Complete surgical resection of the
Complete surgical resection of the tumor at the time of diagnosis is essential towards effective management of PPB. Unfortunately, a large cohort of patients present with an advance stage of disease where the tumor has grown to such an extent to invade surrounding vital structures, hindering the complete surgical removal of the tumor. Nevertheless, offering neoadjuvant chemotherapy to such patients may offer reduction in tumor size, eventually facilitating surgical resection. Alternatively, local radiotherapy to the tumor bed appears controversial in young children presumably due to its potential toxicity in late reacting normal tissues. More importantly, survival rates do not differ significantly between patients who do and do not receive radiotherapy.
Case report A 1.5-year-old boy presented to us complaining of dyspnea, cough and wheezing. Breath sounds were concealed in the right lung due to dullness in the same regions. The patient\'s radiological work-up revealed a mass in the right lower pulmonary lobe. Specifically, chest x-ray showed right hemithorax opacity suggestive of mass. Chest ultrasonography (USG) revealed pleural effusion in the right side, and thoracic computerized tomography (CT) illustrated soft tissue opacity in the apical segment of the right lower lobe measuring 12 × 11 cm2 in cranio-caudal direction, as shown in Fig. 1a. Medially, the mass was abutting the SB 203580 hydrochloride and great vessels with intact intervening fat planes. Posteriorly, the mass was abutting the posterior chest wall with right-sided massive pleural effusion along with mediastinal and hilar lymphadenopathy. Additional testing showed that biochemical measures were within normal limits, as were bone scintigraphy and abdominal USG. For confirmative diagnosis, the patient underwent excision biopsy of the mass via right thoracotomy. The results demonstrated a 17 × 13 × 5 cm3 mass in aggregate. Immunochemical staining illustrated positive thyroid transcription factor-1 (TTF-1), epithelial membrane antigen (EMA) and BCL 2 while negative for KD19, CD34 and S100. Histological diagnosis indicated that the tumor was PPB (Dehner\'s type II as it was comprised of both cystic and solid components). All of the clinical, radiological and histopathological features of the patient\'s PPB tumor are summarized in Table 1. At the therapeutic end, the patient was assessed to determine if he was a good candidate for complete surgical excision of the tumor; however, surgery was not possible as the tumor has infiltrated vital structures (i.e., lungs, aorta, and pleura). Alternatively, neoadjuvant chemotherapy was implemented. Specifically, ICE (ifosfamide 2g/m2 on days 1–3, carboplatin 600 mg/m2 on day 3 and etoposide 150 mg/m2 on days 1–3) alternated with VAC (vincristine 1.5 mg/m2 on day 1, actinomycin-D 15μg/kg on day 1, cyclophosphamide 1.2g/m2 on day 1) were scheduled. Six courses were administered where the ICE and VAC courses were alternated every three weeks for a total of 12 chemotherapy courses. After the first course of chemotherapy was completed, the patient\'s dyspnea and cough resolved markedly. Radiological (CT chest) reevaluation of the patient after the six courses of chemotherapy revealed a 60% reduction in tumor size, as assessed by the radiologist (Fig. 1b). Moreover, no pleural effusion was seen.
Discussion Pleuropulmonary blastoma (PPB) is an exceedingly rare disease with a typically poor prognosis, most probably due to the aggressive nature of the disease. Recently, the key prognostic factors for progression-free survival (PFS) and overall survival (OS) has been documented. In particular, the PSF and OS were found to be significantly correlated to IRS stage (the extent of tumor resection at diagnosis), T-stage (tumor invasiveness) and parietal pleural involvement (p = 0.008, 0.02 and 0.03, respectively). Alternatively, patient age, gender, tumor size, type of chemotherapy, complete resection after chemotherapy and use of radiotherapy did not correlated significantly with outcome. However, for Dehner\'s type II/III tumors, chemotherapy type emerged as a significant factor. Specifically, the 5-year PFS for patients treated with doxorubicin-containing regimen (VAID or IVAD, where D = doxorubicin) was significantly higher (p = 0.01) at 70% compared to all the other regimens at 31.3%. Moreover, histological type of the PPB disease also influences the prognosis. Specifically, Dehner\'s type I PPB (cystic lesion) was found to have better prognosis than type II (cystic plus solid lesion), while type II has a better outcome than type III (solid lesion).