Xiaotong Dong1, Lvcheng Jin1, Ailan Wang2, Liping Wu3, Xintong Fan1, Qian Hou1, Tianbao Li2, Ruilian Zhao4, Yunxiang Zhang1. 1. Department of Pathology, First Affiliated Hospital of Weifang Medical University, Weifang People's Hospital, Weifang 261041, China. 2. Department of Science, Qingdao Geneis Institute of Big Data Mining and Precision Medicine, Qingdao, China. 3. Department of Respiratory, First Affiliated Hospital of Weifang Medical University, Weifang People's Hospital, Weifang 261041, China. 4. Department of General Practice, Affiliated Hospital of Weifang Medical University, Weifang 261031, China.
Abstract
OBJECTIVE: Lymphangioleiomyomatosis (LAM) is a rare low-grade metastatic tumor; however, LAM patients were always found in young age with difficulty for diagnosis. Our study is aimed at observing the clinical characteristics of patients with lymphangiomatosis, including the clinical manifestations, imaging findings, histopathological features, and immunophenotype. METHODS: We did a systematic review on LAM/PLAM cases, especially on male cases, and collected the clinical features and molecular mechanisms of PLAM based on previous findings. RESULTS: Diagnosis criteria were summarized by combining CT scans, MRI, immunohistochemistry results, and gene sequencing results for effectively distinguishing between PLAM and similar diseases. Moreover, our study illustrated the molecular mechanism of PLAM as well as the signaling pathway involved in the disease initials. In addition, a male case was reported with differential diagnosis on the clinical manifestations, microscopic features, immunophenotypes, and genotypes. CONCLUSION: Our review will definitely improve the understanding of diagnosis and treatment in PLAM cases.
OBJECTIVE: Lymphangioleiomyomatosis (LAM) is a rare low-grade metastatic tumor; however, LAM patients were always found in young age with difficulty for diagnosis. Our study is aimed at observing the clinical characteristics of patients with lymphangiomatosis, including the clinical manifestations, imaging findings, histopathological features, and immunophenotype. METHODS: We did a systematic review on LAM/PLAM cases, especially on male cases, and collected the clinical features and molecular mechanisms of PLAM based on previous findings. RESULTS: Diagnosis criteria were summarized by combining CT scans, MRI, immunohistochemistry results, and gene sequencing results for effectively distinguishing between PLAM and similar diseases. Moreover, our study illustrated the molecular mechanism of PLAM as well as the signaling pathway involved in the disease initials. In addition, a male case was reported with differential diagnosis on the clinical manifestations, microscopic features, immunophenotypes, and genotypes. CONCLUSION: Our review will definitely improve the understanding of diagnosis and treatment in PLAM cases.
Authors: Elena A Goncharova; Dmitry A Goncharov; Hua Li; Wittaya Pimtong; Stephen Lu; Irene Khavin; Vera P Krymskaya Journal: Mol Cell Biol Date: 2011-04-11 Impact factor: 4.272
Authors: Paola Crivelli; Roberta Eufrasia Ledda; Silvia Terraneo; Maurizio Conti; Gianluca Imeri; Elena Lesma; Fabiano Di Marco Journal: Respir Med Date: 2019-08-22 Impact factor: 3.415
Authors: Mark Nellist; Rutger W W Brouwer; Christel E M Kockx; Monique van Veghel-Plandsoen; Caroline Withagen-Hermans; Lida Prins-Bakker; Marianne Hoogeveen-Westerveld; Alan Mrsic; Mike M P van den Berg; Anna E Koopmans; Marie-Claire de Wit; Floor E Jansen; Anneke J A Maat-Kievit; Ans van den Ouweland; Dicky Halley; Annelies de Klein; Wilfred F J van IJcken Journal: BMC Med Genet Date: 2015-02-25 Impact factor: 2.103
Authors: Victoria Maria Garcia de Medeiros; Jéssica Gonçalves de Lima; Claudia Rosa; Juliana Rega; Mauro Felippe Felix Mediano; Luiz Fernando Rodrigues Junior Journal: Ann Med Date: 2022-12 Impact factor: 5.348