Kivilcim ERDOGAN, Sevil KARABAĞ, Mehmet Ali DEVECİ, Alper GAMLI, Gülfiliz GÖNLÜŞEN, Hilmi Serdar ÖZBARLAS

Yumuşak doku mikzofibrosarkomunun klinikopatolojik incelemesi

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

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.

PDF

___

1. Lurkin A, Ducimetiere F, Vince DR, Decouvelaere AV, Cellier D, Gilly FN et al. Epidemiological evaluation of concordance between initial diagnosis and central pathology review in a comprehensive and prospective series of sarcoma patients in the RhoneAlpes region. BMC Cancer. 2010;10:150.
2. Weiss SW, Enzinger FM. Myxoid variant of malignant fibrous histiocytoma. Cancer. 1977;39:1672–85.
3. WHO Classification of Tumours Editorial Board. Soft Tissue and Bone tumors. 5th edition, IARC Press, Lyon 2020.
4. Sambri A, Bianchi G, Righi A, Ferrari C, Donati D. Surgical margins do not affect prognosis in high grade myxofibrosarcoma. EJSO. 2016;42:1042–8.
5. Baheti AD, Tirumani SH, Rosenthal MH, Howard SA, Shinagare AB, Ramaiya NH et al. Myxoid softtissue neoplasms: comprehensive update of the taxonomy and MRI features. AJR Am J Roentgenol. 2015;204:374 -85.
6. Gronchi A, Lo Vullo S, Colombo C, Collini P, Stacchiotti S, Mariani L et al. Extremity soft tissue sarcoma in a series of patients treated at a single institution: local control directlyimpacts survival. Ann Surg. 2010;251:506-11.
7. Fletcher CD, Gustafson P, Rydholm A, Willen H, Akerman M. Clinicopathologic re-evaluation of 100 malignant fibrous histiocytomas: prognostic relevance of subclassification. J Clin Oncol. 2001;19:3045–50.
8. Mentzel T, van den Berg E, Molenaar W. Myxofibrosarcoma in: Fletcher C, Unni K, Mertens F, editors. WHO classification of tumors-pathology and genetics, tumors of soft tissue and bone. Lyon: IARC Press. 2002;102-3.
9. Fletcher CDM, Hogendoorm PCW, Mertens F, Bridge J, eds. WHO Classification of Tumors of Soft Tissue and Bone. Lyon, France: IARC Press: 2013.
10. Odei B, Rwigema JC, Eilber FR, Eilber FC, Selch M, Singh A et al. Predictors of local recurrence in patients with myxofibrosarcoma. Am J Clin Oncol. 2018;41:827-31.
11. Dei Tos, Angelo Paolo. Soft Tissue Sarcomas: A Pattern Based Approach to Diagnosis. London, Cambridge Press, 2018.
12. Val-Bernal JF, García-González MR, Mayorga M, Marrero RH, Jorge-Pérez N. Primary renal myxofibrosarcoma. Pathol Res Pract. 2015;211:619- 24.
13. Buccoliero AM, Castiglione F, Garbini F, Degl’Innocenti DR, Moncini D, Franchi A et al. Primary cerebral myxofibrosarcoma: clinical, morphologic, immunohistochemical, molecular, and ultrastructural study of an infrequent tumor in an extraordinary localization. J Pediatr Hematol Oncol .2011;33:e279–83.
14. Udaka T, Yamamoto H, Shiomori T, Fujimura T, Suzuki H. Myxofibrosarcoma of the neck. J Laryngol Otol. 2006,120:872-4.
15. Prasad I, Sharan R. Myxofibrosarcoma of larynx. Indian J Otolaryngol Head Neck Surg. 1981;33:71-71.
16. Qiubei Z, Cheng L, Yaping X, Shunzhang L, Jingpin F. Myxofibrosarcoma of the sinus piriformis: case report and literature review. World J Surg Oncol. 2012;15:245.
17. Nomura T, Sakakibara S, Moriwaki A, Kawamoto T, Suzuki S, Ishimura T et al. Low-grade myxofibrosarcoma of the rectus abdominus muscle infiltrating into abdominal cavity: a case report. Eplasty. 2017;21:17:e6.
18. Tjarks BJ, Ko JS, Billings SD. Myxofibrosarcoma of unusual sites. J Cutan Pathol. 2018;45:104-110.
19. Coindre JM, Grading and staging of sarcomas. In: Fletcher CDM, Bridge JA, Hogendoorn PCW, Mertens F (editors.), World Health Organization Classification of Tumors of Soft Tissue and Bone, Lyon, IARC Press, 2013;17–8.
20. Oda Y, Takahira T, Kawaguchi K, Yamamoto H, Tamiya S, Matsuda S et al. Low-grade fibromyxoid sarcoma versus low-grade myxofibrosarcoma in the extremities and trunk. A comparison of clinicopathological and immunohistochemical features. Histopathology. 2004;45:29-38.
21. Delaney D, Diss TC, Presneau N, Hing S, Berisha F, Idowu BD et al. GNAS1 mutations occur more commonly than previously thought in intramuscular myxoma. Mod Pathol. 2009;22:718–24.
22. de Saint Aubain Somerhausen N, Rubin BP, Fletcher CD. Myxoid solitary fibrous tumor: a study of seven cases with emphasis on differential diagnosis. Mod Pathol. 1999;12:463-71.
23. Billings SD, Giblen G, Fanburg-Smith JC. Superficial low-grade fibromyxoid sarcoma (Evans tumor): a clinicopathologic analysis of 19 cases with a unique observation in the pediatric population. Am J Surg Pathol. 2005;29:204–10.
24. Beane JD, Yang JC, White D, Steinberg S, Rosenberg S, Rudloff U. Efficacy of adjuvant radiation therapy in the treatment of soft tissue sarcoma of the extremity 20-year follow-up of a randomized prospective trial. Ann Surg Oncol. 2014;21:2484–9.
25. Roland CL,Wang WL,Lazar AJ, Torres KE. Myxofibrosarcoma. Surg Oncol Clin N Am. 2016;1- 14.
26. Haglund KE, Raut CP, Nascimento AF, Wang Q, George S, Baldini E. Recurrence patterns and survival for patients with intermediate- and high-grade myxofibrosarcoma. Int J Radiat Oncol Biol Phys. 2012;82:361–7.
27. Mutter RW, Singer S, Zhang Z, Brennan M, Alektiar K. The enigma of myxofibrosarcoma of the extremity. Cancer. 2012;118:518–27.
28. Gronchi A, Lo Vullo S, Colombo C, Collini P, Stacchiotti S, Mariani L et al. Extremity soft tissue sarcoma in a series of patients treated at a single institution: local control directlyimpacts survival. Ann Surg. 2010;251:506-11.
29. Lin CN, Chou SC, Li CF, Tsai KB, Chen WC, Hsiung CY et al. Prognostic factors of myxofibrosarcomas: implications of margin status, tumor necrosis, and mitotic rate on survival. J Surg Oncol .2006;93:294- 303.
30. Sanfilippo R, Miceli R, Grosso F, Fiore M, Puma E, Pennacchioli E et al. Myxofibrosarcoma: prognostic factors and survival in a series of patients treated at a single institution. Ann Surg Oncol. 2011;18:720–5.
31. Huang HY, Lal P, Qin J, Brennan MF, Antonescu CR. Low-grade myxofibrosarcoma: a clinicopathologic analysis of 49 cases treated at a single institution with simultaneous assessment of the efficacy of 3-tier and 4-tier grading systems. Hum Pathol. 2004; 35:612-21.
32. Mentzel T, Calonje E, Wadden C, Camplejohn RS, Beham A, Smith MA et al. Myxofibrosarcoma. Clinicopathologic analysis of 75 cases with emphasis on the low-grade variant. Am J Surg Pathol .1996;20:391–405.
33. Willems SM, Debiec-Rychter M, Szuhai K, Hogendoorn P, Sciot R. Local recurrence of myxofibrosarcoma isassociated with increase in tumour grade and cytogenetic aberrations, suggesting a multistep tumour progression model. Mod Pathol. 2006;19:407-16.
34. Fukunaga M, Fukunaga N. Low-grade myxofibrosarcoma: progressionin recurrence. Pathol Int .1997;47:161-5.
35. Riouallon G, Larousserie F, Pluot E, Anract P. Superficial myxofibrosarcoma: Assessment ofrecurrence risk according to the surgical margin following resection. A series of 21 patients. Orthop Traumatol Surg Res. 2013;99:473-7.
36. Nascimento AF, Bertoni F, Fletcher CD. Epithelioid variant of myxofibrosarcoma: expanding the clinicomorphologic spectrum of myxofibrosarcoma in a series of 17 cases. Am J Surg Pathol .2007;31:99– 105.
37. Scoccianti G, Ranucci V, Frenos F, Greto D, Beltrami G, Capanna R, et al. Soft tissue myxofibrosarcoma: A clinico-pathological analysis of a series of 75 patients with emphasis on the epithelioid variant. J Surg Oncol. 2016; 114:50-5.
38. Mentzel T, Calonge E, Wadden C, Camplejohn RS, Beham A, Smith MA, et al. Myxofibrosarcoma. Clinicopathologic analysis of 75 cases with emphasis on the low grade variant, Am. J. Surg. Pathol. 1996;20:391–405.
39. Dewan V, Darbyshire A, Sumathi V, Jeys L, Grimer R. Prognostic and survival factors in myxofibrosarcomas. Sarcoma. 2012;2012:830879.
40. Mühlhofer HML, Lenze U, Gersing A, Lallinger V, Burgkart R, Obermeier A et al. Prognostic factors and outcomes for patients with myxofibrosarcoma: a 13- year retrospective evaluation. Anticancer Res. 2019;39:2985-92.
41. Tsuchie H, Kaya M, Nagasawa H, Emori M, Murahashi Y, Mizushima E et al. Distant metastasis in patients with myxofibrosarcoma. Ups J Med Sci. 2017;122:190–3.
42. Lohberger B, Stuendl N, Leithner A, Rinner B, Sauer S, Kashofer K et al. Establishment of a novel cellular model for myxofibrosarcoma heterogeneity. Sci Rep. 2017;7:44700.
43. Mizuno T, Susa M, Horiuchi K, Shimazaki H, Nakanishi K, Chibaa K. Spontaneous regression of myxofibrosarcoma of the thigh after open biopsy. Case Rep Oncol. 2019;12:364–9.