Background
Ameloblastoma (AMBL) is an odontogenic tumor, considered to be benign, but aggressive, whose principal risk is a recurrence. The growth can be enormous, and it can extend into the intracranial compartment with serious consequences.
Purpose
The intracranial involvement of AMBL is rare, and it may require an extensive surgery. Although it is a rare condition for the neurosurgeon to treat, knowing this condition can lead to a significant increase in survival for these patients.
Methods
A case of a 56-year-old woman presented with a history of recurrent left maxilla AMBL with intracranial extension and dural involvement of the anterior and medial cranial fossa is reported, followed by a systematic review of the literature with the aim to identify the best surgical treatment.
Results
A total of 32 cases were included in the qualitative analysis. Management is varied and often not described, resulting in an almost complete lack of information and indications for treatment. Radical surgery tends to yield the best outcomes, and it is recommended to have adequate surgical margins when possible.
Conclusions
Intracranial involvement from AMBL compartment is an uncommon manifestation of this rare pathology, but which deserves to be treated in a multidisciplinary way in order to ensure maximum surgical radicality. Recurrence reflects failure of the primary surgical resection. If recurrence is the major consideration, surgeons are encouraged to select radical surgery. Whenever a follicular-type maxillary AMBL is diagnosed, it is advisable to check for intracranial spreading and distant metastases during follow-up.
Introduction. According to the 2016 World Health Organization classification of Tumors of the Central Nervous System, the term Primitive Neuroectodermal Tumor has been replaced by the term Embryonal Tumor (ET). We present a case of disseminated cerebrospinal ET presenting in an adult patient. Illustrative Case. A 49-year-old male presenting with low back pain, dysuria, and hypoesthesia of the lower extremities referred to our emergency department. Brain and whole spine contrast-enhanced MRI documented a diffusively disseminated heterogeneous neoplasm with intradural extra- and intramedullary involvement of the cervicothoracic tract and cauda equina. A primary biopsy of the lumbosacral localization was performed through L5 bilateral laminectomy. Histologic diagnosis was Embryonal Tumor Not Otherwise Specified. The patient underwent chemotherapy with postoperative adjuvant alternating Vincristine-Doxorubicin-Ifosfamide (VAI) and Ifosfamide-Etoposide (IE). Discussion. Spinal ETs are exceedingly rare especially when presenting in the adult patient. Neurosurgical and oncologic management is still unclear. When feasible, surgical removal should always be performed to obtain a histologic diagnosis. Postoperative adjuvant therapy might entail both chemo- and radiotherapy; however a consensus on this matter is still lacking.
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