Primary lung chordoma: a case report

• Published in: Diagnostic pathology Vol. 19; no. 1; pp. 91 - 7
• Main Authors: Shigeta, Naoko, Isaka, Tetsuya, Ono, Kyoko, Tanaka, Mio, Yokose, Tomoyuki, Adachi, Hiroyuki, Usuba, Wataru, Ito, Hiroyuki
• Format: Journal Article
• Language: English
• Published: England BioMed Central Ltd 03.07.2024; BioMed Central; BMC
• Subjects: Adult; Amplification; Antigens; Biomarkers, Tumor - analysis; Brachyury; Case Report; Case reports; Cells; Chordoma - diagnosis; Chordoma - pathology; Computed tomography; Cytokeratin; Cytoplasm; Diagnosis; FISH; Fluorescence in situ hybridization; Humans; Immunohistochemistry; In Situ Hybridization, Fluorescence; Isochromosome 12p; Keratin; Leukocytes (eosinophilic); Lung cancer; Lung chordoma; Lung Neoplasms - diagnosis; Lung Neoplasms - pathology; Lungs; Male; Membrane proteins; Metastases; Metastasis; Neoplasms, Germ Cell and Embryonal - chemistry; Neoplasms, Germ Cell and Embryonal - diagnosis; Neoplasms, Germ Cell and Embryonal - pathology; Nerve proteins; Proteins; S100 protein; Staining; Teratoma; Teratoma - chemistry; Teratoma - diagnosis; Teratoma - pathology; Testes; Testicular cancer; Testicular mixed germ-cell tumor; Testicular Neoplasms - chemistry; Testicular Neoplasms - diagnosis; Testicular Neoplasms - pathology; Tumor cells; Tumors; Tumors, Embryonal; Vimentin; Yolk sac; Brachyury; FISH; Testicular mixed germ-cell tumor; Lung chordoma; Isochromosome 12p
• ISSN: 1746-1596; 1746-1596
• DOI: 10.1186/s13000-024-01522-0

Chordoma, a rare malignant tumor arising from notochordal tissue, usually occurs along the spinal axis. Only a few published reports of primary lung chordomas exist. Herein, we present a case of primary lung chordoma and discuss important considerations for diagnosing rare chordomas. We report a case of primary lung chordoma in a 39-year-old male with a history of testicular mixed germ-cell tumor of yolk sac and teratoma. Computed tomography revealed slow-growing solid lesions in the left lower lobe. We performed wedge resection for suspected germ-cell tumor lung metastasis. Histologically, large round or oval cells with eosinophilic cytoplasm were surrounded by large cells with granular, lightly eosinophilic cytoplasm. Tumor cells were physaliphorous. Immunohistochemistry was positive for brachyury, S-100 protein, epithelial membrane antigen, vimentin, and cytokeratin AE1/AE3, suggesting pulmonary chordoma. Re-examination of the testicular mixed germ-cell tumor revealed no notochordal elements. Although some areas were positive for brachyury staining, hematoxylin and eosin (HE) staining did not show morphological features typical of chordoma. Complementary fluorescence in situ hybridization (FISH) of the lung tumor confirmed the absence of isochromosome 12p and 12p amplification. Thus, a final diagnosis of primary lung chordoma was established. In patients with a history of testicular mixed germ cell tumors, comparison of histomorphology using HE and Brachyury staining of lung and testicular tumors, and analyzing isochromosome 12p and 12p amplification in lung tumors using FISH is pivotal for the diagnosis of rare lung chordomas.