Congenital infantile hemangiopericytoma in the floor of mouth – a treatment dilemma

Objectives: The aims of this presentation were to describe the management of a rare case of infantile hemangiopericytoma (HPC) in a newborn girl, to analyze the literature on pediatric cases of infantile HPC in the head and neck region, and to propose treatment protocols for this age group.

Materials and methods: A newborn girl was evaluated for a congenital floor of mouth swelling with subsequent protrusion of tongue, the airway was unobstructed.

Results: The initial MRI findings were suggestive of an AV malformation, which essentially ruled out the likelihood of malignancy. The pediatric physician opted for a conservative management, and decided to treat the lesion with propranolol, a non-selective β blocker. The lesion continued growing regardless of treatment. A second MRA was performed which was unconclusive. With the patient under general anesthesia, an incisional biopsy was performed and sent for histopathological analysis. It was microscopically diagnosed as an infantile HPC. After the first surgical intervention a management dilemma arose, whether to subject a child to primary chemotherapy or an additional more aggressive surgery shortly after the final histopathological diagnosis was available. Due to subsequent morbidity of chemotherapy a second surgical intervention was performed in which the lesion was completely enucleated, the surgery was uneventful.

Summary: Infantile hemangiopericytoma is a rare soft tissue neoplasm. Almost half of these occur during the first year of life, and is defined as infantile HPC. The uniqueness of this case is the unusual location in the head and neck region. This girl is the youngest patient reported thus far in the literature. Surgical treatment of infants and children for such lesions poses difficult management dilemmas, the most important ones being the concern to minimize morbidity, to permit maximum jaw function and to enable uninterrupted facial growth. Management decision-making was based on the patient’s young age.

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Jan 21, 2018 | Posted by in Oral and Maxillofacial Surgery | Comments Off on Congenital infantile hemangiopericytoma in the floor of mouth – a treatment dilemma
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