Autologous stem cell transplantation as first line treatment after\nincomplete excision of pancreatoblastoma
A 14-year-old girl was hospitalized with nausea, vomiting and abdominal pain. An\nabdominal CT scan showed a heterogeneous mass located in the pancreatic body and tail,\nmeasuring 13.5 x 10.0 x 7.0 cm, spleen enlargement and ascites. Serum\nα-fetoprotein was 1.2 IU/mL (reference: < 11.3 IU/mL) and the\ncarbohydrate antigen (CA) 19.9 was 4.5 IU/mL(reference: < 37 IU/mL) and no\nevidence of metastatic disease. As surgical resection of the tumor was not feasible, a\nbiopsy was performed reaching the histopathologic diagnosis of PBL and\nimmunohistochemical markers were positive for AE1+AE3, vimentin (focal),\nα-1-antitrypsin and CEA. Familial adenomatous polyposis and other genetic\ndiseases were investigated and discarded. Following four cycles of a chemotherapy\nregimen with cisplatin (80 mg/m2) and doxorubicin (60 mg/m2)\nabdominal CT showed that the lesion had decreased to 6.2 x 4.5 x 4.8 cm. She therefore\nunderwent a surgical attempt to resect the tumor; intraoperative findings revealed that\nthe tumor was attached to the meso and with a large quantity of peritumoral fibrosis and\nsplenic vessels adhered to it. The tumor was removed with a microscopic margin. The\npatient's surgical recovery was uneventful and then she received two additional cycles\nof the same chemotherapy combination, followed by ASCT. The conditioning regimen\nconsisted of melphalan (90 mg/m2/d x2), carboplatin (AUC 5) x4 and etoposide\n(200 mg/m2/d x4). A total of 9.6 x 106 CD34 cells were infused.\nShe had engraftment of 500 neutrophils/uL on D+8 and 1000 on day D+12. Platelet\nengraftment > 20000 and > 50000 cells/µL was achieved on D+10 and\nD+13, respectively. Only manageable toxicities were observed after transplantation,\nincluding mucositis, diarrhea, weight loss and neutropenic fever. She had no\nlife-threating events. The patient was discharged on D+20 and followed up in the\noutpatient clinic. The last control abdominal CT was normal two years ago. Four years\nfollowing transplantation she is very well with no evidence of disease and has normal\ncardiac function, no evidence of renal toxicities and normal ovarian function. The only\nlate effect observed is hearing loss for high frequencies.