Ectopic Craniopharyngioma Research Articles

Isolated fourth ventricle craniopharyngioma: Representative case illustration and review of literature

Background: Sella and suprasellar areas are frequently affected by craniopharyngiomas. In this article, eight occurrences have been documented. One is new, and the remaining seven are from previously published articles. Their prevalence in the posterior fossa without expansion from the suprasellar area is unusual. Case Description: We present a case of a primary 4th ventricular craniopharyngioma of the posterior fossa in a 16-year-old male with no association with Gardner’s syndrome. He presented with sudden deterioration of consciousness level and was diagnosed as having a homogeneously contrast enhancing lesion occupying the 4th ventricle with obstructive hydrocephalus. The patient underwent emergency placement of external ventricular drain followed by complete removal of the tumor on the next day by midline suboccipital craniotomy with telovelar approach. Histopathological features were consistent with the adamantinomatous variety of craniopharyngioma. He had complete neurological recovery and no evidence of tumor recurrence in 1-year follow-up. Conclusion: The craniopharyngioma in our case was distinct because it was a solid tumor with no cystic component, exhibited homogeneous contrast enhancement in neuroimaging, developed in the fourth ventricle, and reached the level of foramen magnum, features that had rarely been documented previously. We also reviewed the literature on reported cases of 4th ventricular craniopharyngioma to strengthen knowledge in this area and highlight the embryological basis of ectopic craniopharyngioma.

80. Extracranial infrasellar craniopharyngioma arising in the maxillary antrum: An unusual sinonasal tumour

Craniopharyngiomas are benign developmental epithelium-derived tumours that primarily originate above the pituitary gland and optic chiasm (suprasellar 75%). Ectopic craniopharyngiomas have been described in aberrant intracranial and extracranial locations namely the nasopharynx, ethmoid and sphenoid sinuses.

Adamantinomatous craniopharyngioma of cerebellopontine angle in association with Gardner’s syndrome

Background. Craniopharyngiomas are benign epithelial tumors. According to embryogenetic theory, these neoplasms develop from the remains of the cells of Rathke’s pouch, which connects the cavities of the primary oral tube with the pituitary gland in the embryonic period. The metaplastic theory assumes the occurrence of craniopharyngiomas as a result of metaplasia of epithelial cells in the chiasmal-sellar region.Objective. To present a clinical case of ectopic adamantium-like craniopharyngioma of the right cerebellopontine angle associated with Gardner’s syndrome.Design and methods. The analysis of SCT, MRI, histological, histochemical and other special studies, as well as data from four other similar observations published in the literature, was carried out.Results. We describe a case of a rare case of ectopic adamantium-like craniopharyngioma of the right cerebellopontine angle associated with Gardner’s syndrome (familial adenomatous polyposis, multiple skull osteomas and soft tissue tumors) diagnosed in a 27-year-old patient. It is noted, while inexplicable, the correlation of craniopharyngiomas in patients with Gardner’s syndrome in the region of the cerebellopontine angle.Conclusions. In the case of patients with adamantine-like craniopharyngiomas, the including of collection of a family history and special studies to exclude adenomatous intestinal polyposis and Gardner syndrome in the complex of treatment and diagnostics id highly recommended.

Ectopic craniopharyngioma of the orbit: illustrative case.

BACKGROUNDCraniopharyngiomas are uncommon malformations of the sellar or parasellar region that are partly cystic and calcified and have low histological grade. The typical age of presentation is bimodal, with peak incidence rates in children at age 5 to 14 years and in adults at age 50 to 74 years. The usual clinical manifestations are related to endocrine deficiencies due to mass effect along with visual impairment and increased intracranial pressure. If a tumor is favorably localized, the treatment of choice is complete resection.OBSERVATIONSThe authors presented a unique case of a 61-year-old man with a suspicious cystic lesion in the right orbital roof that was causing right-sided headaches with pressure and pain in the right eye. Both computed tomography and magnetic resonance imaging were used for further evaluation and showed a suspicious lytic bone lesion that had an epicenter within the orbital rim, which was highly suggestive of a tumor of interosseous origin. After removal, the tumor was identified by pathology as a craniopharyngioma.LESSONSThe importance of this case report is in documenting a unique case of an ectopic craniopharyngioma in the orbit, adding to current hypotheses of the pathogenesis of ectopic craniopharyngiomas, and presenting an extensive review of literature.

Primary ectopic parasellar craniopharyngioma: a case report

BackgroundCraniopharyngioma (CP) is a slow-growing, benign tumor of the central nervous system located within the sellar and suprasellar regions. The tumor may extend from the suprasellar region to other areas. CPs are generally believed to originate from squamous remnants of an incompletely involuted craniopharyngeal duct that also develops from Rathke’s pouch. Primary parasellar craniopharyngioma is a relatively rare tumor, and nasal endoscopy, computed tomography, and enhanced magnetic resonance imaging can be applied to better evaluate the invasiveness and characteristics of these tumors.Case presentationWe report a case of right parasellar craniopharyngioma in a 49-year-old female patient with a 10-day history of dizziness and blurred vision. Preoperative imaging examination revealed right parasellar space-occupying lesions, and the patient underwent transnasal neuroendoscopic resection of the right parasellar space-occupying lesion. The postoperative pathological result confirmed craniopharyngioma.ConclusionsPrimary ectopic parasellar craniopharyngioma is a relatively rare tumor, and preoperative imaging examination can assist in the evaluation of tumor characteristics. However, the final diagnosis continues to depend on the histopathological results.

Isolated petrous apex ectopic craniopharyngioma

Primary ectopic craniopharyngioma is a rare entity. Isolated petrous apex bone location has not been reported previously. This study reports a case of 26-year-old male with right abducent nerve palsy. CT and MRI imaging reveal right petrous apex cystic lesion. No sellar or suprasellar region involvement was found. Endoscopic endonasal transphenoid approach has been successfully performed. Histopathology examination confirms the diagnosis of adamantinomatous craniopharyngioma. So far, it’s probably the first case report of primary ectopic craniopharyngioma isolated in the petrous apex. This case report supports the premise that primary ectopic craniopharyngioma is a multifactorial process that starts with an error from migrated embryological cells.

Recurrent ectopic craniopharyngioma in the sylvian fissure thirty years after resection through a pterional approach: a case report and review of the literature.

Local recurrence of craniopharyngiomas after apparently complete resection occurs frequently. Ectopic recurrence remote from the original site has been reported in 18 adult patients. The interval between the original diagnosis and the time of recurrence varies widely in these reports (1-26 years). We report a case of an ectopic recurrence in the sylvian fissure of an adamantinomatous type craniopharyngioma 34 years after the initial presentation and 30 years after the last surgical resection. In addition to this being the latest reported ectopic recurrence, the location of this new lesion in the sylvian fissure is fairly rare, having been reported in only three other cases. We also reviewed the English literature for reports of ectopic recurrent craniopharyngiomas in order to conduct an analysis of surveillance and treatment strategies.

WITHDRAWN: Recurrent ectopic craniopharyngioma in the sylvian fissure thirty years after resection through a pterional approach: a case report and review of the literature.

WITHDRAWN: Recurrent ectopic craniopharyngioma in the sylvian fissure thirty years after resection through a pterional approach: a case report and review of the literature.

An Atypical Rare Case of Extracranial Craniopharyngioma

We present a case of a purely extracranial, infrasellar craniopharyngiomathat initially presented as a mass in nose and nasopharynx. Craniopharyngiomas are usually located within the sella. Purely infrasellar craniopharyngiomas have only rarely been reported in the literature. A 55-year-old man presented with 8-month history of progressive headache and epistaxis. Rhinoscopy revealed polypoidal mass in both the nasal cavities and nasopharynx. Pre-operative biopsy suggested Craniopharyngioma. A battery of tests necessary for the diagnosis of Craniopharyngioma was done which excluded other possibilities. Surgical resection was done and histopathology thereafter was confirmatory of Craniopharyngioma. Adjuvant radiotherapy was given to the patient. The patient is doing well. The Rathke's pouch arises from the roof of the primitive mouth and grows toward the brain at the fourth week of gestation. Normally, it loses its attachment with the stomadeum completely by the eighth week of gestation. The craniopharyngeal canal (CPC) extends from the floor of the sella to the vomer and may rarely give rise to ectopic craniopharyngiomas. This case shows that such ectopic tumors may arise anywhere along the CPC. We are documenting this case as an atypical rare case of craniopharyngioma probably originating from tooth primordia.

Primary intracranial ectopic craniopharyngioma in a patient with probable Gardner's syndrome

The authors describe a patient with an adamantinomatous craniopharyngioma (CPG) arising in the cerebellopontine angle (CPA), who also had probable Gardner's syndrome. This 31-year-old man presented with headache and dizziness. Brain CT and MRI showed a 5 × 4-cm lesion with multiple small calcifications in the left CPA. The patient underwent suboccipital craniotomy with tumor removal. Histopathological findings indicated an adamantinomatous CPG. This patient also showed characteristics of Gardner's syndrome. Although this syndrome is associated with intracranial neoplasms, it is unclear whether patients with both Gardner's syndrome and CPG are part of the heterogeneity of Gardner's syndrome.

Primary cerebellopontine angle craniopharyngioma in a patient with Gardner syndrome

Craniopharyngiomas usually involve the sella and suprasellar space. Ectopic craniopharyngiomas have rarely been reported at the cerebellopontine angle (CPA). We report a rare primary craniopharyngioma of the CPA without extension into the sellar region. The lesion was initially detected by MRI during investigation of multiple scalp fibromas. Multiple osteomas of the skull and face were detected 2 years later, and colonic adenomatous polyposis was detected 4 years later; typical features of Gardner syndrome. This is the third report of a primary CPA craniopharyngioma in a patient with Gardner syndrome.

Primary Rathke’s cleft cyst in the cerebellopontine angle associated with apoplexy

Rathke's cleft cyst (RCC) is a congenital, benign, epithelial tumor and mainly occurs in sellar region and occasionally in suprasellar region; ectopic RCC is exceedingly rare. We report an uncommon RCC in cerebellopontine angle (CPA) associated with RCC apoplexy and investigated the possible hypothesis of its origin. A 12-year-old female student was admitted to hospital for 3-month history of vertigo, headache, nausea, and vomiting and aggravated for 1 week. Magnetic resonance imaging (MRI) revealed a space-occupying lesion with short T1 and long T2 signals in the left CPA and an intracystic floating nodule with hypointensity on T1- and T2-weighted imaging. The patient underwent the total tumor removal via the retrosigmoid approach with a good recovery. Primary RCC was confirmed by pathology. Follow-up MRI showed no recurrence 3.5 years after craniotomy. Primary RCC can occur in CPA and present special neuroimaging features associated with RCC apoplexy. We presumed that a mimicking mechanism of ectopic craniopharyngioma in CPA leads to the formation in the present case. Microsurgical resection is the optimal strategy for management. Further research and longer follow-up are helpful to better understanding the pathogenesis and development history of RCC in CPA.

Extracranial Infrasellar Ectopic Craniopharyngioma: A Case Report and Review of the Literature

We present a case of a purely infrasellar craniopharyngioma that initially presented as a sphenoid sinus mass. Craniopharyngiomas are usually located within the sella. Purely infrasellar craniopharyngiomas have only rarely been reported in the literature. A 25-year-old woman presented with 6-month history of progressive headaches. Initial neuroimaging revealed the presence of a sphenoid sinus mass. Initially, she underwent an endoscopic biopsy of the mass by our ENT service. Pathology was consistent with craniopharyngioma and she was referred to neurosurgery for further surgical management. She then underwent an endoscopic transsphenoidal approach for complete resection of the purely extracranial, infrasellar craniopharyngioma. The Rathke pouch arises from the roof of the primitive mouth and grows toward the brain at the fourth week of gestation. Normally, it loses its attachment with the stomadeum completely by the eighth week of gestation. The craniopharyngeal canal (CPC) extends from the floor of the sella to the vomer and may rarely give rise to ectopic craniopharyngiomas. This case shows that such ectopic tumors may arise anywhere along the CPC. Endoscopic endonasal approach provides an excellent route for the resection of these tumors.

Surgical treatment of ectopic recurrence of craniopharyngioma

Local recurrence following radical resection is one of the most common complications of pediatric craniopharyngioma. Only 28 cases of ectopic recurrence of craniopharyngioma have been reported in the literature, and only 13 cases occurred in patients originally treated as children. In this consecutive series of 86 children who underwent radical resection of primary and recurrent craniopharyngiomas, 4 patients (4.7%) experienced ectopic tumor recurrence, accounting for 27% of all recurrences after gross-total resection. The authors report on the successful surgical treatment of these 4 patients and the impact of ectopic craniopharyngioma recurrence on survival.

Ectopic craniopharyngioma of the fourth ventricle: case report

Background Ectopic craniopharyngioma is an uncommon entity. We report the first case of ectopic craniopharyngioma confined purely within the fourth ventricle, exophytic from pons. Case Description A 12-year-old girl presented with 2 months history of headache and recent evidence of raised intracranial pressure and left abducent palsy. Magnetic resonance imaging suggested intra–fourth ventricular mass with hydrocephalus. The patient underwent midline posterior fossa craniotomy and tumor excision, with prompt relief of gaze palsy. Histologic examination proved the lesion to be craniopharyngioma. We found no evidence to correlate this tumor to the suprasellar region. Conclusion Craniopharyngioma may not always be in primary communication with suprasellar tumor.

Recurrent ectopic craniopharyngioma

A 66-year-old woman developed an asymptomatic mass in the right frontal lobe 5 years after undergoing a right frontal craniotomy and removal of a craniopharyngioma. The mass progressively enlarged over the next 3 years, during which time it became multiloculated and partially cystic. Repeat craniotomy was performed 8 years after the original operation, at which time the mass was found to be an ectopic craniopharyngioma. The lesion probably resulted from seeding of tumour cells along the surgical tract at the time of the initial surgery.

Posterior fossa craniopharyngioma

A case is presented of a craniopharyngioma arising in and wholly confined to the posterior fossa in a 23-year-old man. The origin of this unusually located tumour is discussed and comparison made with previously reported ectopic craniopharyngiomas. Most of the cases appeared to be either direct extensions from the suprasellar region or recurrences in locations distant from the original previously treated suprasellar tumours. We found no evidence in this case to relate the tumour to a sellar/suprasellar origin and the craniopharyngioma is believed to have originated in the posterior fossa.

Postoperative ectopic craniopharyngioma

The case of an ectopic craniopharyngioma arising from a seed of tissue deposited along the operative track is reported. The uniqueness of this lesion is addressed. Ideal therapy and controversies regarding radiation therapy of craniopharyngiomas are discussed in light of this new variation in recurrence.