Güncel Patoloji Dergisi 2022, Cilt 6, Sayı 1
Lymphangioleiomyomatosis and Angiomyolipoma Association Literature Review
Sevilay ÖZMEN 1, Funda DEMIRAĞ 2, Onur CEYLAN 1, Metin AKGÜN 3, Fatif ALPER 4, Yener AYDIN 5, Ömer ARAZ 3
1 Atatürk Üniversitesi Tıp Fakültesi, Tıbbi Patoloji Anabilim Dalı, ERZURUM
2 Sağlık Bilimleri Üniversitesi Atatürk Göğüs Hastalıkları ve Göğüs Cerrahisi Eğitim ve Araştırma Hastanesi, Tıbbi Patoloji Anabilim Dalı, ANKARA
3 Atatürk Üniversitesi Tıp Fakültesi, Göğüs Hastalıkları Anabilim Dalı, ERZURUM
4 Atatürk Üniversitesi Tıp Fakültesi, Radyoloji Anabilim Dalı, ERZURUM
5 Atatürk Üniversitesi Tıp Fakültesi, Göğüs Cerrahisi Anabilim Dalı, ERZURUM
DOI : 10.5146/jcpath.2022.74 Aim: Lymphangioleiomyomatosis is a rare disease seen as sporadic or part of tuberous sclerosis. It is more common in women with tuberous sclerosis and is often diagnosed in patients with renal angiomyolipoma. Renal Angiomyolipomas are benign tumors that can gain large size. Our case was sporadic and it was found worth presenting due to the coexistence of pulmonary lymphangioleiomyomatosis and renal angiomyolipom.

Case: A 26-year-old female patient who applied to the emergency department with a complaint of hemoptysis was observed in the right hemithorax by listening in her examination. On lung radiography, ground glass density and locally consolidated areas were observed in the middle and lower zones, while computed tomography showed thin-walled multiple cystic areas located in both lungs. In the abdomen, a palpable mass was detected. In the microscopic examination of right renal mass mass biopsy, mitosis, necrosis, and cytological atypia were not observed in sections with thick-walled dysmorphic vascular structures between mature adipose tissue islands. A video-assisted thoracoscopic surgical biopsy was performed on the patient whose hemoptysis complaints continued and interstitial lung disease was considered and the diagnosis could not be confirmed with high resolution computed tomography. In the microscopic evaluation of lung biopsy, small cystic cavities in the parenchyma and thickening of the interstitial area and cells in the morphology similar to spindle-like smooth muscle cells were observed around these cysts. In the immunohistochemical study, the diagnosis of lymphangioleiomyomatosis was made upon the detection of reactivity in these cells with HMB-45 and estrogen receptor protein.

Conclusion: Lymphangioleiomyomatosis is a disease that affects only women and is characterized by widespread proliferation of abnormal smooth muscle cells with cystic changes in the lung parenchyma. Giant angimyolipomas, which may be associated with lymphangioleiomyomatosis, usually present with the complaint of bleeding in the clinic. However, in our case, angiomyolipoma was detected incidentally and was found interesting in this respect. Anahtar Kelimeler : Lymphangioleiomyomatosis, Angiomyolipoma, Interstitial lung disease