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Rare Malignant Tumors

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Pediatric Surgery

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Abstract

The pediatric population has a distinct set of rare malignant tumors that require understanding to better our ability to recognize, diagnose, and properly manage these diseases. Recent advancements in medical database recording have allowed large analyses of these rare pediatric cancers.

The majority of breast masses in children are benign; however, the incidence of malignancy has increased in the last two decades. Delay in diagnosis and treatment in children with breast cancer may occur because of reluctance to intervene on the developing breast.

Malignant tumors of the thymus include thymomas, carcinoids, carcinomas, germ cell tumors, neuroendocrine tumors, and lymphomas.

Metastatic disease far outnumbers primary lung cancers, but most primary lung tumors are malignant. Some pediatric pulmonary malignancies are associated with underlying congenital lesions. One third of the patients have distant metastases at presentation.

Gastrointestinal (GI) tumors include lymphomas, carcinomas, carcinoid tumors, sarcomas, and neuroendocrine tumors (NET). The most frequent symptom is abdominal pain, which is construed as a vague complaint and may delay diagnosis.

Pediatric non-Wilms’ renal tumors (NWRT) are a heterogeneous group of neoplasms with variable malignant potential, mortality, and response to therapeutic modalities. This group includes renal cell carcinoma (RCC), clear cell sarcoma of the kidney (CCSK), and malignant rhabdoid tumor (MRT).

The improved outcomes in these rare pediatric cancers may be due to advancements in diagnostic tools and therapeutic measures. Still, a lower threshold for consideration of malignancy is necessary. Early recognition and ongoing investigation may result in improved outcomes.

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References

  • Ahmed HU, Arya M, Levitt G, Duffy PG, Mushtaq I, Sebire NJ. Part I: Primary malignant non-Wilms’ renal tumours in children. Lancet Oncol. 2007;8(8):730–7.

    Article  PubMed  Google Scholar 

  • Allan BJ, Thorson CM, Davis JS, Van Haren RM, Parikh PP, Perez EA, Lew JI, Sola JE. An analysis of 73 cases of pediatric malignant tumors of the thymus. J Surg Res. 2013;184(1):397–403.

    Article  PubMed  Google Scholar 

  • Anders CK, Johnson R, Litton J, Phillips M, Bleyer A. Breast cancer before age 40 years. Semin Oncol. 2009;36(3):237–49.

    Article  PubMed  PubMed Central  Google Scholar 

  • Argani P, Perlman EJ, Breslow NE, Browning NG, Green DM, D’Angio GJ, Beckwith JB. Clear cell sarcoma of the kidney: a review of 351 cases from the National Wilms Tumor Study Group Pathology Center. Am J Surg Pathol. 2000;24(1):4–18.

    Article  CAS  PubMed  Google Scholar 

  • Bethel CA, Bhattacharyya N, Hutchinson C, Ruymann F, Cooney DR. Alimentary tract malignancies in children. J Pediatr Surg. 1997;32(7):1004–8; discussion 1008–1009.

    Article  PubMed  CAS  Google Scholar 

  • Bollard CM, Lim MS, Gross TG, C. O. G. N.-H. L. Committee. Children’s Oncology Group’s 2013 blueprint for research: non-Hodgkin lymphoma. Pediatr Blood Cancer. 2013;60(6):979–84.

    Article  PubMed  Google Scholar 

  • Curtis JL, Burns RC, Wang L, Mahour GH, Ford HR. Primary gastric tumors of infancy and childhood: 54-year experience at a single institution. J Pediatr Surg. 2008;43(8):1487–93.

    Article  PubMed  Google Scholar 

  • Dishop MK, Kuruvilla S. Primary and metastatic lung tumors in the pediatric population: a review and 25-year experience at a large children’s hospital. Arch Pathol Lab Med. 2008;132(7):1079–103.

    PubMed  Google Scholar 

  • Fidani P, De Ioris MA, Serra A, De Sio L, Ilari I, Cozza R, Boldrini R, Milano GM, Garre ML, Donfrancesco A. A multimodal strategy based on surgery, radiotherapy, ICE regimen and high dose chemotherapy in atypical teratoid/rhabdoid tumours: a single institution experience. J Neuro-Oncol. 2009;92(2):177–83.

    Article  Google Scholar 

  • Garcia-Foncillas J, Diaz-Rubio E. Progress in metastatic colorectal cancer: growing role of cetuximab to optimize clinical outcome. Clin Transl Oncol. 2010;12(8):533–42.

    Article  PubMed  CAS  Google Scholar 

  • Gutierrez JC, Housri N, Koniaris LG, Fischer AC, Sola JE. Malignant breast cancer in children: a review of 75 patients. J Surg Res. 2008;147(2):182–8.

    Article  PubMed  Google Scholar 

  • Harting MT, Blakely ML, Herzog CE, Lally KP, Ajani JA, Andrassy RJ. Treatment issues in pediatric gastric adenocarcinoma. J Pediatr Surg. 2004;39(8):e8–10.

    Article  PubMed  Google Scholar 

  • Hayat MJ, Howlader N, Reichman ME, Edwards BK. Cancer statistics, trends, and multiple primary cancer analyses from the Surveillance, Epidemiology, and End Results (SEER) Program. Oncologist. 2007;12(1):20–37.

    Article  PubMed  Google Scholar 

  • Hoot AC, Russo P, Judkins AR, Perlman EJ, Biegel JA. Immunohistochemical analysis of hSNF5/INI1 distinguishes renal and extra-renal malignant rhabdoid tumors from other pediatric soft tissue tumors. Am J Surg Pathol. 2004;28(11):1485–91.

    Article  PubMed  Google Scholar 

  • Jaggers J, Balsara K. Mediastinal masses in children. Semin Thorac Cardiovasc Surg. 2004;16(3):201–8.

    Article  PubMed  Google Scholar 

  • Karnak I, Ciftci AO, Senocak ME, Buyukpamukcu N. Colorectal carcinoma in children. J Pediatr Surg. 1999;34(10):1499–504.

    Article  PubMed  CAS  Google Scholar 

  • Kelly KM, Hodgson D, Appel B, Chen L, Cole PD, Horton T, Keller FG, C. O. G. H. L. Committee. Children’s Oncology Group’s 2013 blueprint for research: Hodgkin lymphoma. Pediatr Blood Cancer. 2013;60(6):972–8.

    Article  PubMed  Google Scholar 

  • Ladd AP, Grosfeld JL. Gastrointestinal tumors in children and adolescents. Semin Pediatr Surg. 2006;15(1):37–47.

    Article  PubMed  Google Scholar 

  • LaQuaglia MP, Heller G, Filippa DA, Karasakalides A, Vlamis V, Wollner N, Enker WE, Cohen AM, Exelby PR. Prognostic factors and outcome in patients 21 years and under with colorectal carcinoma. J Pediatr Surg. 1992;27(8):1085–9; discussion 1089–1090.

    Article  PubMed  CAS  Google Scholar 

  • Leveque J, Meunier B, Wattier E, Burtin F, Grall JY, Kerisit J. Malignant cystosarcomas phyllodes of the breast in adolescent females. Eur J Obstet Gynecol Reprod Biol. 1994;54(3):197–203.

    Article  PubMed  CAS  Google Scholar 

  • Lobeck IN, Jeste N, Geller J, et al. Surgical management and surveillance of pediatric appendiceal carcinoid tumor. J Pediatr Surg. 2017;52(6):925–7.

    Article  PubMed  Google Scholar 

  • McGill TW, Downey EC, Westbrook J, Wade D, de la Garza J. Gastric carcinoma in children. J Pediatr Surg. 1993;28(12):1620–1.

    Article  PubMed  CAS  Google Scholar 

  • Mullen B, Richardson JD. Primary anterior mediastinal tumors in children and adults. Ann Thorac Surg. 1986;42(3):338–45.

    Article  PubMed  CAS  Google Scholar 

  • Neville HL, Hogan AR, Zhuge Y, Perez EA, Cheung MC, Koniaris LG, Thompson WR, Sola JE. Incidence and outcomes of malignant pediatric lung neoplasms. J Surg Res. 2009;156(2):224–30.

    Article  PubMed  Google Scholar 

  • Perez EA, Livingstone AS, Franceschi D, Rocha-Lima C, Lee DJ, Hodgson N, Jorda M, Koniaris LG. Current incidence and outcomes of gastrointestinal mesenchymal tumors including gastrointestinal stromal tumors. J Am Coll Surg. 2006;202(4):623–9.

    Article  PubMed  Google Scholar 

  • Rajan PB, Cranor ML, Rosen PP. Cystosarcoma phyllodes in adolescent girls and young women: a study of 45 patients. Am J Surg Pathol. 1998;22(1):64–9.

    Article  PubMed  CAS  Google Scholar 

  • Richards MK, Goldin AB, Beierle EA, et al. Breast malignancies in children: presentation, management, and survival. Ann Surg Oncol. 2017;24(6):1482–91.

    Article  PubMed  Google Scholar 

  • Rogers DA, Lobe TE, Rao BN, Fleming ID, Schropp KP, Pratt AS, Pappo AS. Breast malignancy in children. J Pediatr Surg. 1994;29(1):48–51.

    Article  PubMed  CAS  Google Scholar 

  • Rothstein DH, Voss SD, Isakoff M, Puder M. Thymoma in a child: case report and review of the literature. Pediatr Surg Int. 2005;21(7):548–51.

    Article  PubMed  Google Scholar 

  • Sausville JE, Hernandez DJ, Argani P, Gearhart JP. Pediatric renal cell carcinoma. J Pediatr Urol. 2009;5(4):308–14.

    Article  PubMed  Google Scholar 

  • Silberstein J, Grabowski J, Saltzstein SL, Kane CJ. Renal cell carcinoma in the pediatric population: results from the California Cancer Registry. Pediatr Blood Cancer. 2009;52(2):237–41.

    Article  PubMed  Google Scholar 

  • Spigland N, Di Lorenzo M, Youssef S, Russo P, Brandt M. Malignant thymoma in children: a 20-year review. J Pediatr Surg. 1990;25(11):1143–6.

    Article  PubMed  CAS  Google Scholar 

  • Spunt SL, Pratt CB, Rao BN, Pritchard M, Jenkins JJ, Hill DA, Cain AM, Pappo AS. Childhood carcinoid tumors: the St Jude Children’s Research Hospital experience. J Pediatr Surg. 2000;35(9):1282–6.

    Article  PubMed  CAS  Google Scholar 

  • Stachowicz-Stencel T, Orbach D, Brecht I, et al. Thymoma and thymic carcinoma in children and adolescents: a report from the European Cooperative Study Group for Pediatric Rare Tumors (EXPeRT). Eur J Cancer. 2015;51(16):2444–52.

    Article  PubMed  Google Scholar 

  • Sultan I, Qaddoumi I, Rodriguez-Galindo C, Nassan AA, Ghandour K, Al-Hussaini M. Age, stage, and radiotherapy, but not primary tumor site, affects the outcome of patients with malignant rhabdoid tumors. Pediatr Blood Cancer. 2010;54(1):35–40.

    Article  PubMed  Google Scholar 

  • Szanto J, Andras C, Tsakiris J, Gomba S, Szentirmay Z, Banlaki S, Szilagyi I, Kiss C, Antall P, Horvath A, Lengyel L, Castiglione-Gertsch M. Secretory breast cancer in a 7.5-year old boy. Breast. 2004;13(5):439–42.

    Article  PubMed  CAS  Google Scholar 

  • Tischer W, Reddemann H, Herzog P, Gdanietz K, Witt J, Wurnig P, Reiner A. Experience in surgical treatment of pulmonary and bronchial tumours in childhood. Prog Pediatr Surg. 1987;21:118–35.

    Article  PubMed  CAS  Google Scholar 

  • Turalba CI, el-Mahdi AM, Ladaga L. Fatal metastatic cystosarcoma phyllodes in an adolescent female: case report and review of treatment approaches. J Surg Oncol. 1986;33(3):176–81.

    Article  PubMed  CAS  Google Scholar 

  • Weldon CB, Shamberger RC. Pediatric pulmonary tumors: primary and metastatic. Semin Pediatr Surg. 2008;17(1):17–29.

    Article  PubMed  Google Scholar 

  • West KW, Rescorla FJ, Scherer LR 3rd, Grosfeld JL. Diagnosis and treatment of symptomatic breast masses in the pediatric population. J Pediatr Surg. 1995;30(2):182–6; discussion 186–187.

    Article  PubMed  CAS  Google Scholar 

  • Wolpin BM, Mayer RJ. Systemic treatment of colorectal cancer. Gastroenterology. 2008;134(5):1296–310.

    Article  PubMed  CAS  Google Scholar 

  • Yang R, Cheung MC, Zhuge Y, Armstrong C, Koniaris LG, Sola JE. Primary solid tumors of the colon and rectum in the pediatric patient: a review of 270 cases. J Surg Res. 2010;161(2):209–16.

    Article  PubMed  Google Scholar 

  • Yao JC, Ajani JA. Adjuvant and preoperative chemotherapy for gastric cancer. Curr Oncol Rep. 2002;4(3):222–8.

    Article  PubMed  Google Scholar 

  • Yaris N, Nas Y, Cobanoglu U, Yavuz MN. Thymic carcinoma in children. Pediatr Blood Cancer. 2006;47(2):224–7.

    Article  PubMed  Google Scholar 

  • Young JL Jr, Miller RW. Incidence of malignant tumors in U. S. children. J Pediatr. 1975;86(2):254–8.

    Article  PubMed  Google Scholar 

  • Zang EA, Wynder EL. Differences in lung cancer risk between men and women: examination of the evidence. J Natl Cancer Inst. 1996;88(3–4):183–92.

    Article  PubMed  CAS  Google Scholar 

  • Zhuge Y, Cheung MC, Yang R, Eldick D, Koniaris LG, Sola JE. Pediatric intestinal foregut and small bowel solid tumors: a review of 105 cases. J Surg Res. 2009a;156(1):95–102.

    Article  PubMed  Google Scholar 

  • Zhuge Y, Cheung MC, Yang R, Koniaris LG. Diagnosing gastrointestinal stromal tumors before the year 2000. Cancer Epidemiol Biomark Prev. 2009b;18(3):1013–4; author reply 1014–1015.

    Article  Google Scholar 

  • Zhuge Y, Cheung MC, Yang R, Perez EA, Koniaris LG, Sola JE. Pediatric non-Wilms renal tumors: subtypes, survival, and prognostic indicators. J Surg Res. 2010;163(2):257–63.

    Article  PubMed  Google Scholar 

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Authors and Affiliations

  1. Division of Pediatric Surgery, DeWitt-Daughtry Family Department of Surgery, Leonard M. Miller School of Medicine, University of Miami, Miami, FL, USA

    Casey J. Allen, Jun Tashiro & Juan E. Sola

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  1. Casey J. Allen
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  2. Jun Tashiro
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  3. Juan E. Sola
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Correspondence to Casey J. Allen .

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Editors and Affiliations

  1. Our Lady’s Children's Hospital, National Children’s Research Centre, Dublin, Ireland

    Prem Puri

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Allen, C.J., Tashiro, J., Sola, J.E. (2020). Rare Malignant Tumors. In: Puri, P. (eds) Pediatric Surgery. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-38482-0_160-1

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  • DOI: https://doi.org/10.1007/978-3-642-38482-0_160-1

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  • Print ISBN: 978-3-642-38482-0

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