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2019

ANUÁRIO DO HOSPITAL
DONA ESTEFÂNIA

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CRANIAL FASCIITIS IN CHILDREN

Miguel Correia1, Amets Sagarribay1, Mário Matos1, António Baptista1, Luís Távora1, Carla Conceição2, Manuela Mafra3.

1 - Neurosurgical Pediatric Unit, Hospital Dona Estefânia, Centro Hospitalar de Lisboa Central, EPE;
2 - Pediatric Neurorradiology Unit, Hospital Dona Estefânia, Centro Hospitalar de Lisboa Central, EPE;
3 - Pathology Department, Hospital São José, Centro Hospitalar de Lisboa Central, EPE.

 - 23rd ESPN Congress Session 14: "Oncology", University of Amsterdam, 1-5 May (Poster).

Objective: cranial fasciitis in childhood is a rare benign fibroblastic tumour of the skull almost exclusively seen in children under 6 years of age. It has to be considered in the differential diagnoses of lytic skull lesions in children. It is histologically similar to nodular fasciitis and has been related to prior trauma in some cases but also as a result of genetical disease, as familial adenomatous polyposis (FAP).

Methods: Cranial fasciitis is a reactive myofibromatous tumour of the skull that tipically presents as a rapidly growing skull mass with significant bone destruction in young children. There are few cases reported in the literature, with a median age at presentation at 21 months an a male predominance (2:1). Surgery is curative even when total removal is not achieved. The histological evaluation is fundamental because it is important to recognize this lesions and differenciate from more agressive lesions as eosinophilic granuloma and malignant skull tumour demanding adjuvant treatment and with a poor prognosis.

Results: We report the case of a 5 years old boy, with a growing subcutaneous bony mass in the parassagital left parietal bone, previously biopsed in another institution but without a clear histological caracterization. This lesion showed a radiological appearence of a lytic lesion growing from the subcutaneous tissue through the bone to the epidural space. At surgery it was a well demarked bony lesion. We did a craniotomy limited to 0,5cm around the perimeter of the lesion and remove the tumour "en bloc" after cutting some adhesions to the external dural sleave. He had an uneventful postoperative period and no recurrence has been reported in the 2 years follow-up period. We show imaging and histological features and review literature.

Conclusions: Cranial fasciitis is a rare benign skull tumour with less than 60 cases reported before, frequently affecting young children under 6 years of age, with a male preponderance, that may mimic other more agressive lesions in radiological studies and also by the rapid growth pattern. Although it is a very rare entity it has to be considered in the differential diagnosis of lytic skull lesions because it´s treatment and prognosis differes from other diseases. Complete surgical excision may be curative but also though incomplete ressection.

Keywords: cranial fasciitis, childhood.