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6. Esthesioneuroblastoma in pediatric and adolescent age. A report from the TREP project in cooperation with the Italian Neuroblastoma and Soft Tissue Sarcoma Committees

Author

Bisogno Gianni, Soloni Pietro, Conte Massimo, Podda Marta, Ferrari Andrea, Garaventa Alberto, Luksch Roberto, and Cecchetto Giovanni

Subjects
Esthesioneuroblastoma, Olfactory neuroblastoma, Rare tumors, Nasal tumors, Chemotherapy, Radiotherapy, Late effects, Endocrine disorders, Neoplasms. Tumors. Oncology. Including cancer and carcinogens, RC254-282
Abstract

Abstract Background Esthesioneuroblastoma (ENB) is a rare, aggressive tumor with no established treatment in children. We analyzed a series of pediatric ENB patients with the aim of improving our knowledge of this disease. Methods 9 patients (6 males; age 0.9-18 years, median 9.9) were identified by searching the AIEOP (Italian Association of Pediatric Hematology and Oncology) registry and the national databases of rare tumors, soft tissue sarcomas (STS) and neuroblastomas. The data on the cases included in STS treatment protocols were collected prospectively and histology was centrally reviewed; the data and histology concerning the other children were reviewed for the purpose of this analysis. Results All tumors occurred in the sinonasal region with bone erosion (7 patients) and intracranial (4) or intraorbital (4) extension. Three patients were in Kadish stage B, and 6 in stage C. Complete tumor resection was very difficult to achieve, but adding chemotherapy and radiotherapy enabled tumor control in 8 patients. Response to chemotherapy was evident in 5/7 evaluable cases. Radiotherapy (48.5-60 Gy) was delivered in all children but one, due to early disease progression. With a median follow-up of 13.4 years (range 9.2-22.9), 7 patients are alive in 1st and one in 2nd complete remission. All surviving patients developed treatment-related sequelae, the most frequent being endocrine dysfunctions (4 patients) and craniofacial growth impairments (4 patients). Conclusions Our findings confirm that ENB in children has an aggressive presentation, but multimodal therapy can cure most patients. Our results are encouraging but future strategies must optimize treatment in terms of survival and related morbidities.

Published
2012
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7. Castleman's disease in childhood: report of three cases and review of the literature

Author

Guerrieri Patrizia, Cecchetto Giovanni, Scibetta Nunzia, Trizzino Antonino, Farruggia Piero, D'Amore Emanuele SG, and D'Angelo Paolo

Subjects
Castleman's disease, childhood, differential diagnosis, histological subgroups, outcome, Pediatrics, RJ1-570
Abstract

Abstract Castleman's disease (CD) is a rare, localized or generalized, lymphoproliferative disorder with a frequent mediastinal location, but possible in any lymph node or extra nodal site. It usually appears in young adults whilst it rarely occurs in childhood. There are only about 100 pediatric cases published, five of them in Italy. We report 3 cases of localized Castleman's disease, investigated in our Department in a 3 years period and reviewed the literature.

Published
2011
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8. Epithelial thymic tumours in paediatric age: a report from the TREP project

Author

Migliorati Roberta, Di Cataldo Andrea, Conte Massimo, Ferrari Andrea, Inserra Alessandro, Carretto Elena, Cecchetto Giovanni, and Bisogno Gianni

Subjects
Medicine
Abstract

Abstract Background Thymic epithelial tumours (thymoma and carcinoma) are exceptionally rare in children. We describe a national multicentre series with a view to illustrating their clinical behaviour and the results of treatment. Methods From January 2000 all patients under 18 years of age diagnosed with "rare paediatric tumours" were centrally registered by the Italian centres participating in the TREP project (Tumori Rari in Età Pediatrica [Rare Tumours in Paediatric Age]). The clinical data of children with a thymic epithelial tumour registered as at December 2009 were analyzed for the purposes of the present study. Results Our series comprised 4 patients with thymoma and 5 with carcinoma (4 males, 5 females; median age 12.4 years). The tumour masses were mainly large, exceeding 5 cm in largest diameter. Based on the Masaoka staging system, 3 patients were stage I, 1 was stage III, 1 was stage IVa and 4 were stage IVb. All 3 patients with stage I thymoma underwent complete tumour resection at diagnosis and were alive 22, 35 and 93 months after surgery. One patient with a thymoma metastasizing to the kidneys died rapidly due to respiratory failure. Thymic carcinomas were much more aggressive, infiltrating nearby organs (in 4 cases) and regional nodes (in 5), and spreading to the bone (in 3) and liver (in 1). All patients received multidrug chemotherapy (platinum derivatives + etoposide or other drugs) with evidence of tumour reduction in 3 cases. Two patients underwent partial tumour resection (after chemo-radiotherapy in one case) and 4 patients were given radiotherapy (45-54 Gy). All patients died of their disease. Conclusions Children with thymomas completely resected at diagnosis have an excellent prognosis while thymic carcinomas behave aggressively and carry a poor prognosis despite multimodal treatment.

Published
2011
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12. Mediastinal Germ Cell Tumors

Author

Schneider, Dominik T., Cecchetto, Giovanni, Olson, Thomas A., Reaman, Gregory H., Series Editor, Smith, Franklin O., Series Editor, Schneider, Dominik T., editor, Brecht, Ines B., editor, Olson, Thomas A., editor, and Ferrari, Andrea, editor

Published
2022
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23. Micro-ct evaluation of morphological degenerative features of sterno-clavicular joint for age-at-death estimation in forensic anthropology – a qualitative analysis

Author

Secco, Laura, primary, Padalino, Pasquale, additional, Franceschetto, Lisa, additional, Viero, Alessia, additional, Pizzi, Marco, additional, De Conti, Giorgio, additional, Viel, Guido, additional, Fais, aPaolo, additional, Giraudo, Chiara, additional, and Cecchetto, Giovanni, additional

Published
2023
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45. Mediastinal Germ Cell Tumors

Author

Schneider, Dominik T., Cecchetto, Giovanni, Olson, Thomas A., Schneider, Dominik T., editor, Brecht, Ines B., editor, Olson, Thomas A., editor, and Ferrari, Andrea, editor

Published
2012
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47. Pediatric nonrhabdomyosarcoma soft tissue sarcomas arising at visceral sites

Author

Ferrari, Andrea, Magni, Chiara, Bergamaschi, Luca, Cecchetto, Giovanni, Alaggio, Rita, Milano, Giuseppe Maria, Bertolini, Patrizia, Basso, Eleonora, Manzitti, Carla, Di Martino, Martina, Giurici, Nauga, Melchionda, Fraia, Cecinati, Valerio, Chiaravalli, Stefano, Affinita, Maria Carmen, Scagnellato, Angela, Casanova, Michela, and Bisogno, Gianni

Published
2017
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