Gamze ERKILINÇ, Şirin BAŞPINAR, Sema BİRCAN, Sedat SOYUPEK, Alim KOŞAR

RENAL ANJİOMYOLİPOMLAR: 15 OLGUNUN ANALİZİ

Amaç Anjiomyolipom (AML) matür yağ dokusu, kalın duvarlı damar yapıları, düz kas dokusu ve melanin sentez leyen özel miyoid hücrelerin değişen oranlarda göz lendiği benign mezankimal bir tümördür. Bu makalede 2008-2018 yılları arasında bölümümüzde renal AML tanısı almış farklı klinik ve mikroskopik özellikleri olan olgular ile patolojide tanı koymada yararı olacak gün cel bilgileri sunmayı amaçladık. Gereç ve Yöntem Tıbbı Patoloji Anabilim Dalında 2008-2018 yılları ara sında formalin ile fikse edilmiş 4-5 mikron incelikte ke sitler ile incelenmiş 15 AML olgusu retrospektif olarak değerlendirilmiştir. Bulgular Onbeş olgunun 2’si erkek 13’ü kadın olup, yaşları 37- 74 (ortalama yaş 59±10) arasında değişmekteydi. Re nal AML’lerin boyutu 0,7 - 15 cm arasında olup ortala ması 5.1±3,2 cm idi. Olgulardan birinde tüberoskleroz olduğu tespit edildi. Yedi olguda kapsül bütünlüğünde kayıp ve bir olguda perforasyon ile uyumlu alanlar iz lendi. Mikroskobik incelemede 13 olguda matür yağ dokusu, kalın duvarlı damar yapıları ve iğsi epiteloid hücreler izlendi. Dört olgu yağ dokudan zengin, 1 olgu iğsi epiteloid hücrelerden zengin ve 3 olgu damar ya pılarından zengindi. Sonuç AML farklı klinik özellikler, mikroskopik ve makros kopik bulgular ile karakterize olan bir tümördür. İnsi dental olarak izlenebileceği gibi çok büyük boyutlara ulaşıp kanama ve perforasyon gibi komplikasyonlarla tespit edilebilir. Tüm komponentlerin her zaman birlik te bulunmayıp belirli komponentlerin baskın olabile ceğini akılda tutmak önemlidir.

RENAL ANGIOMYOLIPOMAS: ANALYSIS OF 15 CASES

Objective Angiomyolipoma (AML) is a benign mesenchymal tumor in which mature adipose tissue, thick walled vasculature, smooth muscle tissue and special myo id cells expressing melanin are observed in varying proportions. In this article, we aimed to present the patients with different clinical and microscopic featu res diagnosed with renal AML in our clinic between 2008-2018 and provide acurrent information in the diagnosis of AML. Material and Methods Formalin-fixed 4-5 micron thick sections of 15 AML cases which were diagnosed at SDÜ Department of Medical Pathology between 2008-2018 were retros pectively evaluated. Results Two of the 15 patients were male and 13 were female and their ages ranged from 37 to 74 years (mean59±10 years). Renal AMLs were between 0.7 and 15 cm in size and averaged 5.1±3,2 cm. One patient had tube rous sclerosis. In 7 cases, capsule integrity was lost and in one case perforation was observed. Microsco pic examination revealed mature adipose tissue, thick walled vein structures and spindle epithelioid cells in 13 cases. Four cases were rich in adipose tissue, 1 case was rich in spindle epithelioid cells and 3 cases were rich in vascular structures. Conclusion AML is a tumour which is characterized by different clinical features, microscopic and macroscopic findin gs. It can be observed incidentally, and can be detec ted by complications such as bleeding and perforation when it reaches a very large size. It is important to keep in mind that not all components can always co exist and that certain components can be dominant.

PDF

___

1. Ashebu SD, Dahniya MH, Elshebiny YH, Varro J, Al-khawari H. Giant bleeding renal angiomyolipoma: Diagnosis and management. Australasian Radiology 2002;46:115-8.
2. Victor E. Reuter, Paul B. Gaudin. Adult renal tumors. In Stenberg SS, editor. Diagnostic Surgical Pathology. 3 rd ed. Lippincott Williams and Wilkins 1999;1785-1824.
3. Nelson G. Ordonez, Rosai J. Urinary tract. In: Rosai j, Editor. Ackerman’s Surgical Pathology. 9 th ed. St Louis: Mosby 2004;1163-1359.
4. Behzatoğlu K, Dönmez M, Erdem Huq Gülben, Toktaş G. Tek böbrekte anjiomyolipom ve böbrek hücreli karsinom birlikteliği. İstanbul Tıp Dergisi 2005;4:43-46.
5. Morgan GS, Straumfjord JV, Hall EJ. Angiomyolipoma of the kidney. J Urol. 1951;65:525-7.
6. Kori C, Akhtar N, Vamsidhar P.N, Gupta S, Kumar V. Giant Exophytic Renal Angiomyolipoma Mimicking as Retroperitoneal Sarcoma; A Case Report with Review of Literature. J Clin Diagn Res. 2015; 9(4):1-2.
7. Hornick JL, Pan CC. “PEComa” in World Health Organization classification of tumours of soft tissue and bone, 4th, Fletcher CDM, Brodge JA, Hogendoom PCW, Mertens F (eds), IARC, Lyon 2013; 230.
8. Fittschen A, Wendlik I, Oeztuerk S, Kratzer W, Akinli AS, Haenle MM et al Prevalence of sporadic renal angiomyolipoma: a retrospective analysis of 61,389 in-and out-patients. AbdomImaging 2014;39:1009-1013.
9. Koo KC, Kim WT, Ham WS, Lee JS, Ju HJ, Choi YD. Trends of presentation and clinical out come of treated renal angiomyolipoma. Yonsei Med J2010;51:728-734.
10. Arslan B, Gürkan O, Çetin B, Arslan ÖA, Göv T, Yazıcı G et al. Evaluation of ABO blood groups and blood-based biomarkers as a predictor of growth kinetics of renal angiomyolipoma. International Urology and Nephrology 2018;2012-9.
11. Flum AS, Hamoui N, Said MA, Yang XJ, Casalino DD, McGuire BB et al. Update on the diagnosis and management of renal angiomyolipoma. J Urol 2016;195:834-846.
12. Goldblum JR, Folpe AL, Weiss S. Enzinger and Weiss’s Soft Tissue Tumors, 6th ed. China, Mosby; 2014.
13. Duran FY, Duran Ö, Öztürk U, Demir G, Kozacıoğlu Z, Gülden İ et al. Spontan Anjiomyolipom Rüptürüne Bağlı Akut Retroperitoneal Hematom: Olgu Sunumu. The Cystoscope 2015; 271- 273.
14. Başpınar Ş, Bircan S, Bozkurt KK, Yavuz G, Tuncer SS, Oksay T, Koşar A. Renal angiomyolipoma: Report of two cases. Süleyman Demirel Üniversitesi Yaşam Dergisi 2010; 2(2):04-08.
15. Martignoni G, Amin MB, Eble N, Sauter G, Epstein JI, Sesterhenn IA. “Angiomyolipoma” in World Health Organization Classification of Tumours: Pathology and Genetics of Tumours of the Urinary System and Male Genital Organs. IARC Press, Lyon, France, 2004;65–67.
16. Henske EP, Ao X, Short MP. Frequent progesterone receptor immunoreactivity in tuberous sclerosis-associated renal angiomyolipomas. ModPath1998;11:665-668.
17. Wang SF, Lo WO. Benign neoplasm of kidney: Angiomyolipoma. J Med Ultrasound 2018;26:119-22.
18. Çimen S, Ediz C, Taşdemir C, Coşkun Eİ. Sezeryan sonrası ağrı ve hayatı tehdit eden masif hemoraji ile prezente olan renal anjiomyolipom: olgu sunumu. Journal of contemporary medicine 2014;4:72-75.
19. Kontos S, Politis V, Fokitis I, Lefakis G, Koritsiadis G, Simaioforidis V, et al. Rapture of renal angiomyolipoma during pregnancy: A case report. Cases J 2008;1:245.
20. Kushwaha R, Dhawan I, Arora R, Gupta K, Dhupia JS. Multifokal renal angiomyolipoma presenting as massive intraabdominal hemorrhage. Indian J Pathol Microbiol 2010;53:340-341
21. Nelson CP, Sanda MG. Contemporary diagnosis and management of renal angiomyolipoma. J Urol 2002;168:1315-25.
22. Dickinson M, Ruckle H, Beaghler M. Renal angiomyolipoma: optimal treatment based on size and symptoms. Clin Nephrol 1998;49:281-6.
23. Winterkorn EB, Daouk GH, Anupindi S, Thiele EA. Tuberous sclerosis complex and renal angiomyolipoma: case report and review of the literature. Pediatr Nephrol 2006; 21: 1189-1193.
24. Bernstein J, Robbins TO. Renal involvement in tuberous sclerosis. Ann New York Acad Sci 1991;615:36-49.
25. Cox RM, MAGİ-Galluzzi C Jesse KM et al. Immunohistochemical pitfalls in genito urinary pathology: AdvAnatPathol. 2018;25:387-399.
26. Martignoni G, Bonetti F, Chilosi M, Brunelli M, Segala D, Amin BM et al. Cathepsin K expression in the spectrum of perivascular epithelioid cell (PEC) lesions of the kidney. Mod Pathol. 2012;25:100–111.
27. Asch-Kendrick RJ, Shetty S, Goldblum JR, Sharman R, Epstein JI, Argani P et al. A subset of fat-predominant angiomyolipomas label for MDM2: a potential diagnostic pitfall. Hum Pathol. 2016;57:7–12.
28. Korğalı E, Ayan S. Renal anjiomyolipomlara güncel yaklaşım. Üroonkoloji bülteni. 2010; 3.
29. Kavaney PB, Fielding I. Angiomyolipoma and renal cell carcinoma in same kidney. Urology 1975;6:643-646.
30. Dong A, Wang Y, Zuo C. Synchronous pure epithelioid angiomyolipoma of the kidney and retroperitoneal schwannoma in the same patient on 18F-FDG PET/CT imaging. Clin Nucl Med. 2014;39(1):106.
31. Makhlouf HR, Remotti HE, Ishak KG. Expression of C-KlT (CD117) in angiomyolipoma. Am J Surg Patbol 2002;26( 4 ):493-497.
32. Patil PA, McKenney JK, Trpkov K, Hes O, Montironi R, Scarpelli M, et al. Renal leiomyoma: a contemporary multi-institution study of an infrequent and frequently misclassified neoplasm. Am J Surg Pathol. 2015; 39:349–356.
33. Theodosopoulos T, Yiallourou A, Kyriazi M, Anastasopoulos G, Kairi-Vassilatou E, Dafnios N, et al. Unilateral simultaneous renal oncocytoma and angiomyolipoma: case report.Cases J. 2009;26;2:90-93.
34. Jun SY, Cho KJ, Kim CS, Ayala AG , Ro JY. Triple synchronous neoplasms in one kidney: report of a case and review of the literature. Ann Diagn Pathol. 2003;7(6):374-80.
35. Val-Bernal JF, Hernández-Nieto E, Garijo MF. Association of cystic nephroma with angiomyolipoma in the same kidney. MF Pathol Res Pract. 2000;196(8):583-8.
36. Prando A, Prando D, Prando P. Renal cell carcinoma: unusual imaging manifestations. Radiographics. 2006;26(1):233-44.
37. Ljungberg B, Bensalah K, Canfield S, Dabestani S, Hofmann F, Hora M et al EAU guidelines on renal cell carcinoma: EurUrol2014; 67(5):913–924.
38. Şen S, Sarkık B, Şimşir A, Kısmalı E, Gökmen E. Böbrekte kitle nedeniyle yapılan iğne kor biyopsileri ve tanı zorlukları. Türk Patoloji Dergisi 2009;25:5-18.
39. Jimenez H V. Coexistence between renal cell cancer and Hodgkin’s lymphoma: A rare coincidence. BMC Urol. 2006;6:10.
40. Sayın B, Yıldırım N, Dede D.Kabaçam G, Soydinç P, Bostanoğlu S. Dev Renal Anjiomyolipoma Rüptürü Acta Oncologica Turcica 2007; 40: 48 -51.
41. Cibas ES, Goss GA, Kulke MH. Malignant epithelioid angiomyolipoma (‘‘sarcoma ex angiomyolipoma’’) of the kidney: a case report and review of the literature. Am J Surg Pathol 2001;25:121–6.
42. Osterling JE, Fishman EK, Goldman SM. The management of renal angiomyolipoma. J Urol 1986;135:1121-4.
43. Boorjian SA, Frank I, Inman B, Lohse CM, Cheville JC, Leibovich BC, Blute ML. The role of partial nephrectomy for the management of sporadic renal angiomyolipoma. Urology. 2007;70(6):1064-8.