Korean J Pathol.  2013 Apr;47(2):172-176.

Congenital Peribronchial Myofibroblastic Tumor: A Case Study and Literature Review

• Kim Y
• Park HY
• Cho J
• Han J
• Cho EY

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.

Keyword

Congenital tumor; Lung; Peribronchial myofibroblastic tumor

MeSH Terms

Cartilage
Diaphragm
Electrons
Follow-Up Studies
Humans
Infant
Lung
Mesoderm
Myofibroblasts
Phenotype
Recurrence