Pulmonary blastoma in adult: Dramatic but transient response to doxorubicin plus ifosfamide

A 22-year-old woman presented in June 2006 with a massive hemoptysis, revealing a large pulmonary right tumour. An urgent right pneumectomy was required with a pericardiectomy extended to the left auricle. Brain, chest and abdominal CT-scan and 18 F-fl uorodeoxyglucose (FDG) Positron Emission Tomography (PET) did not reveal metastatic extension. The initial histopathology report showed a 12 cm pulmonary blastoma biphasic subtype with no lymph node involvement and the resection was complete. A second histological examination confi rmed the pulmonary blastoma diagnosis, well-differentiated with rhabdomyoblastic, chondroid, and osteoid differentiation. As the margins were clear, no adjuvant treatment was offered. On November 2006, CT-scan, PET scan and liver MRI showed a massive local recurrence with liver extension through diaphragm (Figure 1, size 44 mm x 39 mm). The fi rst-line treatment consisted of six cycles of combined chemotherapy (Ifosfamide 2.5 g/m 2 on day 1 plus Doxorubicin 20 mg/m 2 once daily for three consecutive days every 21 days with lenograstim support d6 to d13). Successive tumour assessments demonstrated major tumour shrinkage. After the sixth cycle, the size of the residual tumour was 21 mm x 14 mm (Figure 2). The patient then received two additional cycles of doxorubicin (70 mg/m 2 /d q3wks) followed with stereotactic radiotherapy on the residual tumour (40 Gy). At the end of radiotherapy, the largest size of the residual mass was 11 mm without FDG-fi xation. Nevertheless, a second local relapse with thoracic vertebra and scapula involvement was diagnosed in May 2008. The patient received three successive ineffective lines of chemotherapy (Carboplatin  Vincristine/ Cyclophosphamide  Actinomycine-D/docetaxel gemcitabine). She died in November 2008.