Olgu Sunumu
BibTex RIS Kaynak Göster

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

Yıl 2020, Ağız Kanserleri Özel Sayısı, 187 - 193, 28.09.2020

Murat Kaan Erdem Cengiz Evli Mehmet Eray Kolsuz Erdal Erdem

https://doi.org/10.20515/otd.769055

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.

Kaynakça

  • Seper L, Bürger H, Vormoor J, et al. Agressive fibromatosis involving the mandible-case report and review of the literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod, 2005;99:30-38.
  • Kruse AL, Luebbers HT, Gratz KW, et al. Aggressive fibromatosis of the head and neck: a new classification based on a literature review. Oral Maxillofac Surg. 2010;14:227-232.
  • Said-Al-Naief N, Fernandes R, Louis P, et al. Desmoplastic fibroma of the jaws. Oral Surg Oral Med Oral Pathol 2006;101:82-94.
  • Günhan Ö. (2015) Oral ve maksillofasiyal patoloji, Ankara: Quintessence Publishing, p. 257-259.
  • Hoos A, Lewis JJ, Urist MJ, et al. Desmoid tumors of the head and neck – a clinical study of a rare entity. Head &Neck 2000; 22(8):814-21
  • Abdelkader M, Riad M, Williams A. Aggressive fibromatosis of the head and neck (desmoid tumours) The Journal of Laryngology & Otology 2001;115 (10):772-6
  • Suresh CS, Ali AA. Desmoid tumor of the tongue Med Oral Patol Oral Cir Bucal. 2008;13(12):E761-4
  • Richter M, Chausse JM. Infrequent tumors of the mandible. removal and reconstruction. Acta Stomatol Belg. 1989;86(4):265-74
  • Angiero F, Benedicenti S, Stefani M. Fibromatosis of the head and neck: morphological, immunohistochemical and clinical features. Anticancer Research, 2008;28:1725-32.
  • MacKenzie DH. The fibromatoses: a clinicopathological concept. British Medical Journal, 1972;4:277-81.
  • Pinheiro LV, Fagundes JJ, Coy CS, et al. Multiple desmoid tumors in a patient with Gardner's syndrome. report of a case. Int J Surg Case Rep. 2014;5(7):370–374.
  • Hu T, Jing G, Lv K. Aggressive fibromatosis in the maxilla. Br J Oral Maxillofac Surg. 2009;47(2):129-31.
  • Ballo MT, Zagars GK, Pollack A, et al. Desmoid tumor: prognostic factors and outcome after surgery, radiation therapy, or combined surgery and radiation therapy. J Clin Oncol. 1999;17:158–167.
  • Watzinger F, Turhani D, Wutzl A, et al. Aggressive fibromatosis of the mandible: a case report. Int J. Oral Maxillofac. Surg. 2005; 34:211-213.
  • Bataini JP, Belloir C, Mazabraud A, et al. Desmoid tumors in adults:the role of radiotheraphy in their management.The American Journal of Surgery 1988;155(6):754-60
  • Suit HD. Radiation dose and response of desmoids tumors. Int J Radiat Oncol Biol Phys. 1990;19(1):37-40
  • Zlotecki RA, Scarborough MT, Morris CG, et al. External beam radiotheraphy for primary and adjuvant management of aggressive fibromatosis. Int J Radiat Oncol Biol Phys. 2002;54:177-181
  • Sherman NE, Romsdahl M, Evans H, et al. Desmoid tumors: a 20-year radiotheraphy experience. Int j Radiat Oncol Biol Phys. 1990;19(1):37-40
  • Mc Collough WM, Parson JT, Van der Griend R, et al. Radiation therapy for aggressive fibromatosis. J Bone Joint Surg Am. 1991;73(5) 717-725.
  • Nuyttens JJ, Rust PF, Thomas CR Jr, Turrisi AT III. Surgery versus radiation therapy for patients with aggressive fibromatosis or desmoid tumors: a comparative review of 22 articles. Cancer 2000; 88: 1517–1523. 
  • Tullio A, Sesenna E, Raffaini M. Aggressive juvenile fibromatosis. Minerva Stomatol. 1990;39:7777–81
  • Kasper B, Baumgarten C, Garcia J, et al. An update on the management of sporadic desmoid-type fibromatosis: a European Consensus Initiative between Sarcoma Patients EuroNet (SPAEN) and European Organization for Research and Treatment of Cancer (EORTC)/Soft Tissue and Bone Sarcoma Group (STBSG). Ann Oncol. 2017;28(10):2399–2408.

Maksillada Görülen Agresif Fibromatozis, Vaka Sunumu ve Literatür İncelemesi

Yıl 2020, Ağız Kanserleri Özel Sayısı, 187 - 193, 28.09.2020

Murat Kaan Erdem Cengiz Evli Mehmet Eray Kolsuz Erdal Erdem

https://doi.org/10.20515/otd.769055

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

  • Seper L, Bürger H, Vormoor J, et al. Agressive fibromatosis involving the mandible-case report and review of the literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod, 2005;99:30-38.
  • Kruse AL, Luebbers HT, Gratz KW, et al. Aggressive fibromatosis of the head and neck: a new classification based on a literature review. Oral Maxillofac Surg. 2010;14:227-232.
  • Said-Al-Naief N, Fernandes R, Louis P, et al. Desmoplastic fibroma of the jaws. Oral Surg Oral Med Oral Pathol 2006;101:82-94.
  • Günhan Ö. (2015) Oral ve maksillofasiyal patoloji, Ankara: Quintessence Publishing, p. 257-259.
  • Hoos A, Lewis JJ, Urist MJ, et al. Desmoid tumors of the head and neck – a clinical study of a rare entity. Head &Neck 2000; 22(8):814-21
  • Abdelkader M, Riad M, Williams A. Aggressive fibromatosis of the head and neck (desmoid tumours) The Journal of Laryngology & Otology 2001;115 (10):772-6
  • Suresh CS, Ali AA. Desmoid tumor of the tongue Med Oral Patol Oral Cir Bucal. 2008;13(12):E761-4
  • Richter M, Chausse JM. Infrequent tumors of the mandible. removal and reconstruction. Acta Stomatol Belg. 1989;86(4):265-74
  • Angiero F, Benedicenti S, Stefani M. Fibromatosis of the head and neck: morphological, immunohistochemical and clinical features. Anticancer Research, 2008;28:1725-32.
  • MacKenzie DH. The fibromatoses: a clinicopathological concept. British Medical Journal, 1972;4:277-81.
  • Pinheiro LV, Fagundes JJ, Coy CS, et al. Multiple desmoid tumors in a patient with Gardner's syndrome. report of a case. Int J Surg Case Rep. 2014;5(7):370–374.
  • Hu T, Jing G, Lv K. Aggressive fibromatosis in the maxilla. Br J Oral Maxillofac Surg. 2009;47(2):129-31.
  • Ballo MT, Zagars GK, Pollack A, et al. Desmoid tumor: prognostic factors and outcome after surgery, radiation therapy, or combined surgery and radiation therapy. J Clin Oncol. 1999;17:158–167.
  • Watzinger F, Turhani D, Wutzl A, et al. Aggressive fibromatosis of the mandible: a case report. Int J. Oral Maxillofac. Surg. 2005; 34:211-213.
  • Bataini JP, Belloir C, Mazabraud A, et al. Desmoid tumors in adults:the role of radiotheraphy in their management.The American Journal of Surgery 1988;155(6):754-60
  • Suit HD. Radiation dose and response of desmoids tumors. Int J Radiat Oncol Biol Phys. 1990;19(1):37-40
  • Zlotecki RA, Scarborough MT, Morris CG, et al. External beam radiotheraphy for primary and adjuvant management of aggressive fibromatosis. Int J Radiat Oncol Biol Phys. 2002;54:177-181
  • Sherman NE, Romsdahl M, Evans H, et al. Desmoid tumors: a 20-year radiotheraphy experience. Int j Radiat Oncol Biol Phys. 1990;19(1):37-40
  • Mc Collough WM, Parson JT, Van der Griend R, et al. Radiation therapy for aggressive fibromatosis. J Bone Joint Surg Am. 1991;73(5) 717-725.
  • Nuyttens JJ, Rust PF, Thomas CR Jr, Turrisi AT III. Surgery versus radiation therapy for patients with aggressive fibromatosis or desmoid tumors: a comparative review of 22 articles. Cancer 2000; 88: 1517–1523. 
  • Tullio A, Sesenna E, Raffaini M. Aggressive juvenile fibromatosis. Minerva Stomatol. 1990;39:7777–81
  • Kasper B, Baumgarten C, Garcia J, et al. An update on the management of sporadic desmoid-type fibromatosis: a European Consensus Initiative between Sarcoma Patients EuroNet (SPAEN) and European Organization for Research and Treatment of Cancer (EORTC)/Soft Tissue and Bone Sarcoma Group (STBSG). Ann Oncol. 2017;28(10):2399–2408.
Toplam 22 adet kaynakça vardır.

Ayrıntılar

Birincil Dil Türkçe
Konular Sağlık Kurumları Yönetimi
Bölüm OLGU SUNUMLARI / CASE REPORTS
Yazarlar

Murat Kaan Erdem 0000-0001-7852-3758

Cengiz Evli 0000-0003-4391-2408

Mehmet Eray Kolsuz 0000-0001-8872-1897

Erdal Erdem 0000-0001-8131-4519

Yayımlanma Tarihi 28 Eylül 2020
Yayımlandığı Sayı Yıl 2020 Ağız Kanserleri Özel Sayısı

Kaynak Göster

Vancouver Erdem MK, Evli C, Kolsuz ME, Erdem E. Maksillada Görülen Agresif Fibromatozis, Vaka Sunumu ve Literatür İncelemesi. Osmangazi Tıp Dergisi. 2020;42(5):187-93.


13299        13308       13306       13305    13307  1330126978