First report of pulmonary sclerosing pneomucytoma with malignant transformation in both cuboidal surface cells and stromal round cells: a case report

BACKGROUND: Pulmonary sclerosing pneumocytoma (PSP) is a rare benign tumor. Although lymph node metastasis has been reported, it is still considered benign. No malignant transformation has been reported. This is the first case of malignant transformation of both cuboidal surface cells and stromal round cells. CASE PRESENTATION: A 64-year-old male had been complaining of intermittent hemoptysis several times per day for eight months. Chest computed tomography scan showed parenchymal infiltration with cystic lesion in the right lower lobe accompanied by enlarged right hilar lymph nodes. Lobectomy and systemic lymph node dissection was performed. On grossly pathological examination, the lesion was 50 mm from the bronchial stump. It was a mixture of both cystic and solid components and 30 mm * 20 mm in size with unclear border. Microscopically, the cuboidal surface cells transformed to adenocarcinoma. The stromal round cells also had a malignant transformation. The Ki-67 proliferation index in malignant cuboidal surface cells and stromal round cells were 70 and 55%, respectively. Furthermore, E-cadherin was negative in primary tumor but positive in metastatic lymph node, which suggested that the mesenchymal to epithelial transition may play an important role in lymph node metastasis. CONCLUSIONS: To our knowledge, we present the first case of malignant transformation of both cuboidal surface cells and stromal round cells in PSP. The process of mesenchymal to epithelial transition may play an important role in lymph node metastasis.