Benign notochordal cell tumor of the lung: Report of a case

Abstract

A surgical case of a benign notochordal cell tumor of the lung is reported. The patient was an asymptomatic 41‐year‐old man, who was incidentally found to have a small tumor in the subpleural region of the left lingular segment. Since wedge resection of the tumor, the patient has been free from recurrence. The tumor measured 12 mm in diameter and showed a central cystic change. It consisted of a diffuse proliferation of polygonal cells with abundant, uni‐ or multi‐vacuolated cytoplasm and bland nuclei. The tumor did not show a lobular architecture and lacked a myxoid or fibrous connective tissue containing blood vessels. In the peripheral region of the tumor, a small number of alveolar epithelial cells were entrapped. The nuclei of tumor cells were immunoreactive for brachyury, and the cytoplasm was positive for cytokeratin and S‐100 protein. The entrapment of alveolar epithelial cells suggests infiltrative growth of the tumor, and the almost complete absence of blood vessels within the tumor may have restricted tumor growth and induced a cystic change in the central region.

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

“…The whole-body CT, bone scintigraphy, and (18) F-fluorodeoxyglucose-positron emission tomography would be helpful. Other primary pulmonary neoplasms with myxoid background and secondary clear cell tumors, such as myoepithelioma, hamartoma, pleomorphic adenoma, perivascular epithelioid cell tumor, pulmonary myxoid sarcoma with EWSR1-CREB1 fusion should be considered in the differential diagnosis [ 3 , 8 – 10 ]. But these were easily ruled out on the basis of pathological and clinical findings and the immunohistochemistry.…”

“…In our case, the brachyury-positive pattern was consistent with most of the literatures aforementioned, and CK (AE1/AE3), EMA, S100 were positively stained, strongly suggested notochordal differentiation. Reported skull BNCT typically had a Ki-67 index range from <1 to 3% [ 17 – 19 ], while the lung BNCTs was 1 to 5.2% [ 3 , 8 – 10 ], which was even higher. However, the follow-up results showed that no recurrence occurred in patients with a higher Ki-67 index, indicating the benign biological behavior of this tumor [ 19 ].…”

“…The distribution of BNCT is similar to chordomas, axial skeleton, especially the lumbo-sacral region and clivus are the most common sites. Extraosseous chordomas have been reported in soft tissue and lung [ 5 – 7 ], while extraosseous BNCTs were only occurred in lung [ 3 , 8 – 10 ], and there were only five reported cases. The pulmonary primary BNCT was first reported by Kikuchi [ 3 ] in 2011.…”

Cytological detection of metastatic chordoma cells in pleural effusions: A case report

“…15 The main differential diagnosis of chordoma cells metastatic to the lung includes benign notochordal cell tumor (BNCT) and primary chordoma of the lung. [16][17][18] Given the morphological and immunohistochemical overlap, it may be difficult to distinguish metastatic chordoma from these tumors. BNCT of the lung shows a solitary, well-demarcated mass with an asymptomatic clinical course in general.…”

Multiple benign notochordal cell tumors in lung with cystic change

“…BNCT is a benign lesion derived from notochordal cells and it is relatively common in intraosseous lesion, being found in 20% of adults at autopsy [1] . Although some cases of BNCT arising in lung has been reported recently [2] , [3] , [4] , [5] , [6] , [7] , there is no report of multiple pulmonary BNCTs. We present a case of 54-year-old male with multiple pulmonary nodules showing cystic change and finally diagnosed of pulmonary BNCT.…”