title: Pulmonary epithelioid inflammatory myofibroblastic sarcoma with multiple bone metastases: case report and review of literature.
abstract: Epithelioid inflammatory myofibroblastic sarcoma (EIMS) is a rare variant of inflammatory myofibroblastic tumor with distinctive morphological features and malignant clinical behavior. Only a few such cases have been described in the literature. We report here a case of unusual pulmonary EIMS with multiple bone metastases. A 21-year-old Chinese male patient presented with complaints of general fatigue and rapid weight loss, and a huge tumor arising in the left lower lobe of lung was detected by chest computed tomography. The mass of lung was totally resected. Microscopically, the tumor cells were rounded and epithelioid in shape. Myxoid stroma and inflammatory infiltration was also present. The tumor cells were immunopositive to anaplastic lymphoma kinase (ALK) in smooth cytoplasmic pattern. Fluorescence in situ hybridization (FISH) assay revealed the presence of rearrangement of ALK gene. Three months after lung surgery, there were multiple bone metastases and intraspinal mass found by positron emission tomography. The second surgical treatment was performed to remove the intraspinal lesion. The histological and immunohistochemical features of intraspinal mass were similar to those of pulmonary tumor. The diagnosis of pulmonary EIMS with multiple bone metastases was made. To the best of our knowledge, it may be the first case of an EIMS arising in lung. Awareness of EIMS in respiratory tract and its distinctive features is important for pathologists to avoid a diagnostic pitfall caused by histologic similarities to other ALK-positive tumors. ALK inhibitor is a promising treatment for this aggressive tumor regardless of its potential acquired resistance.
authors.affiliation: Department of Pathology, The First Affiliated Hospital, Guangzhou Medical University, 151, Yanjiang Road, Guangzhou, 510120, China.
authors.forename: Xinge
authors.initials: X
authors.lastname: Fu
authors.affiliation: Department of Pathology, The First Affiliated Hospital, Guangzhou Medical University, 151, Yanjiang Road, Guangzhou, 510120, China.
authors.forename: Juhong
authors.initials: J
authors.lastname: Jiang
authors.affiliation: School of Chinese Medicine, Hong Kong Baptist University, 7, Baptist University Road, Kowloon Tong, Hong Kong, China.
authors.forename: Xiao-ying
authors.initials: XY
authors.lastname: Tian
authors.affiliation: Department of Pathology, The First Affiliated Hospital, Sun Yat-sen University, 58, Zhongshan Road II, Guangzhou, 510080, China. lizhi@mail.sysu.edu.cn.
authors.forename: Zhi
authors.initials: Z
authors.lastname: Li
mesh_terms: D000077548:Anaplastic Lymphoma Kinase; D014408:Biomarkers, Tumor / Q000032:analysis / Q000235:genetics; D001859:Bone Neoplasms / Q000737:chemistry / Q000235:genetics / Q000556:secondary* / Q000601:surgery; D015622:Epithelioid Cells / Q000737:chemistry / Q000473:pathology*; D017809:Fatal Outcome; D015321:Gene Rearrangement; D006801:Humans; D007150:Immunohistochemistry; D017404:In Situ Hybridization, Fluorescence; D008175:Lung Neoplasms / Q000737:chemistry / Q000235:genetics / Q000473:pathology* / Q000601:surgery; D008297:Male; D058628:Myofibroblasts / Q000737:chemistry / Q000473:pathology*; D011013:Pneumonectomy; D049268:Positron-Emission Tomography; D020794:Receptor Protein-Tyrosine Kinases / Q000235:genetics; D012086:Reoperation; D012509:Sarcoma / Q000737:chemistry / Q000235:genetics / Q000556:secondary* / Q000601:surgery; D013997:Time Factors; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D014408:Biomarkers, Tumor; C000626173:ALK protein, human; D000077548:Anaplastic Lymphoma Kinase; D020794:Receptor Protein-Tyrosine Kinases