Paediatric Pathology Online: Head and Neck Neoplasms

Atypical Fibroxanthoma

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. Visit: Paediatric Pathology Online

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, 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.

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.

Fetal Rhabdomyoma

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.

Rhabdomyosarcoma

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.

Ewing's sarcoma / PNET

Neuroblastoma may arise primarily from the cervical sympathetic chain or present as a metastatic deposit.

Neuroblastoma

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.

Pediatric head and neck masses. Top Magn Reson Imaging. 2004;15(2):95-101.

Most neck masses in the pediatric head and neck region are benign. Congenital, developmental, and inflammatory lesions make up most of the masses in the pediatric head and neck. For example, neck masses due to inflammatory lymphadenitis are common in children because of the frequency of upper respiratory tract infections. Although many of the malignant tumors in children are found in the head and neck, they account for only a small portion of the neck masses. The choice of the imaging modality is based on a number of factors, several of which are unique to the pediatric population. Although the bulk of disease entities are adequately evaluated by CT, MRI can provide additional vital information in many cases. MRI provides better soft tissue characterization than CT, has multiplanar capabilities. In this article, we will attempt to provide an overview of conditions that present as neck masses.

Evaluation of neck masses in children. Am Fam Physician.1995;51(8):1904-12.

Neck masses in children may be inflammatory, neoplastic or congenital. Although most of these masses are benign inflammatory nodes, an asymptomatic neck mass is the most common presentation of head and neck malignancies in children. Cystic lesions are usually pharyngeal cleft remnants or vascular malformations, whereas solid lesions are generally inflammatory or neoplastic. While the history and the physical examination are the most important parts of the evaluation of neck masses in children, biopsy may be necessary to establish the diagnosis.

Pediatric neck masses: guidelines for evaluation.Int J Pediatr Otorhinolaryngol. 1988 Dec;16(3):199-210.

Neck masses are frequent findings in the pediatric population. Unlike the adult, there are few established guidelines for evaluation of these children. The etiology of cervical masses includes many conditions. Knowledge of these conditions and their clinical presentations is essential. To elucidate the clinical characteristics which may help in establishing a correct diagnosis, the charts of 445 patients with biopsies of neck masses performed at the Children's Hospital of Philadelphia were analyzed. There were 244 (55%) congenital lesions, 118 (27%) inflammatory lesions, 23 (5%) non-inflammatory benign masses, 12 (3%) benign neoplasms, and 48 (11%) malignancies. The preoperative diagnosis was correct in 270 (61%) patients. Guidelines are established for the evaluation of the child presenting with a neck mass.