Göğüs Duvarının Desmoid Tip Tümörlerin İncelenmesi: Tek Merkez Deneyimi

Yıl 2025, Cilt: 7 Sayı: 2, 198 - 204, 23.06.2025

Kadri Ceberut , Burak Hasgül

https://doi.org/10.52827/hititmedj.1610954

Öz

Amaç: Göğüs duvarı desmoid tipi tümörleri nadir görülür ve yüksek nüks oranları nedeniyle yönetimlerinde önemli zorluklar oluşturur. Bu çalışmanın amacı, bu tümörlerle ilgili klinik deneyimlerimizi aktarmak ve ilgili literatürü gözden geçirmektir.
Gereç ve Yöntem: Gaziosmanpaşa Üniversitesi Tıp Fakültesi’ nde 2007-2022 yılları arasında ameliyat edilen ve histopatolojik olarak desmoid tipi göğüs duvarı tümörü tanısı almış hastalar retrospektif olarak incelendi. Tedavide tüm hastalara cerrahi uygulandı ve iki hastaya ikinci nüksünden sonra radyoterapi verildi. Demografik bulgular, klinik özellikler, cerrahi yöntemler, lokal nüksler ve takip kriterleri kaydedildi. Gardner sendromunu dışlamak için tüm hastalara gastroskopi ve kolonoskopi yapıldı.
Bulgular: Çalışmamızdaki hastaların beşi kadın ve biri erkekti. Çalışma grubunun yaş aralığı 14 ile 76 yaş aralığındaydı. Nüks oranı %33,3 olarak hesaplandı. İki hastada üçer kez tümör nüksü görüldü. Bu hastalara ikinci ve üçüncü nüksleri arasında radyoterapi uygulandı. Bir hasta, tedavi ve takip sürecinde miyokard enfarktüsü nedeniyle hayatını kaybetti. Diğer hastalarda herhangi bir majör komplikasyon izlenmedi, tüm hastalar sağ olarak halen takiptedir.
Sonuç: Tedavideki birincil hedef mümkün olduğunca geniş bir rezeksiyon yapılması olmalıdır. Ancak bu tek başına nüksleri öngörmek veya önlemek için yeterli değildir. Desmoid tip tümörlerin yönetimi için hastaların yakın takibi önemlidir.

Anahtar Kelimeler

Agresif fibromatozis , desmoid tümörler , epidemiyoloji , torasik cerrahi , yumuşak doku tümörü

Etik Beyan

Bu çalışma Tokat Gaziosmanpaşa Üniversitesi Tıp Fakültesi Klinik Araştırmalar Etik Kurulu tarafından onaylandı (Onay No: 22-Kaek-096).

Evaluation of Desmoid-type Chest Wall Tumors: A Single Center Experience

Öz

Objective: While chest wall desmoid-type tumors are rare, they do pose significant challenges in their management due to their high rate of recurrence. The purpose of this study is to evaluate our clinical experience of these tumors while reviewing the relevant literature.
Materials and Methods: A retrospective study was conducted of patients diagnosed with desmoid-type chest wall tumors who underwent surgery at Gaziosmanpasa University Faculty of Medicine between 2007 and 2022. While all patients underwent surgical management, two patients received radiotherapy after their second recurrence. Demographic findings, clinical features, operative methods, local recurrences, and follow-up criteria were recorded as part of the study. Gastroscopies and colonoscopies were performed on all patients to exclude Gardner syndrome.
Results: Five of the patients in our study were female and one was male, which represented 16.6% of the group. The age range of the study group was between 14 and 76 years old. The recurrence rate was 33.3%, with two patients experiencing three tumor recurrences who received radiotherapy between the second and third recurrences. One patient, who still had a tumor, died due to a myocardial infarction. We did not observe any major complications in the other patients. All of the patients under follow-up are still alive.
Conclusion: While the primary goal of the treatment should be to perform as wide a resection as possible, this is insufficient for the prediction or prevention of recurrences, and close follow-up is necessary for the management of locally invasive tumors.

Anahtar Kelimeler

Aggressive fibromatosis , desmoid tumors , epidemiology , soft-tissue tumor , thoracic surgery

Destekleyen Kurum

YOKTUR

Kaynakça

• Zehani-Kassar A, Ayadi-Kaddour A, Marghli A et al. Desmoid-type chest wall fibromatosis. A six cases series. Orthop Traumatol Surg Res 2011;97(1):102-107.
• Kabiri EH, Al Aziz S, El Maslout A, Benosman A. Desmoid tumors of the chest wall. Eur J Cardiothorac Surg 2001 May;19(5):580-583.
• Zisis C, Dountsis A, Nikolaides A, Dahabreh J. Desmoid Tumors of the Chest Wall. Asian Cardiovascular and Thoracic Annals 2006;14(5):359-362.
• Abbas AE, Deschamps C, Cassivi SD et al. Chest-wall desmoid tumors: results of surgical intervention. Ann Thorac Surg 2004 Oct;78(4):1219-1223.
• Napolitano A, Mazzocca A, Spalato Ceruso M et al. Recent Advances in Desmoid Tumor Therapy. Cancers (Basel) 2020 Aug 1;12(8):2135.
• Mangla A, Agarwal N, Schwartz G. Desmoid Tumors: Current Perspective and Treatment. Curr Treat Options Oncol 2024 Feb;25(2):161-175.
• Sakamoto K, Okita M, Takeuchi K, Nishida S, Mikami Y, Hayashi Y. Desmoid tumor of the chest wall infiltrating into the brachial plexus: report of a resected case. Kyobu Geka 2001 Feb;54(2):160-163.
• Choi KA, Yeong YA. Desmoid tumor of the chest wall mimicking recurrent breast cancer: multimodality imaging findings. Iran J Radiol 2016;13:e31649.
• Douira-Khomsi W, Mascard E, Adamsbaum C. Une masse pariétale lombaire chez un enfant [A lumbar cutaneous mass in a child]. Arch Pediatr 2009;16(9):1295-1297.
• Abrão FC, Waisberg DR, Fernandez A et al. Desmoid tumors of the chest wall: Surgical challenges and possible risk factors. Clinics (Sao Paulo) 2011;66:705–708.
• Lev D, Kotilingan D, Wei C et al. Optimizing treatment of desmoid tumors. J Clin Oncol 2007;25:1785–1791.
• Ma D, Li S, Fu R et al. Long-term outcomes of 47 patients with aggressive fibromatosis of the chest treated with surgery. Eur J Surg Oncol 2016 Nov;42(11):1693-1698.
• Shin SH, Ko KR, Cho SK, Choi YL, Seo SW. Surgical outcome of desmoid tumors: adjuvant radiotherapy delayed the recurrence, but did not affect long-term outcomes. J Surg Oncol 2013;108(1):28-33.
• Ballo MT, Zagars GK, Pollack A, Pisters PW, Pollack RA. Desmoid tumor: prognostic factors and outcome after surgery, radiation therapy, or combined surgery and radiation therapy. J Clin Oncol 1999 Jan;17(1):158-167.
• Xiao Y, He J, Gong C, Liu L, Huang S. Desmoid-type fibromatosis of the chest wall: a case report. Ann Transl Med 2020 Oct;8(20):1322.
• Goy BW, Lee SP, Fu YS, Selch MT, Eilber F. Treatment results of unresected or partially resected desmoid tumors. Am J Clin Oncol 1998 Dec;21(6):584-590.
• Tsuchishima S, Nishizawa H, Matsumoto Y, Kitagawa S, Shimizu T, Yuasa K. A desmoid tumor of the chest wall. Kyobu Geka 1998;51(12):1055-1059.
• Schlemmer M. Desmoid tumors and deep fibromatoses. Hematol Oncol Clin North Am 2005;19(3):565-571.
• Ganeshan D, Amini B, Nikolaidis P, Assing M, Vikram R. Current Update on Desmoid Fibromatosis. J Comput Assist Tomogr 2019;43(1):29-38.