1 - Serviço de Neurorradiologia, Hospital Dona Estefânica e Hospital são José, CHULC
2 - Serviço de Neurocirurgia, Hospital Dona Estefânica, CHULC
- XV Congresso Nacional da SPNR, 28-29 Junho 2019, Coimbra
- Neuroradiology (2020) 62:109–127. https://doi.org/10.1007/s00234-019-02317-7
Introduction: Craniopharyngiomas are histologically benign tumors located primarily within the sellar and parasellar region. They are thought to arise form epithelial remnants of Rathke’s pouch (adamantinomatous type) or from metaplasia of squamous epithelial cells (squamous papillary type). The imaging diagnosis is mainly based on the three components of the tumor (cystic, solid and calcified). Metastasis of craniopharyngiomas are quite rare, with only a few reports of seeded tumor along the surgical route.
Material and Methods: We present the case of an eight year-old boy who presented to our hospital with tiredness, headache and vomiting.
Results: Physical examination of the patient documented a short stature (below the 5th percentile) and blood tests revealed both high levels of cholesterol and low levels of IGF-1. The CT scan and MRI revealed a large suprasellar and intrasellar expansive lesion, with III ventricle extension, mainly cystic with peripheral calcification and solid components, with associated ventricular system mass effect. Cystic components showed hyperintensity in T1, T2 and FLAIR sequences, with typical “motor oil cyst” aspect. The patient underwent a three-step surgical treatment, with an anterior transcallosal, right supraorbital/subfrontal and endoscopic transsphenoidal approaches, with total removal of the tumor. Histological examination of the surgical specimen documented an adamantinomatous craniopharyngioma (grade I, WHO). One year later a control MRI of the brain was performed showing no recurrence of the tumor in the original locations, but a new cystic lesion in the subarachnoid space adjacent to the right clinoid process, with ring-shape enhancement. A new surgery was performed with complete removal and confirmation of a seeded craniopharyngioma.
Conclusion: Although exhibiting a benign histopathological pattern, craniopharyngiomas can be clinically aggressive with local invasion and possible recurrences. Metastasis or cerebrospinal fluid dissemination are extremely rare and mainly related with seeded tumor along the previous surgical access.
Palavras Chave: Craniopharyngioma; children; seeding; MRI