Pediatric Head and NeckPediatric Head and Neck
MalignanciesMalignancies
Elizabeth J. Rosen, MDElizabeth J. Rosen, MD
Ronal...
Pediatric CancerPediatric Cancer
22ndnd
leading cause of death in age range ofleading cause of death in age range of
5-14 ...
Pediatric CancerPediatric Cancer
Albright, et al in Archives of Oto-HNSAlbright, et al in Archives of Oto-HNS
June 2002Jun...
Pediatric Head and NeckPediatric Head and Neck
MalignanciesMalignancies
Lymphoma = 59%Lymphoma = 59%
Rhabdomyosarcoma = 13...
Non-Hodgkin’s LymphomaNon-Hodgkin’s Lymphoma
Lymphoma = 11.5% of all pediatricLymphoma = 11.5% of all pediatric
cancerscan...
Non-Hodgkin’s LymphomaNon-Hodgkin’s Lymphoma
Low-, intermediate-, and high-gradeLow-, intermediate-, and high-grade
lesion...
Non-Hodgkin’s LymphomaNon-Hodgkin’s Lymphoma
Large cell lymphomaLarge cell lymphoma
– 27% pediatric cases27% pediatric cas...
Non-Hodgkin’s LymphomaNon-Hodgkin’s Lymphoma
LymphoblasticLymphoblastic
lymphomalymphoma
– 29% pediatric29% pediatric
case...
Non-Hodgkin’s LymphomaNon-Hodgkin’s Lymphoma
Small cell noncleavedSmall cell noncleaved
– 34% pediatric cases34% pediatric...
Non-Hodgkin’s LymphomaNon-Hodgkin’s Lymphoma
Presentation in the H&N in 5-10% ofPresentation in the H&N in 5-10% of
casesc...
Non-Hodgkin’s LymphomaNon-Hodgkin’s Lymphoma
EvaluationEvaluation
– H&PH&P
– BiopsyBiopsy
TonsillectomyTonsillectomy
Lymph...
Non-Hodgkin’s LymphomaNon-Hodgkin’s Lymphoma
Non-Hodgkin’s LymphomaNon-Hodgkin’s Lymphoma
Multiagent ChemotherapyMultiagent Chemotherapy
– CyclophosphamideCyclophospha...
Non-Hodgkin’s LymphomaNon-Hodgkin’s Lymphoma
SurvivalSurvival
– Overall Stage I and II NHL = 85-95%Overall Stage I and II ...
Hodgkin’s DiseaseHodgkin’s Disease
Less common than NHLLess common than NHL
More frequently in 15-20 y/o populationMore fr...
Hodgkin’s DiseaseHodgkin’s Disease
Nodular SclerosingNodular Sclerosing
From, Diagnostic Surgical Pathology of the Head an...
Hodgkin’s DiseaseHodgkin’s Disease
Mixed CellularityMixed Cellularity
From, Diagnostic Surgical Pathology of the Head and ...
Hodgkin’s DiseaseHodgkin’s Disease
PresentationPresentation
– Asymmetric lymphadenopathy—90%Asymmetric lymphadenopathy—90%...
Hodgkin’s DiseaseHodgkin’s Disease
EvaluationEvaluation
– H&PH&P
– Biopsy = Reed-Biopsy = Reed-
Sternberg cellsSternberg c...
Hodgkin’s DiseaseHodgkin’s Disease
Hodgkin’s DiseaseHodgkin’s Disease
Localized diseaseLocalized disease
– Extended field XRTExtended field XRT
Disseminated ...
Hodgkin’s DiseaseHodgkin’s Disease
SurvivalSurvival
– Stages I, II, and III = 90%Stages I, II, and III = 90%
– Stage IV = ...
RhabdomyosarcomaRhabdomyosarcoma
Most common soft tissue sarcoma inMost common soft tissue sarcoma in
childrenchildren
4.5...
RhabdomyosarcomaRhabdomyosarcoma
EmbryonalEmbryonal
– Most common in kids:Most common in kids:
60-70% of cases60-70% of ca...
RhabdomyosarcomaRhabdomyosarcoma
AlveolarAlveolar
– 20% of pediatric cases20% of pediatric cases
– Chromosomal translocati...
RhabdomyosarcomaRhabdomyosarcoma
BotryoidBotryoid
– 5-10% of pediatric5-10% of pediatric
casescases
– Grape-like tumorGrap...
RhabdomyosarcomaRhabdomyosarcoma
Most common site of presentation isMost common site of presentation is
H&N--40% of casesH...
RhabdomyosarcomaRhabdomyosarcoma
EvaluationEvaluation
– H & PH & P
– BiopsyBiopsy
– CT/MRI of primaryCT/MRI of primary
– M...
RhabdomyosarcomaRhabdomyosarcoma
Intergroup Rhabdomyosarcoma StudyIntergroup Rhabdomyosarcoma Study
Clinical Grouping Clas...
RhabdomyosarcomaRhabdomyosarcoma
RhabdomyosarcomaRhabdomyosarcoma
TreatmentTreatment
– SurgerySurgery
Goal = complete excision with marginGoal = complete e...
RhabdomyosarcomaRhabdomyosarcoma
TreatmentTreatment
– ChemotherapyChemotherapy
Low-risk: vincristine, dactinomycin, +/-Low...
RhabdomyosarcomaRhabdomyosarcoma
TreatmentTreatment
– Radiation TherapyRadiation Therapy
Postoperative microscopic disease...
RhabdomyosarcomaRhabdomyosarcoma
SurvivalSurvival
– Before 1970 = 33%Before 1970 = 33%
– Currently = 70%Currently = 70%
– ...
NeuroblastomaNeuroblastoma
Most common extracranial solid tumor inMost common extracranial solid tumor in
childrenchildren...
NeuroblastomaNeuroblastoma
““Small blue round cell” tumorSmall blue round cell” tumor
Immunohistochemical stains:Immunohis...
NeuroblastomaNeuroblastoma
2-5% in the H&N region—most often as2-5% in the H&N region—most often as
lateral neck masslater...
NeuroblastomaNeuroblastoma
EvaluationEvaluation
– H & PH & P
– BiopsyBiopsy
– Urine catecholamine studiesUrine catecholami...
NeuroblastomaNeuroblastoma
NeuroblastomaNeuroblastoma
TreatmentTreatment
– SurgerySurgery
– ChemotherapyChemotherapy
Intermediate- or High-riskInterm...
NeuroblastomaNeuroblastoma
Prognostic FactorsPrognostic Factors
– Age at diagnosisAge at diagnosis
– Stage at diagnosisSta...
EsthesioneuroblastomaEsthesioneuroblastoma
100 pediatric cases in the literature100 pediatric cases in the literature
Teen...
EsthesioneuroblastomaEsthesioneuroblastoma
HistologyHistology
From, Surgical Pathology of the Head and Neck, Lippincott Wi...
EsthesioneuroblastomaEsthesioneuroblastoma
100 pediatric cases in the literature100 pediatric cases in the literature
Teen...
Nasopharyngeal CarcinomaNasopharyngeal Carcinoma
5% of pediatric H&N malignancies5% of pediatric H&N malignancies
Teenager...
Nasopharyngeal CarcinomaNasopharyngeal Carcinoma
WHO ClassificationWHO Classification
– Type I – squamous cellType I – squ...
Nasopharyngeal CarcinomaNasopharyngeal Carcinoma
PresentationPresentation
– Neck mass and hearing lossNeck mass and hearin...
Nasopharyngeal CarcinomaNasopharyngeal Carcinoma
EvaluationEvaluation
– H & PH & P
– EndoscopyEndoscopy
– BiopsyBiopsy
– C...
Nasopharyngeal CarcinomaNasopharyngeal Carcinoma
Nasopharyngeal CarcinomaNasopharyngeal Carcinoma
TreatmentTreatment
– Radiation TherapyRadiation Therapy
Primary and local...
Nasopharyngeal CarcinomaNasopharyngeal Carcinoma
SurvivalSurvival
– Overall 5-year = 40%Overall 5-year = 40%
– Prognostic ...
Soft-tissue SarcomasSoft-tissue Sarcomas
4.5% of pediatric H&N malignancies4.5% of pediatric H&N malignancies
Diverse grou...
Soft-tissue SarcomasSoft-tissue Sarcomas
PresentationPresentation
EvaluationEvaluation
StagingStaging
TreatmentTreatment
S...
Primitive NeuroectodermalPrimitive Neuroectodermal
TumorsTumors
RareRare
42% involve the H&N region42% involve the H&N reg...
Primitive NeuroectodermalPrimitive Neuroectodermal
TumorsTumors
PresentationPresentation
EvaluationEvaluation
StagingStagi...
ConclusionConclusion
Rare diseasesRare diseases
Broad differential diagnosisBroad differential diagnosis
High index of sus...
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Pediatric Head and Neck Malignancies

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  • Dear MDs Rosen and Deskin, this slides shows a very good approach to the H&N Pediatric malignancies in several aspects as the most comon cancer in frequency, incidence, diagnosis and therapy, I really loved.
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Pediatric Head and Neck Malignancies

  1. 1. Pediatric Head and NeckPediatric Head and Neck MalignanciesMalignancies Elizabeth J. Rosen, MDElizabeth J. Rosen, MD Ronald W. Deskin, MDRonald W. Deskin, MD 4/9/034/9/03
  2. 2. Pediatric CancerPediatric Cancer 22ndnd leading cause of death in age range ofleading cause of death in age range of 5-14 years5-14 years 1/333 children diagnosed annually1/333 children diagnosed annually 11,000 new cases in children under 2011,000 new cases in children under 20 years of age each yearyears of age each year Head and Neck Malignancies make up 5%Head and Neck Malignancies make up 5% of pediatric cancer cases = 500 kids/yearof pediatric cancer cases = 500 kids/year
  3. 3. Pediatric CancerPediatric Cancer Albright, et al in Archives of Oto-HNSAlbright, et al in Archives of Oto-HNS June 2002June 2002 – Overall annual incidence of pediatricOverall annual incidence of pediatric cancercancer 1973-1975 = 11.22 cases/100,000 person/years1973-1975 = 11.22 cases/100,000 person/years 1994-1996 = 14.03 cases/100,000 person years1994-1996 = 14.03 cases/100,000 person years Increase of 25%Increase of 25% – Annual incidence of pediatric H&N cancerAnnual incidence of pediatric H&N cancer 1973-1975 = 1.10 cases/100,000 person/years1973-1975 = 1.10 cases/100,000 person/years 1994-1996 = 1.49 cases/100,000 person/years1994-1996 = 1.49 cases/100,000 person/years Increase of 35%Increase of 35%
  4. 4. Pediatric Head and NeckPediatric Head and Neck MalignanciesMalignancies Lymphoma = 59%Lymphoma = 59% Rhabdomyosarcoma = 13%Rhabdomyosarcoma = 13% Thyroid Malignancies = 10%Thyroid Malignancies = 10% Nasopharyngeal Carcinoma = 5%Nasopharyngeal Carcinoma = 5% Neuroblastoma = 5%Neuroblastoma = 5% Nonrhabdomyosarcoma Soft-tissue SarcomaNonrhabdomyosarcoma Soft-tissue Sarcoma = 4.5%= 4.5% Salivary Gland Malignancies = 2.5%Salivary Gland Malignancies = 2.5% Malignant Teratoma = 1%Malignant Teratoma = 1% OthersOthers
  5. 5. Non-Hodgkin’s LymphomaNon-Hodgkin’s Lymphoma Lymphoma = 11.5% of all pediatricLymphoma = 11.5% of all pediatric cancerscancers NHL occurs in 60% of lymphoma casesNHL occurs in 60% of lymphoma cases M:F = 3:1M:F = 3:1 Peak incidence between 7-11 years of agePeak incidence between 7-11 years of age Increased risk with T-cell deficiencyIncreased risk with T-cell deficiency – Congenital immunodeficiency syndromesCongenital immunodeficiency syndromes – Acquired immunodeficiency syndromeAcquired immunodeficiency syndrome – Immunosuppressive drug therapyImmunosuppressive drug therapy
  6. 6. Non-Hodgkin’s LymphomaNon-Hodgkin’s Lymphoma Low-, intermediate-, and high-gradeLow-, intermediate-, and high-grade lesionslesions 90% of children with NHL have high-grade90% of children with NHL have high-grade disease at presentationdisease at presentation High-gradeHigh-grade – Large cell lymphomaLarge cell lymphoma – Lymphoblastic lymphomaLymphoblastic lymphoma – Small cell noncleaved lymphomaSmall cell noncleaved lymphoma
  7. 7. Non-Hodgkin’s LymphomaNon-Hodgkin’s Lymphoma Large cell lymphomaLarge cell lymphoma – 27% pediatric cases27% pediatric cases – t(2;5) anomalyt(2;5) anomaly – Rare presentation inRare presentation in H&NH&N From, Diagnostic Surgical Pathology of the Head and Neck,From, Diagnostic Surgical Pathology of the Head and Neck, W.B.Saunders, p 762.W.B.Saunders, p 762.
  8. 8. Non-Hodgkin’s LymphomaNon-Hodgkin’s Lymphoma LymphoblasticLymphoblastic lymphomalymphoma – 29% pediatric29% pediatric casescases – t(7 or 14)t(7 or 14) – Mediastinal massMediastinal mass From, Diagnostic Surgical Pathology of theFrom, Diagnostic Surgical Pathology of the Head and Neck, W.B.Saunders, p 759.Head and Neck, W.B.Saunders, p 759.
  9. 9. Non-Hodgkin’s LymphomaNon-Hodgkin’s Lymphoma Small cell noncleavedSmall cell noncleaved – 34% pediatric cases34% pediatric cases – Burkitt’s lymphomaBurkitt’s lymphoma Epstein-Barr virusEpstein-Barr virus t(8;2,14,22)t(8;2,14,22) MandibleMandible Head and NeckHead and Neck From, Surgical Pathology of the Head and Neck,From, Surgical Pathology of the Head and Neck, Lippincott Williams & Wilkins, p 161.Lippincott Williams & Wilkins, p 161.
  10. 10. Non-Hodgkin’s LymphomaNon-Hodgkin’s Lymphoma Presentation in the H&N in 5-10% ofPresentation in the H&N in 5-10% of casescases – Cervical lymphadenopathyCervical lymphadenopathy – Salivary gland, larynx, sinuses, orbit, scalpSalivary gland, larynx, sinuses, orbit, scalp – Waldeyer’s ringWaldeyer’s ring Asymmetric tonsils-how concerning is it?Asymmetric tonsils-how concerning is it? – Associated symptomsAssociated symptoms Fever, night sweats, weight lossFever, night sweats, weight loss
  11. 11. Non-Hodgkin’s LymphomaNon-Hodgkin’s Lymphoma EvaluationEvaluation – H&PH&P – BiopsyBiopsy TonsillectomyTonsillectomy Lymph nodeLymph node – Staging w/uStaging w/u Blood studiesBlood studies Lumbar punctureLumbar puncture Bone marrow biopsyBone marrow biopsy CT chest/abdomen/pelvisCT chest/abdomen/pelvis Bone scanBone scan
  12. 12. Non-Hodgkin’s LymphomaNon-Hodgkin’s Lymphoma
  13. 13. Non-Hodgkin’s LymphomaNon-Hodgkin’s Lymphoma Multiagent ChemotherapyMultiagent Chemotherapy – CyclophosphamideCyclophosphamide – DoxorubicinDoxorubicin – VincristineVincristine – PrednisonePrednisone – +/- Methotrexate+/- Methotrexate – XRT—not routinely usedXRT—not routinely used
  14. 14. Non-Hodgkin’s LymphomaNon-Hodgkin’s Lymphoma SurvivalSurvival – Overall Stage I and II NHL = 85-95%Overall Stage I and II NHL = 85-95% – Overall Stage III and IV NHL = 65-75%Overall Stage III and IV NHL = 65-75% – Stage III and IV BL = 75-85%Stage III and IV BL = 75-85%
  15. 15. Hodgkin’s DiseaseHodgkin’s Disease Less common than NHLLess common than NHL More frequently in 15-20 y/o populationMore frequently in 15-20 y/o population 4% under 10 years4% under 10 years M:F = 3:1M:F = 3:1 Association with EBVAssociation with EBV
  16. 16. Hodgkin’s DiseaseHodgkin’s Disease Nodular SclerosingNodular Sclerosing From, Diagnostic Surgical Pathology of the Head and Neck,From, Diagnostic Surgical Pathology of the Head and Neck, W.B.Saunders, p 750 & 764.W.B.Saunders, p 750 & 764. Lymphocyte PredominantLymphocyte Predominant
  17. 17. Hodgkin’s DiseaseHodgkin’s Disease Mixed CellularityMixed Cellularity From, Diagnostic Surgical Pathology of the Head and Neck,From, Diagnostic Surgical Pathology of the Head and Neck, W.B.Saunders, p 750.W.B.Saunders, p 750. Lymphocyte DepletedLymphocyte Depleted
  18. 18. Hodgkin’s DiseaseHodgkin’s Disease PresentationPresentation – Asymmetric lymphadenopathy—90%Asymmetric lymphadenopathy—90% Firm, rubberyFirm, rubbery Supraclavicular fossaSupraclavicular fossa – Spleen, liverSpleen, liver – Constitutional symptoms—1/3 of casesConstitutional symptoms—1/3 of cases Fever, night sweats, anorexia, weakness, weightFever, night sweats, anorexia, weakness, weight lossloss
  19. 19. Hodgkin’s DiseaseHodgkin’s Disease EvaluationEvaluation – H&PH&P – Biopsy = Reed-Biopsy = Reed- Sternberg cellsSternberg cells – Staging w/uStaging w/u Similar to NHLSimilar to NHL LaparotomyLaparotomy – ControversialControversial From, Principles and Practice of PediatricFrom, Principles and Practice of Pediatric Oncology, Lippincott Williams & Wilkins,Oncology, Lippincott Williams & Wilkins, P 640.P 640.
  20. 20. Hodgkin’s DiseaseHodgkin’s Disease
  21. 21. Hodgkin’s DiseaseHodgkin’s Disease Localized diseaseLocalized disease – Extended field XRTExtended field XRT Disseminated diseaseDisseminated disease – MOPP = nitrogen mustard, vinblastine,MOPP = nitrogen mustard, vinblastine, procarbazine, prednisoneprocarbazine, prednisone – ABVD = adriamycin bleomycin, vincristine,ABVD = adriamycin bleomycin, vincristine, dacarbazinedacarbazine
  22. 22. Hodgkin’s DiseaseHodgkin’s Disease SurvivalSurvival – Stages I, II, and III = 90%Stages I, II, and III = 90% – Stage IV = 75-80%Stage IV = 75-80%
  23. 23. RhabdomyosarcomaRhabdomyosarcoma Most common soft tissue sarcoma inMost common soft tissue sarcoma in childrenchildren 4.5 cases/1,000,000 children under 144.5 cases/1,000,000 children under 14 yearsyears Majority diagnosed before age 10Majority diagnosed before age 10 M:F = 1.5:1M:F = 1.5:1
  24. 24. RhabdomyosarcomaRhabdomyosarcoma EmbryonalEmbryonal – Most common in kids:Most common in kids: 60-70% of cases60-70% of cases – Chromosome 11p15Chromosome 11p15 deletiondeletion – Lack of geneLack of gene amplificationamplification – Hyperdiploid DNAHyperdiploid DNA From, Surgical Pathology of the Head and Neck, LippincottFrom, Surgical Pathology of the Head and Neck, Lippincott Williams & Wilkins, p 157.Williams & Wilkins, p 157.
  25. 25. RhabdomyosarcomaRhabdomyosarcoma AlveolarAlveolar – 20% of pediatric cases20% of pediatric cases – Chromosomal translocation:Chromosomal translocation: t(2;13) or t(1;13)t(2;13) or t(1;13) – Gene amplificationGene amplification – Tetraploid DNATetraploid DNA From, Surgical Pathology of the Head and Neck, Lippincott Williams & Wilkins,From, Surgical Pathology of the Head and Neck, Lippincott Williams & Wilkins, p 157.p 157.
  26. 26. RhabdomyosarcomaRhabdomyosarcoma BotryoidBotryoid – 5-10% of pediatric5-10% of pediatric casescases – Grape-like tumorGrape-like tumor massesmasses PleomorphicPleomorphic – Rare in childrenRare in children From, Diagnostic Surgical Pathology of the Head and Neck,From, Diagnostic Surgical Pathology of the Head and Neck, W.B.Saunders, p 554.W.B.Saunders, p 554.
  27. 27. RhabdomyosarcomaRhabdomyosarcoma Most common site of presentation isMost common site of presentation is H&N--40% of casesH&N--40% of cases 1/3 of cases involve the orbit1/3 of cases involve the orbit Oral cavity & oropharynx, face & neck,Oral cavity & oropharynx, face & neck, middle ear & mastoid, nose & paranasalmiddle ear & mastoid, nose & paranasal sinusessinuses Localized swelling, proptosis, nasalLocalized swelling, proptosis, nasal obstruction, epistaxis, otorrhea, hearingobstruction, epistaxis, otorrhea, hearing loss, fetor and cranial nerve deficitsloss, fetor and cranial nerve deficits
  28. 28. RhabdomyosarcomaRhabdomyosarcoma EvaluationEvaluation – H & PH & P – BiopsyBiopsy – CT/MRI of primaryCT/MRI of primary – Metastatic w/uMetastatic w/u Chest CTChest CT Bone scanBone scan Bone marrow biopsyBone marrow biopsy
  29. 29. RhabdomyosarcomaRhabdomyosarcoma Intergroup Rhabdomyosarcoma StudyIntergroup Rhabdomyosarcoma Study Clinical Grouping Classification (IRSCGC)Clinical Grouping Classification (IRSCGC)
  30. 30. RhabdomyosarcomaRhabdomyosarcoma
  31. 31. RhabdomyosarcomaRhabdomyosarcoma TreatmentTreatment – SurgerySurgery Goal = complete excision with marginGoal = complete excision with margin Consider morbidity of surgeryConsider morbidity of surgery – Cranial nervesCranial nerves – CosmesisCosmesis DebulkingDebulking Exception is orbital rhabdomyosarcoma—surgeryException is orbital rhabdomyosarcoma—surgery offers no advantage over chemo/XRToffers no advantage over chemo/XRT
  32. 32. RhabdomyosarcomaRhabdomyosarcoma TreatmentTreatment – ChemotherapyChemotherapy Low-risk: vincristine, dactinomycin, +/-Low-risk: vincristine, dactinomycin, +/- cyclophosphamidecyclophosphamide Intermediate- and High-risk: vincristine,Intermediate- and High-risk: vincristine, dactinomycin and cyclophosphamidedactinomycin and cyclophosphamide
  33. 33. RhabdomyosarcomaRhabdomyosarcoma TreatmentTreatment – Radiation TherapyRadiation Therapy Postoperative microscopic diseasePostoperative microscopic disease – 4,000-4,500 cGy4,000-4,500 cGy Gross diseaseGross disease – 4,500-5,000 cGy4,500-5,000 cGy Hyperfractionated XRTHyperfractionated XRT BrachytherapyBrachytherapy
  34. 34. RhabdomyosarcomaRhabdomyosarcoma SurvivalSurvival – Before 1970 = 33%Before 1970 = 33% – Currently = 70%Currently = 70% – Intergroup Rhabdomyosarcoma StudyIntergroup Rhabdomyosarcoma Study – Prognostic factorsPrognostic factors Tumor sizeTumor size Regional node statusRegional node status Margins after surgeryMargins after surgery Genetic factorsGenetic factors
  35. 35. NeuroblastomaNeuroblastoma Most common extracranial solid tumor inMost common extracranial solid tumor in childrenchildren 8-10% of childhood cancers8-10% of childhood cancers 90-95% of cases diagnosed before age 1090-95% of cases diagnosed before age 10 More common in boys and CaucasiansMore common in boys and Caucasians ? Genetic or environmental factors? Genetic or environmental factors
  36. 36. NeuroblastomaNeuroblastoma ““Small blue round cell” tumorSmall blue round cell” tumor Immunohistochemical stains:Immunohistochemical stains: neurofilament proteins,neurofilament proteins, synaptophysin, NSEsynaptophysin, NSE Electron microscopy:Electron microscopy: neurosecretory granules,neurosecretory granules, microtubules and filamentsmicrotubules and filaments Chromosome 1 deletions orChromosome 1 deletions or N-N- mycmyc oncogene amplificationoncogene amplification From, Principles and Practice of Pediatric Oncology, Lippincott Williams & Wilkins,From, Principles and Practice of Pediatric Oncology, Lippincott Williams & Wilkins, p 903.p 903.
  37. 37. NeuroblastomaNeuroblastoma 2-5% in the H&N region—most often as2-5% in the H&N region—most often as lateral neck masslateral neck mass Airway obstruction, aspiration, dysphagia,Airway obstruction, aspiration, dysphagia, Horner’s syndrome, proptosis, periorbitalHorner’s syndrome, proptosis, periorbital ecchymosis, opthalmoplegia, conjunctivalecchymosis, opthalmoplegia, conjunctival or eyelid edema, papilledemaor eyelid edema, papilledema Heterochromia iridesHeterochromia irides
  38. 38. NeuroblastomaNeuroblastoma EvaluationEvaluation – H & PH & P – BiopsyBiopsy – Urine catecholamine studiesUrine catecholamine studies – Metastatic w/uMetastatic w/u CXRCXR Bone marrow biopsyBone marrow biopsy Bone scanBone scan CT or MRICT or MRI
  39. 39. NeuroblastomaNeuroblastoma
  40. 40. NeuroblastomaNeuroblastoma TreatmentTreatment – SurgerySurgery – ChemotherapyChemotherapy Intermediate- or High-riskIntermediate- or High-risk Low-risk with recurrenceLow-risk with recurrence Cyclophosphamide, ifosfamide, doxorubicin,Cyclophosphamide, ifosfamide, doxorubicin, teniposide, etoposide, cisplatin or carboplatinteniposide, etoposide, cisplatin or carboplatin – Radiation TherapyRadiation Therapy Limited useLimited use
  41. 41. NeuroblastomaNeuroblastoma Prognostic FactorsPrognostic Factors – Age at diagnosisAge at diagnosis – Stage at diagnosisStage at diagnosis Overall, Stage I or II = 75-90%Overall, Stage I or II = 75-90% Infants: Stage III = 80-90%; Stage IV = 60-Infants: Stage III = 80-90%; Stage IV = 60- 75%75% Children: Stage III = 50%; Stage IV = 15%Children: Stage III = 50%; Stage IV = 15%
  42. 42. EsthesioneuroblastomaEsthesioneuroblastoma 100 pediatric cases in the literature100 pediatric cases in the literature Teenagers, boys > girlsTeenagers, boys > girls PresentationPresentation HistologyHistology StagingStaging TreatmentTreatment
  43. 43. EsthesioneuroblastomaEsthesioneuroblastoma HistologyHistology From, Surgical Pathology of the Head and Neck, Lippincott Williams & Wilkins, p 86.From, Surgical Pathology of the Head and Neck, Lippincott Williams & Wilkins, p 86.
  44. 44. EsthesioneuroblastomaEsthesioneuroblastoma 100 pediatric cases in the literature100 pediatric cases in the literature Teenagers, boys > girlsTeenagers, boys > girls PresentationPresentation HistologyHistology StagingStaging TreatmentTreatment
  45. 45. Nasopharyngeal CarcinomaNasopharyngeal Carcinoma 5% of pediatric H&N malignancies5% of pediatric H&N malignancies Teenagers, M=F, African AmericansTeenagers, M=F, African Americans Significantly higher incidence in ChineseSignificantly higher incidence in Chinese – HLA-A2, HLA-B-Sin 2HLA-A2, HLA-B-Sin 2 – Smoke, dust, nitrosamine rich salted fishSmoke, dust, nitrosamine rich salted fish EBVEBV From, Diagnostic Surgical Pathology of theFrom, Diagnostic Surgical Pathology of the Head and Neck, W.B.Saunders, p 43.Head and Neck, W.B.Saunders, p 43.
  46. 46. Nasopharyngeal CarcinomaNasopharyngeal Carcinoma WHO ClassificationWHO Classification – Type I – squamous cellType I – squamous cell carcinomacarcinoma – Type II – non-keratinizingType II – non-keratinizing squamous cell carcinomasquamous cell carcinoma – Type III – undifferentiatedType III – undifferentiated or lymphoepitheliomaor lymphoepithelioma From, Diagnostic Surgical Pathology of the Head and Neck,From, Diagnostic Surgical Pathology of the Head and Neck, W.B.Saunders, p 43W.B.Saunders, p 43
  47. 47. Nasopharyngeal CarcinomaNasopharyngeal Carcinoma PresentationPresentation – Neck mass and hearing lossNeck mass and hearing loss – Nasal obstruction, rhinorrhea, epistaxis,Nasal obstruction, rhinorrhea, epistaxis, headache, otalgiaheadache, otalgia – Cranial neuropathyCranial neuropathy Abducens palsyAbducens palsy CN III, IV, VCN III, IV, V CN IX, X, XIICN IX, X, XII
  48. 48. Nasopharyngeal CarcinomaNasopharyngeal Carcinoma EvaluationEvaluation – H & PH & P – EndoscopyEndoscopy – BiopsyBiopsy – CT/MRI for local extentCT/MRI for local extent – Metastatic w/uMetastatic w/u CT chest/abdomenCT chest/abdomen Bone scanBone scan
  49. 49. Nasopharyngeal CarcinomaNasopharyngeal Carcinoma
  50. 50. Nasopharyngeal CarcinomaNasopharyngeal Carcinoma TreatmentTreatment – Radiation TherapyRadiation Therapy Primary and local lymphaticsPrimary and local lymphatics 6,500-7,000 cGy6,500-7,000 cGy – ChemotherapyChemotherapy Advanced diseaseAdvanced disease Vincristine, doxorubicin, cyclophosphamide,Vincristine, doxorubicin, cyclophosphamide, cisplatin, 5-fluorouracilcisplatin, 5-fluorouracil – SurgerySurgery
  51. 51. Nasopharyngeal CarcinomaNasopharyngeal Carcinoma SurvivalSurvival – Overall 5-year = 40%Overall 5-year = 40% – Prognostic FactorsPrognostic Factors PositivePositive – Locally confined diseaseLocally confined disease – Ipsilateral nodesIpsilateral nodes NegativeNegative – Bilateral nodesBilateral nodes – CNS penetrationCNS penetration
  52. 52. Soft-tissue SarcomasSoft-tissue Sarcomas 4.5% of pediatric H&N malignancies4.5% of pediatric H&N malignancies Diverse group of tumorsDiverse group of tumors fibrosarcomafibrosarcoma epitheloid sarcomaepitheloid sarcoma synovial sarcomasynovial sarcoma chondrosarcomachondrosarcoma dermatofibrosarcoma protuberansdermatofibrosarcoma protuberans osteosarcomaosteosarcoma leiomyosarcomaleiomyosarcoma hemangiopericytomahemangiopericytoma liposarcomaliposarcoma clear-cell sarcomaclear-cell sarcoma
  53. 53. Soft-tissue SarcomasSoft-tissue Sarcomas PresentationPresentation EvaluationEvaluation StagingStaging TreatmentTreatment SurvivalSurvival
  54. 54. Primitive NeuroectodermalPrimitive Neuroectodermal TumorsTumors RareRare 42% involve the H&N region42% involve the H&N region ““small blue round cell” tumorsmall blue round cell” tumor From, Diagnostic Surgical Pathology of the Head and Neck, W.B.Saunders, p 527.From, Diagnostic Surgical Pathology of the Head and Neck, W.B.Saunders, p 527.
  55. 55. Primitive NeuroectodermalPrimitive Neuroectodermal TumorsTumors PresentationPresentation EvaluationEvaluation StagingStaging TreatmentTreatment SurvivalSurvival
  56. 56. ConclusionConclusion Rare diseasesRare diseases Broad differential diagnosisBroad differential diagnosis High index of suspicionHigh index of suspicion Early diagnosisEarly diagnosis Accurate stagingAccurate staging Multimodality therapyMultimodality therapy Improved prognosisImproved prognosis

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