Tumor-third ventricular relationships in supradiaphragmatic craniopharyngiomas: correlation of morphological, magnetic resonance imaging, and operative findings

OBJECTIVE: To disclose the relationships of primarily supradiaphragmatic craniopharyngiomas with the third ventricular floor (3rdVF) by means of preoperative magnetic resonance imaging (MRI) and thus to select the surgical approach avoiding the hypothalamic structures. METHODS: MRI findings in 76 consecutive patients with craniopharyngiomas operated on between June 1991 and December 2002 were interpreted on the basis of the results of the authors' own previous microanatomic studies. The assumed tumor-3rdVF relationships were then correlated with the operative findings. MRI features characteristic for different topographical relationships were analyzed in 44 patients (18 children, 26 adults) with exclusively supradiaphragmatic tumors. RESULTS: In 14 of 15 patients with the tumor located below the 3rdVF (suprasellar extraventricular craniopharyngioma), the anterior communicating artery was displaced upward and indirectly indicated the position of the chiasm between the prechiasmatic and the retrochiasmatic tumor portions. Hydrocephalus was absent in 14 patients, including those with giant tumors. The anterior part of the third ventricular cavity was found in front of the level of the foramina of Monro in 6 patients. All 28 tumors growing partially inside and partially outside the third ventricular cavity (intraventricular and extraventricular craniopharyngioma) were retrochiasmatic. They caused severe or moderate hydrocephalus in 20 patients and mild hydrocephalus in 2. One purely intraventricular tumor caused severe hydrocephalus. CONCLUSION: The position of the optic chiasm and the size of the lateral ventricles on preoperative MRI enable us to determine the position of the 3rdVF or its remnants in relation to the supradiaphragmatic craniopharyngiomas and to select the proper surgical approach allowing exposure of the tumor while avoiding the hypothalamic structures.     

Intraventricular craniopharyngioma: report of two cases and review of the literature.

Two unusual cases of purely intraventricular craniopharyngioma are presented. Both patients complained of headache as a sign of increasing intracranial pressure, but neither other neurological deficits nor hormonal disorders were present. Magnetic resonance images showed a mass lesion located within the third ventricle. Surgery confirmed that these two tumors were completely confined within the third ventricle, and histologically they proved to be squamous papillary craniopharyngiomas. Review of the literature demonstrates that craniopharyngiomas at this location have many common features and would appear to form a distinct entity.     

"Moustache" appearance in craniopharyngiomas: unique magnetic resonance imaging and computed tomographic findings of perifocal edema

This report describes two cases of craniopharyngioma with perifocal edema. In both patients, computed tomography and magnetic resonance imaging (MRI) revealed that the tumors occupied the suprasellar cistern, invaginated the floor of the 3rd ventricle and were tightly adherent to the ventricular walls. The intraventricular portions of the tumors were cystic, containing protein-rich fluid as suggested by MRI and confirmed by operative findings. There was perifocal edema in the hypothalamus adjacent to the intraventricular tumor, the optic tracts, and the posterior limbs of the internal capsules, resembling the shape of a moustache on axial computed tomographic and MRI scans. The perifocal edema subsided after treatment of the intraventricular tumor by surgical resection or radiation therapy. The "moustache" appearance seems a unique, characteristic feature of perifocal edema, which is observed infrequently with certain craniopharyngiomas.     

Extended transsphenoidal surgery for suprasellar craniopharyngiomas: infrachiasmatic radical resection combined with or without a suprachiasmatic trans-lamina terminalis approach

BACKGROUND: An extended transsphenoidal approach allowed for direct midline exposure of the parasellar structures such as the hypothalamic-pituitary axis and the third ventricle. To evaluate the capability of this approach for removal of suprasellar craniopharyngiomas, surgical outcomes were retrospectively analyzed. METHODS: During a 9-year period, 20 consecutive patients with suprasellar craniopharyngioma underwent transsphenoidal tumor resection. The average follow-up period was 55 months. No patient had a purely intrasellar tumor, 9 had prechiasmatic tumors, 9 had retrochiasmatic tumors, and 2 had purely intraventricular tumors. RESULTS: Total resection was achieved in 86% of operations. Even in mostly intraventricular cases, a transsphenoidal trans-lamina terminalis approach afforded complete resection. Visual improvement and preservation of the pituitary stalk were achieved in 84% and 95% of cases, respectively. New postoperative deterioration of pituitary function occurred in about 65% of cases, and no patient resolved their preoperative hormonal disturbance after surgery. The overall percentage of patients with diabetes insipidus increased to 61% postoperatively from 11% preoperatively. Nonendocrinologic surgical complications were observed: worsening of vision in 3 patients, hyperphagia in 3 patients, short-term memory loss in 2 patients, and cerebrospinal fluid leakage in 3 patients. Recurrence after total resection occurred in 2 (11%) patients with retrochiasmatic tumors. CONCLUSIONS: Reasonable surgical results in this study suggest that the extended transsphenoidal approach is safe and effective for removal of craniopharyngiomas. Although preservation of the pituitary stalk can be achieved in a high percentage of patients, postoperative endocrinopathy still remains as a significant problem after radical removal of the craniopharyngioma.     

Spontaneous intraventricular rupture of craniopharyngioma cyst

BACKGROUND: Rupture of a cystic craniopharyngioma is a rare phenomenon. The rupture of the cyst causes decompression of the adjacent neural structures resulting in spontaneous improvement of the visual symptoms or level of sensorium. The leakage of its contents into the subarachnoid space gives rise to meningismus. We report an extremely rare phenomenon of an intraventricular rupture of a cystic craniopharyngioma, which resulted in acute neurological deterioration and chemical ventriculitis. CASE DESCRIPTION: A 38-year-old lady presented with a 1-year history of frontal lobe dysfunction and bilateral primary optic atrophy. The CT scan showed a multi-loculated, hyperdense lesion in the region of the third ventricle and suprasellar cistern. She suffered acute deterioration of neurological status; computed tomography (CT) scan showed a hypodense lesion in the suprasellar cistern with persistent hydrocephalus. She was treated with ventricular drainage, steroids and anticonvulsants. Ventricular fluid showed high cholesterol and LDH levels. The diagnosis of craniopharyngioma was subsequently verified histologically. CONCLUSIONS The intraventricular rupture of a cystic craniopharyngioma can result in acute clinical deterioration and morbidity because of chemical ventriculitis. This is unlike the rupture in the subarachnoid space or sphenoid sinus which usually results in symptomatic improvement, although chemical meningitis may occur. This rare phenomenon should be recognized, and prompt ventricular drainage is advised. The literature is reviewed, and management of this condition is discussed.     

Endoscopic, endonasal extended transsphenoidal, transplanum transtuberculum approach for resection of suprasellar lesions

OBJECT: The extended transsphenoidal approach is a less invasive method for removing purely suprasellar lesions compared with traditional transcranial approaches. Most advocates have used a sublabial incision and a microscope and have reported a significant risk of cerebrospinal fluid (CSF) leakage. The authors report on a series of purely endoscopic endonasal surgeries for resection of suprasellar supradiaphragmatic lesions above a normal-sized sella turcica with a low risk of CSF leakage. METHODS: A purely endoscopic endonasal approach was used to remove suprasellar lesions in a series of 10 patients. Five lesions were prechiasmal (three tuberculum sellae and two planum sphenoidale meningiomas) and five were post-chiasmal (four craniopharyngiomas and one Rathke cleft cyst). The floor of the planum sphenoidale and the sella turcica was reconstructed using a multilayer closure with autologous and synthetic materials. Spinal drainage was performed in only five cases. Complete resection of the lesions was achieved in all but one patient. The pituitary stalk was preserved in all but one patient, whose stalk was invaded by a craniopharyngioma and who had preoperative diabetes insipidus (DI). Vision improved postoperatively in all patients with preoperative impairment. Six patients had temporary DI; in five, the DI became permanent. Four patients with craniopharyngiomas required cortisone and thyroid replacement. After a mean follow up of 10 months, there was only one transient CSF leak when a lumbar drain was clamped prematurely on postoperative Day 5. CONCLUSIONS: A purely endoscopic endonasal approach to suprasellar supradiaphragmatic lesions is a feasible minimally invasive alternative to craniotomy. With a multilayer closure, the risk of CSF leakage is low and lumbar drainage can be avoided. A larger series will be required to validate this approach.     

Intraventricular craniopharyngioma: a long-term follow-up of six cases

Intraventricular craniopharyngiomas are rare tumours. They are wholly within the third ventricle and can be distinguished from suprasellar lesions which extend into the third ventricle by the presence of an intact floor of the third ventricle. They are attached to the wall of the third ventricle to a variable extent, most commonly in the region of the tuber cinereum. The long-term follow-up on six cases treated by one of the authors is presented. Headache and visual disturbance were the most common presenting features but, unlike the more common suprasellar lesions, symptomatic endocrine disturbances were not a common presenting feature. Total surgical removal can cause hypothalamic damage and the resulting morbidity can be serious and sometimes life-threatening. Subtotal removal followed by radiotherapy is probably the treatment of choice for these lesions.     

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.     

Neuroendoscopic treatment of cystic craniopharyngioma in the third ventricle.

The third ventricle is a relatively uncommon location for craniopharyngiomas. Generally, craniotomy has been considered the procedure of choice in such cases. We describe a girl in whom a cystic third ventricular craniopharyngioma was successfully treated by evacuation of the cyst contents via a flexible neuroendoscope and precise placement of an Ommaya reservoir catheter within the tumor.     

Craniopharyngioma: a pathologic, clinical, and surgical review

Craniopharyngioma is a rare and mostly benign epithelial tumor of the sellar and suprasellar region. Two principal patterns of craniopharyngioma are recognized: papillary and adamantinomatous. Papillary craniopharyngiomas are encountered in adults and may lack the cystic spaces filled with "motor oil" as well as the palisading peripheral rows of epithelial cells, keratinization, or calcification typical of pediatric adamantinomatous craniopharyngioma. Secondary to their anatomic location, craniopharyngiomas may present with endocrinologic dysfunction and visual disturbances. Differential diagnosis includes Rathke's cleft cyst, pituitary adenoma, dermoid/epidermoid cysts, and other rare sellar/suprasellar lesions as pituicytomas. Many controversies exist concerning the preferred surgical approach for these tumors. Endoscopic endonasal surgery is no longer reserved only for sellar or small cystic suprasellar lesions. Prechiasmatic/preinfundibular lesions are effectively removed using an endonasal transtuberculum/transplanum approach; subchiasmatic/transinfundibular tumors require the addition of a transellar approach with inferior pituitary transposition; and retrochiasmatic/retroinfundibular lesions are better accessed performing an endonasal superior pituitary transposition. Compared with well-established trancranial approaches (pterional, subfrontal, presigmoid), endoscopic endonasal surgery combines the virtues of the caudocranial and midline approaches, allowing for appropriate infrachiasmatic exposure without the need for manipulation of surrounding neurovascular structures to access the tumor. This anatomic advantage, combined with high-definition wide-angle visualization, exquisite endonasal microsurgical techniques, and devoted instrumentation facilitates a high rate of endocrine function preservation and visual improvement, while concurrently achieving comparable resections. Endoscopic skull base reconstruction with the vascularized nasoseptal flap has dramatically reduced the incidence of cerebrospinal fluid leak, consolidating endoscopic endonasal surgery as an effective and safe alternative for the treatment of these challenging tumors.     

Giant Suprasellar Rathke's Cleft Cyst Mimicking Craniopharyngioma: Implications for a Spectrum of Cystic Epithelial Lesions of Ectodermal Origin

Cystic epithelial lesions such as Rathke's cleft cysts (RCCs) and craniopharyngiomas may be difficult to distinguish on a clinical, radiographic, and sometimes histopathological basis. We describe a case of a giant 6.5 cm suprasellar cystic lesion that was presumed to be a craniopharyngioma based on the neuroimaging findings. The lesion extended from the anterior skull base and sella turcica to the lateral ventricle and sylvian fissure resulting in obstructive hydrocephalus. Complete surgical removal of the suprasellar lesion was achieved using an extended frontotemporal transbasal skull base approach. Intraoperatively, the cyst wall was thickened and partially calcified, resembling a craniopharyngioma. However, the histopathological examination revealed findings most consistent with a RCC with additional features of extensive squamous metaplasia, metaplastic bone formation, and chronic inflammation. The case raises the issue of whether there is a pathologic continuum of parasellar ectodermal lesions which may account for the overlap of features and transitional states. In this report, we discuss the possible spectrum between RCCs and craniopharyngiomas, and also emphasize the importance of complete resection of the cyst wall in RCCs that exhibit squamous metaplasia, inflammation, or ossification to minimize the probability of recurrence.     

Third ventricular lesion masquerading as suprasellar disease

We discuss the case of a patient who presented with a bitemporal visual field disturbance thought to arise from chiasmatic compression secondary to a suprasellar mass. The patient was ultimately diagnosed with medulloblastoma with diffuse intraventricular disease. Careful review of magnetic resonance (MR) findings in this case demonstrate the apparent suprasellar mass to be within the suprachiasmatic recess of the third ventricle. The role of MR imaging in distinguishing between suprasellar disease involving the third ventricle and primary third ventricular lesions is discussed.     

Neuroendoscopic transventricular surgery for suprasellar cystic mass lesions such as cystic craniopharyngioma and Rathke cleft cyst

Cystic mass lesions in the suprasellar cistern are often associated with neurological deficits, cognitive disorders, and endocrinological impairments. Many surgical approaches are available to treat these mass lesions, but are technically difficult and cannot remove the lesion completely without risking damage to neurological and endocrinological functions due to the proximity to the surrounding structures. Neuroendoscopic transventricular surgery was performed using a ventricular fiberscope for three patients with craniopharyngiomas and two patients with Rathke cleft cysts, with gamma knife radiosurgery for craniopharyngiomas. The endoscopic transventricular approach is safe and minimally invasive for congenital benign suprasellar cystic lesions, especially arachnoid cysts.     

Tumours of the third ventricle

Summary Primary intraventricular tumors, including colloid cysts, choroid plexus papillomas, and ependymomas have their attachment within the IIIrd ventricle and stay within its confines. Secondary intraventricular tumours arise adjacent to the IIIrd ventricle, often in the suprasellar region, and include craniopharyngiomas, optic nerve gliomas, and meningiomas. Approaches to the ventricle include subfrontal, subtemporal, anterior transventricular, anterior and posterior transcallosal, and transtentorial routes. Memory problems have been noted with anterior dorsal methods, implicating damage to one or both anterior forniceal columns. Methods of opening the roof of the third ventricle must evaluate risk to the deep ventricular veins, the fornix, and the choroid plexus. The basal subfrontal method may be modified by opening the lamina terminalis or including a lateral approach from the pterional area. The application of these approaches in a series of 100 third ventricle tumours is analyzed.     

Infrasellar Craniopharyngiomas: Case Report and Review of the Literature

Although craniopharyngiomas account for a large percentage of pediatric intracranial tumors, there is a bimodal age distribution. Most of these neoplasms are suprasellar or sellar in location. In this report we describe an unusual case of an infrasellar craniopharyngioma in a child. Only four previous cases of infrasellar craniopharyngiomas with no sellar involvement have been described. Infrasellar craniopharyngiomas are part of the continuum representing intracranial craniopharyngiomas and ameloblastomas of the jaw. A transnasal endoscopic biopsy was performed with a preliminary diagnosis of craniopharyngioma. The patient then underwent a radical resection of the infrasellar tumor via a subfrontal transbasal approach. This case illustrates a rare and unusual location for a craniopharyngioma. Craniopharyngiomas should be considered in the differential diagnosis of infrasellar neoplasms. Infrasellar craniopharyngiomas compromise part of the spectrum of tumors originating from enamel-forming neural crest cells.     

110例颅咽管瘤手术经验

目的 探讨手术全切除颅咽管瘤的有效方法。方法 110例颅咽管瘤患者,男性62例,女性58例。年龄15．0－67．0岁,平均34．4岁。其中102例患者取翼点入路,5例经额下入 路,3例经纵裂从胼胝体至穹窿间隙和第三脑室前部切除肿瘤。术中根据肿瘤位置,从视交叉前间隙、视神经外侧间隙和颈内动脉外侧间隙切除肿瘤。术中注意保护下丘脑神经结构和防止穿通动脉的 损伤。结果 101例患者达到肿瘤全切除,6例次全切除,3例大部切除。术中垂体柄保留57例,断裂29例,未见垂体柄24例。术后54例患者有多饮多尿,52例3个月内恢复,2例持续1年以上;52例患者有血钠紊乱,均在3个月内恢复;3例术后视力减退加重;3例动眼神经麻痹,其中1例1年以上仍无恢复,2例暂短性肢体瘫;4例死亡。术后随访1个月－5．0年,平均1．1年。101例肿瘤全切除患者,有4例复发。本组患者术后99例能参加日常工作,5例生活自理,2例需要生活照顾。结论 选择合适的手术入路,保护下丘脑结构和功能,是颅咽管瘤全切除并获得良好效果的关键。     

A case of metastatic suprasellar tumor with edema along the optic pathway

A rare case in presented of a patient with edema formation along the optic pathway, which was caused by metastatic suprasellar tumor mimicking craniopharyngioma. A 63-year-old female with visual disturbance, diabetes insipidus, and symptomatic panhypopituitarism was admitted to our hospital. CT images showed a suprasellar mass lesion. MR images demonstrated a heterogeneously enhanced tumor and edema along the optic nerve and tract. Although no cystic component is shown in radiological findings, these features are commonly observed in cases of craniopharyngioma. The patient underwent removal of the tumor by the transcranial approach. Since histological examination revealed adenocarcinoma, the tumor was diagnosed as metastatic brain tumor originating from non-recurrent rectal cancer operated on eight years previously. Conventional radiotherapy with a dose of 50GY was performed for the residual tumor. The tumor size was unchanged, but the edema had disappeared 7 months after the operation. Edema spreading along the optic tract was reported as a characteristic MR finding for diagnosis of craniopharyngiomas as distinguished from other common parasellar tumors. However, it is suggested that edema of the optic pathway seems to be caused not only by craniopharyngioma but also other suprasellar lesions. Even if there was a characteristic radiological finding for some specific diseases, metastasis should be kept in mind as a differential diagnosis, because of its various appearance in neuroimages.     

经眶-额-颞入路显微手术切除颅咽管瘤

目的 总结经眶-额-颞入路显微手术切除颅咽管瘤的经验,探索肿瘤全切除的手术技巧。方法 采用经眶-额-颞入路显微手术切除颅咽管瘤18例。肿瘤位于鞍上7例、鞍上-第三脑室6例、鞍上-鞍内4例、第三脑室1例。12例肿瘤有钙化、13例囊性变、5例完全实质性。术中根据肿瘤的生长方向,分别从不同的间隙切除肿瘤。结果 肿瘤全切除14例,次全切除2例,部分切除2例。术后17例患者获随访,随访时间8～41个月。肿瘤全切除的14例未见肿瘤复发;次全切除和部分切除的3例于术后1个月内行辅助放疗,在随访期间肿瘤未见明显增大。Karnofsky预后评分(KPS)80～90分12例,60～70分5例,50分1例,无一例死亡。结论 经眶-额-颞入路能清楚地显露鞍区颅咽管瘤及其周围结构,减少对脑组织的牵拉和无效脑暴露。显微手术技巧是安全切除肿瘤、避免穿通动脉损伤和保护下丘脑功能的重要保障,也是手术成败的关键。     

Ventriculostomy in a tumor involving the third ventricular floor

Recently, endoscopic management has gotten preference over open surgical treatment in selected cases of intraventricular tumors. Endoscopic third ventriculostomy (ETV) appears unfeasible when tumors extend to the third ventricular floor region due to the risk of perforators and injury to the basilar artery. We report the case of a 12-year-old male with symptoms of acute, chronic, raised intracranial pressure. Computed tomography (CT) and magnetic resonance imaging (MRI) showed a posterior third ventricular tumor involving the aqueductal and floor regions. The ETV was done after clearing the floor by partial tumor resection, keeping the dorsum sellae as the major anatomical landmark. The patient improved satisfactorily and was given adjuvant radiotherapy, and the need for an external shunt was completely eliminated. We conclude that ETV appears worth trying, even in third ventricular tumors involving the floor region if they can be cleared from the tumor keeping the dorsum sellae as the major anatomical landmark.     

Intraventricular craniopharyngioma: morphological analysis and outcome evaluation of 17 cases

Purpose  

There is still some confusion with regard to the tumor–third ventricle floor (3rd VF) relationship of craniopharyngiomas located exclusively within the third ventricle. This study aims to provide some evidence to clarify the growth pattern of intraventricular craniopharyngiomas (IVC), and to summarize the surgical strategy and outcome.