Primary pulmonary hyalinizing clear cell carcinoma of bronchial submucosal gland origin

Joaquín J. García, Long Jin, Shawn B. Jackson, Brandon T. Larsen, Jean E. Lewis, William R. Sukov, Anja C. Roden

Research output: Contribution to journalArticlepeer-review

22 Scopus citations


Hyalinizing clear cell carcinoma (HCCC) has only been described in salivary glands of the head and neck. We report a 38-year-old man with a 2.6-cm lung tumor that was growing in a peribronchial location and had morphologic features of HCCC. The tumor cells expressed cytokeratin 7 and keratin AE1/AE3, and the vast majority of tumor cells marked also with p63 and p40. They were negative for cytokeratin 20, S-100, smooth muscle actin, napsin A, and thyroid transcription factor-1. Fluorescence in situ hybridization revealed Ewing Sarcoma Breakpoint Region 1 (EWSR1) rearrangement, and reverse-transcription polymerase chain reaction confirmed the presence of the EWSR1-Activating Transcription Factor 1 (ATF1) fusion transcript, which was subsequently sequenced. The morphologic, immunophenotypic, cytogenetic, and molecular findings together with the patient's history and location of the tumor support a diagnosis of primary pulmonary HCCC of bronchial submucosal gland origin. It is our understanding that this is the first report of HCCC arising as a primary tumor outside the head and neck region.

Original languageEnglish (US)
Pages (from-to)471-475
Number of pages5
JournalHuman Pathology
Issue number3
StatePublished - Mar 1 2015


  • EWSR1 rearrangement
  • EWSR1-ATF1 fusion
  • FISH
  • RT-PCR
  • Salivary gland neoplasm

ASJC Scopus subject areas

  • Pathology and Forensic Medicine


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