Extra-Axial Chordoma Presenting as a Lung Mass

Park, Seung Yong; Kim, So Ri; Choe, Yeong Hun; Lee, Ka Young; Park, Seoung Ju; Lee, Heung Bum; Jin, Gong Yong; Jang, Kyu Yun; Lee, Yong Chul

doi:10.1159/000134306

Cited by 21 publications

(22 citation statements)

References 16 publications

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“… 3 , 7 , 8 Besides soft tissue, there are a few reports of chordomas that originate in the lungs, which is currently the only reported visceral organ where extraosseous chordomas occur. 9 , 10 Interestingly, Kikuchi et al 11 recently reported 2 cases of incidental notochordal tumors of the lung that were indicative of BNCTs, as evidenced by brachyury expression. Furthermore, Lee et al 12 reported an additional case of BNCTs in both the lungs.…”

Section: Discussionmentioning

confidence: 99%

Extraosseous Benign Notochordal Cell Tumor Originating in the Lung

et al. 2015

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Benign notochordal cell tumors (BNCTs) are tumors originating in the axial skeleton, where chordomas occur. Although very rare, some cases of extraosseous chordoma, such as in the soft tissue and lungs, have been reported. We report a case of a primary tumor showing the notochordal characteristics of BNCTs within the axial skeleton.An asymptomatic 57-year-old woman presented with an abnormal shadow on her chest radiograph; chest computed tomography revealed a well-defined round nodule. The resected sample tissue contained a jelly-like small nodule. Histologically, it was identified as a BNCT, based on minimal nuclear atypia, extremely low mitotic activity within the tumor cells lying in a sheet-like arrangement, and focal immunopositivity for brachyury.This is the third case report of BNCT originating in the lungs; BNCTs are considered asymptomatic tumors that are identified by using highly developed chest imaging technology; however, our findings also suggest that these notochordal tumors may potentially originate from extraosseous sites that lack ideal precursor cells. Our case suggests that notochordal tumors can arise from organs that are unrelated to known notochordal development.

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Section: Discussionmentioning

confidence: 99%

Extraosseous Benign Notochordal Cell Tumor Originating in the Lung

et al. 2015

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“…Besides Kawahara et al's17 case report of exfoliative cytology of metastatic chordoma appearing in the sputum, Maynard reports the autopsy findings in a case of chordoma with pulmonary metastases 21. In 2009, Park et al18 has reported an extra‐axial chordoma presenting as a primary lung mass. Chordomal metastases to the lung normally present as multiple cannonball lesions on radiological imagining 2, 3.…”

Section: Discussionmentioning

confidence: 99%

“…We agree with Kawahara et al17 that physaliferous cells are perhaps not essential for the confirmatory diagnosis of chordoma in pulmonary aspirates and sputum cytology if it is supported by a documented past history with the availability of previous histology for comparison and review. In the absence of a documented history of chordoma, an extra‐axial chordoma presenting as a primary lung mass is another diagnostic possibility 18. Cytologically, especially with limited sampling associated with the lack of typical “physaliferous cells” chordomas in the lung can mimic other myxoid neoplasms such as well‐differentiated chondrosarcomas, metastatic mucinous adenocarcinoma, renal cell carcinoma, myxoid liposarcoma, and myxopapillary ependymoma 9–11, 14.…”

Section: Discussionmentioning

confidence: 99%

Fine‐needle aspiration cytology with histological correlation of chordoma metastatic to the lung: A diagnostic dilemma

Kanthan

Senger

2010

Diagnostic Cytopathology

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Chordoma is an uncommon tumor initially believed to be benign due to the rarity of its metastasis. Cytological, morphological, and immunohistochemical features of chordoma, relating to its origin from notochordal remnants, allows for its accurate diagnosis. A 75-year-old man with a known history of tuberculosis (TB) presented with shortness of breath and a dry cough. A chest X-ray demonstrated a diffuse, infiltrative miliary pattern in both lungs. Bronchial washings submitted for culture and cytological examination did not identify any tuberculous bacilli. Fine needle aspiration cytology (FNAC) showed focal areas of myxoid regions with small, round, uniform mononuclear cells. There was a documented past history of chordoma arising from the L2 vertebrae. Because of the rarity of this lesion in the lung and the limited diagnostic material available with a clinical history of TB and lumbar chordoma, the pathological report rendered on the FNAC was "atypical cells suspicious for metastatic chordoma." A wedge biopsy of the lung confirmed the presence of metastatic pulmonary chordoma. Despite palliative treatment, he died within a year. Although the metastatic potential of chordomas has been recognized and documented, to the best of our knowledge, metastasis of chordoma to the lung diagnosed by FNAC with cytohistological correlation has not been previously reported in the English literature.

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“…The pathological basis of how extra-axial chordomas develop is unclear. They are believed to arise from migrating or ectopic notochord, but have been documented arising from distal bones and soft tissue, including lung parenchyma [6][7][8]. A summary of radiological characteristics of tumors included in the differential diagnosis is highlighted in Table 1.…”

Section: Discussionmentioning

confidence: 99%

Chordomas and Their Consideration in the Radiographic Differential of Extra-Axial Lesions of the Central Nervous

Lang¹,

Thawani²,

Sayah³

et al. 2012

OJMN

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Due to the rare occurrence of chordomas extra-axially, these lesions have not earned a great deal of consideration in the clinical and radiographic differential diagnoses of extra-axial paraspinal lesions. We describe a case of a patient with a surgically resected extra-axial chordoma and review the radiologic characteristics of chordomas as an entity in the spectrum of extra-axial lesions. A 47-year-old man presented to our institution with four months of intermittent parethesias, pain, and subjective weakness in the left leg. MR imaging of the lumbar spine demonstrated a large heterogeneously- enhancing paraspinal lesion extending from the left L3-4 neural foramen into the psoas muscle. The patient underwent a CT-guided needle biopsy demonstrating features consistent with a chordoma. He then underwent a left retroperitoneal approach and en bloc resection of the lesion from the vertebral column. Pathology confirmed chordoma as the diagnosis. EACs are a rare but important consideration in the diagnosis of extra-axial lesions of the central nervous system

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Extra-Axial Chordoma Presenting as a Lung Mass

Cited by 21 publications

References 16 publications

Extraosseous Benign Notochordal Cell Tumor Originating in the Lung

Extraosseous Benign Notochordal Cell Tumor Originating in the Lung

Fine‐needle aspiration cytology with histological correlation of chordoma metastatic to the lung: A diagnostic dilemma

Chordomas and Their Consideration in the Radiographic Differential of Extra-Axial Lesions of the Central Nervous

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