Aggressive Fibromatosis Involving the Maxilla, Case Report and Review of the Literature

Öz

Desmoid tumors (aggressive fibromatosis) are nonmalignant neoplasms with locally aggressive growth, but clinically don’t display any metastatic potential. In head and neck region desmoids are very rare and described to have a high tendency for recurrance following surgery ranging between 46% and 62%. A little is reported about clinical features and outcome of desmoid tumors of the head and neck region. The aim of this case report is to review the literature on desmoid tumors in the oral region and to evaluate the diagnosis and treatment methods. A 17-year-old male patient was admitted to our clinic with the complaint of slowly growing, painless mass on the posterior region of the maxilla presetented for 2 months. Medical history was not contrubitory. Clinical examination revealed a firm, regionally ulcerated painless mass on the left premolar and molar (corresponding the vestibule of teeth 24-26) region of the maxilla. After total excision with 5 mm safety margins (function-protective surgery), the lesion was sent to the pathology laboratory and diagnosed as desmoid tumor (Aggressive Fibromatosis). Adjuvant radiotherapy was applied following the operation. There was no evidence of recurrence in the postoperative 10 year follow-up and healing was uneventful. Few cases have been reported about clinical features and treatment outcomes of desmoid tumors of the head and neck. Many alternatives have been described in the treatment of desmoid tumors and no common treatment strategy has been uniformly agreed upon. Although surgical and oncotherapy combinations are often successful in the treatment of these tumors, more case reviews are needed to obtain definitive results in their treatment.

Anahtar Kelimeler

• Desmoid tumor; aggressive fibromatosis; maxilla; radiotherapy.

Maksillada Görülen Agresif Fibromatozis, Vaka Sunumu ve Literatür İncelemesi

Öz

Desmoid tümörler (agresif fibromatozis) lokal agresif büyüme gösteren benign neoplazmlardır ve klinik olarak metastatik potansiyel göstermemektedirler. Baş ve boyun bölgesinde desmoid tümörler çok nadir görülmekte olup, ameliyat sonrası %46 ile %62 arasında değişen yüksek nüks eğilimi gösterdikleri tanımlanmıştır. Baş ve boyundaki desmoid tümörlerin klinik özellikleri ve sonuçları hakkında çok az vaka bildirilmiştir. Bu olgu sunumunun amacı günümüze kadar oral bölgede görülen desmoid tümörlerin literatür taraması ve olgu sunumuyla teşhis ve tedavi yöntemlerinin tartışılmasıdır. 17 yaşında erkek hasta kliniğimize üst çene sol arka bölgede 2 aydır iyileşmeyen, yavaş gelişen ağrısız şişlik şikayetiyle başvurmuştur. Alınan anamnezde hastanın herhangi bir sistemik rahatsızlığı olmadığı öğrenilmiştir. Yapılan klinik muayenede maksilla sol premolar molar bölgede (24-26 nolu dişlerin vestibülünde) mukozadan kabarık, sert, üzerinde yer yer ülserasyon gözlenen ağrısız lezyonun varlığı tespit edilmiştir. 5 mm'lik güvenlik sınırlarıyla (fonksiyon koruyucu cerrahi yöntemiyle) yapılan total eksizyon sonrasında lezyon patoloji laboratuvarına gönderilip gelen rapor doğrultusunda Desmoid Tümör (Agresif Fibromatozis) teşhisi konulmuştur. Ameliyata ek radyoterapi uygulanmıştır. Ameliyat sonrası 10 yıllık takipte nüks bulgusu yoktur ve ilgili bölge tamamen iyileşmiştir. Baş ve boyundaki desmoid tümörlerin klinik özellikleri ve tedavi sonuçları hakkında çok az vaka bildirilmiştir. Desmoid tümörlerin tedavisinde birçok seçenek tanımlanmış olup, ortak bir tedavi stratejisine varılamamıştır. Bu tümörlerin tedavilerinde çoğu zaman cerrahi ve onkoterapi kombinasyonları ile başarılı sonuçlar elde edilse de tedavilerinde kesin sonuçlar çıkarmak için daha çok vaka derlemelerinin yapılması gerekmektedir.

Anahtar Kelimeler

• Desmoid tümör
• agresif fibromatozis
• maksilla
• radyoterapi

Kaynakça

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