Renal Angiomyolipoma and Lung Lymphangiomyomatosis: Case Report

Öz

Angiomyolipoma is a benign tumoral lesion of the kidney which microscopically contains fat, smooth muscle and blood vessels, and may result in retroperitoneal hemorrhage. Lymphangiomyomatosis is a lesion that may lead to diffuse involvement of lungs and cause respiratory failure, spontaneous pneumothorax or pleural effusion. It is characterized by proliferation of a mixture of lymphatic and smooth muscle. Both lesions may be components of tuberous sclerosis, an uncommon syndrome. Our case is 41-year-old female presented with chest pain and dyspnea. Thin walled air cysts were determined in lung parenchyma by thorax tomography and images of enlarged kidneys and mass lesions including areas of adipose density were supplied by abdominal tomography. Right nephrectomy was performed because of intraabdominal hemorrhage. Infiltrative tumor microscopically composed of mature lipomatose, myomatose components and congested blood vessels, and reactive for HMB-45 [Melanoma (gp100) Ab-3 (clone HMB45+HMB50), Neomarkers] was diagnosed as angiomyolipoma. Microscopic evaluation of the lung lesions revealed cystic lymphatic containing smooth muscle cells. The tumor with presence of smooth muscle in the walls of lymphatic shown with trichrome and was diagnosed as lymphangiomyomatosis. The patient died because of irreversible cardiopulmonary arrest on the postoperative 16th day. Key words: Angiomyolipoma, Lymphangiomyomatosis, Tuberosclerosis

Anahtar Kelimeler

• Angiomyolipoma, Lymphangiomyomatosis, Tuberosclerosis

Renal Anjiomiyolipoma ve Akciğerde Lenfanjiomiyomatozis: Olgu Sunumu+

Öz

Anjiyomiyolipoma böbrekte, retroperitoneal hemoraji yapabilen, mikroskopik olarak damar yapıları, düz kas ve yağ dokuları içeren benign tümöral lezyondur. Lenfanjiyomiyomatozis ise; akciğerleri difüz tutabilen, respiratuar yetmezlik, spontane pnömotoraks veya şilöz plevral efüzyon izlenebilen lezyondur. Mikroskopik olarak lenf damarları ve düz kas elementlerinin karışımının proliferasyonları ile karakterizedir. Her iki lezyon da az rastlanan bir sendrom olan tuberoskleroz kompleksinin komponenti olarak izlenebilir. Olgumuz göğüs ağrısı, nefes darlığı şikayeti olan 41 yaşında bayan hastadır. Toraks tomografisinde akciğer parankiminde, ince duvarlı hava kistleri, abdominal tomografide böbreklerde büyüme ve yağ dansitesinde alanlar içeren lezyonlar belirlenmiştir. İntraabdominal kanama nedeniyle sağ nefrektomi uygulanmıştır. Mikroskobide, böbrek parankimindeki infiltratif tümörün matür lipomatöz, miyomatöz komponentler ve konjesyone vasküler yapılardan oluştuğu izlenmiş, immünohistokimyasal incelemede tümör hücrelerinde HMB-45 ile reaksiyon gözlenen olguya anjiyomiyolipoma tanısı konulmuştur. Akciğer lezyonlarının mikroskobik incelemesinde, kistik dilatasyonlu, düz kas hücreleri içeren lenf damarları izlenmiştir. Trikrom ile lenf damarları duvarında düz kas yönünde boyanma gözlenmiş ve lenfanjiyomiyomatozis olarak değerlendirilmiştir. Olgumuz postoperatif 16. günde gelişen kardiyopulmoner arest nedeni ile eksitus kabul edilmiştir. Anahtar kelimeler: Anjiomiyolipoma, Lenfanjiomiyomatozis, Tuberoskleroz

Anahtar Kelimeler

• Anjiomiyolipoma, Lenfanjiomiyomatozis, Tuberoskleroz

Kaynakça

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