Neuroblastoma in the adult: the Italian experience with 21 patients.
作者信息
Sorrentino Stefania, Gigliotti Anna R, Sementa Angela R, Morsellino Vera, Conte Massimo, Erminio Giovanni, Buffa Piero, Granata Claudio, Mazzocco Katia, Garaventa Alberto, De Bernardi Bruno
机构信息
Departments of *Hematology and Oncology ‡Pathology §Surgery, Istituto Giannina Gaslini †Service of Clinical Epidemiology and Biostatistics ∥Service of Diagnostic Imaging, Istituto Giannina Gaslini, Genova, Italy.
出版信息
J Pediatr Hematol Oncol. 2014 Nov;36(8):e499-505. doi: 10.1097/MPH.0000000000000144.
BACKGROUND
Neuroblastoma in the adult is rare. No established therapeutic guidelines exist for these patients and the literature on this issue is scant and contradictory.
MATERIALS AND METHODS
Between 1986 and 2011, 21 adults (18 to 38 y; median, 23) diagnosed with neuroblastoma were referred to our hospital. Three of the 21 were classified as neuroblastoma, not otherwise specified, 13 as neuroblastoma, schwannian stroma-poor, and 5 as ganglioneuroblastoma, nodular. Nine patients had a resectable (stage 1/2) and 6 an unresectable primary tumor (stage 3); 6 had disseminated disease (stage 4).
RESULTS
Of 9 stage 1/2 patients, 6 underwent surgery alone (2 survive, 4 died), 2 received adjuvant chemotherapy (both survive), and 1 received radiation therapy (alive). Four of the 6 stage 3 patients received chemotherapy and died, 1 underwent partial tumor resection only and died, and 1 received radiation therapy after partial tumor resection and is alive. The 6 stage 4 patients received chemotherapy with/without radiotherapy, and all died. Event-free survival at 10 years was 33.3% for stage 1/2, 16.7% for stage 3, and 0% for stage 4 patients. The 10-year overall and event-free survival rates were 39.8% and 19.1%, respectively.
CONCLUSIONS
The outcome of neuroblastoma in adults is poorer than in younger patients at all stages. The clinical course seems modestly influenced by therapy.
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