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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CT and magnetic resonance imaging are equally effective in following desmoid tumors. Intra-abdominal fibromatosis Pelvic tumor. These treatments include radiotherapy, chemotherapy anthracycline, vinblastine and methotrexatehormonal therapy tamoxifennon-steroidal anti-inflammatory drugs NSAIDsinterferon and imatinib mesylate 8. Other complications include bowel perforation, fistulization, bleeding and ureteral obstruction.
An invasive mass adherent to the fjbromatosis tissue was visualized intraoperatively and extensively debulked. More recently, Imatinib fibromatlsis been tested on advanced aggressive fibromatosis.
Case Reports in Vascular Medicine
Only about one third of abdominal desmoids cause pain, although the most common symptom is abdominal pain. Written informed consent was obtained from the patient for inclusion in the present study. The diagnosis of desmoid tumor, or aggressive fibromatosis, should alert members of the sarcoma team to the potential underlying diagnosis of FAP.
T1-weighted magnetic resonance imaging of the axial plane. Once the mass was isolated from the antero-lateral surface of the liver we proceeded with resection of the last four ribs, from the sternum to the posterior axillary line. Analysis of thirty cases followed up for ten or more years.
Inneoadjuvant treatments were introduced since they could be associated with improved patient outcome. Desmoids do not metastasize but may infiltrate adjacent structures, extend along fascial planes, attach to and fibormatosis bones, and engulf and compress blood vessels, nerves, ureters, and other hollow organs of the abdomen.
Tohuku J Exp Med. The mass was markedly inhomogeneous with the fibromatozis of hyperdense areas indistinguishable from the muscle and bone. This case report documents an extremity desmoid tumor that had been diagnosed as a vascular tumor based on its magnetic resonance imaging MRI characteristics.
The lesion extraxbdominal was large, gray-white, fibrotic, and irregular. Introduction Desmoid tumors, also known as aggressive fibromatosis, are an extremely rare entity.
Interferon has been shown to be effective in increasing the period of disease-free survival in certain patients 1.
Extra-abdominal fibromatosis: Clinical and therapeutic considerations based on an illustrative case
Although it lacks metastatic potential, it can grow aggressively in a locally infiltrating pattern. Excision with wide margins and possibly frozen section evaluation of margins Ann Surg Oncol ; After appropriate recognition, wide local excision may be the most appropriate treatment for fibromatosis of the extremity. The post-operative recovery was regular, and the patient figromatosis discharged on day 8.
Frequently, desmoid tumors occur in young females during or after pregnancy and can regress spontaneously in menopause or after hormonal therapy with tamoxifen 3. T2-weighted magetic resonance imaging MRI showing a non-homogeneous high signal intensity tumor. Chest wall desmoid tumors: Arch Pathol Lab Med.
The peak incidence of desmoids in FAP is between 28 and 31 years, although they may occur at any age. Furthermore, some families with APC mutations exhibit desmoids as their only disease manifestation.
Chemotherapy is used in those patients in whom the tumor exhibits rapid growth or in those who are heavily symptomatic, and it is associated with the use of NSAIDs or COX2 inhibitors.
To receive news and publication updates for Case Reports in Vascular Medicine, enter your email address in the box below. Micke O, Seegenschmiedt MH. Wilcken N, Tattersall MH. Herniation of solid tissue through the anterior intercostal spaces is apparent.
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 ].
Extraabdoominal 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. It usually is a fixed tumor, and total resection often is impossible without compromising nearby structures, as encountered in our case.
Results of surgical intervention. Desmoids may occur in any musculoaponeurotic tissue structures of the body, although they tend to be fibromaosis extremities and spinal areas in the general population and in the abdomen in FAP. Because intra-abdominal desmoids often involve the mesentery or encase vessels or organs, medical therapies are often first attempted. The internal structure of the tumor is composed of abundant collagen material mixed with spindle cells and fibroblasts with abundant eosinophilic cytoplasm 4.
Multimodality management of desmoid tumors: Fibromatosis of the breast. Clonal fibroblastic proliferation of deep soft tissue with infiltrative extraqbdominal “Desmos” Greek means tendon-like Locally aggressive local recurrence, but no metastases See also: No infiltration of fibromwtosis surrounding large vessels was identified, but the tumor invested nerves in the popliteal fossa.
Extra abdominal mammary fibromatosis Desmoid tumor of breast in an elderly female. Desmoid tumors can either be primitive or secondary to trauma, including surgical procedures, or hormonal stimulation.