The magnetic resonance imaging of the brain revealed a large heterogeneous solid-cystic mass in the left temporal lobe with no connection to the craniopharyngeal
duct, the suprasellar, or intrasellar regions, although there was no significant edema or mass effect (Figure 1).
They were named by Harvey Cushing and described as being derived from "epithelial rests ascribable to an imperfect closure of the hypophysial or craniopharyngeal
duct".1 Since this early description by Cushing, the treatment strategies for these tumours have undergone significant advancement.
Benign teratomas arising from the midline nasal septum have been well described and resected endoscopically [4,7]; however, a skull base teratoma on a neurovascular stalk arising from the craniopharyngeal
duct, superimposed on a KFS background, presents a singular challenge.
There are multiple theories attempting to explain the formation of these basal encephaloceles [1, 4, 5], including the incomplete closure of the neural tube leading to herniation of meninges and neural tissue, and the persistence of the craniopharyngeal
It is supposed to develop owing to transformation of embryonic squamous cell structures along the path of the craniopharyngeal
Their localization can be intrasellar, suprasellar or parasellar and basically anywhere alongside the craniopharyngeal
canal [1, 2].
In 1904, Erdheim postulated that craniopharyngiomas developed from squamous remnants of the obliterated craniopharyngeal
duct, suggesting that these tumors may arise at any point along the tract of migration of Rathke's pouch from the vomer, the roof of the nasopharynx, or through the midline sphenoid bone beneath the floor of the sella turcica.
Hypothalamic hamartoma associated with a craniopharyngeal
The medial aspect of the temporal bone may fail to develop, resulting in a persistent lateral craniopharyngeal
canal (also known as the Sternberg canal) between the middle cranial fossa and the pneumatised inferolateral recess of the sphenoid sinus.3 Idiopathic intracranial hypertension (pseudotumour cerebri) and empty sella syndrome are also known to occur in these patients,2 suggesting that chronically increased intracranial pressure with localised thinning of the bone may also play a role in the pathogenesis of this condition.4
The index case was observed earlier in the year than in previous years, and clinical manifestations of the cases were unusual (absence of craniopharyngeal
syndrome and bleeding from gastrointestinal tract that are typical for CCHF patients from Bulgaria); in the fatal case, autopsy of the patient showed hemorrhages only in the lungs.
Craniopharyngiomas arise from remnants of Rathke's pouch along the path of the craniopharyngeal
Transsphenoidal encephaloceles have often been attributed to persistence of the craniopharyngeal