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Infantile haemangiopericytoma: a rare congenital cervical tumour

Published online by Cambridge University Press:  10 February 2014

E M Farrar*
Affiliation:
Paediatric ENT Department, Royal Manchester Children's Hospital, Central Manchester University Hospitals NHS Foundation Trust, Manchester, UK
J E H Bates
Affiliation:
Paediatric ENT Department, Royal Manchester Children's Hospital, Central Manchester University Hospitals NHS Foundation Trust, Manchester, UK
S Bitetti
Affiliation:
Department of Paediatric Histopathology, Royal Manchester Children's Hospital, Central Manchester University Hospitals NHS Foundation Trust, Manchester, UK
I A Bruce
Affiliation:
Paediatric ENT Department, Royal Manchester Children's Hospital, Central Manchester University Hospitals NHS Foundation Trust, Manchester, UK Respiratory and Allergy Centre, Institute of Inflammation and Repair, University of Manchester, Manchester, UK
*
Address for correspondence: Ms Eleanor M Farrar, Paediatric ENT Department, Royal Manchester Children's Hospital, Oxford Road, Manchester, M139WL E-mail: [email protected]

Abstract

Objective:

Infantile haemangiopericytoma is a rare childhood vascular tumour of borderline malignancy and unpredictable clinical course. It can present a diagnostic challenge due to indeterminate clinical, radiological and pathological features. This report presents the case of a large congenital haemangiopericytoma of the neck in a neonate, and discusses diagnosis, imaging, pathology and surgical management.

Clinical presentation:

A full-term neonate presented with a large posterior neck mass at birth. Pre-operative radiological appearances were suggestive of teratoma, but following surgical excision the diagnosis of infantile haemangiopericytoma was confirmed on histological analysis. There were no signs of recurrence at 12-month follow up.

Conclusion:

Haemangiopericytoma can follow an aggressive course in adults, including local recurrence and metastasis. The infantile variant is rare but typically follows a distinct clinical course, and is associated with more benign behaviour compared with similar tumours in adults and children over one year. Congenital haemangiopericytoma can be effectively treated with surgery, without requiring adjuvant therapy.

Type
Clinical Records
Copyright
Copyright © JLO (1984) Limited 2014 

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