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KMID : 0357920130470020172
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Korean Journal of Pathology
2013 Volume.47 No. 2 p.172 ~ p.176
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Congenital Peribronchial Myofibroblastic Tumor: A Case Study and Literature Review
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Kim Yu-Il
Park Ha-Young
Cho Jun-Hun
Han Joung-Ho
Cho Eun-Yoon
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Abstract
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Congenital peribronchial myofibroblastic tumor (CPMT) is a benign pulmonary spindle cell neoplasm of intrauterine and perinatal period, which is thought to arise from primitive peribronchial mesenchyme. We present a case detected incidentally in a one-month-old infant. The solid and partially necrotic tumor involved the right middle and lower lobes of the lung with extension to the diaphragm. Histologically, the tumor was composed of fasciculated monotonous spindle cells, proliferating peribronchiolar cartilage and round cells with rich vasculature, and high mitotic activity was identified in the round cell area. Immunohistochemical and electron microscopic studies showed that the spindle cells were myofibroblastic in phenotype. Although the tumor showed several malignant pathological features, recurrence was not observed in the two-year follow-up period, consistent with the benign clinical behavior of CPMT.
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KEYWORD
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Congenital tumor, Lung, Peribronchial myofibroblastic tumor
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