ABSTRACT
El blastoma pleuropulmonar es una neoplasia mesenquimal rara que se debe considerar siempre en el escenario de una masa pulmonar sólida o quística en niños menores de 5 años. Se localiza usualmente en la periferia del pulmón; sin embargo, puede existir compromiso extrapulmonar de mediastino, diafragma y pleura. Se clasifica según su patrón histológico e imagenológico en tres tipos: quístico, mixto (sólido-quístico) y sólido. La cirugía radical es el tratamiento de elección y la única que ha demostrado disminución en la tasa de recurrencia. Se indica el uso de la quimioterapia neoadyuvante y la radioterapia según el tipo histológico y las márgenes libres de tumor. Se estudia el caso de una niña de 3 años con hallazgos, en radiografía convencional y tomografía de tórax, de masa sólida con diagnóstico histopatológico de blastoma pleuropulmonar.
Pleuropulmonary blastoma is a rare mesenchymal neoplasm that should always be considered in the setting of a solid or cystic lung mass in children under 5 years old. It is usually located in the periphery of the lung. However extra pulmonary involvement of the mediastinum, diaphragm, and pleura can exist. It is classified according to its histological and imaging pattern in three types: cystic, mixed (solidcystic) and solid. Radical surgery is the treatment of choice and the only one that has demonstrated a decrease in the rate of recurrence. The use of neoadjuvant chemotherapy and radiotherapy are defined according to the histological type and presence of tumor-free margins. We present the case of a 3-year-old female patient with findings on conventional radiography and thoracic tomography of a solid mass with histopathological diagnosis of pleuropulmonary blastoma.
Subject(s)
Humans , Pulmonary Blastoma , Thoracic Surgery , Lung , NeoplasmsABSTRACT
PURPOSE: Incel (biricodar, VX-710) restores drug sensitivity to P-glycoprotein (MDR1) and multidrug-resistance-associated protein (MRP1) expressing cells. This phase II study evaluated the safety/tolerability, pharmacokinetics, and efficacy of VX-710 plus paclitaxel in women with advanced ovarian cancer refractory to prior paclitaxel therapy. EXPERIMENTAL DESIGN: Eligible patients had paclitaxel-refractory disease defined as progressive disease after a minimum of two cycles of paclitaxel (weekly or 3-week schedule) or relapsed disease within 4 months of prior paclitaxel therapy. Patients received 80 mg/m(2) paclitaxel over 3 h starting 4 h after initiation of a 24-h continuous intravenous infusion of 120 mg/m(2)/h VX-710. Cycles were repeated every 3 weeks. RESULTS: Fifty patients received treatment and 45 were evaluable for response. VX-710 + paclitaxel therapy was generally well tolerated. Myelosuppression was the principal toxicity, with a median Cycle 1 nadir absolute neutrophil count of 0.27 x 10(9) cells/L and a 47% overall incidence of Grade 4 neutropenia. Mild to moderate peripheral neuritis or neuropathy was the primary nonhematologic toxicity, affecting 62% of patients. Other nonhematologic toxicities were generally mild to moderate and reversible. Paclitaxel area under the concentration-versus-time curve (AUC) (16 +/- 5.3 microg x h/mL) during the first treatment cycle was comparable to standard 175 mg/m(2) paclitaxel administered over 3 h. Of the 3 patients who achieved partial responses, 2 had progressed during prior paclitaxel therapy. Twelve patients maintained stable disease and 14/45 (31%) of patients had CA-125 reductions of 50-90% for up to 24 weeks. The median time-to-disease progression was 10 weeks for the intent-to-treat population and 20.7 weeks for the CA-125 responders. CONCLUSIONS: The results suggest that VX-710 with paclitaxel has modest activity in paclitaxel-resistant ovarian cancer. Further research is warranted in less heavily treated patients.
