Understanding of the basic biology and natural history of these tumors has increased substantially over the past decade. It is usually benign which means that it does not metastasize spread to other areas of the body, but it can invade and attach to surrounding tissue, and intertwine with organs and structures. The management of desmoid tumours european journal of cancer. The myofibroblast is the cell responsible for the desmoid tumor. Desmoid tumor of tongue med oral patol oral cir bucal. While each child or adult may experience symptoms differently, the following are the most common. It originates from the musculoaponeurotic structures found in the entire body. Others grow quickly and are treated with surgery, radiation therapy. Desmoid tumor, alternative medicine cures, prevention and. Some desmoid tumors are slow growing and dont require immediate treatment. Accordingly, medical and surgical management of desmoid tumors has also evolved. Abdominal desmoid tumor is a type of desmoid tumor that involves the abdominal wall. Notably, molecular studies have demonstrated that desmoid tumors in autosomal inherited fap disease show clonal neoplasms arising from germline mutations or changes in the apc gene.
The natural history of desmoid tumors, and the available data utilizing initial observation are discussed in detail elsewhere. What is desmoid fibromatosiscausestreatmentsurgery. The mayo clinic web site provides further information on desmoid tumor. The term desmoid, coined by muller in 1838, is derived from the greek word desmos, which means tendonlike desmoid tumors often appear as infiltrative, usually welldifferentiated, firm overgrowths of fibrous tissue, and they are locally aggressive. Desmoid comes from the greek word desmos, which means tendon or bandlike. Extraabdominal desmoid tumor edt is a rare con dition, caused by. Mortality of intraabdominal desmoid tumors in patients. This website is maintained by the national library of medicine.
The desmoid tumor research foundation dtrf, suffern, new york. Desmoid fibromatosis are basically a medical condition in which abnormalities in fibroblasts give rise to tumors also referred as desmoid tumors. In individuals lacking germline mutations the development of desmoid tumors requires a rare combination of events. A total of 154 patients with intraabdominal desmoid tumors were included in the study. Desmoid tumor genetic and rare diseases information. It is not intended to be and should not be interpreted as medical advice or a diagnosis of any health or fitness problem, condition or disease. Desmoid tumors most often occur in the abdomen, arms and legs. Desmoid tumors occur in up to 20 percent of people with familial adenomatous polyposis of the colon.
Desmoid tumor is called aggressive fibromatosis as it has similarities with a malignant cancerous tumor called fibrosarcoma. Desmoid tumors are noncancerous growths that occur in the connective tissue. These tumors tend to occur in women in their thirties, but can occur in anyone at any age. Desmoid tumor article about desmoid tumor by the free. Desmoid tumor is a rare type of cancer that develops in the tissues that form tendons and ligaments, usually in the arms, legs or abdomen and sometimes in the chest. Desmoid tumors are rare softtissue tumors of mesenchymal origin, with an estimated incidence of 24 per million people per year. Palpation revealed a nontender firm mass approximately 3 x 3 cm in diameter. Desmoids in these areas may cause unique clinical problems.
Desmoid tumor definition desmoid tumor is a tendonlike benign fibrous neoplasms. Also known as aggressive fibromatosis, desmoid tumors typically form as a single, firm but rubbery mass in the connective tissue in the arms, legs or trunk. Know the causes, diagnosis, and treatment of desmoid fibromatosis. In various studies, 2869% of desmoids were intraabdominal mesenteric or pelvic or abdominal wall, and the remaining were extraabdominal 7. Pdf desmoid tumor aggressive fibromatosis of the neck. Desmoid tumor is a tendonlike benign fibrous neoplasms. There are even instances of spontaneous regression. Review diagnosis and management of desmoid tumors and. In some cases, they appear as part of various syndromes, such as familial adenomatous polyposis fap. A hallmark of aggressive fibromatosis af or desmoid tumors is its unpredictable. Desmoid tumors in children are most commonly diagnosed between ages 15 and 16. Tailoring desmoid treatment repub, erasmus university repository.
Media in category desmoid tumor this category contains only the following file. The tumor is present as a mass on the abdomen the cause of tumor formation is general unknown, but several contributory factors that relate to genetic abnormalities, physical trauma, and hormonal issues have been proposed. Race and ethnic background do not seem to affect whether a person gets these tumors. Desmoids do not have the ability to metastasize but can cause significant morbidity and mortality by local invasion. Extraabdominal desmoid tumor is a type of desmoid tumor that occurs outside the abdomen such as in the limbs and shoulders.
Desmoid tumor commonly develops in the fibrous connective tissues of the body that connect. She is a healthy, 40 year old female who noticed that something was wrong after slowly developing more pain and discomfort during hiking and other physical activity a couple years ago. Figure 2 recurrencefree survival s curve of patients subjected to surgical treatment of extraabdominal desmoid tumor according to the surgical margin. The probability of survival free of local recurrence is calculated for three years, five years, and seven years after surgery. Our desmoid fibromatosis local recurrence nomogram is a tool designed to predict the likelihood of survival without the desmoid tumor returning at the site of initial surgery after the tumor is removed through surgery. Extraabdominal desmoid tumors associated with familial. Desmoidtype fibromatosis df is sometimes called desmoid tumour or aggressive fibromatosis. Desmoid tumors are rare benign, softtissue tumors that usually affect adults. Desmoid tumors may affect all sites, including the extremities, trunk, and abdomen. These tumors, also called aggressive fibromatosis, are considered benign because they typically dont spread to other parts of the body. They may occur at any site in the body, outside of the abdomen they can cause a wideranging set of signs and symptoms depending on their location.
Desmoid tumors are characterized by proliferation of fibroblastic cells that arise from the fascia or aponeurosis of muscle. It appears as firm overgrowths of tissues, usually fibrous, locally aggressive, well differentiated and infiltrative. Desmoid fibromatosis is a rare but locally aggressive tumor comprised of myofibroblasts. Desmoid tumors develop in the tissues that form tendons and ligaments, usually in the arms, legs or abdomen and sometimes in the chest. Desmoid tumor of tongue the ventral surface appeared smooth. Ben alman, steven attia, christina baumgarten, charlotte benson, jeanyves blay, sylvie.
For example, only 5% of sporadic desmoid tumors are intraabdominal, but 80% of patients with familial adenomatous polyposis fapassociated desmoid tumors develop intraabdominal disease. The desmoid tumor research foundation web site offers information on desmoid tumors. Extraabdominal desmoid tumors are a significant cause of morbidity in patients with familial adenomatous polyposis syndrome. These tumors may occur throughout the body, but are commonly found on the abdominal wall and within the intestinal mesentery. The clinical features and the pathological changes of desmoid tumors were studied to point out the key factors affecting the recurrence. The term desmoid, coined by muller in 1838, is derived from the greek word desmos, which means tendonlike. Pdf desmoid tumor fibromatosis of the head and neck. Desmoid tumors are cytologically bland fibrous neoplasms originating from the musculoaponeurotic structures throughout the body. Prognostic factors influencing progressionfree survival determined from a series. Possible clinical factors related to the postoperative recurrence were analyzed statistically. Aggressive fibromatosis is a rare condition marked by the presence of desmoid tumors. These tumors, also called aggressive fibromatosis, are a type of soft tissue sarcoma and are considered benign because they typically dont spread to. Tumors that form in the abdominal wall are called abdominal desmoid tumors.
Df can occur anywhere in the body but it is mostly found in the arms, legs and abdomen tummy. These are a type of cell that provide cell support for the bodys tissues. Desmoid tumors that are associated with fap, or more specifically the gardner syndrome variant of fap, have identical characteristics on ct imaging as the tumors that are not associated with a genetic condition kawashima et al. Df, also known as aggressive fibromatosis, is a rare tumor characterized by proliferation of myofibroblasts arising from the deep muscle fascia, aponeurosis, tendons, and scar tissue. Pdf desmoid tumors describe a rare monoclonal, fibroblastic proliferation characterized by a variable. Primary asymptomatic desmoid tumor of the mesentery. The content on this site is presented in a summary fashion, and is intended to be used for educational and entertainment purposes only. Desmoid tumors fall under this category because they have an annual incidence of 0. The desmoid tumor research foundation dtrf facebook. Intraabdominal and abdominal wall desmoid fibromatosis. Feedback controls limit cell division after a certain number of cells have developed, allowing for tissue repair click the link for more information. These are associated with a biallelic apc mutation in the affected tissue 2, 8. A desmoid tumor, also called aggressive fibromatosis, is a mass rather than a lump that can occur anywhere in the body, and for many reasons. Desmoid tumors are usually considered benign not cancer because they rarely spread to different parts of your body.
Tumors are also called neoplasms, which means that they are composed of new and actively growing tissue. Another term for desmoid tumors is aggressive fibromatosis. Treatment for a tumor depends upon its location and characteristics, as well as the patients preferences. Desmoid tumor of the tongue med oral patol oral cir bucal.
Typically, a single tumor develops, although some people have multiple tumors. Desmoid tumor commonly develops in the fibrous connective tissues of the body that connect, support, and surround other body parts and organs. Imaging of intra and extraabdominal desmoid tumors. Desmoid fibromatosis is known to affect women more as compared to men. This paper analyzes recent evidence pertaining to the. In another study, it was demonstrated that the 5year recurrencefree.
Review diagnosis and management of desmoid tumors and fibrosarcomasandra l. Desmoid tumors may invade surrounding tissues and be difficult to control, and they may develop at any body site. Desmoid tumors may arise at any site in the body, but are most commonly found in the trunk and extremities. Desmoid tumor is also called aggressive fibromatosis, as it has similarities with a malignant cancerous tumor called. The treatment of desmoid tumors annals of oncology. Genetics home reference ghr contains information on desmoid tumor. However, it is considered benign because it does not metastasize spread to other parts of the body. Management and recurrence patterns of desmoids tumors. Aggressive fibromatosis, desmoid tumor, advanced disease. Desmoid tumors account for 3% of all soft tissue tumors and 0. Desmoid tumor definition of desmoid tumor by medical. They are most commonly found in the abdomen of adults, arising from the anterior abdominal wall, mesentery, or retroperitoneum. Desmoid tumors arise from cells called fibroblasts, which are found throughout the body and provide structural support, protection to the vital organs, and play a critical role in wound healing.
A few weeks ago i met a patient who came to see me for treatment recommendations for a desmoid tumor in her hip. Each tumor was staged and each patient was categorized according to the stage of their most advanced tumor. As an example, in a placebocontrolled randomized trial of sorafenib in patients with disease progression or symptomatic desmoid fibromatosis, 20 percent of patients developed disease regression on the placebo arm. Desmoid tumours aggressive fibromatosis, deep musculoaponeurotic. According to the who, desmoid tumors are defined as clonal fibroblastic proliferations that arise in the deep soft tissue and are characterized by infiltrative growth and tendency toward local. A desmoid tumor is an abnormal growth that arises from connective tissue, which is the tissue that provides strength and flexibility to structures such as bones, ligaments, and muscles. Natural cure for desmoid tumor and alternative treatments. For that reason, desmoids are not extensively discussed in the literature.
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