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.
|Published (Last):||18 April 2005|
|PDF File Size:||1.66 Mb|
|ePub File Size:||7.74 Mb|
|Price:||Free* [*Free Regsitration Required]|
Thus, function-sparing surgery became a “reasonable” choice when feasible without leaving macroscopic residual disease. Sporadic desmoid tumors of the chest: Discrepancies between the results of the impact of the quality of surgery suggest that additional factors may influence the eextraabdominal history of these tumours. After appropriate recognition, wide local excision may be the most appropriate treatment for fibromatosis of the extremity.
Radiation therapy in the management of desmoid tumors. 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.
No necrosis or pleomorphism is present [ 7 ]. Support Center Support Center.
Pathology Outlines – Fibromatosis – deep (desmoid type)
Images hosted on PathOut server: Keywords Desmoid tumor, Mammary fibromatosis, Extra abdominal, Adjuvant radiotherapy. World J Surg Oncol. Combination chemotherapy is effective and can be offered for unresectable cases that fail to respond to this therapy. 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.
The tumors frequently recur after surgical excision, which remains the treatment of choice. In our case, the patient had no identifiable risk factors for the development of a desmoid tumor. CT and magnetic resonance imaging are equally effective in following desmoid tumors.
This application of personalized medicine in fibromatosis could lead to the right treatment at the right time for the optimal outcome. Axial image of MRI shows a large, expansive heterogeneous soft tissue mass with contrast, closely applied to the muscular structures, and infiltration and obliteration of adjacent structures white asteriks. Extra-abdominal fibromatosis occurs more often in females and has a higher incidence between puberty and the fourth decade of life.
Extra-abdominal fibromatosis: Clinical and therapeutic considerations based on an illustrative case
The definitive histological examination showed evidence consistent with extra-abdominal fibromatosis: Br J Plast Surg. They can mimic vascular tumors given their typical clinical and radiographic appearance. On cut section, it is hard and tan-white. Furthermore, the lesion tended to herniate in the space between the costal cartilages.
Intraoperatively, the patient was placed in the supine position under epidural anesthesia. Through a right subcostal laparotomy, the voluminous neoplasm of the right subdiaphragmatic thoracic wall was excised.
Desmoid tumors may be the first manifestation of FAP in some patients and families.
There was a problem providing the content you requested
Predominantly fibrotic with less cellularity. Arises outside abdomen and abdominal wall, usually in muscles of shoulder, chest wall, back and thigh, equal gender frequencies Intra-abdominal fibromatosis: The patient refused any subsequent radiotherapy or chemotherapy, and he was discharged on the 9th postoperative day.
The mass was markedly inhomogeneous with the presence of hyperdense areas indistinguishable from the muscle and bone. The disease occurs more often in females and has a higher incidence between puberty and the fourth decade of life. 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.
A distinctive histopathology has been demonstrated in desmoid tumors of young FAP patients, whose first manifestation of the condition was often this lesion. Fewer than half of tumors so treated recur, and further therapy with radiation and repeat excision is usually successful.
Oberman, Atlas of Tumor Pathology. 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.
Eur J Cardiothorac Surg. This wide range shows the great variability extraabdoinal accrual, treatments and follow-up in such a rare disease which has never been investigated in a controlled randomized study.
The role of imatinib mesylate in adjuvant therapy of extra-abdominal desmoid tumors. Extra-abdominal and abdominal wall desmoids are best treated by surgery with adequate extraabsominal of margins. MRI represents the gold standard to identify the fibromatous lesion and its anatomical association with the adjacent tissues.
The use of adjuvant chemotherapy treatment was excluded due to the lack of literature in this regard. The distal extremity was stiff and tender to palpation. Articles from Oncology Letters are provided here courtesy of Spandidos Fibromatksis.
The histologic appearance of the tumor is usually consistent in various microscopic fields within a given case as well as from case to case.