Paediatric Pathology Online
Pathology of Head and Neck Neoplasms
Teratomas in the newborn usually present in midline or lateral neck or as thyroid masses.
They are immature and benign in behavior unless there is a yolk sac component.
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The rare melanotic neuroectodermal tumour of infancy is a neural crest derived tumour that recapitulates retinal development and usually presents in the maxilla.
Lobulated and non-encapsulated it varies in colour from white to brown.
Microscopically there is a fibrous stroma with two populations of cells-large epithelioid cells with cytoplasmic melanin and vesicular nuclei, and small dark neuroblasts with hyperchromatic nuclei.
Both cell types are positive for NSE (Neuron-specific enolase) and epithelioid cells are also positive for cytokeratin, vimentin and HMB45.
The majority are benign in behaviour but they may recur and very rarely metastasise.
Nodular fasciitis is a rare benign tumor that can present in the head and neck in children. It should be considered in the evaluation and treatment of head and neck soft-tissue masses in children.
Fetal rhabdomyoma, a rare benign tumor with a predilection for the head and neck region of children, is classically composed of small striated muscle fibres resembling fetal myotubules in a fibromyxoid stroma. Occasionally however it may be difficult to differentiate from rhabdomyosarcoma.
Rhabdomyosarcoma is the commonest sarcoma of the head and neck. The majority are embryonal type. The botryoid subtype forms polypoid masses within the oral or nasal cavity.
Peripheral primitive neuroectodermal tumour, alveolar rhabdomyosarcoma including the solid variant, a variety of other "soft tissue" benign and malignant tumours and fibromatoses, may involve the different compartments of the head and neck region.
Neuroblastoma may arise primarily from the cervical sympathetic chain or present as a metastatic deposit.
Lymphomas may be extra-nodal.
As many of these tumours are of small blue cell or spindle cell type, a broad panel of antibodies including muscle, neural, epithelial, vascular and lymphoid markers play an important role together with routine histology to establish the correct diagnosis.
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