medigraphic.com
Boletín Clínico de la Asociación Médica del Hospital Infantil del Estado de Sonora

2018, Number 1

<< Back

Bol Clin Hosp Infant Edo Son 2018; 35 (1)

Pancreatoblastoma Unusual malignancy in pediatrics

Quero-Hernández A, Reyes-Gómez U, López-Cruz G, Álvarez- Solís RM, Quero-Estrada A, Reyes-Hernández KL, Guerrero-Becerra M, de León-Herrera SR, Plascencia-Hernández A, Mercado-Uribe MC, Martínez-Arce P, Luévanos-Velázquez A

Language: Spanish
References: 16
Page: 70-76
PDF size: 480.11 Kb.


ABSTRACT

Primary malignant tumors of the pancreas are rare in children and adolescents. They include adenocarcinoma, acinar cell carcinoma, adenosquamous carcinoma, solid pseudopapillary tumor and pancreatoblastoma.
The objective is to present the clinical characteristics and treatment outcome of a patient with pancreatoblastoma. Male of 3 years with 4 months of evolution with abdominal pain and increase of it. Abdominal CT showed a pancreatic tumor involving body and tail, which was completely resected and received adjuvant chemotherapy based on Vincristine, Doxorubicin and Cisplatin, with bi-monthly tomographic evaluation and remains disease-free at 36 months of follow-up. Pancreatoblastoma requires a multidisciplinary approach. Complete control is achieved with complete tumor resection, in addition to a neoadjuvant and adjuvant chemotherapy regimen with Cisplatin and Anthracyclines. 5-year disease-free survival, however, is still considered unsatisfactory. The prognosis is linked to the complete resection of the lesion and the absence of mestastatic disease at the time of diagnosis.


REFERENCES

  1. Pérez EA, Gutiérrez JC, Koniaris LG, Neville HL, Thompson WR, Sola JE. Malignant pancreatic tumors: incidence and outcome in 58 pediatric patients. J Pediatr Surg, 2009; 44 (1): 197-203.

  2. Glick RD, Farzana DP, Pappo A, LaQuaglia M. Management of pancreatoblastoma in children and young adults. J Pediatr Hematol Oncol, 2012; 34: S47-S50.

  3. Rojas Y, Warneke CL, Dhamne CA, Tsao K,Nuchtern JG, Lally KP, et al. Primary malignant pancreatic neoplasms in children and adolescents: a 20 year experience. J Pediatr Surg, 2012; 47 (12): 2199-2204.

  4. Shorter NA, Glick RD, Klimstra DS, Brennan MF, LaQuaglia M. Malignant pancreatic tumors in childhood and adolescence: The Memorial Sloan-Kettering experience, 1967 to present. J Pediatr Surg, 2002; 37 (6): 887-892.

  5. Defachelles AS, Rocourt N, Branchereau S, Peuchmaur M. Pancreatoblastoma in children: diagnosis and therapeutic management. Bull Cancer, 2012; 99 (7-8): 793-799.

  6. Bien E, Godzinski J, Dall´igna P, Defachelles AS, Stachowicz ST, Orbach D, et al. Pancreatoblastoma: a report from the European cooperative study group for paediatric rare tumours (EXPeRT). Eur J Cancer, 2011; 47 (15): 2347-2352.

  7. Dhebri AR, Connor S, Campbell F, Ghaneh P, Sutton R, Neoptolemos JP. Diagnosis, treatment of pancreatoblastoma. Pancreatology, 2004; 4 (5): 452-453.

  8. Lee YJ, Hah JO. Long term survival of pancreatoblastoma in children. J Pediatr Hematol Oncol, 2007; 29 (12): 845-847.

  9. Xu C, Zhong L, Wang Y, Wang Y, Yang Z, Kang X. Clinical analysis of childhood pancreatoblastoma arising from the tail of the pancreas. J Pediatr Hematol Oncol, 2012; 34 (5): 177-181.

  10. Howlader N, Noone AM, Krapcho M, Garshell J, Miller D, Altekruse SF, Kosary CL, Yu M, Ruhl J, Tatalovich Z,Mariotto A, Lewis DR, Chen HS, Feuer EJ, Cronin KA (eds). SEER Cancer Statistics Review, 1975- 2012, National Cancer Institute. Bethesda, MD. Disponible en: http://seer.cancer.gov/csr/1975_2012/ (based on November 2014 SEER data submission, posted to the SEER web site, April 2015).

  11. Wood LD, Klimstra D. Pathology and genetics of pancreatic neoplasms with acinar differentiation.Semin Diagn Pathol, 2014; 31 (6): 491-497.

  12. Chung EM, Travis M, Conran RM. Pancreatic tumors in children: radiologic-pathologic correlation. Archive AFIP, 2006; 26 (4): 1211-1238.

  13. Roul M, Dall´gna P, Alaggio R, Toffolutti T, Cattarossi L, Gamba PG. Congenital pancreatoblastoma: a case report. J Ped Surg, 2015; 3: 120-122.

  14. Défachelles AS, Martin de L, Boutard P, Nelken B, Schneider P, Patte C. Pancreatoblastoma in childhood: clinical course and therapeutic management of seven patients. Med Pediatr Oncol, 2001; 37 (1): 47-52.

  15. Nasher O, Hall NJ, Sebire NJ, de Coppo P, Pierro A. Pancreatic tumours in children: diagnosis, treatment and outcome. Pediatr Surg Int, 2015; 31 (9): 831-835.

  16. Dall´Igna P, Cecchetto G, Bisogno G, Conte M, D´Angelo P, De Leonardis F. Pancreatic tumors in children and adolescents: The Italian TREP project experience. Pediatr Blood Cancer, 2010; 54 (5): 675-680.




2020     |     www.medigraphic.com