Pleuropulmonary Blastoma: A Report of Two Rare Cases

TL;DR

This paper reports two rare cases of pleuropulmonary blastoma, a malignant lung tumor, and describes their clinical and pathological features.

Contribution

The novelty lies in presenting two distinct immunohistochemical profiles of pleuropulmonary blastoma cases with improved patient outcomes following chemotherapy.

Findings

Both cases showed a pleural-based mass with malignant mesenchymal tumor features confirmed by biopsy.

Tumor cells were positive for vimentin and showed variable positivity for BCL2, α-1 antitrypsin, and desmin.

Both patients improved after starting neoadjuvant chemotherapy.

Abstract

Pleuropulmonary blastoma (PPB) is a rare malignant tumor arising from the lung and pleura. It has three types based on the solid and cystic components. The prognosis of PPB varies depending on the type. Here, we present two female patients who come with complaints of breathlessness. Contrast-enhanced computed tomography (CECT) chest showed a pleural-based mass. Biopsy from the pleural-based mass showed a tumor with features of the malignant mesenchymal tumor. Tumor cells in both cases were positive for vimentin and negative for PanCK. In addition, tumor cells of one case showed positive for BCL2 and α-1 antitrypsin and negative for desmin, CD99, NSE, and p53. Tumor cells of another case are negative for CD99, WT-1, S100, synaptophysin, and chromogranin. In addition, some of the cells have abundant eosinophilic cytoplasm. Desmin shows positive in many cells and highlights rhabdomyoblasts. Morphological and immunohistochemical findings were correlated with the CECT diagnosis of PPB. Both cases were started on neoadjuvant chemotherapy and kept under follow-up. Both the patient's condition improved.

Figures