ABSTRACT
Objective: To investigate the clinicopathological features of perivascular epithelioid cell tumor (PEComa) of the lung. Methods: Eight PEComa cases of the lung diagnosed at the First Affiliated Hospital of Soochow University, Suzhou, China from July 2008 to December 2021 were collected and subject to immunohistochemical staining, fluorescence in situ hybridization and next generation sequencing. The relevant literature was reviewed and the clinicopathological features were analyzed. Results: There were 5 males and 3 females, aged from 18 to 70 years (mean 39 years). There were 3 cases of the right upper lung, 3 cases of the left lower lung, 1 case of the left upper lung and 1 case of the right middle lung. Seven cases were solitary and 1 case was multifocal (4 lesions). Seven cases were benign while one was malignant. The tumors were all located in the peripheral part of the lung, with a maximum diameter of 0.2-4.0 cm. Grossly, they were oval and well circumscribed. Microscopically, the tumor cells were oval, short spindle-shaped, arranged in solid nests, acinar or hemangiopericytoma-like patterns, with clear or eosinophilic cytoplasm. The stroma was rich in blood vessels with hyalinization. Coagulated necrosis and high-grade nuclei were seen in the malignant case, and calcification was seen in 2 cases. Immunohistochemically, the tumor cells were positive for Melan A (8/8), HMB45 (7/8), CD34 (6/8), TFE3 (4/7), and SMA (3/8). All cases were negative for CKpan and S-100. TFE3 (Xp11.2) gene fusion was examined using the TFE3 break-apart fluorescence in situ hybridization in 5 cases, in which only the malignant case was positive. The next generation sequencing revealed the SFPQ-TFE3 [t(X;1)(p11.2;p34)] fusion. Follow-up of the patients ranged from 12 to 173 months while one patient was lost to the follow-up. The malignant case had tumor metastasis to the brain 4 years after the operation and then received radiotherapy. Other 6 cases had no recurrence and metastasis, and all the 7 patients survived. Conclusions: Most of the PEComas of the lung are benign. When there are malignant morphological features such as necrosis, high-grade nuclei or SFPQ-TFE3 gene fusion, close follow-up seems necessary.
Subject(s)
Male , Female , Humans , In Situ Hybridization, Fluorescence , Perivascular Epithelioid Cell Neoplasms/pathology , Lung/pathology , Basic Helix-Loop-Helix Leucine Zipper Transcription Factors/genetics , Necrosis , Biomarkers, Tumor/analysisABSTRACT
To evaluate the correlation between the radiomics signature of hepatobiliary phase imaging of gadolinium-ethoxybenzyl diethylenetriaminepentaacetic acid(Gd-EOB-DTPA)enhanced magnetic resonance imaging(MRI)and Child-Pugh of liver cirrhosis,establish nomogram prediction model,and assess the predictive value of quantitative assessment of liver reserve function of patients with liver cirrhosis. One hundred patients with liver cirrhosis who met the inclusion criteria were divided into 52 patients with Child-Pugh grade A and 48 patients with Child-Pugh grade B+C according to Child-Pugh classification criteria,and were randomly divided into training set and test set at a proportion of 7∶3.The AK software was used to extract the imaging features of the Gd-EOB-DTPA-enhanced MRI hepatobiliary images of the patients in the training set,and the least absolute shrinkage and selection operator feature selection algorithm was used to reduce the dimension of the data,select the features,and construct the radiomics tags.According to the radiomics label Rad-score,a line chart(nomogram)prediction model was established to predict the Child-Pugh B+C level of liver reserve function.The model was applied to the training set and test set respectively,and the diagnostic efficiency was quantitatively evaluated by receiver operating characteristic(ROC)curve. After dimension reduction and screening of 396 texture feature parameters extracted by AK software,7 image feature parameters were obtained.According to the above characteristics,the radiomics tag Rad-score was constructed and the nomogram prediction model was created.The differences of Rad-score scores between Child-Pugh A and Child-Pugh B+C groups in training set and test set were statistically analyzed by Wilcoxon rank sum test(=0.000, =0.001).The diagnostic efficacy of nomogram prediction model for predicting Child-Pugh B+C grade of liver reserve function in the ROC curve of training set and test set was 0.88 and 0.86 respectively. The nomogram prediction model created according to the radiomics tag Rad-score of patients with liver cirrhosis with different liver reserve functions can be used as a more accurate and reliable auxiliary detection tool for liver reserve function.It provides a new means for clinicians to evaluate liver reserve function more accurately.
