Pediatric Malignancies of Head and Neck

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Pediatric Malignancies of Head and Neck

Pediatric Otolaryngology
Author Credentials 

William O. Collins, MD
Associate Professor
Department of Otolaryngology
University of Florida College of Medicine
Gainesville, FL

Module Summary

Although most head and neck masses in children are benign, malignant neoplasms can occur. If diagnosis is delayed, significant morbidity and mortality can result. Thus it is important to recognize common presenting signs and symptoms of head and neck malignant neoplasms in children in order to institute prompt diagnostic evaluation. Early diagnosis can lead to earlier therapeutic intervention with both medical and surgical treatments.

Module Learning Objectives 
  1. Recognize common anatomic areas in the pediatric head and neck which can develop neoplastic processes.
  2. Properly stage malignant neoplasms of the head and neck in pediatric patients.
  3. Develop treatment strategies for pediatric head and neck malignant neoplasms.

Anatomy

Learning Objectives 

To understand the anatomic distribution of malignant neoplasms of the head and neck in pediatric patients, including patterns of locoregional metastasis.

References 
  1. Albright JT, Topham AK, Reilly JS. Pediatric head and neck malignancies: U.S. incidence and trends over 2 decades. Arch Otolaryngol Head Neck Surg. 2002;128:655.
  2. Cunningham MJ, Myers EN, Bluestone CD. Malignant tumors of the head and neck in children: A 20 year review. Int J Pediatr Otorhinolaryngol. 1987 Oct;13(3):279-92.
  3. Whittemore KR, Cunningham MJ. Malignant Tumors of the Head and Neck. In Pediatric Otolaryngology, Fourth Edition. Ed. Bluestone CD, Stool SE, Alper CM, et al. Saunders, Philadelphia, PA. 2003; p. 1703-1736.

Pathogenesis

Learning Objectives 

To recognize the abnormal molecular processes which lead to malignant transformation of pediatric head and neck lesions.

Basic Science

Learning Objectives 

To understand the histologic cell types affected in pediatric head and neck malignancies.

References 
  1. Cunningham MJ, Myers EN, Bluestone CD. Malignant tumors of the head and neck in children: a 20 year review. Int J Pediatr Otorhinolaryngol. 1987 Oct;13(3):279-92.
  2. Albright JT, Topham AK, Reilly JS. Pediatric head and neck malignancies: U.S. incidence and trends over 2 decades. Arch Otolaryngol Head Neck Surg. 2002 Jun;128(6):655-9.
  3. Sandlund JT, Downing JR, Crist WM. Non-Hodgkin’s lymphoma in childhood. N Engl J Med. 1996 May 9;334(19):1238-48.
  4. Tubergen DG, Krailo MD, Meadows AT, et al. Comparison of treatment regimens for pediatric lymphoblastic non-Hodgkin’s lymphoma: a Children’s Cancer Group study. J Clin Oncol. 1995 Jun;13(6):1368-76.
  5. Weiner ES. Head and neck rhabdomyosarcoma. Seminar Ped Surg. 1994 Aug;3(3):203-6.
  6. De Keyser L, Van Herle A. Differentiated thyroid cancer in children. Head Neck Surg. 1985;8(2):100.
  7. O’Riordian DS, O’Brien T, Weaver AL, et al. Medullary thyroid carcinoma in multiple endocrine neoplasia types 2A and 2B. Surgery. 1994 Dec;116(6):1017-23.
  8. Feinmesser R, Lubin E, Segal K, et al. Carcinoma of the thyroid in children - a review. J Pediatr Endocrinol Metab. 1997 Nov-Dec;10(6):561-8.
  9. Tom LWC, Anderson GJ, Womer RB, et al. Nasopharyngeal malignancies in children. Laryngoscope. 1992 May;102(5):509-14.
  10. Byers R, Piorkowski R, Luna M. Malignant parotid tumors in patients under 20 years of age. Arch Otolaryngol Head Neck Surg. 1984; 110:232.
  11. Friedman[[initial?]], Levin B, Grybauskas V, et al. Malignant tumors of the major salivary glands. Otolaryngol Clin North Am. 1986 Nov;19(4):625-36.
  12. Albright JT, Topham AK, Reilly JS. Pediatric head and neck malignancies. Arch Otolaryngol Head Neck Surg. 2002 Jun;128(6):655-9.

Incidence

Learning Objectives 

To identify the incidence of pediatric head and neck malignancies:

  1. Lymphomas:
    1. Hodgkins
    2. Non-Hodgkins
  2. Sarcomas: rhabdomyosarcoma
  3. Thyroid carcinoma
  4. Nasopharyngeal carcinoma
  5. Neuroblastoma
References 
  1. Whittemore KR, Cunningham MJ. Malignant Tumors of the Head and Neck. In Pediatric Otolaryngology, Fourth Edition. Ed. Bluestone CD, Stool SE, Alper CM, et al. Saunders, Philadelphia, PA. 2003; p. 1703-1736.
  2. Albright JT, Topham AK, Reilly JS. Pediatric head and neck malignancies. Arch Otolaryngol Head Neck Surg. 2002 Jun;128(6):655-9.

Genetics

Learning Objectives 

To recognize genetic mutations which can lead to malignant transformation in pediatric head and neck malignancies.

References 
  1. Givens DJ, Buchmann LO, Agarwal AM, et al. BRAF V600E does not predict aggressive features of pediatric papillary thyroid carcinoma. Laryngoscope. 2014;124(9): e389-e393.

Patient Evaluation

Learning Objectives 

To comprehensively evaluate a pediatric patient with a suspected malignant neoplasm of the head and neck.

References 
  1. Maris JM, Matthay KK. Molecular biology of neuroblastoma. J Clin Oncol. 1999;17:2264.
  2. O’Riordian DS, O’Brien T, Weaver AL, et al. Medullary thyroid carcinoma in multiple endocrine neoplasia types 2A and 2B. Surgery. 1994;116:1017.
  3. Raney RB, Anderson JR, Barr FG, et al. Rhabdomyosarcoma and undifferentiated sarcoma in the first two decades of life: a selective review of Intergroup Rhabdomyosarcoma Study Group experience and rationale for Intergroup Rhabdomyosarcoma Study V. Am J Pediatr Hematol Oncol. 2001;23:215.
  4. Tubergen DG, Krailo MD, Meadows AT, et al. Comparison of treatment regimens for pediatric lymphoblastic non-Hodgkin’s lymphoma: a Children’s Cancer Group study. J Clin Oncol. 1995;13:2023.
  5. Weiner ES. Head and neck rhabdomyosarcoma. Semin Pediatr Surg. 1994 Aug;3(3):203-6.
  6. Shapiro NL, Bhattacharyya N. Clinical characteristics and survival for major salivary gland malignancies in children. Otolaryngol Head Neck Surg. 2006;134:631-634.
  7. Thompson GB, Hay ID. Current strategies for surgical management and adjuvant treatment of childhood papillary thyroid carcinoma. World J Surg. 2004;28:1187-1198.
  8. Benoit MM, Bhattacharyya N, Faquin W, et al. Cancer of the nasal cavity in the pediatric population. Pediatrics. 2008;121:e141-e145.
  9. Collini P, Mattavelli F, Pellegrinelli A, et al. Papillary carcinoma of the thyroid gland of childhood and adolescence: morphologic subtypes, biologic behavior and prognosis: a clinicopathologic study of 42 sporadic cases treated at a single institution during a 30-year period. Am J Surg Pathol. 2006;30:1420-1426.
  10. Rahbar R, Grimmer JF, Vargas SO, et al. Mucoepidermoid carcinoma of the parotid gland in children: a 10 year experience. Arch Otolaryngol Head Neck Surg. 2006;132:375-380.

Imaging

Learning Objectives 

To describe the complementary roles of different imaging modalities in the evaluation of pediatric head and neck malignancies:

  • Ultrasound
  • CT scanning
  • MRI imaging
  • Radionuclide scanning
References 
  1. Whittemore KR, Cunningham MJ. Malignant Tumors of the Head and Neck. In Pediatric Otolaryngology, Fourth Edition. Ed. Bluestone CD, Stool SE, Alper CM, et al. Saunders, Philadelphia, PA. 2003; p. 1703-1736.

Pathology

Learning Objectives 

To describe the histologic abnormalities of common pediatric head and neck malignancies.

References 
  1. Feinmesser R, Lubin E, Segal K, et al. Carcinoma of the thyroid in children - a review. J Pediatr Endocrinol Metab. 1997;10:561.
  2. Harris N. Hodgkin’s lymphomas: classification, diagnosis and grading. Semin Hematol. 1999;36:220.
  3. Sandlund JT, Downing JR, Crist WM. Non-Hodgkin’s lymphoma in childhood. N Engl J Med. 1996;334:1238.
  4. Tubergen DG, Krailo MD, Meadows AT, et al. Comparison of treatment regimens for pediatric lymphoblastic non-Hodgkin’s lymphoma: a Children’s Cancer Group study. J Clin Oncol. 1995;13:2023.
  5. Weiner ES. Head and neck rhabdomyosarcoma. Semin Pediatr Surg. 1994 Aug;3(3):203-6.

Treatment

Learning Objectives 

To describe treatment strategies for pediatric head and neck malignancies:

  1. Lymphomas:
    1. Hodgkins
    2. Non-Hodgkins
  2. Sarcomas: rhabdomyosarcoma
  3. Thyroid carcinoma
  4. Nasopharyngeal carcinoma
  5. Neuroblastoma
References 
  1. Castleberry RP. Biology and treatment of neuroblastoma. Pediatr Clin North Am. 1997 Aug;44(4):919-37.
  2. Fearon B, Forte V, Brama I. Malignant nasopharyngeal tumors in children. Laryngoscope. 1990; 100:470.
  3. Raney RB, Anderson JR, Barr FG, et al. Rhabdomyosarcoma and undifferentiated sarcoma in the first two decades of life: a selective review of Intergroup Rhabdomyosarcoma Study Group experience and rationale for Intergroup Rhabdomyosarcoma Study V. Am J Pediatr Hematol Oncol. 2001 May;23(4):215-20.
  4. Sandlund JT, Downing JR, Crist WM. Non-Hodgkin’s lymphoma in childhood. N Engl J Med. 1996;334:1238.
  5. Tom LWC, Anderson GJ, Womer RB, et al. Nasopharyngeal malignancies in children. Laryngoscope. 1992 May;102(5):509-14.
  6. Tubergen DG, Krailo MD, Meadows AT, et al. Comparison of treatment regimens for pediatric lymphoblastic non-Hodgkin’s lymphoma: a Children’s Cancer Group study. J Clin Oncol. 1995 Jun;13(6):1368-76.
  7. Wei WI. Nasopharyngeal cancer: current status of management. A New York Head and Neck Society lecture. Arch Otolaryngol Head Neck Surg. 2001;127:766.
  8. Weiner ES. Head and neck rhabdomyosarcoma. Semin Pediatr Surg. 1994;3:203.
  9. Whittemore KR Jr., Cunningham MJ. Cancer of the head and neck in the pediatric population. In: Myers EN, Suen JY, Hanna E, et al., editors. Cancer of the head and neck. 4th ed. Philadelphia: W.B. Saunders Co.; 2002.
  10. Whittemore KR Jr., Cunningham MJ. Malignant tumors of the head and neck. In: Bluestone CD, Alper CM, Arjmand EM, et al, editors. Pediatric otolaryngology. 4th ed. Philadelphia: W.B. Saunders Co.; 2002.

Medical Therapies

Learning Objectives 

To describe medical therapies for pediatric head and neck malignancies:

  • Chemotherapy
  • Immunotherapy
  • Antiangiogenesis
References 
  1. Wei WI. Nasopharyngeal cancer: current status of management. A New York Head and Neck Society lecture. Arch Otolaryngol Head Neck Surg. 2001;127:766.
  2. Walterhouse DO, Pappo AS, Baker KS, et al. Rhabdomyosarcoma of the parotid region occurring in childhood and adolescence. Cancer. 2001;92:3135-3146.

Surgical Therapies

Learning Objectives 

To develop surgical strategies for treatment of:

  1. Lymphomas:
    1. Hodgkins
    2. Non-Hodgkins
  2. Sarcomas: rhabdomyosarcoma
  3. Thyroid carcinoma
  4. Nasopharyngeal carcinoma
  5. Neuroblastoma
References 
  1. Byers R, Piorkowski R, Luna M. Malignant parotid tumors in patients under 20 years of age. Arch Otolaryngol Head Neck Surg. 1984;110:232.
  2. Cunningham MJ. Salivary gland surgery. In: Bluestone CD, Rosenfeld RM, editors. Surgical atlas of pediatric otolaryngology. 2nd ed. Hamilton, Ontario: BC Decker; 2002: 515.
  3. Cunningham MJ. Thyroidectomy. In: Bluestone CD, Rosenfeld RM, editors. Surgical atlas of pediatric otolaryngology. 2nd ed. Hamilton, Ontario: BC Decker; 2002: 539.
  4. Daya H, Chan HSL, Sirkin W, et al. Pediatric rhabdomyosarcoma of the head and neck: is there a place for surgical management? Arch Otolaryngol Head Neck Surg. 2000; 126:468.
  5. Feinmesser R, Lubin E, Segal K, et al. Carcinoma of the thyroid in children - a review. J Pediatr Endocrinol Metab. 1997 Nov-Dec;10(6):561-8.
  6. Friedman[[initial?]], Levin B, Grybauskas V, et al. Malignant tumors of the major salivary glands. Otolaryngol Clin North Am. 1986 Nov;19(4):625-36.
  7. Lallier M, St-Vil D, Giroux M, et al. Prophylactic thyroidectomy for medullary thyroid carcinoma in gene carriers of MEN 2 syndromes. J Pediatr Surg 1997; 6:42.
  8. Vassilopoulou-Sellin R, Goepfert H, Raney B, et al. Differentiated thyroid cancer in children and adolescents: clinical outcome and mortality after long-term follow-up. Head Neck. 11998 Sep;20(6):549-55.
  9. O’Riordian DS, O’Brien T, Weaver AL, et al. Medullary thyroid carcinoma in multiple endocrine neoplasia types 2A and 2B. Surgery. 1994 Dec;116(6):1017-23.
  10. Raney RB, Anderson JR, Barr FG, et al. Rhabdomyosarcoma and undifferentiated sarcoma in the first two decades of life: a selective review of Intergroup Rhabdomyosarcoma Study Group experience and rationale for Intergroup Rhabdomyosarcoma Study V. Am J Pediatr Hematol Oncol. 2001; 23:215.
  11. Seifert G, Sobin LH. The World Health Organization’s histologic classification of salivary gland tumors. Cancer. 1992 Jul 15;70(2):379-85.
  12. Weiner ES. Head and neck rhabdomyosarcoma. Semin Pediatr Surg. 1994 Aug;3(3):203-6.

Staging

Learning Objectives 

To classify common pediatric head and neck malignancies according to well established staging systems:

  • Ann Arbor staging system for Hodgkins disease
  • Non-Hodgkins staging
  • Clinical group staging for rhabdomyosarcoma
  • WHO staging system for nasopharyngeal carcinoma
References 
  1. Harris N. Hodgkin’s lymphomas: classification, diagnosis and grading. Semin Hematol. 1999;36:220.
  2. Sandlund JT, Downing JR, Crist WM. Non-Hodgkin’s lymphoma in childhood. N Engl J Med. 1996;334:1238.
  3. Tubergen DG, Krailo MD, Meadows AT, et al. Comparison of treatment regimens for pediatric lymphoblastic non-Hodgkin’s lymphoma: a Children’s Cancer Group study. J Clin Oncol. 1995;13:2023.
  4. Weiner ES. Head and neck rhabdomyosarcoma. Semin Pediatr Surg. 1994;3:203.
  5. Whittemore KR, Cunningham MJ. Malignant Tumors of the Head and Neck. In Pediatric Otolaryngology, Fourth Edition. Ed. Bluestone CD, Stool SE, Alper CM, et al. Saunders, Philadelphia, PA. 2003; p. 1703-1736.

Case Studies

  1. A 16 year old female presents with a slowly enlarging low, midline neck mass. Ultrasound imaging confirms that it is a thyroid nodule. What size nodule would warrant fine needle aspiration? What ultrasonographic findings would be suspicious for malignancy? What are the surgical treatment strategies for treatment of well differentiated thyroid carcinoma in children?
  2. A 2 year old male has a mass in his right parotid gland, with associated facial nerve weakness. What imaging modalities would assist in the diagnostic assessment of this patient?  What surgical treatment strategies are available to treat a young child with a parotid gland malignancy? What adjuvant therapies could be considered?

Complications

Learning Objectives 

To recognize complications related to treatment of pediatric head and neck malignancies, as well as the long term survival rates.

References 
  1. Paulino AC, Simon JH, Zhen W, et al. Long-term effects in children treated with radiotherapy for head and neck rhabdomyosarcoma. Int J Radiation Oncology Biol Phys. 2000;48:1489-1495.

Review

Review Questions 
  1. What are the most common head and neck malignancies in children and adolescents, stratified by age?
  2. What are the most common anatomic regions of the head and neck affected?
  3. What are the survival rates for these most common head and neck malignancies?
Resources 

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