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Pediatric Temporal Bone Malignancies
Built from Haynes-Glasscock-Shambaugh Surgery of the Ear, Seventh Edition
What’s inside
9 sections · 126 slides
Scope of the problem
- Malignant neoplasms of the pediatric temporal bone
- Topics covered
- Malignant tumors of the temporal bone
- Malignant tumors of the temporal bone (continued)
Rhabdomyosarcoma
- Frequency of rhabdomyosarcoma in childhood
- Site distribution in Intergroup Rhabdomyosarcoma Study IV
- Age distribution and sex of temporal bone rhabdomyosarcoma
- Familial syndromes associated with rhabdomyosarcoma
- Cell of origin and pathogenesis
- Histologic variants of rhabdomyosarcoma
- Embryonal subtypes and middle ear predominance
- Molecular characteristics of embryonal and alveolar tumours
- Prognostic value of PAX-FOXO1 fusion status
- Distinguishing rhabdomyosarcoma from other small round cell tumours
- Anti-myogenin nuclear labeling in alveolar rhabdomyosarcoma
- Alveolar rhabdomyosarcoma histology
- Embryonal rhabdomyosarcoma histology
Patterns of tumour spread
- Biological behaviour of temporal bone rhabdomyosarcoma
- Nodal and distant metastatic disease
- Sites of distant metastasis
- Structures at risk from contiguous extension
- Routes of local and intracranial spread
- Anterior spread along the Eustachian tube
Clinical presentation
- Presentation mimicking chronic otitis media
- Prat and Gray series of middle ear rhabdomyosarcoma
- Wiatrak and Pensak and Durve series
- Presenting signs and symptoms of temporal bone rhabdomyosarcoma
- Additional presenting symptoms
- Delay from symptom onset to diagnosis
- Indications for biopsy in a child with chronic ear disease
Evaluation and imaging
- Initial clinical assessment
- Cranial nerve deficits that localise disease
- Baseline systemic investigations
- Sequence of imaging and biopsy
- Complementary roles of computed tomography and magnetic resonance imaging
- Computed tomography appearance of rhabdomyosarcoma
- Right temporal bone rhabdomyosarcoma on computed tomography
- Magnetic resonance signal characteristics of rhabdomyosarcoma
- Right temporal bone rhabdomyosarcoma on magnetic resonance imaging
- Imaging characteristics of malignant temporal bone tumors
- Extent of disease evaluation after diagnosis
- Role of FDG positron emission tomography
- Imaging under anaesthesia in the young child
Staging and prognosis
- Three-step staging framework
- Cooperative group history behind the staging systems
- Rhabdomyosarcoma tumor staging definition of terms
- Rhabdomyosarcoma tumor staging definition of terms (continued)
- Rhabdomyosarcoma Soft Tissue Sarcoma Committee of the Children’s Oncology Group: pretreatment staging system
- Rhabdomyosarcoma Soft Tissue Sarcoma Committee of the Children’s Oncology Group: surgical–pathologic group system
- Rhabdomyosarcoma Soft Tissue Sarcoma Committee of the Children’s Oncology Group: surgical–pathologic group system (continued)
- Rhabdomyosarcoma Soft Tissue Sarcoma Committee of the Children’s Oncology Group: risk group classification
- Why temporal bone rhabdomyosarcoma is never low stage
- Findings from IRS I to IV middle ear cohort
- Event-free survival by risk group
- Favourable features in metastatic disease
- Oberlin adverse prognostic factors
Management of rhabdomyosarcoma
- Shift from radical surgery to multimodal therapy
- Survival trend in middle ear rhabdomyosarcoma
- Current standard of care
- Role of surgery in rhabdomyosarcoma generally
- Limits of surgery in parameningeal disease
- Delayed primary excision
- Selected candidates for surgical excision
- Management of the neck
- VAC chemotherapy backbone
- Chemotherapy by risk group
- Strategies in high-risk disease
- Indications and techniques for radiation therapy
- Proton beam therapy in parameningeal rhabdomyosarcoma
- Radiation dose for parameningeal tumours
- Whole-brain irradiation and intrathecal therapy
- Timing of radiation therapy
- Summary of rhabdomyosarcoma management
Other malignancies of the temporal bone
- Ewing sarcoma: incidence and site
- Reported temporal bone Ewing sarcoma
- Presentation of Ewing sarcoma
- Evaluation and pathology of Ewing sarcoma
- Imaging characteristics of Ewing sarcoma
- Management of Ewing sarcoma
- Local control in temporal bone Ewing sarcoma
- Osteosarcoma: incidence at craniofacial sites
- Reported pediatric cranial osteosarcoma
- Pathology and classification of osteosarcoma
- Predisposing conditions for osteosarcoma
- Imaging features of osteosarcoma
- Prognosis of temporal bone osteosarcoma
- Determinants of outcome in osteosarcoma
- Treatment of osteosarcoma
- Non-rhabdomyosarcoma soft tissue sarcomas
- Reported non-rhabdomyosarcoma sarcomas of the temporal bone
- Management principles for NRSTS
- Granulocytic sarcoma: nature and associations
- Clinical significance of granulocytic sarcoma
- Imaging and treatment of granulocytic sarcoma
- Lymphoma in the pediatric head and neck
- Subtypes and rarity of primary temporal bone lymphoma
- Presentation of primary temporal bone lymphoma
- Imaging and management of temporal bone lymphoma
- Pediatric parotid malignancy
- Management of parotid tumours invading the temporal bone
Conclusion
- Take-home points
- Practical rules to carry away
- References
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- Haynes-Glasscock-Shambaugh Surgery of the Ear, Seventh Edition
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