To improve the recognization of clinical, radiological and pathological characteristics in lymphangioleiomyomatosis (LAM) complicated with renal angiomyolipomas (AML), and to decrease misdiagnosis and missed diagnosis.

The clinical , radiological and pathological data of 41 patients of LAM complicated with renal AML in Guangzhou Institute of Respiratory Disease from 2011 to 2017 were retrospectively analyzed.

All patients were divided into two groups: ten cases in tuberous sclerosis complex (TSC)-AML group and 31 cases in LAM-AML group. In TSC-AML group, patients were all female and their mean age was 39.8 years old. Clinical manifestations included hematuria (six cases), lumbago (five cases), abdominal mass (three cases). All individuals had bilateral leisions and over three tumors.Maximum length of tumor was 20 cm. In LAM-AML group, patients were also female and their mean age was 37.5 years old. Clinical manifestations included hematuria (eight cases), lumbago (six cases), abdominal mass (three cases). 12 cases had bilateral leisions and 19 cases had unilateral leisions. Quantity of tumor was 1-3 in 17 cases (54.8%). There were 22 patients (71.0%) whose length of tumor was less than 3 cm. Maximum length of tumor was 18 cm. CT scans of chest in all patients indicated diffuse cystic lung.Enhanced CT scans of abdomen in all patients indentified multiple renal AML, including nine cases with enormous AML. Typical pathological features of lung included cystic change of lung and abnormal proliferation of multiple immature smooth muscle spindle cells and perivascular epithelial cells.Typical immunohistochemisty of lung biopsy included SMA(+ ), HMB45(+ ), ER(+ ), and PR(+ ). Typical pathological manifestinations of kidney included fat cell accumulation and abnormal proliferation of multiple immature smooth muscle cells and perivascular epithelial cells. Immunohistochemisty of kidney biopsy included SMA(+ ) and HMB45(+ ). Mean value of serum vascular endothelial growth factor-D concentration in TSC-AML group and LAM-AML group was respectively 2 030.9 ng/L (IQR: 1 243.1~3 124.4 ng/L) and 1 592.9 ng/L (IQR: 845.2~1 904.9 ng/L), which was higher than that in healthy control group [268.1 ng/L (IQR: 190.8~364.1 ng/L), all P<0.001].

The main renal leision of LAM is renal AML. Enhanced CT scans of abdomen, serum vascular endothelial growth factor-D and renal biopsy contribute to diagnosis of renal AML.

Lymphangioleiomyomatosis; Pulmonary; Angiomyolipomas; Kidney