Treatment of choice in Desmoid tumor?
|D||Wide excision + Radiotherapy|
a. Desmoid tumors are histologically benign fibrous neoplasms originating from the musculoaponeurotic structures throughout the body.
b. Desmoid tumors often appear as infiltrative, usually well-differentiated, firm overgrowths of fibrous tissue, and they are locally aggressive.
c. The synonym aggressive fibromatosis describes the marked cellularity and aggressive local behavior.
d. The familial polyposis gene on chromosome 5 has been extensively studied.
e. An endocrine etiology is suggested.
i. Desmoid tumors most commonly appear in young women during or after pregnancy.
ii. The tumors regress during menopause and after tamoxifen treatment.
iii. Desmoid tumors may regress after exposure to oral contraceptives.
a. Aggressive, wide surgical resection is the treatment of choice.
b. Complete surgical excision of desmoid tumors is the most effective method of cure.
c. Primary surgery with negative surgical margins is the most successful primary treatment modality for desmoid tumors. Positive margins after surgery reflect a high risk for recurrence.
d. In those patients who refuse surgery or are not surgical candidates the following options may be considered:
Radiation therapy may be used as a treatment of recurrent disease or as primary therapy to avoid mutilating surgical resection.
i. Pharmacologic therapy with antiestrogens and prostaglandin inhibitors may also be used.
ii. In cases of recurrent extra-abdominal desmoid tumors in which surgery is contraindicated or in cases of recurrence, a chemotherapeutic regimen of doxorubicin, dacarbazine, and carboplatin may be effective. Intra-abdominal desmoid tumors as a part of Gardener syndrome may respond to systemic doxorubicin, and ifosfamide can be useful for patients with complications from the tumor.