Benign+Fibromatosis

Desmoid tumor: Keloid: Peyronie’s disease: Plantar fibromatosis: Keloid: Peyronie’s disease: Plantar fibromatosis: If the tumor is more to the surface a mass or lump may present as a smooth uniform mass. || A low-grade, locally invasive, nonmetastasizing tumor of connective tissue. [4] || A diagnosis of fibromatosis is benign and non-metastasizing.[4] || **Keloid tumors** usually do not need treatment. They, however, can be reduced in size by: [7] **Benign superficial plantar Fibromatosis** may regress naturally without treatment. [5] []. ||
 * **Epidemiolgy:** || Fibromatosis includes desmoid tumor, also known as aggressive fibromatosis, keloids, Peyronie’s disease, plantar and palmar fibromatosis.
 * Accounts for 0.03% of all neoplasms
 * Women after childbirth
 * Twice as common in females than in male
 * More common in persons aged 10-40 years [1]
 * Greater frequency in blacks, Hispanics and Asians
 * As high as 16% prevalence in black and Hispanic population
 * Formation approximately 15 times greater in highly pigmented ethnic groups than in whites [1]
 * Relatively common, develops in 25% of middle-aged or eldery persons
 * Whites affected more than other groups
 * Affects men 10 times more than women [1] ||
 * **Etiology:** || Desmoid tumor:
 * Commonly arise from the rectus abdominis muscle in postpartum women and in scars due to abdominal surgery
 * Can arise in skeletal muscle
 * May be derived from mesenchymal cells
 * Occur at a rate of 10-15% in patients with familial adenomatous polyposis (FAP) [1]
 * Tissue trauma
 * Infection and excessive wound tension
 * Genetic factors (most recently overexpression of DeltNp63 and underexpression of p3) [1]
 * Cause unknown
 * May be a connective tissue disorder similar to Dupuytren’s contracture [1]
 * Repeated trauma
 * Long-term alcohol consumption
 * Chronic liver disease
 * Diabetes
 * Epilepsy
 * Dupuytren contracture
 * Knuckle pads
 * Peyronie’s disease [1] ||
 * ** Signs & Symptoms: ** || Desmoid tumors can develop anywhere in the body and are typically painless presenting as a lump in the tendons and ligaments of the body. Once these tumors “push” into neighboring structures these symptoms may occur
 * obstruction of bowels (if in abdomen)
 * Pain/soreness in muscles (compression of nerves)
 * Limping (if in legs or thighs)
 * **Diagnostic Procedures:** || After a medical exam the physician can order several diagnostic evaluations to better stage and identify the tumor.
 * Tissue biopsy (most conclusive)
 * MRI
 * CT scan
 * X-rays
 * APC mutational analysis ||
 * **Histology:** || Low-power view of fibromatosis infiltrating skeletal muscle and fat. [4]
 * **Lymph node drainage:** || Tumors are usually localized but occasionally spread to local lymph nodes (location of lymph node drainage depends on tumor location).[4] ||
 * **Metastatic spread:** || A low-grade, locally invasive, nonmetastasizing tumor of connective tissue[4]
 * **Grading:** || Grading involves analyzing the cellular structure of a lesion to determine the abnormality and rate of growth of the tissues envolved . A tumors grade effects the decision to treat or not and what options may be available for the patient. Grading also effect the predicted prognosis of the patient. Since a benign diagnosis is not cancerous, grading is not available.[2] ||
 * **Staging:** || Staging describes the extent or severity of the tumor and spread. This included the primary tumor site, size, nodal involvement, cell grade, and metastasis. Benign lesions are not cancerous and no staging is available.[2] ||
 * **Radiation side effects:** || Uninvolved anatomical structures should be shielded to prevent damage or malignacy. For Peyronie’s disease, the gonads, pubic hair and glans should be shielded if not involved to prevent damage.[3] ||
 * **Prognosis:** || Fibromatosis has a favorable prognosis, although slow progression is not uncommon. [5] ||
 * **Treatments:** || **Desmoid tumors** primary treatment option is surgery. Extensive cases may require surgery plus chemotherapy, and repeat surgery. [6]
 * Corticosteroid injections
 * Freezing (cryotherapy)
 * Laser treatments
 * Radiation
 * Surgical removal
 * Peyronie's disease ** can be treated with radiation therapy. The penis can be placed through a hole in a lead sheet, so there is careful shielding of the gonads. A single dorsal field can be used. The dose that is given is 5 Gy in 1 fraction, which can be repeated in 1 month, to 3 Gy daily for 6 or 7 fractions. [3]
 * **TD 5/5:** || Desmoid tumors, Keloid tumors, and Plantar Fibromatosis are not treated with radiation. In the case of Peyronie's disease the dose limit they would be interested in would be testes spermatogonia, which could lead to sterility after 2 Gy. [3] ||
 * **References:** || # Drugs, Diseases & Procedures. Medscape. http://emedicine.medscape.com/article/. Accessed June 27, 2012.
 * 1) Cancer staging/ grading//.// Available at: []. Accessed on July 2,2012.
 * 2) Chao KS, Perez CA, Brady LW. Radiation Oncology: Management Decisions. Philadelphia, PA: Lippincott Williams & Wilkins; 2002.
 * 3) Fibromatosis. September 2007. Available at:[]. Accessed on: July 5, 2012.
 * 4) Plantar Fibromatosis Follow-up. Medscape. Available at: []. Accessed July 2, 2012.
 * 5) Desmoid Tumor Treatment & Management. Medscape. Available at: [|http://emedicine.medscape.com/article/1060887-treatment#showall]. Accessed July 2, 2012.
 * 6) Keloids. PubMed Health. Available at: []. Accessed July 2, 2012. ||

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