Dermatofibrosarcoma Protuberans (DFSP)

Dermatofibrosarcoma Protuberans??

Pernah x pembaca mendengar tentang penyakit ini? Jarang2 sekali kita mendengar isu yg dibangkitkan mengenai penyakit ini, tambahan pula nama saintifiknya sangat panjang dan sukar untuk diingati malah disebut. Namun, sedikit masa dulu nama yg unik ini sungguh sinonim dgn penulis walaupun penulis sendiri tidak berapa mengatahui asal usul serta simptom2 nya. Baru2 ini penulis tergerak hati untuk mengatahu dgn lebih lanjut tentang penyakit ini dan bebesar hati untuk berkongsi informasi dgn para pembaca.

Dermatofibrosarcoma protuberans (DFSP) is a relatively uncommon soft tissue neoplasm with intermediate-to-low grade malignancy. Although metastasis rarely occurs, DFSP is a locally aggressive tumor with a high recurrence rate. Maknanya, tumor jenis ini jarang merebak tapi berpotensi untuk tumbuh kembali selepas pembedahan dan apabila 'metastasis' berlaku, this cancerous cell berpotensi untuk merebak from original site to one or more sites elsewhere in the body. DFSP is a very slowly growing tumor. Because of the slow progression, the diagnosis is often delayed dan tumor ini sering kali berlaku kepada golongan dewasa yang berumur 20-25 tahun. Tambahan pula, tumor jenis ini tidak mengenali jantina mahupun bangsa. It may start as a small asymptomatic papule, which is likely ignored. The tumor may gradually enlarge into a lumpy nodule, or it may evolve into an atrophic and/or sclerotic plaque.

DFSP biasanya agak besar and presents as a large indurated plaque several centimeters in diameter. It is composed of firm, irregular nodules varying in color from flesh to reddish brown. Sometimes, it may present as a morphealike, atrophic, sclerotic, violaceous plaque without nodularity that may ulcerate as it slowly increases in size. DFSP most commonly occurs on the trunk, followed by the proximal extremities. It rarely occurs above the neck. Although metastasis of DFSP is rare (only 1-4% reported), almost all metastatic cases have been associated with local recurrence and a poor prognosis. Most of the patients with metastatic DFSP have died within 2 years. A small subset of DFSP patients presents with fibrosarcomatous progression. This fibrosarcomatous progression DFSP variant is more aggressive in nature and the clinical outcome usually is poor.

Tumor jenis ini jarang 2 berlaku dan mengikut statistic, 3 dripada setuja manusia berpotensi untuk menjadi pesakit. DFSP is a cutaneous malignancy that arises from the dermis and invades deeper subcutaneous tissue (eg, fat, fascia, muscle, bone). Bagaimanapun, punca kepada penyakit ini masih tidak dikenalpasti. It was reported that in 10-20% of patients with this tumor, trauma at the site seems to be incriminated. Surgical and old burn scars and sites of vaccinations have all been reported as sites of DFSP.

Treatment?

a) Radiation therapy (Radiotherapy) has had a limited role in the past, but, recently, it has been used as an adjunct to surgery. Radiation therapy may be recommended for patients if the margins of resection are positive or for situations where adequate wide excision alone may result in major cosmetic or functional deficits. Close follow-up care after radiation therapy is warranted because some of the tumors may become more aggressive.

b) Imatinib mesylate (molecularly targeted therapy) has been found to have significant therapeutic value in the treatment of DFSP. Imatinib is a potent and specific inhibitor of several protein-tyrosine kinases, including the PDGF receptors. Imatinib mesylate is indicated for the treatment of adult patients with unresectable, recurrent, and/or metastatic DFSP and the recommended dose is 800 mg/d.

c) Surgical care such as Surgical excision remains the mainstay of treatment for DFSP. Recently, Mohs micrographic surgery has been increasingly accepted as the treatment of choice. a wide excision of 3 cm or more of the margins, down to and including the fascia, is recommended for the treatment of DFSP. Ini kerana, cancer cell tersebut berpotensi untuk membiak dgn lebih agresif lagi apabila ada sebahagian akar cell yg tertinggal tambahn pula DFSP commonly extends far beyond the clinical margins.

Uish.. cuba para pembaca teliti gambar yg penulis selitkan bersama entri ni. Nampak mcm tahi lalat dan lebiahan daging biasa. Sapa tahu rupa2nya bende2 mcm tu boley membawa kemudaratan. Dari info yg penulis baca, tumor jenis ni mgkin xlah berapa membahayakan tp manala tau kalo2 tidak bernasib baik boleh memudaratkan nyawa. So, sentiasalah alert dgn segala perubahan yg berlaku kat badan kita.

Thank You~