Preoperative evaluation included abdominal ultrasound, magnetic resonance. Imaging of intra and extraabdominal desmoid tumors. Unlike a fatty tumor that grows under your skin called a lipoma, this kind of tumor develops in the fibrous tissue that makes up your tendons and ligaments. Tumor desmoide intraabdominal cirugia espanola elsevier.
The myofibroblast is the cell responsible for the desmoid tumor. A metastasis of the ovariantumor, a lymphoma or a sarcoma was discussed as differential diagnosis. Genetics home reference ghr contains information on desmoid tumor. Giant desmoid tumor of the abdominal wall in a patient with gardner syndrome. Superficial desmoids tend to be less aggressive than deep desmoids abdominal, extra abdominal, mesenteric. We report on two female patients with desmoid tumor of the abdominal wall who underwent primary resection. Review diagnosis and management of desmoid tumors and. Some tumors dont grow, and some even get smaller on their own. Desmoid tumoursaggressive fibromatosis dtaf are infrequent softtissue neoplasms. Introduction the first case of desmoid tumor aggressive fibromatosis was described by mcfarlane 1832 1, who reported the disease occurring in the anterior abdominal wall in a young woman after childbirth. A classic presentation of desmoid includes a postpartum woman with an abdominal wall mass. Extraabdominal periosteal desmoid tumor of the third toe. If youre diagnosed with a desmoid tumor, your doctor will recommend one of the following.
Development of desmoids is most commonly associated with genetic predisposition, pregnancy, hormonal exposure, and physical factors such as trauma andor surgery 6, 7. Desmoid tumors are rare lesions without any metastatic potential but a strong tendency to invade locally and to recur. Most commonly used for intraabdominal tumors and associated complications like small bowl obstruction soft tissue mass with well to poorly defined margins, variable attenuation and mild to moderate enhancement mri. Desmoid tumors are slow growing deep fibromatoses with aggressive infiltration of adjacent tissue but without any metastatic potential. An ethmoidal desmoid tumor has been described in a. Aggressive fibromatosis is a rare condition marked by the presence of desmoid tumors. Tumors that form in the abdominal wall are called abdominal desmoid tumors. 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. A desmoid tumor rarely metastasizes spreads to other parts of the body. These tumors are associated with women of fertile age, especially during and after pregnancy. The mrt demonstrated the abdominal wall tumor originating from the abdominal transversal muscle and internal abdominal oblique muscle fascia.
Rarely, a desmoid tumor may develop during or soon after. The term desmoid, coined by johannes mueller in 1838 8 was used to describe its tendonlike consistency. Desmoids associated with fap and gardner syndrome are typically in traabdominal, usually located in the small. The patient underwent a surgical biopsy with the histology and the immunohistochemical staining confirmed the diagnosis of a desmoid tumor. Desmoid tumors are responsible for 9 to 11 percent of deaths among patients with fap. These tumors tend to occur in women in their thirties, but can occur in anyone at any age. Bennet first pointed out its histologic features 1. Both patients had a history of an earlier abdominal surgery. 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.
Tumors are also called neoplasms, which means that they are composed of new and actively growing tissue. The role of local treatment is limited and only a few drugs have shown activity. The severe mutilations of the abdominal wall require different techniques of repair, often combining plastic procedures and the use of prosthetic material. Desmoid tumor genetic and rare diseases information. Desmoid definition of desmoid by medical dictionary. Desmoid tumors most often happen in the abdomen, arms and legs. They are benign but locally aggressive, and wide local surgical excision is the treatment of choice owing to the high rate of recurrence in the lower extremities.
The diagnosis of desmoid tumor should be considered in patients with an abdominal mass, a history of abdominal surgery or injury, or gardner syndrome. A nodule or relatively large mass of unusually firm. Desmoid tumors are neoplasms of the conjunctive tissue that are characterized by exclusive locoregional growth, frequent recurrence and minimal metastatic potential. Intraabdominal presentations can be associated with mass effect, intestinalobstruction,ormucosalischemia. Intraabdominal and abdominal wall desmoid fibromatosis. 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. Rbcp aggressive fibromatosis desmoid tumor associated. Fappatientsoftenpresent with concomitant intraabdominal and abdominal wall disease 17. 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.
Symptoms of intestinal, vascular, ureteric, or neural involvement may be the initial manifestations. Desmoid tumor is a rare soft tissues neoplasia characterized by local invasiveness and by a tendency towards local recurrence although not towards metastasization. This type is more common in individuals with familial adenomatous polyposis fap, a genetic condition that leads to the development of colon cancer. Segun su localizacion, podemos dividirlos en 2 grupos. People with familial adenomatous polyposis fap have an increased risk of desmoid tumors. They mainly affect individuals with familial adenomatous polyposis of the colon, and. This article will focus on the abdominal presentation of this fibroblastic tumor. The terms desmoid tumor and aggressive fibromatosis are occasionally used synonymously by some authors 9. Direttore nicola picardi desmoid tumor of the abdominal wall.
The treatment of these infiltrating and recurrent connective tissue tumors can only be surgical, by means of wide parietal excision. Desmoid tumors, also known as aggressive fibromatosis, are uncommon mesenchymal neoplasms. Td extrabdominales miembros, tronco, cabeza y cuello, pared toracicaabdominal y td. Giant desmoid tumor of the abdominal wall in a patient. Noncytotoxic drug therapy for intraabdominal desmoid tumor in patients with familial adenomatous polyposis. Desmoid tumors in children are most commonly diagnosed between ages 15 and 16.
At sonography, desmoids have variable echogenicity, with smooth, welldefined margins. Abdominal desmoid tumor is a type of desmoid tumor that involves the abdominal wall. A tumor of the tissue that surrounds muscles, usually in the abdomen. However, they may grow locally infiltrating or compressing adjacent structures. Tumores desmoides abdominales en las paredes del abdomen vientre. Desmoid tumors occur in up to 20 percent of people with familial adenomatous polyposis of the colon. Typically, a single tumor develops, although some people have multiple tumors. For that reason, desmoids are not extensively discussed in the literature. Gardner syndrome gs is a rare entity characterized by a triad of familial colonic polyposis, multiple osteomas and soft tissue tumors, including desmoid tumor dt. There are even instances of spontaneous regression. Preferred modality for abdominal wall and extraabdominal tumors. We report the outcome of two patients affected by progressive dtaf treated with the angiogenesis. In various studies, 2869% of desmoids were intraabdominal mesenteric or pelvic or abdominal wall, and the remaining were extraabdominal 7. Also called aggressive fibromatosis, especially when the tumor is outside the abdomen.
Desmoid tumors are cytologically bland fibrous neoplasms originating from the musculoaponeurotic structures throughout the body. The lump gradually increased in size and an ultrasound taken at 24 weeks of gestation revealed a welldefined hypoechoic lesion measuring 8. The majority of cases of abdominal wall desmoid tumor are sporadic in nature, but there are several associated factors. This article describes a case of an extraabdominal desmoid tumor occurring in the third toe of a healthy adult woman with no medical or familial predisposing history. Pazopanib is an active treatment in desmoid tumour. The desmoid tumor research foundation web site offers information on desmoid tumors. Lowdose chemotherapy with vinblastine and methotrexate in childhood desmoid tumors. However, tumor invasion into vital structures andor organs can result in substantial morbidity and may be fatal, especially in the case of intraabdominal desmoids, particularly those arising in patients with familial adenomatous polyposis fap. Desmoid tumor symptoms differ based on where the tumors occur. Treatment for a tumor depends upon its location and characteristics, as well as the patients preferences. The authors report 7 cases of desmoid tumors of the abdominal wall treated between 1975 and 1996. Desmoid tumor, first described by macfarlane in 1832 7, is a locally invasive but histologically benign dense fibrous neoplasm arising from and within the fascial sheaths of striated muscle.
Desmoid tumors originate from the monoclonal proliferation of. Desmoid tumor definition of desmoid tumor by medical. The mayo clinic web site provides further information on desmoid tumor. Otherwellcharacterized,thoughrare,sitesincludetheheadandneck region18andthebreast19. The ct revealed an ovariantumor, combined with a right abdominal wall tumor of unknown dignity. Fap is caused by a gene mutation that can be passed down from parents to children. A pregnancyassociated desmoid tumor is an even less common disease very rarely reported in published articles. Race and ethnic background do not seem to affect whether a person gets these tumors.