously and was associated with gravative pain.

Extra-abdominal fibromatosis occurs more often in females and has a higher incidence between puberty and the fourth decade of life. Mesenteric, pelvic or retroperitoneal locations, associated with Gardner’s syndrome familial adenomatous polyposis, multiple osteomas Often postsurgical.

Intraoperatively, the patient was placed in the supine position under epidural anesthesia. Magnetic resonance imaging MRI is the gold standard technique for diagnosis 2.

Aggressive surgical resection with safe margins 2—4 cm R0 is the prime treatment. The tumors frequently recur after surgical excision, which remains the treatment of choice. Ultrasonography US and thoracoabdominal computed tomography CT were performed.

Fibromatosis and Desmoid Tumors

Published online Sep 8. Despite the multifactorial etiology, certain genetic factors have been identified. Furthermore, some families with APC mutations exhibit desmoids as their only disease manifestation.

They are slow growing and histologically benign, but tend to be locally invasive at various anatomic sites. Radiation therapy in the management of desmoid tumors.


During the last 10 years, the following concepts evolved from individual series. The lesion was felt to represent a vascular mass. The internal fibrromatosis of the tumor is composed of abundant collagen material mixed with spindle cells and fibroblasts with abundant eosinophilic cytoplasm 4.

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Herniation of solid tissue through extraabominal anterior intercostal spaces is apparent. Management of desmoid tumours. Desmoid tumors fibromatoses of the breast: Discussion 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.

A to Z of desmoid tumors. Desmoid tumors are described as dense, collagenous lesions with intertwining bundles of spindle cells without epithelial components.

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

Extra abdominal mammary fibromatosis Desmoid tumor of breast in an elderly female. The patient’s remote medical history reported an endometriosis initially treated fibromtaosis hormonal therapy and then with surgery; in addition, the lesion occurred soon after the birth of the patient’s first child.

A report of cases. Desmoid tumors originate most frequently from abdominal fascial or musculoaponeurotic structures, although they may appear at extra-abdominal sites.

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

Abstract Aggressive fibromatosis is a rare soft tissue tumor. The definitive histological examination showed evidence consistent with extra-abdominal fibromatosis: Surgery is difficult, and sometimes impossible, in cases of intra-abdominal desmoids but remains as an important option in selected cases. There are ongoing studies evaluating the effectiveness of pre-operative radiotherapy in reducing the dimensions of the tumors and in affecting the risk of local recurrence This “non-aggressive” protocol was applied on a larger multi-institutional series of patients 9 and its implementation fibtomatosis aggressive surgery or tibromatosis on the majority of primary tumours.

Through a right subcostal laparotomy, the voluminous neoplasm of the right subdiaphragmatic thoracic wall was excised. Microscopic tumor margins were negative, and there was no evidence of malignant change Figure 3. Interferon has been shown to be effective in increasing the period of disease-free survival in certain patients 1. The present study reports the case of a year-old female with a voluminous mass diagnosed as an extra-abdominal fibromatosis.


Recently, 10 the prevalence of the mutations of the gene encoding beta-catenin was evaluated in a large cohort of sporadic desmoids patients. 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. A multi-disciplinary approach is highly recommended for difficult desmoids, especially those that are intra-abdominal.

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.

Chest wall desmoid tumors: The thoraco-abdominal wall reconstruction was performed and an intrabdominal-diaphragmatic prosthesis Dual-Mesh was positioned to restore the continuity.

Treatment is undertaken for symptoms, cosmetic issues, functional disruption or imminent risk to adjacent structures.

Postoperatively, pathologic examination demonstrated widespread proliferation of spindle-shaped cells and collagen fibers. No infiltration of the surrounding large vessels was identified, but the tumor invested nerves in the popliteal fossa. Molecular determinants of fibromatosis recurrence or progression remain obscure. Generally, histopathological examination is necessary extraabdomminal definitive diagnosis, as in our case.

Click here for patient related inquiries. The peak incidence of desmoids in FAP is between 28 and 31 years, although they may occur at any age. Severe and even fatal clinical problems are sometimes caused by these tumors, especially if mesenteric vessels or other abdominal organs fxtraabdominal obstructed.

J Comput Assist Tomogr. Uniform spindle cells with moderate collagen. Furthermore, the lesion tended to herniate in the space between the costal cartilages. If clinical features are typical, the differential diagnosis typically includes vascular and fibromatossis tissue tumors such as fibrosarcomas or neurofibromas.

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