Submandibular lipoblastoma: Case report of a rare tumor in childhood

Abstract

Lipoblastoma is a rare, benign tumor usually occurring in childhood. It is essentially localized in the extremities and trunk, with few cases reported in the neck. We report the case of a 2-year-old girl with a rapidly enlarging, painless neck mass. Magnetic resonance imaging (MRI) revealed a 3-cm mass in the right submandibular region. Review of literature, diagnostic methods, and genetics of lipomatous tumors are discussed. Complete surgical excision via a lateral cervical approach demonstrated a white soft tissue with an adherent ganglion. Histology and immunohistochemistry confirmed the diagnosis of lipoblastoma. Cervical lipoblastoma is rare, and typically asymptomatic, rarely causing nerve compression or airway obstruction. MRI can help identifying the lipomatous nature of the mass, but the findings can be inconsistent due to variable maturity of fat cells and the mesenchymal content of the tumor. Diagnosis is always based on pathological examination. Further chromosomal analysis is useful in differentiating lipoblastoma from liposarcoma. Complete surgical excision is the recommended treatment.

Keywords: Benign tumor; head and neck; immunohistochemestry; lipoblastoma; liposarcoma.

Figure 1

Magnetic resonance imaging demonstrated a well-defined mass of 36 mm × 23 mm × 39 mm in the right submaxillary angle showing hyperintense signal on T1.

Figure 2

Benign tumor composed of lobular aggregation of regular mature adipocytes and immature myxoid mesenchymal tissue

Figure 3

Tumoral cells showed diffuse expression of PS 100