Multiple primary chordomas of the lung

Ohya, Maki; Yoshida, Kazuo; Shimojo, Hisashi; Shiina, Takayuki

doi:10.1016/j.rmcr.2018.08.012

Cited by 3 publications

(4 citation statements)

References 8 publications

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“…Macroscopically, lung chordomas are usually well-demarcated, solid to cystic, with a yellow, transparent, gelatinous appearance [ 9 ]. Microscopically, HE staining of tumor sections show that intraosseous chordoma cells float in sheets, cords, or alone in an abundant myxoid stroma surrounded by a fibrous band.…”

Section: Discussionmentioning

confidence: 99%

Primary lung chordoma: a case report

Shigeta,

Isaka,

Ono

et al. 2024

Diagn Pathol

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Background 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. Case presentation 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. Conclusions 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.

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Section: Discussionmentioning

confidence: 99%

Primary lung chordoma: a case report

Shigeta,

Isaka,

Ono

et al. 2024

Diagn Pathol

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“…Pulmonary chordoma is extremely rare, to our knowledge, there were only three reported cases [ 6 , 7 , 11 ]. The pathogene sis is unclear, one possible mechanism may derive from multipotent cells in the lung parenchyma or a notochordal remnant with aberrant migration from midline [ 6 ].…”

Section: Discussionmentioning

confidence: 99%

Pulmonary tumor with Notochordal differentiation: a case report and morphologic, Immunohistochemical and molecular study of benign Notochordal cell tumor originating in the lung

Song

et al. 2021

Diagn Pathol

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Background Extraosseous benign notochordal cell tumor is extremely rare, and there are only five reported cases worldwide. The presented case of pulmonary primary benign notochordal cell tumor is the sixth case, but the first to report the deletion mutation of EGFR gene exon 19. Case presentation The patient was a 50-year-old asymptomatic woman, who had been followed up for 3 years for a nodule in the right lung. After ten months of the wedge resection, the patient is alive without evidence of recurrence or metastasis. The tumor was 7 mm in diameter and was well demarcated. The tumor was consisted of a sheet of large round vacuolated cells with small and bland nuclei. No connective tissue containing blood vessels or inflammatory cell infiltration was detected in the stroma. The tumor was positive for CK AE1/AE3, Vimentin, S100 and Brachyury. EGFR gene mutation and amplification were not detected. Conclusions We firstly reported the positive immunohistochemical staining for EGFR and the negative molecular results of EGFR gene of pulmonary primary benign notochordal cell tumor. Due to the rarity of this tumor, more reports are needed to explore pathological characteristics, especially the molecular characteristics, in order to better understand the nature of tumors.

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“…Pulmonary chordoma is extremely rare, to our knowledge, there were only three reported cases [6,7,11]. The pathogene sis is unclear, one possible mechanism may derive from multipotent cells in the lung parenchyma or a notochordal remnant with aberrant migration from midline [6].…”

Section: Discussionmentioning

confidence: 99%

“…Lobule structure containing cords or nests of atypia notochordal cells and extracellular myxoid matrix are the important histological features of chordoma, while the BNCT lacks such structures. Intraosseous BNCT has recently been recognized to be a potential precursor of classic chordoma [2,12], while it has not been documented that chordomas arise from BNCT in the lung, pulmonary BNCT is considered a potential precursor of classic chordoma [3,9,11].…”

Section: Discussionmentioning

confidence: 99%

Pulmonary Tumor With Notochordal Differentiation: A Case Report and Morphologic, Immunohistochemical and Molecular Study of Benign Notochordal Cell Tumor Originating in the Lung

Song

et al. 2021

Preprint

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Background: Extraosseous benign notochordal cell tumor is extremely rare, and there are only five reported cases worldwide. The presented case of pulmonary primary benign notochordal cell tumor is the sixth case, but the first to report the deletion mutation of EGFR gene exon 19. Case presentation: The patient was a 50-year-old asymptomatic woman, who had been followed up for 3 years for a nodule in the right lung. Six months after the wedge resection, the patient is alive without evidence recurrence or metastasis. The tumor was 7 millimeters in diameter and was well demarcated. The tumor was consisted of a sheet of large round vacuolated cells with small and bland nuclei. No connective tissue containing blood vessels or inflammatory cell infiltration was detected in the stroma. The tumor were positive for CK AE1/AE3, Vimentin, S100 and Brachyury. Deletion mutation of EGFR gene exon 19 was first detected and reported. Conclusions: We firstly reported the EGFR mutation of pulmonary primary benign notochordal cell tumor. Due to the rarity of this tumor, more reports are need to explore pathological characteristics, especially the molecular characteristics, in order to better understand the nature of tumors

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Multiple primary chordomas of the lung

Cited by 3 publications

References 8 publications

Primary lung chordoma: a case report

Primary lung chordoma: a case report

Pulmonary tumor with Notochordal differentiation: a case report and morphologic, Immunohistochemical and molecular study of benign Notochordal cell tumor originating in the lung

Pulmonary Tumor With Notochordal Differentiation: A Case Report and Morphologic, Immunohistochemical and Molecular Study of Benign Notochordal Cell Tumor Originating in the Lung

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