Year : 2003 | Volume
: 13 | Issue : 4 | Page : 427--429
Lateral ventricle craniopharyngioma - MRI demonstration of migratory nature of ectopic craniopharyngioma from the suprasellar region
J Singh, K Ganesan, SB Desai, A Kohli, E Kaushik
Department of MRI, Jaslok Hospital And Research Centre, 15, Dr. G. Deshmukh Marg, Mumbai - 400026, India
Department of MRI, Jaslok Hospital And Research Centre,15, Dr.G.Deshmukh Marg, Mumbai-400026
Ectopic craniopharyngiomas are located within the third ventricle, nasopharynx, sphenoid bone, cerebellopontine angle and pineal region. Some of these ectopic locations are explained by migration of the craniopharyngioma from the site of the craniopharyngeal duct from which these tumors arise. We report a case of a lateral ventricle craniopharyngioma with pre-operative imaging demonstration of this migratory nature of the tumor from the suprasellar region.
|How to cite this article:|
Singh J, Ganesan K, Desai S B, Kohli A, Kaushik E. Lateral ventricle craniopharyngioma - MRI demonstration of migratory nature of ectopic craniopharyngioma from the suprasellar region.Indian J Radiol Imaging 2003;13:427-429
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Singh J, Ganesan K, Desai S B, Kohli A, Kaushik E. Lateral ventricle craniopharyngioma - MRI demonstration of migratory nature of ectopic craniopharyngioma from the suprasellar region. Indian J Radiol Imaging [serial online] 2003 [cited 2020 Jun 4 ];13:427-429
Available from: http://www.ijri.org/text.asp?2003/13/4/427/28727
Craniopharyngiomas are neoplasms arising from the craniopharyngeal duct. They are usually located in the suprasellar and sellar regions and can be found anywhere from the floor of the third ventricle to the pituitary gland. They can extend into the anterior (2-5%), middle (2%) or posterior (1-4%) cranial fossa. Ectopic locations include the third ventricle, nasopharynx, sphenoid bone, cerebellopontine angle and the pineal region . We report a case of a craniopharyngioma essentially within the right lateral ventricle, an uncommon site, with a small stalk in the suprasellar cistern suggesting the true site of origin.
An 11-year-old girl was referred to our hospital with a ninemonth history of repeated episodes of headache associated with vomiting that had worsened over the past one week and generalized tonic clonic convulsions since the past nine months. There were no visual complaints or limb weakness. On examination she was conscious and cooperative. The higher functions were normal. Cranial nerves examination revealed right eye nystagmus, normal visual field and acuity. There was no sensory or motor deficit. Bilateral cerebellar signs were present. Fundoscopy revealed bilateral papilloedema.
An MRI was performed which revealed a large well-defined lobulated mass lesion in the temporal and occipital horns and body of right lateral ventricle [Figure 1] with a small extension into the suprasellar cistern [Figure 2]. The mass was hyperintense on both T1 and T2 weighted images and revealed rim enhancement on administration of gadolinium [Figure 3]. The lesion caused distortion and displacement of the brainstem anteriorly and to the left causing aqueductal compression and obstructive hydrocephalus [Figure 4]. The third ventricle and right thalamus were displaced to the left [Figure 5] and the cerebellum downward by this mass lesion.
The patient underwent a right basal temporal craniectomy with partial excision of the intraventricular tumor. At surgery it was noted that the tumor had encroached the ventricular system via the right temporal choroidal fissure. The tumor was cystic with characteristic machine-oil fluid content. The cyst wall revealed some calcification and was densely adherent to the ventricular wall permitting only partial excision. The histopathology confirmed the lesion to be an admantinomatous craniopharyngioma.
Craniopharyngiomas comprise nearly 1%-3% of all intracranial tumors and arise from the squamous epithelial rests along remnants of Rathke's cleft (craniopharyngeal duct).They have a bimodal age distribution and i commonly occur in children (peak incidence 5-1 Oyears) . There is seen a second smaller peak in middle aged adults. Most craniopharyngiomas arise in the suprasellar and sellar regions. The ectopic sites reported so far include the nasopharynx, sphenoid bone, pineal region, cerebellopontine angle and third ventricle,,,. Craniopharyngioma within the third ventricle with extension into the lateral ventricles has also been reported. However, intraventricular craniopharyngioma with only lateral ventricle involvement as in our case is an uncommon finding. Craniopharyngiomas, although benign tumors, can be locally aggressive and invasive  and rarely metastatic. Altinores et al described a case of a 14-year-old boy with a cerebellopontine angle craniopharyngioma presenting with progressive hearing loss in the right ear and right facial weakness who developed diabetes insipidus in the postoperative period. They postulated a possible extension of the tumor in the suprasellar region in view of this post-operative complication and its migration from this site of origin. In our case we were able to demonstrate by imaging this uncommon migratory nature of a craniopharyngioma pre-operatively. Our patient suffered no post-operative endocrine dysfunction as only a partial excision was performed. However this increases the chances of recurrence.
Craniopharyngiomas are of two histological subtypes; the admantinomatous type and the papillary type. Admantinomatous tumors are grossly cystic and may have both solid and cystic components. The cyst content is cholesterol rich and is typically described as like machine-oil. These tumors contain cords of columnar or squamous epithelium with wet keratin formation. Calcification, fibrosis and inflammation are common features. They are more likely to be adherent and invasive. Recurrence is also common with this subtype. In contrast the papillary type are usually solid and show no calcification and wet keratin formation. They are more commonly situated within the third ventricle and affect the adult age group.
On imaging craniopharyngiomas are usually cystic with wall or solid component calcification and may appear hyperintense on both T1 and T2 weighted images. The hyperintensity on T1 weighted spin echo sequences has been attributed to protein, hemorrhage, cholesterol and other fatty components. Ahmadi et al have demonstrated that this T1 hyperintensity best correlates with high protein content (concentration greater than 90g/L), presence of free methemoglobin or both . On administration of gadolinium contrast the wall and solid portions of the tumor show enhancement.
In our case the characteristic morphology of the tumor and the extension into the suprasellar cistern allowed us to make the diagnosis of craniopharyngioma preoperatively despite the unusual location of the tumor.
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