Congenital Peribronchial Myofibroblastic Tumor: A Case Study and Literature Review
김유일, 박하영, 조준훈, 한정호, 조은윤,
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김유일 ( Kim Yu-Il )
Sungkyunkwan University School of Medicine Samsung Medical Center Department of Pathology
박하영 ( Park Ha-Young )
Sungkyunkwan University School of Medicine Samsung Medical Center Department of Pathology
조준훈 ( Cho Jun-Hun )
Sungkyunkwan University School of Medicine Samsung Medical Center Department of Pathology
한정호 ( Han Joung-Ho )
Sungkyunkwan University School of Medicine Samsung Medical Center Department of Pathology
조은윤 ( Cho Eun-Yoon )
Sungkyunkwan University School of Medicine Samsung Medical Center Department of Pathology
KMID : 0357920130470020172
Abstract
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.
키워드
Congenital tumor; Lung; Peribronchial myofibroblastic tumor
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