Fibromatosis+(Benign)

The cause of desmoid tumors is uncertain and may be related to trauma or hormonal factors, or they may have a genetic association. -the familial polyposis gene on chromsome 5 has been extensively studied -an endocrine etiology is suggested; desmoid tumors most commonly appear in young women during or after pregnancy; the tumors regress during menopause and after tamoxifen treatment; desmoid tumors may regress after exposure to oral contraceptives -the proliferative response of fibroblasts to estrogen has been established. 2 The origin of desmoid tumors are probably related to other fibromatoses such as keloids, Peyronie's disease, plantar and palmar fibromatosis, fibromatosis coli, and progressive myositis fibrosa. 1 || -can arise in scars due to abdominal surgery -clusters of cases in families without evidence of any associated syndromes -a history of trauma (often surgical) to the site of the desmoid tumor. __Peripheral desmoid tumors__ are firm, smooth and mobile; they often adhere to surrounding structures; the overlying skin is usually unaffected; the presence of such a soft tissue growth should alert the clinician to delve more deeply into the family history for evidence of familial polyposis coli and Gardner syndrome; extra-abdominal desmoid tumors are rare and may be first evident as gradually increasing leg swelling. __Intra-abdominal and extra-abdominal desmoid turmors__ may be seen; extra-abdominal desmoid tumors may also be seen (rarely) in the urological system, including in the bladder and the scrotum; intra-abdominal desmoind tumors remain asymptomatic until their growth and infiltration cause visceral compression; symptoms of intestinal, vascular, ureteric or neural involvement may be the initial manifestations; an ethmoidal desmoid tumor has been described in a pediatric patient. 2 || -immunostaining with vimentin, alpha smooth muscle actin, muscle actin and desmin are helpful in distinguishing the tumors in the differential diagnosis of desmoid tumors. -APC mutational analysis -CT or MR scan -biopsy -electron microscopy 2 || been named “aggressive fibromatosis” for this disease. 5 || 50 to 55 Gy at 1.6 to 1.8 Gy/fraction, with wide margins. 1 ||
 * Epidemiology: || Benign fibromatosis (specifically desmoid tumors) account for 0.03% of all neoplasms. Occur most often in persons from 10-40 years of age. Incidence is twice as common in females than males. 2 ||
 * Etiology: || Desmoid tumor (aka aggressive fibromatosis):
 * Signs & Symptoms: || -Although desmoid tumors can arise in any skeletal muscle, they most commonly develop in the anterior abdominal wall and shoulder girdle.
 * Diagnostic Procedures: || The workup for desmoid tumors can include:
 * Histology: || Well-differentiated fibrous neoplasms 1 ||
 * Lymph Node Drainage: || These tumors are usually localized but occasionally spread to local lymph nodes (location of lymph node drainage depends on tumor location). 3 ||
 * Metastatic Spread: || A diagnosis of fibromatosis is benign and non-metastasizing. 1 ||
 * Grading: || A desmoid is a benign growth of connective tissue. Locally infiltrating growth has
 * Staging: || <span style="color: #ff0000; font-family: Arial,Helvetica,sans-serif; font-size: 10pt;">“Radiotherapy is indicated in cases of local inoperability.” <span style="color: #ff0000; font-family: Arial,Helvetica,sans-serif; font-size: 80%; vertical-align: super;">5 ||
 * Radiation Side Effects: || Acute side effects consist of erythema and skin irritation. Possible long-term side effects include secondary malignancy. 6 ||
 * Prognosis: || Prognosis is excellent. A study showed a recurrence rate of 8.3%, but these patients underwent salvage surgery and re-irradiation. Ultimately, the 10-year recurrence-free survival rate in this study was 100%. 6 ||
 * Treatments: || Surgery and Radiation Therapy
 * TD5/5: || Desmoid tumors may occur anywhere in the body. Sites of radiation treatment may include the head and neck, trunk, or extremities. The organs of interest depends on the location of the tumor. 6 ||
 * Planning Photos: || <span style="color: #008000; font-family: Arial,Helvetica,sans-serif;">The following images show the isodose distribution and PA and Left Lateral DRR's of an external beam radiation plan created for a diagnosis of fibromatosis (specifically a desmoid tumor) in the patient's upper back. <span style="color: #008000; font-family: Arial,Helvetica,sans-serif; font-size: 80%; vertical-align: super;">4

|| <span style="font-family: Arial,Helvetica,sans-serif;">1. Chao KS, Perez CA, Brady LW. //<span style="font-family: Arial,Helvetica,sans-serif;">Radiation Oncology - Management Decisions. // <span style="font-family: Arial,Helvetica,sans-serif;"> 2nd ed. Philadelphia: Lippincott, Williams & Wilkins, 2002: 677-688. 2. eMedicine. Desmoid Tumor. [] Accessed February 15, 2010. 3. CureSearch. Desmoid Tumors. [] Access February 15, 2010. <span style="font-family: Arial,Helvetica,sans-serif;">4. Digitally Reconstructed Radiographs courtesy of Bridget Keehan, RT(T), The Cancer Team at Bellin Health. 5. Gunderson and Tepper. Clinical Radiation Oncology. 2nd Edition. Philadelphia, PA: Churchill Livingstone. 2007. 6. Park H, Pyo H, Shin K, Suh C. Radiation Treatment for Aggressive Fibromatosis: Findings from Observed Patterns of Local Failure. //Oncology// [serial online]. May 2003;64(4):346-352. Available from: Academic Search Complete, Ipswich, MA. Accessed February 21, 2010.
 * References**