What are the desmoid tumors?

What are the desmoid tumors? Desmoid benign tumors are histologically malignant fibrous structures musculoaponeurotice origins of the body. Desmoid is derived from the Greek term signifying desmos like tendons. Desmoid infiltrative tumors are often well differentiated, firm fibrous tissue proliferation, locally aggressive. Despite the benign histologic appearance and negligible metastatic potential desmoide tumors tend to cause local infiltration is significant, resulting in distortion, morbidity and mortality effects of compression and obstruction of vital organs.
Although desmoide tumors can occur in any skeletal muscle, most occur in the anterior abdominal wall. Peripheral skin tumors are firm, mobile and smooth. Surrounding skin is unaffected. The cause is uncertain but are recognized association with trauma or hormonal factors.
The indicated treatment is surgical excision. Radiation may be used for recurrent disease, antiestrogenii and prostaglandin inhibitors. It is also used to inhibit proliferation of tumor and anticancer. Local Recurrence is 70%.
Pathogenesis and causes
Although tumors occur most often desmoide straight abdominal muscles in postpartum women after abdominal scars from surgery, can occur in skeletal muscles. Tumors tend to infiltrate the muscle bundles forcing their degeneration. Although skin lesions are seen setting is normal. Miofibroblastul is considered responsible for tumor cell desmoide. Gradner syndrome or familial adenomatous polimatoza adenomatous colorectal polyps are characterized by neoplasms and soft tissue and hard. These may include hundreds or thousands. Desmoide tumors occur in 10% of these patients. These shows legacy APC gene defect.
Case desmoide tumors is uncertain and may be associated with trauma or hormonal factors, genetic or association. It suggests a genetic etiology: desmoide tumors occur mostly in young women during or after pregnancy, menopause and they regress after treatment with tamoxifen or exposure to oral contraceptives. The stability of the fibroblast proliferative response to estrogen.
Signs and symptoms
Desmoide tumors are common after delivery are twice as common in women than men and have an average age of getting between 10-40 years. Although desmoide tumors may arise from any skeletal muscle most often develops in the abdominal wall. Retroperitoneal neoplasms are more common in familial polyposis and Gardner syndrome abdominal soup. Whole families have been reported suffering from these tumors. History of trauma at the tumor is present in 1 in 4 cases.
Physical Exam Desmoide peripheral tumors: -Are firm, smooth and mobile -Often adhere to adjacent structures -Normal skin that covers them Desmoide-extra-abdominal tumors are rare.
Desmoide abdominal tumors: -They can be seen in the urological system, including bladder and scrotal Growth-remain asymptomatic until events cause compression and infiltration -Symptoms of neural damage, intestinal, vascular, ureter may be early manifestations -Pediatric patients have been described desmoide ethmoid tumors.
Diagnosis
Studies: -Imunotiparea with vimentin, alpha smooth muscle actin, desmina are helpful to differentiate tumor APC gene mutations-evaluation Magnetic resonance-computertomografia and are used to diagnose tumors -They can detect the extent and relationship with adjacent structures -Can make the needle biopsy of the tumor and electronic microscopic examination Gardner-in syndrome indicate colonoscopy.
Histological examination. Tumors are composed of collagen surrounding fibroblast cells. Cells shows multiple mitoses and atypical nuclei. Are present in peripheral macrophages, giant cells and lymphocytes. The differential diagnosis is made with the following conditions: Gardner syndrome, fibrosarcoma, familial polyposis of the colon.
Treatment
Primary surgery with negative margins is the first option. Positive margins are at increased risk of recurrence. In patients who refuse surgery or are not candidates for radiotherapy can choose to treat recurrent disease, pharmacologic therapy with antiestrogen and prostaglandin inhibitors. In case of recurrent extra-abdominal tumors in which surgery is contraindicated indicate a chemotherapy regimen with doxorubicin, and caboplatin decarbazina. Desmoide abdominal tumors as part of Gardner syndrome may respond to systemic doxorubicin and ifosfamide.