Fibromatozis tümör serimizin dağılımı ve patolojik özellikleri
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Date
2013
Authors
Altıntaş, Emine Kıyım
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Publisher
Uludağ Üniversitesi
Abstract
Fibromatozisler kökeni fibroblast olan, yüzeyel ve derin dokulardan kaynaklanan, çoğunlukla erişkinlerde görülen, nüks oranı yüksek, biyolojik davranışı benign, patogenezi tam olarak aydınlatılamamış mezenkimal yumuşak doku tümörleridir. Fibromatozislerin etiyolojisinde kesin olmamakla birlikte travma, genetik, endokrin ve fiziksel faktörlerin rol aldığı düşünülmektedir. Diabetik komplikasyonlarla ilişkili olarak gelişebilir. Enzinger ve Weiss sınıflamasında, köken aldıkları dokuya göre yüzeyel (fasyadan kaynaklanan) ve derin (muskuloaponörotik dokudan kaynaklanan) olmak üzere başlıca 2 gruba ayrılır. Derin fibromatozisler, yüzeyel fibromatozislerden daha nadir görülürler. Çalışmanın amacı; fibromatozis tanısı almış olguların dağılımı ve patolojik özelliklerinin literatür bulguları eşliğinde değerlendirilmesidir. Çalışmaya dahil edilen fibromatozis tanılı toplam 125 olgunun 90?ı yüzeyel, 35?i derin fibromatozis vakasından oluşmaktadır. Yüzeyel fibromatozislerin 84?ü palmar, 6?sı plantar, derin fibromatozislerin ise 25?si ekstra-abdominal, 6?sı abdominal, 4?ü intra-abdominal fibromatozis vakasından oluşmaktaydı. Hastaların yaş ortalaması 50,4±17,4 yıl (aralık 10-84 yaş) olarak bulundu. Palmar fibromatozisli 84 olgunun 43?ü 5. ve 6. dekatlardaydı. Yüzyirmibeş olgunun 80?i erkek, 45?i kadındı. Yüzeyel fibromatozisli 16 hasta aynı zamanda diabet hastasıydı. Palmar fibromatozisli 5, derin fibromatozisli 8 olguda travma öyküsü mevcuttu. Gardner Sendromu?yla ilişkili olduğunu düşündüğümüz daha öncesinde FAP nedeniyle opere olmuş 2 kadın hastamız mevcuttu. Yüzeyel fibromatozislerde ortalama tümör çapı 3,4±1,5 cm iken derin fibromatozislerde 8,8±5,4 cm ölçüldü. Palmar fibromatozisli 84 olgunun 12?sinde, plantar fibromatozisli 5 olgunun 1?inde ve derin yerleşimli 35 fibromatozis olgusunun 9?unda nüks saptandı. Sonuç olarak tüm bulgular bir bütün halinde değerlendirildiğinde; fibromatozis tümör serimizin dağılımının ve patolojik özelliklerinin genelde literatür ile uyumlu olmasına karşın daha uzun süreli ve geniş hasta popülasyonlarında ileri çalışmaların yararlı olacağı kanısına varıldı.
Fibromatoses are benign mesenchymal soft tissue tumors which originate from the fibroblasts in the superficial and deep soft tissues, that have unknown etiopathogenesis. They generally ocur in adults. Trauma, genetic, and endocrinologic hormonal changes (i.e. with diabetes as a complication) were suspected in the etiology. In Enzinger-Weiss classification fibromatoses evaluated in two main groups according to the location: Superficial fibromatosis, which originate from fascia and, deep fibromatosis which are seen rarely and originate from musculoaponeurotic tissues. The aim of this study is to investigate the distirution of fibromatosis cases and their pathological features and review of the findings with the literature. Of the 125 fibromatoses in our study, 90 were superficial located, and 35 were deep located. Of the 90 superficial fibromatoses, 84 (67,2%) were palmar fibromatosis, 6 (4,8%) were plantar fibromatosis. Of the 35 deep fibromatosis, they were located in extra-abdominal in 25 (20%) patients, abdominal in 6 (4,8%) patients, and intra-abdominal in 4 (3,2%) patients. The mean age of the patients was 50,4 ±17,4 (range 10-84 years). Of 125 patients, 80 (64%) were male and 45 (36%) were female. There was co-existent diabetes mellitus in 16 (19%) cases with superficial fibromatosis. There was a reported trauma history in 5 patients with palmar fibromatosis and 8 (22,8%) patients with deep fibromatosis, retrospectively. 2 of the patient underwent surgery because of FAP which were probably related to Gardner syndrome. The mean tumor diameter in those with superficial fibromatosis and deep fibromatosis were 3,4±1,5 and 8,8±5,4 cm, respectively. In our study, we observed recurrences in 12 patients (14,3%) with palmar fibromatosis and 1 patient (20%) with plantar fibromatosis had recurrences, while 9 (25,7%) out of 35 patients with deep fibromatosis had recurrences. In conclusion, despite the main pathological characteristics and distribution of fibromatosis cases are compatible with the literature, future studies are needed to wide-range of patient population and in a long follow-up period.
Fibromatoses are benign mesenchymal soft tissue tumors which originate from the fibroblasts in the superficial and deep soft tissues, that have unknown etiopathogenesis. They generally ocur in adults. Trauma, genetic, and endocrinologic hormonal changes (i.e. with diabetes as a complication) were suspected in the etiology. In Enzinger-Weiss classification fibromatoses evaluated in two main groups according to the location: Superficial fibromatosis, which originate from fascia and, deep fibromatosis which are seen rarely and originate from musculoaponeurotic tissues. The aim of this study is to investigate the distirution of fibromatosis cases and their pathological features and review of the findings with the literature. Of the 125 fibromatoses in our study, 90 were superficial located, and 35 were deep located. Of the 90 superficial fibromatoses, 84 (67,2%) were palmar fibromatosis, 6 (4,8%) were plantar fibromatosis. Of the 35 deep fibromatosis, they were located in extra-abdominal in 25 (20%) patients, abdominal in 6 (4,8%) patients, and intra-abdominal in 4 (3,2%) patients. The mean age of the patients was 50,4 ±17,4 (range 10-84 years). Of 125 patients, 80 (64%) were male and 45 (36%) were female. There was co-existent diabetes mellitus in 16 (19%) cases with superficial fibromatosis. There was a reported trauma history in 5 patients with palmar fibromatosis and 8 (22,8%) patients with deep fibromatosis, retrospectively. 2 of the patient underwent surgery because of FAP which were probably related to Gardner syndrome. The mean tumor diameter in those with superficial fibromatosis and deep fibromatosis were 3,4±1,5 and 8,8±5,4 cm, respectively. In our study, we observed recurrences in 12 patients (14,3%) with palmar fibromatosis and 1 patient (20%) with plantar fibromatosis had recurrences, while 9 (25,7%) out of 35 patients with deep fibromatosis had recurrences. In conclusion, despite the main pathological characteristics and distribution of fibromatosis cases are compatible with the literature, future studies are needed to wide-range of patient population and in a long follow-up period.
Description
Keywords
Fibromatozis, Yüzeyel, Derin, Nüks, Fibromatosis, Superficial, Deep, Recurrence
Citation
Altıntaş, E. K. (2013). Fibromatozis tümör serimizin dağılımı ve patolojik özellikleri. Yayınlanmamış uzmanlık tezi. Uludağ Üniversitesi Tıp Fakültesi.