What is the treatment of choice in desmoid tumors ?
Types of desmoid tumors Peripheral desmoid tumors o Peripheral desmoid tumors are firm, smooth, and mobile. o They often adhere to surrounding structures. The overlying skin is usually unaffected. L Intra-abdominal and extra-abdominal desmoid tumors o Intra-abdominal desmoid tumors remain asymptomatic until their growth and infiltration cause visceral compression. Symptoms of intestinal, vascular, ureteric, or neural involvement may be the initial manifestations. L Breast desmoid tumors o Desmoid tumors account for 0.2% of primary breast tumors, developing from muscular fasciae and aponeuroses. o Desmoid tumors may mimic breast cancer. Treatment Radiation therapy may be used as a treatment for recurrent disease or as primary therapy to avoid mutilating surgical resection. It may be used postoperatively, preoperatively, or as the sole treatment. -- Pharmacologic therapy with antiestrogens and prostaglandin inhibitors may also be used. -- chemotherapeutic regimen: Recurrent extra-abdominal: doxorubicin, dacarbazine, and carboplatin may be effective Intra abdominal: May respond to systemic doxorubicin, and ifosfamide Differential diagnosis Fibrosarcoma: atypia or mitotic figures present L GIST: strong CD117+, CD34+ b Idiopathic retroperitoneal fibrosis inflammatory, strangles the ureters Leiomvoma: bright pink cytoplasm of smooth muscle, desmin+ L Low grade fibromvxoid sarcoma: heavily collagenized stroma with abrupt transition to myxoid areas, often epithelioid areas or poorly formed but large collagen rosettes; beta catenin negative Neurofibroma: no myofibroblasts, S100+ Schwannoma: palisading Schwann cells, usually minimal collagen, S100+ L Sclerosing omentitis: grows like panniculitis, beta catenin negative Wide excision Ref : Bailey and love surgery text book 27th Ed.
Irradiation
Wide excision
Local excision
Local excision following radiation
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