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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Case Reports in Vascular Medicine
Introduction Desmoid tumors, also known as aggressive fibromatosis, are an extremely rare entity. Predominantly fibrotic with less cellularity. Extra-abdominal fibromatosis occurs more often in females and has a higher incidence between puberty and the fourth decade of life. Case report A year-old female patient was admitted to the Department of Extrraabdominal 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.
The lesion shows poor vascularization and a typical light-brown color, tibromatosis aids in the differentiation from the adjacent tissue. Expression profiles of sex steroid receptors in desmoid tumours.
Desmoid fibromatosis showing fascicular arrangement of bland fibroblasts, which are interrupted by extraabxominal, compressed vascular channels resulting in an appearance akin to a hypocellular scar.
Mesenteric, pelvic or retroperitoneal locations, associated with Gardner’s syndrome familial adenomatous polyposis, multiple osteomas Often postsurgical. Molecular determinants of fibromatosis recurrence or progression remain obscure. A and B T2-weighted sequences in the axial planes at two different levels confirming the solid nature of the lesion.
The present study reports the case of a year-old female with a voluminous mass diagnosed as an extra-abdominal fibromatosis. New author database being installed, click here for details. National Comprenhensive Cancer Network guidelines suggest the use of post-operative radiotherapy only for tumors of large dimensions and with positive resection margins 9. A report of cases. Histological examination showed evidence consistent with extra-abdominal fibromatosis.
Oberman, Atlas of Tumor Pathology. Abdominal fibromatosis Extension into adjacent muscle. Arises within abdominal wall of women during or after pregnancy May see with cesarean section scar Extra-abdominal fibromatosis: View at Google Scholar R.
Pathology Outlines – Fibromatosis – deep (desmoid type)
Support Center Support Center. It is not always possible to obtain disease-free resection margins, particularly if the tumor involves noble structures such as the spinal column, brachial plexus, major vessels or structures of the fascia of the neck 5. Uniform spindle cells with moderate collagen.
Eztraabdominal desmoid tumor may be primary or secondary to trauma, including surgery, or hormonal stimuli 3. C T2-weighted sequences in the coronal plane confirming the obtuse angles in relation to the liver and aiding in the measurement of the cranio-caudal diameter of the lesion, which appears to be fibeomatosis localized in the abdomen.
Trisomy 20 and 8.
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The possibility of an extremity desmoid tumor extraabdomijal be kept in mind when evaluating an extremity mass, but the diagnosis should be made only on the basis of a detailed histological examination. The technique is also useful in the post-operative follow-up evaluation of the patient 2. Extra-abdominal fibromatosis, also known as a desmoid tumor, is a rarely observed lesion of benign biological significance, characterized as a non-metastatic lesion with local invasiveness 1.
No infiltration of the surrounding large vessels was identified, but the tumor invested nerves in the popliteal fossa. Genetic susceptibility is believed to play a role in the development of the disease, but genetic risk factors have not been identified [ 5 ]. Firm, white lobulated fusiform mass. Large, firm, white cut surface, infiltrative borders Often in muscular fascia, cuts with gritty sensation, 5 – 10 cm. 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.
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.
To receive news and publication updates for Case Reports in Vascular Medicine, enter your email address in the box below. The subgroup of progressive or recurrent tumours might have the same aggressive biological characteristics, whatever their treatment.
The incidence of desmoid tumors has been reported as 2—4 cases per 1 million [ 12 exrtaabdominal.