Desmoid tumors occur in up to 20 percent of people with familial adenomatous polyposis of the colon. While each child or adult may experience symptoms differently, the following are the most common. Remission of rapidly growing desmoid tumors after tamoxifen therapy. Tumors that form in the abdominal wall are called abdominal desmoid tumors. Im still waiting to hear if its fap but im pretty convinced its post pregnancy related. Desmoplastic small round cell tumors dsrct are aggressive tumors that typically begin in the abdomen or pelvis.
Desmoid tumors that grow can extend to involve nearby tissues and organs, causing signs, symptoms and complications. I am 40 years old and had a rather large desmoid tumor 16 cm removed from right paraspinal muscle three weeks ago, the margins were clear, but very close, at some points, just millimeters. An intraabdominal desmoid tumor difficult to distinguish. Desmoids are infiltrative, locally destructive, soft tissue tumors. Race and ethnic background do not seem to affect whether a person gets these tumors. 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. Desmoplastic small round cell tumors dsrct memorial. Data merge with the other 2 observational studies evaluate the natural history of dt during pregnancy on a larger scale. Aggressive fibromatosis is a rare condition marked by the presence of desmoid tumors.
While clonality does not definitively establish neoplasia, combining the lack of selflimited growth and locally aggressive behavior indicates that this tumor is best. These tumors tend to occur in women in their thirties, but can occur in anyone at any age. These tumors often occur in women in their 30s, but can occur in anyone at any age. Asymptomatic, not growing incidental at laparotomyor ct scan nsaid or surgery without removal significant amount of intestine stage ii. The mayo clinic web site provides further information on desmoid tumor. Surgery is the therapeutic mainstay, but there has been much discussion of late regarding its proper application. 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.
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. The most common symptoms include pain or a mass in the abdomen. In the united states, 900 to 1,500 new cases are diagnosed per year. Desmoid tumors are associated with a variable and unpredictable clinical course. The term desmoid originates from the greek word desmos, meaning band or tendonlike and was first applied in the 1800s to describe tumors with a tendonlike consistency. Desmoid tumors are benign fibroelastic tumors with potential local invasion and recurrence after excision. This subset of desmoid tumours warrants specific discussion due to the. Desmoid tumours can arise from any musculoaponeurotic sheath in any part of the body, and are commonly classified by anatomical locations as intraabdominal, extraabdominal or abdominal wall tumours. Desmoid tumors or aggressive fibromatosis are infrequent conditions, with a large.
However, it is considered benign because it does not metastasize spread to other parts of the body. An update on the management of sporadic desmoidtype. Desmoid tumor genetic and rare diseases information. Desmoid tumor definition of desmoid tumor by medical. Desmoidtype fibromatosis df is sometimes called desmoid tumour or aggressive fibromatosis. There are even instances of spontaneous regression. In individuals lacking germline mutations the development of desmoid tumors requires a rare combination of events. 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. Medical center drive, 3310 ccc, ann arbor, michigan. It originates from the musculoaponeurotic structures found in the entire body. Genetics home reference ghr contains information on desmoid tumor. Desmoidtype fibromatosis is a rare and locally aggressive monoclonal. Dsrct usually occurs in white adolescent and young men between the ages of ten and 30.
Extraabdominal desmoid tumors are a significant cause of morbidity in patients with familial adenomatous polyposis syndrome. Desmoid tumors are responsible for 9 to 11 percent of deaths among patients with fap. Recent controversies exist regarding their optimal treatment. Understanding of the basic biology and natural history of these tumors has increased substantially over the past decade. These tumors are more aggressive in younger patients, with re currence rates up to 87% 3. These tumors, also called aggressive fibromatosis, are considered benign because they typically dont spread to other parts of the body. Although they do not metastasize, the incidence of local recurrence is quite high. Intraabdominal desmoid tumors usually occur at the mesentery or retroperitoneum, and may morphologically mimic gastrointestinal. Desmoid tumors account for 3% of all soft tissue tumors and 0. It should combine several modalities to prevent the important risk of recurrences. Material on cancer care and has formal eortc board approval. Desmoidtype fibromatosis df is a rare entity that predominantly involves the extremities, the trunk, and the abdominal cavity. The incidence of desmoid tumors in the general population is 24 cases per million per year, with a slight female pre ponderance and peak incidence in the third and fourth decades 2. Pdf sporadic abdominal wall desmoid tumor after caesarean.
Highdose tamoxifen and sulindac as firstline treatment for desmoid tumors. Extraabdominal desmoid tumors associated with familial. In some cases, they appear as part of various syndromes, such as familial adenomatous polyposis fap. Can wait and see be the standard of care for initial. The term desmoid, coined by muller in 1838, is derived from the greek word desmos, which means tendonlike. Df can occur anywhere in the body but it is mostly found in the arms, legs and abdomen tummy. Extraabdominal desmoid lesions, otherwise known as aggressive fibromatosis, are slowgrowing benign lesions which may be encountered in clinical practice. Typically, a single tumor develops, although some people have multiple tumors. Junctional desmoid tumours are a subset of extraabdominal desmoid tumours. These are associated with a biallelic apc mutation in the affected tissue 2, 8. Unfortunately, the pathology of this lesion wasnt available and other differential diagnoses include lymphoma, sarcoma and metastasis. Pdf management of a large abdominal wall desmoid tumor. Desmoid tumors in children are most commonly diagnosed between ages 15 and 16. Desmoid tumors are benign fibroblastic neoplasms with no metastatic potential, but a propensity for local recurrence even after complete surgical resection.
Hi, ive had 2ops this year and radiotherapy for an abdominal wall desmoid. Desmoid tumors of the chest wall oxford academic journals. Pdf desmoid tumor of the small bowel and the mesentery. It is a nonmetastasizing, sporadic mesenchymal tumor with high tendency to recurrence and often is categorized as lowgrade sarcoma. I am now looking at postop radiation therapy, which comes with its own set of risks, and then hormone therapy. 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 definition desmoid tumor is a tendonlike benign fibrous neoplasms. Desmoid comes from the greek word desmos, which means tendon or bandlike. Another term for desmoid tumors is aggressive fibromatosis. Although the imaging appearance may be suggestive, histopathologic confirmation. An update on the management of sporadic desmoidtype fibromatosis. I had significant weight loss and night sweats for nearly a year following the arrival of our second child.
Introduction desmoid tumors are deepseated, benign, slowly growing fibroblastic neoplasms that arise from musculoaponeurotic stromal elements. This website is maintained by the national library of medicine. Accordingly, medical and surgical management of desmoid tumors has also evolved. These lesions can develop at any site in the body, and commonly occur in the intraabdominal area.
Desmoid tumor dt is a common manifestation of gardners syndrome gs, although it is a rare condition in the general population. Desmoid tumor is called aggressive fibromatosis as it has similarities with a malignant cancerous tumor called fibrosarcoma. Review diagnosis and management of desmoid tumors and. These are a type of cell that provide cell support for the bodys tissues. 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. Hansmann a, adolph c, vogel t, unger a, moeslein g. Primary asymptomatic desmoid tumor of the mesentery.
It appears as firm overgrowths of tissues, usually fibrous, locally aggressive, well differentiated and infiltrative. Radiotherapy in desmoid tumors university of helsinki research. The differential diagnosis of desmoid tumors includes other neoplasms, such as lymphoma, pleomorphic sarcoma, fibrosarcoma, and giant cell tumor of the tendon sheath 1. 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. Request pdf desmoids tumors desmoid tumor can be defined as a pseudoencapsulated infiltrative growth of welldifferentiated collagenous fibroblasts and fibrocytes arising. Desmoid tumors are cytologically bland fibrous neoplasms originating from the musculoaponeurotic structures throughout the body. Dt in patients with gs is usually located in the abdominal wall andor intraabdominal cavity. Desmoid tumors are noncancerous growths that occur in the connective tissue. We report a case of a 37yearold woman with an abdominal wall desmoid tumor that appeared and grew rapidly during her pregnancy, diagnosed by trucut core biopsy.
Natural cure for desmoid tumor and alternative treatments. Its intestinal lesions are specially prominent, but extracolonic manifestations may also occur, such as osteomas, skin tumors, supernumerary teeth, congenital hypertrophy of the retinal pigment epithelium and desmoid tumor. Tailoring desmoid treatment repub, erasmus university repository. Surgery is the best primary treatment, but sometimes the tumor is nonresectable. The majority of tumors had attenuation values equal to 47% or greater than 41% the attenuation of muscle on contrastenhanced ct scans. Fap staging intraabdominal desmoid tumors in fap patients stage i. Multiple desmoid tumors in a patient with gardners. Treatment for a tumor depends upon its location and characteristics, as well as the patients preferences. Desmoid tumor of the abdominal wall radiology case.
Desmoid tumors most often occur in the abdomen, arms and legs. Diagnosis is usually difficult, and must combine medical history, semiology and imaging, though only. Desmoid tumors of the chest wall european journal of cardio. Desmoid tumors are usually considered benign not cancer because they rarely spread to different parts of your body. Complications attributable to the desmoid were commonly detectable on ct hydronephrosis occurred in 36% and smallbowel obstruction in 20%. 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 tumors can arise in virtually any part of the body. Given their benign nature, is major debulking surgery justified, or is it worth administering chemotherapy for a disease process which unusually defies common teaching and.
Desmoid tumors, characterized by aggressive local infiltration of surrounding tissues, are uncommon benign neoplasms with no metastatic potential, that occasionally may attain large size. 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. Epidemiology, risk factors, molecular pathogenesis, clinical presentation, diagnosis, and local therapy, section on familial adenomatous polyposis and gardner syndrome. 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. Background desmoid tumors aggressive fibromatosis are rare soft tissue. These tumors, also called aggressive fibromatosis, are a type of soft tissue sarcoma and are considered benign because they typically dont spread to. This paper analyzes recent evidence pertaining to the. Combining surgery with rt yielded a higher lo cal control rate. 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 probability of survival free of local recurrence is calculated for three years, five years, and seven years after surgery. Desmoid tumors are rare, affecting an estimated 1 to 2 per 500,000 people worldwide. Tumors are also called neoplasms, which means that they are composed of new and actively growing tissue.
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