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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Main Menu for Phones Long-term follow-up of 28 multimodally treated patients. The definitive histological examination showed evidence consistent with extra-abdominal fibromatosis: A to Z of desmoid tumors.

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

The use of extraabdominao, sulindac, and tamoxifen for the treatment of desmoid tumors associated with familial polyposis.

Furthermore, the lesion tended to herniate in the space between the costal cartilages. The technique is also useful in the post-operative follow-up evaluation of the patient 2. 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 had appeared 4—5 months previously and was associated with gravative pain.

To receive extraabdominap and publication updates for Case Reports in Vascular Medicine, enter your email address in the box below. More recently, Imatinib has been tested on advanced aggressive fibromatosis. The internal structure of the tumor is composed of abundant collagen material mixed with spindle cells and fibroblasts with abundant eosinophilic cytoplasm 4. Extra-abdominal desmoid tumour of the breast: Management of desmoid tumours.

Microscopic tumor margins were negative, and there was no evidence of malignant change Figure 3. Chest wall desmoid tumors: This wide range shows the great variability of accrual, treatments and follow-up in such a rare disease which has never been investigated in a controlled randomized study.


Upon CT of the chest, a coarse solid expansive formation was observed. Arises within abdominal wall of women during or after pregnancy May see with cesarean section scar Extra-abdominal fibromatosis: US, which was performed due to the difficult accessibility of the anatomical site, identified the presence of a solid mass fkbromatosis central necrosis, that appeared hypoechoic and confounding.

exfraabdominal The subgroup of progressive or recurrent tumours might have the same aggressive biological characteristics, whatever their treatment. Interferon has been shown to be effective in increasing the period of disease-free survival in certain patients 1. No necrotic outbreaks were identified. Desmoids most commonly occur following surgery after an average of 4.

Fibbromatosis report a case of mammary fibromatosis in an elderly female in which no associated risk factor was discernible. For this reason, tumors should be removed as soon as possible after identification in order to achieve the most optimal resection possible.

An optimal resection of the tumor involving the thoracic wall, as in the present case, is required to include the excision of disease-free ribs, one above and one underneath the lesion 5. Medical observation alone was first proposed for recurrent but stable lesions.

The relationship of desmoids in FAP to sporadic cases is uncertain in view of the distinctive early histopathology and the observation that APC gene mutations are uncommon in sporadic desmoids.

It is difficult to achieve complete resection because of their propensity for local invasion. 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 ].

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Fibromatosis and Desmoid Tumors

Results of surgical intervention. The final objective is to use molecular findings of tumours to individualize the selection of management protocols. Aggressive fibromatosis is a rare soft tissue tumor. Studies have been conducted on the use of imatinib mesylate a selective inhibitor of the tyrosine kinase receptor in the inoperable forms of the tumor and in those patients who refused chemo-radiotherapy or hormonal therapy.

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

Pathology Outlines – Fibromatosis – deep (desmoid type)

Click here for information on linking to our website or using our content or images. Identification of biological factors predictive of response to imatinib mesylate in aggressive fibromatosis.

The relative risk for desmoids in FAP patients compared with the general population is Generally, desmoid tumors present as a mass of hard consistency that is not well-defined, and is typically infiltrative and adherent to the surrounding tissue.

Extra-abdominal fibromatosis is a rare, benign disease that is characterized by a local but not metastatic invasivity.

What is the Initiative? Received Oct 14; Accepted Jul 7. Abstract Extra-abdominal fibromatosis is a rare, benign disease that is characterized by a local but not metastatic invasivity. Predominantly cellular with relatively less fibrosis. Magnetic resonance imaging MRI is the gold standard technique for diagnosis 2.

Large, firm, white cut surface, infiltrative borders Often in muscular fascia, cuts with gritty sensation, 5 – 10 cm.