Table 1 Most frequent malignant tumors of the head and neck in children [2, 5]
| Â | Diagnosis | Diagnostic and surveillance tools | Treatment |
|---|---|---|---|
Rhabdomyosarcoma | Is the most frequent common soft tissue sarcoma in children and adolescents  • More than 1/3 are located in head and neck region   • Peaks: 2–6 years and 10–18 years   • Blue cell tumors   • Embryonal form at birth: spindle cell or botryoid variants and carries a better prognosis The alveolar form peaks in childhood and adolescence: chromosomal translocation PAX3 FKHR gene has poorer prognosis, especially with metastasis | • Needle or open biopsy. Radiological imaging for staging. (location, size, extent, absence, or the presence of metastasis)  CT or MRI or fluorodeoxyglucose PET scan: after 6 weeks of completion of therapy | Multidisciplinary approach Intergroup rhabdomyosarcoma studies (IRS 1-IV): prognostic groups  • Multimodal treatment (chemotherapy and either surgery or radiotherapy)  • Residual cases could have post-chemoradiotherapy surgery |
Neuroblastoma | Most common extracranial malignant tumor in children Less than 5% in the head and neck Arise from the sympathetic cervical ganglia: presents as retropharyngeal or lateral mass Orbital metastatic lesions could be observed Overall survival, with a median follow-up of 4Â years: 91% (same in cervical and cervicothoracic neuroblastoma) | Diagnostic: plain radiography, ultrasound, CT, and MRI Metastatic evaluation: bone marrow study, bone scan, scintigraphy with 131 I-meta-Iodo-benzyl-Mandelic (MIBG) | Treatment involves surgery in most cases, with adjuvant chemotherapy and occasional chemotherapy, depending on the stage of the disease Surgery is advised in localized primary or secondary head and neck neuroblastoma, because it provides more favorable prognosis In extensive metastatic lesions, multimodal treatment is decided in multidisciplinary reunions -MIBG is used in high-risk neuroblastoma as an induction therapy |
Thyroid cancer | • 2% of cases of thyroid cancers are children and adolescents. Sporadic-differentiated thyroid cancer is the most common. (papillary and papillary follicular)  • Female predominance  • Postpubertal children  • Pure follicular carcinoma, anaplastic and undifferentiated, is extremely rare Medullary thyroid carcinoma should be thought of in children with multiple endocrine neoplasia (MEN) type 2 A and B | Risk factors  • Radiation exposure: radiation, leukemia, and lymphoma treatment, especially before 10 years old and in those receiving doses superior to 30 Gy  • Iodine deficiency: increases incidence, reduces latency, and influences aggressiveness of radio-induced thyroid neoplasm  • Diagnosis: solitary thyroid nodule (20% malignancy)  • Initial investigation: TSH, calcitonin, neck ultrasound, and fine needle aspiration (FNA) in children is related to limited data | Therapeutic decision  • FNA  • Malignancy  • Surgery indicated  • Indeterminate or inadequate  • Repeat in 3–6 months or surgery after discussion of benefit-risk ratio in multidisciplinary reunions Surgery is indicated in children with higher risk of malignancy  • < 10 years old  •Radiation exposure  • Family history of thyroid cancer |
Salivary gland malignancy |  • -Malignancy is frequent in vascular lesions  • -Other types of salivary gland malignancies are rare  • More common in older children  • Five-year survival 90% (similar to adults) |  • Most frequent localization is in the parotid gland, in which the most common histological finding in mucoepidermoid carcinoma  • The most common radiotherapy-induced cancer in children is mucoepidermoid  • Most submandibular and minor gland lesions are benign Other malignant lesions described include acinic cell carcinoma | Extracapsular dissection with nerve monitoring allows resection of malignant lesions, preserving full nerve function |
Nasopharyngeal carcinoma | • -Common in southern China, Southeast Asia, the Mediterranean Basin, and Alaska • Median age in children: 13 years • -Higher in males | -Factors implicated the following: *Genotype (specific human leukocyte antigen subtypes) *Environment: salted fish high in nitrosamine *Infection (EBV) WHO classification  • Type 1: keratinizing squamous cell carcinoma  • Type 2: non-keratinizing carcinoma  • Type 3: undifferentiated carcinoma | Treatment has been established for adults: height dose radiotherapy to the nasopharynx and involved neck lymph nodes In children, NPC is different with its association with EBV, predominance of type 3, high incidence of advanced stage disease Which has led to increase trials of adjuvant, neoadjuvant, and concomitant chemotherapy |
Non-Hodgkin’s lymphoma |  • 25% of cases in children less than 10 years old  • Increasing incidence with age and male predominance  • Histological varieties are divided into low, intermediate, and high grade  • -High grade is the most prevalent in children  • 90% mature B-cell lymphomas. (Burkitt 40% et diffuse large cell 10%)  • Immunodeficiency increases the risk, predominance of mature B cell, associates with EBV | Open lymph node excision is the gold standard The use of fine needle biopsy is controversial | Treatment based on aggressive multiagent chemotherapy directed by histology and disease stage. (CHOP-based regimens) Long-term survival enhanced could reach 80% |
Hodgkin’s lymphoma | Especially in adolescents Only 5% in children under 10 years old No gender difference Association between Hodgkin’s lymphoma and EBV infection EBV in genome of Reed-Stemberg cell |  + Open lymph node biopsy is the gold standard  + Histologically: 2 categories -Classical Hodgkin’s lymphoma (90%) CD15, CD30, Reed-Stemberg -Lymphocyte predominant Hodgkin’s: lymphoma (10%), CD20, presentation in early stages, with distinctive subtypes (survival 95%) |  • Treatment based on stratification of risk groups: Based on histology, stage, and presentation, number of involved sites, disease blk, and the presence or absence of symptoms  • Low-risk classical HL ca be treated by limited cycles of chemotherapy  • High-risk classical HL intense chemotherapy regimes with diverse agents, combined with radiotherapy  • Free disease survival of 90% have been achieved |