Aynı Kitle İçerisinde Dermatofibrosarkom Protuberans Ve Miksofibrosarkom, Çok Nadir Olgu Sunumu

Year 2021, Volume: 11 Issue: 1, 94 - 99, 01.04.2021

Elif Gökçe Devecioğlu Nazlı Sena Şeker

Abstract

Biz aynı kitle içerisinde dermatofibrosarkom protuberans ve orta dereceli miksofibrosarkom birlikteliğinin izlendiği çok nadir bir kompozit tümör vakası sunmaktayız. Vakamız, 58 yaşında kadın hastaya ait, sağ göğüs altında yıllardır mevcut olan, son zamanlarda büyüme gösteren deri üzerinde protrüde kitledir. Makroskopik olarak yan yana olan her iki tümör bileşeninin de birbiri ile devamlılığı mevcut olup, subkutan dokuya kadar uzanmaktadır. İki tümör arasında mikroskobik olarak da belirgin bağlantı alanları görülmüştür. Dermatofibrosarkom protuberans vakalarında fibrosarkom transformasyonu yanı sıra nadiren pleomorfik sarkom transformasyonu gösteren olgularda bildirilmektedir. Bizim olgumuzda aynı tümör içerisinde iki ayrı tümörün izlenmesi dermatofibrosarkom protuberanslar vakalarının daha önce literatürde bildirilmemiş miksofibrosarkoma da dönüşebileceğini düşündürmektedir.

Keywords

Dermatofibrosarkom prıtuberans, miksofibrosarkom, mezenkimal tümör

Dermatofibrosarcoma Protuberans And Myxofibrosarcoma In The Same Mass, Very Rare Case Report

Abstract

We present a very rare case of composite tumor consisting of dermatofibrosarcoma protuberans and intermediate grade myxofibrosarcoma with in the same mass. Our case is a protruded mass on the skin, which has been present for years under the right chest of a 58-year-old female patient, and has been growing recently. Both tumor components, which are adjacent to each other macroscopically, have continuity with each other and extend to the subcutaneous tissue. Microscopically, distinct connection areas were also observed between them. Besides known fibrosarcoma transformation of dermatofibrosarcoma protuberans, cases that are rarely transformed into pleomorphic sarcoma are known. However, in our case, the existing of two different tumors with in the same mass suggests that dermatofibrosarcoma protuberans may also transform into a myxofibrosarcoma, which has not been previously reported in the literature.

Keywords

Dermatofibrosarcoma precuberance, myxofibrosarcoma, mesenchymal tumor

References

• 1. Madden C, Spector A, Siddiqui S, Mirkin G, Yim J, Hao X. Dermatofibrosarcoma Protuberans on Adult Toes: A Case Report and Review of the Literature. Anticancer Research, 2019. 39(4): p. 2105-2111.
• 2. BrooksJ, Ramsey ML. Dermatofibrosarcoma Protuberans. StatPearls; 2020
• 3. Llombart B, Serra-Guillén C, Monteagudo C, López Guerrero JA, Sanmartín O. Dermatofibrosarcoma protuberans: a comprehensive review and update on diagnosis and management. Semin Diagn Pathol. 2013 Feb;30(1):13-28.
• 4. Mentzel T, Schärer L, Kazakov DV, Michal M. Myxoid dermatofibrosarcoma protuberans: clinicopathologic, immunohistochemical, and molecular analysis of eight cases. Am J Dermatopathol. 2007 Oct;29(5):443-8.
• 5. Nakamura I, Kariya Y, Okada E, Yasuda M, Matori S, Ishikawa O, et al. A novel chromosomal translocation associated with COL1A2-PDGFB gene fusion in dermatofibrosarcoma protuberans: PDGF expression as a new diagnostic tool. JAMA Dermatol. 2015 Dec 1;151(12):1330-1337.
• 6. Lopez LV, Yatsenko SA, Burgess M, Schoedel K, Rao UNM. Dermatofibrosarcoma protuberans with fibrosarcomatous transformation: our experience, molecular evaluation of selected cases, and short literature review. Int J Dermatol. 2019 Nov;58(11):1246-1252.
• 7. Llombart B, Serra C, Requena C, Alsina M, Morgado-Carrasco D, Través V, et al. Guidelines for Diagnosis and Treatment of Cutaneous Sarcomas: Dermatofibrosarcoma Protuberans. Actas Dermosifiliogr. 2018 Dec;109(10):868-877.
• 8. Fletcher CD, Mertens F, Unni K. World Health Organization classification of tumours: pathology and genetics tumours of soft tissue and bone. In: Fletcher CDM, Bridge JA, Hogendoorn PCW, Mertens; 2002: IARC Publications.
• 9. Fletcher CD, Mertens F, Unni K. World Health Organization classification of tumours: pathology and genetics tumours of soft tissue and bone. In: Fletcher CDM, Bridge JA, Hogendoorn PCW, Mertens; 2002: 102-104
• 10. Qiubei Z, Cheng L, Yaping X, Shunzhang L, Jingping F. Myxofibrosarcoma of the sinus piriformis: case report and literature review. World J Surg Oncol. 2012 Nov 15;10:245.
• 11. Willems SM, Debiec-Rychter M, Szuhai K, Hogendoorn PC, Sciot R. Local recurrence of myxofibrosarcoma is associated with increase in tumour grade and cytogenetic aberrations, suggesting a multistep tumour progression model. Mod Pathol. 2006 Mar;19(3):407-16.
• 12. Trojani M, Contesso G, Coindre JM, Rouesse J, Bui NB, de Mascarel A, et al. Soft‐tissue sarcomas of adults; study of pathological prognostic variables and definition of a histopathological grading system. Int J Cancer. 1984 Jan 15;33(1):37-42.
• 13. 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 Apr;20(4):391-405.
• 14. Wiszniewska J, Roy A, Masand RP. Myxoid dermatofibrosarcoma protuberans of the vulva: case report of a rare variant in an unusual location, with unusual morphologic and immunohistochemical features. Am J Dermatopathol. 2016 Mar;38(3):226-30.
• 15. Szollosi Z, Nemes Z. Transformed dermatofibrosarcoma protuberans: a clinicopathological study of eight cases. J Clin Pathol. 2005 Jul;58(7):751-6.
• 16. Voth H, Landsberg J, Hinz T, Wenzel J, Bieber T, Reinhard G, et al. Management of dermatofibrosarcoma protuberans with fibrosarcomatous transformation: an evidence‐based review of the literature. J Eur Acad Dermatol Venereol. 2011 Dec;25(12):1385-91.
• 17. Swaby MG, Evans HL, Fletcher CD, Prieto VG, Patel KU, Lev DC, et al, Dermatofibrosarcoma protuberans with unusual sarcomatous transformation: a series of 4 cases with molecular confirmation. Am J Dermatopathol. 2011 Jun;33(4):354-60.
• 18. Terrier-Lacombe MJ, Guillou L, Maire G, Terrier P, Vince DR, de Saint Aubain Somerhausen N, et al., Dermatofibrosarcoma protuberans, giant cell fibroblastoma, and hybrid lesions in children: clinicopathologic comparative analysis of 28 cases with molecular data: a study from the French Federation of Cancer Centers Sarcoma Group. Am J Surg Pathol. 2003 Jan;27(1):27-39.
• 19. Warbrick-Smith J, Hollowood K, Birch J. Dermatofibrosarcoma protuberans recurring as a hybrid dermatofibrosarcoma/giant cell fibroblastoma in an adult: A case report. J Plast Reconstr Aesthet Surg. 2010 Nov;63(11):e785-7.
• 20. Frierson HF, Cooper PH. Myxoid variant of dermatofibrosarcoma protuberans. Am J Surg Pathol. 1983 Jul;7(5):445-50.
• 21. Reimann JD, Fletcher CD. Myxoid dermatofibrosarcoma protuberans: a rare variant analyzed in a series of 23 cases. Am J Surg Pathol. 2007 Sep;31(9):1371-7.
• 22. Smith SC, Poznanski AA, Fullen DR, Ma L, McHugh JB, Lucas DR, et al. CD34‐positive superficial myxofibrosarcoma: a potential diagnostic pitfall. J Cutan Pathol. 2013 Jul;40(7):639-45.
• 23. Zámečník M, Michal M, Mukenšnábl P. Composite tumor consisting of dermatofibrosarcoma protuberans and myxoflbrosarcoma. J Cutan Pathol. 1998 Sep;25(8):445-9.