Desmoid Tumours: Our Experience of Six Cases and Review of Literature | oneFAPvoice

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Desmoid Tumours: Our Experience of Six Cases and Review of Literature

key information

source: Journal of clinical and diagnostic research

year: 2014

authors: Kallam A R, Ramakrishna B V, Roy G K, Karthik K R

summary/abstract:

Desmoid tumours represent aggressive fibroblastic proliferation of the musculoaponeurotic structures commonly from the anterior abdominal wall. These tumours infiltrate locally, recur frequently but do not metastasize. Antecedent trauma, pregnancy and estrogens play a role in the etiopathogenesis of these tumours. In familial adenomatous polyposis (FAP) genetic history associated with chromosomal abnormality and familial incidence as in Gardner’s syndrome is reported and most of these tumours are intraperitoneal either in the mesentery or pelvis and may be multiple and they carry poor prognosis. Surgery is the most preferred treatment and requires wide excision with 1 cm margin followed by reconstruction of the defect in the anterior abdominal wall either with local musculoaponeurotic layers or with synthetic mesh. In intra-abdominal cases associated with FAP in addition to surgery, hormonal treatment, chemotherapy and Radiotherapy are also advised depending upon the particular condition but usually prognosis is not encouraging. In this article we present our personal experience in the successful treatment of six cases of sporadic desmoids, five in females of child bearing age, and all in the anterior abdominal wall and one extra abdominal in a child aged 13 y in the gluteal region (Case 6). It is very interesting and unique to see two desmoid tumours developing in the same patient (Case2)one in each of the Rectus abdominal muscles (Right & Left).

organisation: Alluri Sitarama Raju Institute of Medical Sciences

DOI: 10.7860/JCDR/2014/9610.5032

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