Pulmonary epithelioid inflammatory myofibroblastic sarcoma with multiple bone metastases: Case report and review of literature

Xinge Fu, Juhong Jiang, Xiao Ying TIAN, Zhi Li*

*Corresponding author for this work

Research output: Contribution to journalJournal articlepeer-review

24 Citations (Scopus)


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.

Original languageEnglish
Article number106
JournalDiagnostic Pathology
Issue number1
Publication statusPublished - 16 Jul 2015

Scopus Subject Areas

  • Pathology and Forensic Medicine
  • Histology

User-Defined Keywords

  • Anaplastic lymphoma kinase
  • Differential diagnosis
  • Epithelioid inflammatory myofibroblastic sarcoma
  • Inflammatory myofibroblastic tumor
  • Prognosis


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