Yumuşak doku mikzofibrosarkomunun klinikopatolojik incelemesi

Yıl 2021, Cilt: 46 Sayı: 1, 116 - 124, 31.03.2021

Kivilcim Erdogan , Sevil Karabağ , Mehmet Ali Deveci , Alper Gamlı Gülfiliz Gönlüşen , Hilmi Serdar Özbarlas

Öz

Amaç: Bu çalışmada amaç, farklı mikzofibrosarkom vakalarının epidemiyolojik verileri yanı sıra patolojik özelliklerini vurgulayarak bu tümörlerin klinik gidişi ile ilişkisini değerlendirmektir.
Gereç ve Yöntem: Tek merkezli çalışmaya 22 mikzofibrosarkom olgusu dahil edilmiştir. Olguların yaş, cinsiyet, tümör yerleşimi, çapı, histopatolojik özellikleri yanı sıra tedavi yöntemleri, klinik takibi de araştırılmıştır. Patoloji değerlendirmesinde ayırıcı tanıya yönelik ek immünohistokimyasal çalışmalar yapılmıştır.
Bulgular: Bu seride ortalama yaş 54 olup kadın-erkek oranı eşittir. Ekstremiteler en sık yerleşim yeridir. Yüzeyel yerleşmiş tümörler, derin yerleşim gösterenlere kıyasla belirgin olarak iyi seyretmektedir. Patolojik değerlendirme sonucu verilen grade ile klinik gidiş yakın ilişkilidir. Ortalama rekürrens süresi 16 ay olduğu için olguların uzun dönem takibi gereklidir. Metastaz görülen tüm olgularda öncesinde rekürrens mevcuttur. Hastalıktan ölüm sadece yüksek dereceli olanlarda gerçekleşmiştir.
Sonuç: Sonuçta bu tümörler çok nadir görülmekle birlikte tanıda zorluk yaratması bakımından önem teşkil etmektedir. Histopatolojik olarak reaktif lezyonlarla bile karışabilmesi nedeniyle doğru tanı koymak hasta yönetiminde şarttır. Bu tümör grubunda bizim serimizde yüzeyel olanlar ve derecesi düşük olanlar çok iyi seyirli olup, doğru tanı ve tedavi ile bu hastaların iyi prognozlu da olabileceği literatüre katkı olarak sunulmuştur.

Anahtar Kelimeler

myxofibrosarcoma, soft tissue, pathology

Clinicalopathological evaluation of soft tissue myxofibrosarcoma

Öz

Purpose: The aim of this study is to evaluate the relationship between the clinical course of these tumors by emphasizing the epidemiological data as well as the pathological features of different myxofibrosarcoma cases.
Materials and Methods: 22 cases of myxofibrosarcoma were included in the single center study. Age, gender, tumor location, size, histopathological features as well as treatment modalities and clinical follow-up of the cases were also evaluated. Additional immunohistochemical studies were applied for the differential diagnosis in pathological evaluation.
Results: The mean age of the patients was 54 years old and there is no sex predilection. The extremities are the most common location. Deep located myxofibrosarcoma were highly aggressive unlike superficial counterparts. The grade given as a result of pathological examination is closely related to the clinical course. Since the mean recurrence period is 16 months, long term follow up is required. Recurrence is present in all metastatic cases. Death from the disease occurred only in those with a higher grade.
Conclusion: Myxofibrosarcomas are rarely seen but diagnostically challenging. Accurate diagnosis is essential to differentiate myxofibrosarcoma from other pleomorphic sarcomas, and to manage the treatment of patients since it may mimic even reactive lesions by histopathologically. Superficial and low grade myxofibrosarcoma of present study showed excellent prognosis and it has been presented as a contribution to the literature that these patients may have a good prognosis with correct diagnosis and treatment.

Kaynakça

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