ISPUB.COM The Internet Journal of Radiology Volume 12 Number 2 1 of 8 Intracranial Extension Of Giant Multicystic Maxillomandibular Ameloblastoma: A Case Report M Akinkunmi, E Okoturo, M Emmanuel, O Ogunbanjo Citation M Akinkunmi, E Okoturo, M Emmanuel, O Ogunbanjo. Intracranial Extension Of Giant Multicystic Maxillomandibular Ameloblastoma: A Case Report. The Internet Journal of Radiology. 2009 Volume 12 Number 2. Abstract Maxillomandibular Ameloblastoma (A) is a slow growing but locally aggressive odontogenic tumour with a high recurrence rate, which can cause severe facial disfigurement and functional impairment. We report a case of histologically confirmed giant multicystic maxillomandibular A with intracranial extension in a 35-year- old woman of the Yoruba ethnic group in Nigeria, which measured 24 x 19 x 15 cm in diameter. Intracranial extension of the multicystic subtype of A is extremely rare. This to the best of our knowledge is probably the sixth recorded intracranial extension of a multicystic A. She had a recurrence after a year of mandibulectomy. She delayed to seek medical attention for five years after the recurrence, during which the A grew to an enormous size with the complaints of pain, severe headache and loss of vision in the right eye. The radiologist plays a crucial role in the diagnosis and treatment of A. We advocate early radical surgery from the onset to prevent recurrence in multicystic A, and long term follow-up for early detection of possible relapses. INTRODUCTION Ameloblastoma (A) is an odontogenic epithelial tumour affecting the mandible and maxilla, which is slow growing but locally aggressive 1 . It can cause severe facial disfigurement and functional impairment 1 . It is the second most common odontogenic neoplasm after odontoma 2 . It has a high rate of recurrence of up to 80%; and recurrences have occurred as late as 50 years after resection 3,4 . There are three histological variants of A namely plexiform, follicular, and granular cell variants. A is much more common in the mandible than in the maxilla, especially in the premolar- molar region, exhibit no sex predilection, and the most common histologic type is follicular A 5 . Based on biological behavior and histopathologic characteristics, maxillomandibular A has been divided into three subtypes, namely solid or multicystic A, unicystic A, and malignant A 6 . Intracranial extension or metastasis of all the three subtypes of A is extremely rare. To the best of our knowledge, intracranial extension of multicystic A has only been reported in five patients in the medical literature. Oka K et al reported the first case of mandibular A with intracranial extension in 1986 7 . Phillip et al described intracranial extension of A in a patient in 1992 8 . Zarbo et al reported intracranial metastasis in a patient with A in 2003 9 . Hughes et al from the United States of America described a giant A that measured 15.2 x 11.4 x 12.0 cm with no intracranial extension 10 . Dunn J L et al reported a giant A which measured 17 x 15 x 13 on CT, but no intracranial extension was observed 11 . We report a case of giant maxillomandibular A with intracranial extension in a Nigerian woman. CASE REPORT TM is a 35 year old woman who presented at the Oral and Maxillofacial Surgery Unit of the Lagos State University Teaching Hospital, Ikeja, Lagos, Nigeria, with a ten year history of right craniomandibular swelling (Figures 1 and 2). She noticed this was associated with pain and severe headache, a few years ago. There was also progressive loss of vision in the right eye. The swelling was first observed in the right lower jaw at the onset. She had right segmental mandibulectomy four years after the onset of symptoms at a private hospital in Lagos, and the diagnosis of A was histologically confirmed. She had a recurrence of tumour growth a year after surgery. The swelling has since been slowly and progressively increasing in size to the current size. She is married with children. Physical examination revealed a depressed-looking woman, in no obvious distress, mildly pale, afebrile and not jaundiced. There was total loss of vision in the right eye with no light perception. Extra-oral examination showed a mass located at the right craniomandibular region with proptosis.