Intrinsic brainstem choroid plexus papilloma. Case report

The authors report an intrinsic brainstem lesion that was diagnosed initially as a pontine cavernoma, which finally proved to be a choroid plexus papilloma. Choroid plexus papillomas are rare tumors of the central nervous system and are usually intraventricular in location. The occurrence of this tumor in an intraparenchymal location is extremely rare, and its occurrence within the brainstem is previously unreported. The authors also report a trial of chemotherapy with lomustine in the management of the residual tumor.     

Radiological diagnosis of the lateral ventricle tumor (author's transl)

The radiological characteristics of intraventricular tumors were studied with special reference to differential diagnosis from paraventricular tumors by careful analysis of seven cases intraventricular tumors and five cases paraventricular tumors. As, in the radiological term, intraventricular tumors have been dividied into two groups according to their location by Bernasconi et al., seven cases intraventricular tumors reported in this paper were also divided into two groups; 1) 2 cases ependymom occupying the frontal horn and the body of lateral ventricle, 2) 4 cases meningioma and one plexus papilloma occupying the trigone and the adjucent regions. In case of tumors belonged to group 1, the filling defect showing the circumference of tumors and the associated unilateral hydrocephalus were characteristic findings on the ventriculogram, while the depression of ventricular wall was observed in paraventricular tumors. Angiographic characteristics in group 1 tumors were the splay of subependymal veins of the medial group from those of lateral group and the increased density of deep veins due to raised circulation, and these findings demonstrated in all cases of ependymoma reported here. On the other hand, in all meningiomas belonged to group 2, the hypertrophia of choridal arteries was noticed in the angiogram. Moreover, the anterior choroidal artery was shown to be divided into two branches at its plexal segments, and the tumor stain at the trigone always lied between these two branches. We named this dissociation of these two plexal branches due to tumor as "flare sing". This is considered to be specific finding for a trigone meningioma, and this "flare sing" was observed in all of our 4 cases meningioma. In two cases out of four, the inferior ventricular vein was displaced anteriorly delineating the anterior margin of the tumor. This again is considered as a sign indicating the tumor occupying the trigone and adjacent regions.     

Peripheral aneurysms of the lateral posterior choroidal artery: clinical presentation and endovascular treatment: report of two cases

OBJECTIVE AND IMPORTANCE: To describe the clinical presentation and endovascular management of peripheral aneurysms of the lateral posterior choroidal artery. Aneurysms in this location are exceptionally rare and optimal treatment may be difficult. CLINICAL PRESENTATION: Two patients with peripheral aneurysms of the distal portion of the lateral posterior choroidal artery presented with headaches from extensive intraventricular hemorrhage. INTERVENTION: Endovascular surgical therapy by use of superselective n-butylcyanoacrylate embolization of the aneurysm and adjacent distal parent artery was successful in both patients. CONCLUSION: Patients with peripheral aneurysms of the lateral posterior choroidal artery usually present with intraventricular hemorrhage. They may be difficult to treat by open surgical techniques owing to their intraventricular location and the frequent inability to preserve the parent artery by aneurysm clipping. Instead, it is typical that either proximal parent artery occlusion or aneurysm trapping must be used. An equivalent endovascular surgical technique may be an attractive alternative method of management.     

Intraventricular meninigiomas: a report of 25 cases

In order to study the clinical characteristics of intraventricular meningiomas, we analyzed retrospectively 25 patients and reviewed the literature with regard to incidence, clinical manifestation, imaging features, preoperative diagnosis, surgical findings, and histopathological results. Intraventricular meningiomas are quite rare, but they represent an important differential diagnosis of intraventricular neoplasms. Computed tomography or magnetic resonance imaging enable a correct diagnosis of intraventricular meningiomas in most of the cases. The tumors often grow slowly to a substantial size before they become symptomatic. The operative route should be selected according to the tumors location. Out of the 24 lateral ventricular meningiomas in our series, 20 were resected via a posterior parieto-occipital transcortical approach, two were resected via a transcallosal approach, and another two tumors, located in the frontal horn and body of the lateral ventricle, were resected via a frontal approach. A median suboccipital craniotomy was performed for the fourth ventricular meningioma. The parieto-occipital route for lateral ventricular meningiomas is a safe surgical approach, which is not necessarily associated with postoperative visual deficits. Piecemeal removal of the tumor can be safely and easily performed and special attention should be paid to the choroidal vessels intraoperatively.     

Intraventricular neurocytoma: a clinical and pathological study of three cases and review of the literature

Three patients with a recently described tumor of neuronal origin, intraventricular neurocytoma, are presented. These tumors occur as intraventricular lesions in young patients, and the prognosis after surgical treatment is favorable. The initial pathological diagnosis of intraventricular neurocytoma may be difficult because of the striking resemblance of these tumors to oligodendroglioma and, to a lesser extent, ependymoma on light microscopic examination. Despite the use of wide-ranging panels of monoclonal antibodies, previous authors have not found any characteristic immunohistochemical staining patterns, but in our three patients, the use of synaptophysin, glial fibrillary acidic protein, and Leu-7 demonstrated staining patterns that may be useful in the diagnosis of this tumor. The monoclonal antibody Ki-67 was used to stain one tumor and showed a low cell proliferation rate. We have reviewed the clinical, radiological, and pathological features of these 3 patients and 17 previously described patients in an attempt to determine the important diagnostic features of intraventricular neurocytoma. Intraventricular neurocytoma should be considered in any young patient with symptoms of raised intracranial pressure and radiological evidence of an intraventricular lesion. Pathological diagnosis requires the use of electron microscopy to show features of neuronal differentiation; however, immunohistochemical demonstration of a neuronal phenotype is also a useful adjunct to diagnosis. Failure to use specialized techniques for pathological diagnosis will lead to misdiagnosis of these lesions as oligodendrogliomas, as was the initial diagnosis in 2 of our patients before review.     

Primary oligodendroglioma of the lateral ventricle with computed tomographic and positron emission tomographic evaluations

Two cases of primary intraventricular oligodendrogliomas are presented. Total excisions of well-demarcated large tumors in the lateral ventricle were successfully performed in young women by means of a frontal transventricular approach. An evaluation by computed tomography and positron emission tomography was attempted to obtain definite diagnosis of not only the location of the tumor but also the histologic grade of malignancy.     

Epidermoid tumor in the lateral ventricle

After a review of the literature on the origin of epidermoid tumors, a case report is presented of an intraventricular localization of such a tumor. Two theories are discussed as an explanation for this rare site. This case report, in addition to some data from the literature, suggests that it is unacceptable to assume that epidermoids have a primary location within the lateral ventricle.     

Primary extrarenal Wilms' tumor in children

We report three additional cases of primary extrarenal Wilms' tumor and review those cases previously documented. Analysis of the location, histopathology, treatment, and survival of these cases supports the following conclusions: Wilms' tumor may occur in an extrarenal location without primary renal involvement and must be included in the differential diagnosis of abdominal, pelvic, and inguinal masses; an extrarenal location supports a more frequent occurrence of ectopic metanephric blastema than was previously recognized or origin of Wilms' tumor from a more primitive mesodermal tissue; and the natural history and prognosis of extrarenal and renal Wilms' tumors appears similar.     

Minimally invasive management of ependymoma of the aqueduct of Sylvius: therapeutic considerations and management.

A major concern in the neuroendoscopic approach to an intraventricular tumor is the histological confirmation from a limited biopsy. However, the effort to excise the whole bulk of the tumor should be made for the minimally invasive management of selected intraventricular tumors. The case of an adult male with focal aqueductal ependymoma who presented with the clinical syndrome of hydrocephalus is reported. This may be of particular interest because it represents the first case of aqueductal ependymoma that has been successfully treated with endoscopic surgery.     

Recurrence of bladder tumors following surgery for transitional cell carcinoma of the upper urinary tract.

A retrospective analysis of 53 patients with an upper urinary tract (UUT) transitional cell carcinoma, which was treated surgically, was performed in relation to the development of a subsequent bladder tumor. In 19 of the 53 patients (35.8%) bladder tumors developed following surgery of a UUT tumor. The simultaneous occurrence of a bladder tumor and more than two tumors in the UUT had a significant influence on the rate of bladder tumor recurrence. On the other hand, location, mode of growth, grade, stage and vascular invasion of the UUT tumor, and history of bladder tumors did not seem to be related to the frequency of subsequent bladder tumors. These findings suggest that the diversity of UUT tumors at the time of diagnosis is an important factor in bladder tumor recurrence. Therefore, clinical and pathological examinations should be carefully performed in patients with UUT tumors.     

Massive preoperative pulmonary embolism and suprasellar brain tumor: case report and review of the literature

A 57-year-old man presented to our facility with the diagnosis of a suprasellar mass and died on the 2nd post-admission day from a massive pulmonary embolism. At autopsy, the mass was diagnosed as craniopharyngioma. This case report and review of the literature suggest an intimate relation between brain tumors and thromboembolic complications as evidenced by the high incidence of this association, the frequent occurrence of coagulopathies in patients with brain tumors, and the prevalence of tumors in the suprasellar location. The specific mechanisms that may be at play are reviewed.     

Surgical approaches to tumors of the lateral ventricle

Lateral ventricular tumors are rare lesions of the central nervous system, and because most tumors are benign or low grade, permanent cure can be achieved with complete removal. After adequate preoperative imaging discloses a lateral ventricular mass, the neurosurgeon has several options to choose from when determining the ideal surgical approach to the tumor. The surgical approach cannot be standardized, because the specific location, size, and vascularization of these deep-seated tumors are fundamental elements influencing the choice of surgical approach. Although access to the lateral ventricles may require additional preoperative considerations and planning, the combination of proper knowledge of the cortical and intraventricular anatomy with the familiarity and selection of an appropriate surgical approach will optimize the surgical outcome.     

Intraventricular ganglioglioma: a short illustrated review

The following review of the literature describes the ganglioglioma, an uncommon mixed glioneuronal neoplasm, most often of low-grade histology, with a small, albeit well-documented, malignant potential. These tumors exhibit a strong epileptogenic propensity and most often present as new onset seizures or are discovered after a long history of refractory epilepsy. Despite their indolent course, the importance of gross total resection is well recognized to prevent anaplastic and malignant degeneration. Morphologically, the neoplasm is often cystic with an enhancing mural nodule, but can also be entirely solid. They are most often found in the temporal lobe but have been found throughout the neuraxis. An exceedingly rare location of the ganglioglioma is within the lateral ventricle. A systematic literature search revealed only eight reports documenting the occurrence of a ganglioglioma within the lateral ventricle. We describe an illustrative case of an intraventricular ganglioglioma with a prominent cystic component and enhancing mural nodule, which represents the classic radiographic appearance of gangliogliomas described in other locations. A superior parietal lobule approach offered excellent surgical access for tumor removal and the patient has remained free of neurological deficits following surgery. Regardless of location within the central nervous system, ganglioglioma should be on the differential diagnosis for any cystic mass with a mural nodule, particularly in the setting of epilepsy.     

Gamma knife radiosurgery for intraventricular meningiomas

Purpose  Intraventricular meningiomas are relatively rare tumors that may benefit from stereotactic radiosurgery as a minimally invasive treatment strategy. We report our experience using gamma knife radiosurgery (GKR) for intraventricular meningiomas. Methods  Over a 16-year period, we identified 9 patients with intraventricular meningiomas who were eligible for GKR out of a total management experience of 1,045 patients. The mean patient age was 51 years (range, 14 to 81). Three had radiosurgery for recurrent tumors after prior resection, and GKR was used as an adjunctive after subtotal resection in 1 patient. In the other 5 patients, GKR was used as primary management. Two had a diagnosis of meningioma confirmed by biopsy. The median tumor volume at GKR was 3.9 cc (range, 0.8–11.8). A median margin dose of 16.0 Gy (range, 14.0–22.5) was delivered to the tumor margin. Results  The average follow-up was 64 months. None of the patients developed hydrocephalus or treatment-related morbidity. The progression-free periods after radiosurgery varied from 7 to 160 months (mean, 60). Four tumors regressed and 2 remained unchanged. Three patients showed delayed tumor progression. Meningioma growth control was obtained in 7 out of 9 patients, but 1 patient required two radiosurgical procedures. Conclusions  Gamma knife radiosurgery may be an additional minimally invasive management option for small intraventricular meningiomas in patients who either fail or are unsuitable for resection.     

Intraventricular schwannoma: Case report and review of literature

We report here a case of intraventricular schwannoma. This location is rare. Our patient was a 68-year-old female with a large intraventricular lesion of the body of the lateral ventricle on the right side. Brain magnetic resonance imaging (MRI) revealed this lesion. After a right parietotemporal craniotomy, microsurgical excision using neuronavigation was performed to completely remove the tumor. Histological and immunohistochemical examination confirmed the diagnosis of intraventricular schwannoma devoid of atypical features. Postoperative MRI showed macroscopically complete tumor removal with no recurrence after 12 months of follow-up. A review of the literature identified 32 such cases published to date.     

Tuberous sclerosis with intraventricular tumor: report of 2 cases

It is well known that intraventricular tumors are occasionally seen in patients with tuberous sclerosis. We have experienced two cases of tuberous sclerosis with intraventricular tumor. Case 1: an 8-year-old girl was admitted to our clinic because of headache and vomiting of one month's duration. She had adenoma sebaceum, mental retardation and seizures clinically, and a large tumor was found in the right lateral ventricle by pneumoventriculography. Partial removal of the tumor was performed by the right frontal transcortical approach, but she later died of pneumonia. Necropsy revealed hamartomatous disease characterized by multiple focal tumor-like malformations in various organs including the brain, kidneys, heart, lungs and liver. The histological diagnosis of the intraventricular tumor was subependymal giant cell astrocytoma. Case 2: a 6-year-old boy was admitted to our clinic because of headache of one year's duration. He had also suffered from seizures since 6 months of age. Adenoma sebaceum was noted in the cheeks. CT revealed a medium sized tumor at the right foramen of Monro and the moderately dilated right lateral ventricle and several calcified deposits in the lateral ventricle wall. The tumor was subtotally removed by the right frontal transcortical approach. The pathological examination showed subependymal giant cell astrocytoma. Now 6 years after the operation he is enjoying a normal school life. In our cases, intraventricular tumors associated with tuberous sclerosis were of a typically benign histological appearance, that is subependymal giant cell astrocytoma.(ABSTRACT TRUNCATED AT 250 WORDS)     

A case of metastatic choroid plexus tumor from cholangiocellular carcinoma

Metastatic intraventricular tumor located in the choroid plexus is very rare. Only a few cases have been reported in the past. According to past reports, these tumors originated from lung, colon, and so on, but not from the bile duct. This is the first case report of choroid plexus metastasis from cholangiocellular carcinoma. A 57-year-old woman who had a history of cholagiocellular carcinoma, demonstrated intraventricular tumor. Although sufficient examination was performed, the tumor was difficult to diagnose as being a metastatic tumor or a choroid plexus carcinoma. Because of this, we performed endoscopic biopsy of the intraventricular tumor. However intraoperative findings were not helpful in distinguishing metastatic tumor and choroid plexus carcinoma. Postoperatively, histological examination was performed. However it was still difficult to differentiate this rare tumor from choroid plexus carcinoma by only hematoxylin and eosin stain. For further examination, Ber EP-4 stain was performed. Ber EP-4 showed strongly positive which indicates metastatic tumor. This method led us to make an appropriate diagnosis of this extremely rare tumor. We considered that in order to diagnose this rare tumor, appropriate histopathological examination, including immunohistopathological examination should be performed.     

Central neurocytoma: histopathological variants and therapeutic approaches.

The central neurocytoma has recently been added to the differential diagnosis of intraventricular tumors. Histopathologically, this tumor is characterized by a uniform neoplastic cell population with features of neuronal differentiation. Central neurocytomas occur in young adults, develop in the area of the foramen of Monro, and are usually associated with the septum pellucidum. Initial reports appeared to indicate that these tumors are benign lesions with a favorable postoperative prognosis. The authors present clinical and neuropathological findings in a series of eight patients with central neurocytoma. An anterior transcallosal microneurosurgical approach yielded good outcomes. Postoperative radiation therapy was restricted to two patients with a malignant variant of central neurocytoma and one patient with a recurrent tumor. Observations of anaplastic variants of this neoplasm in two cases and local tumor recurrences in three indicate that the biological behavior and postoperative prognosis of central neurocytoma may not always be as favorable as previously assumed.     

Odontogenic myxoma of nasosinusal localization in a pediatric patient

In the present study we report and discuss a case of odontogenic myxoma in a 13-month-old patient. Only two other reports have been published in the literature describing the occurrence of this type of tumor at an earlier age. Odontogenic myxoma is a benign mesenchymal neoplasm of the maxilla more common between the third and fourth decades of life. The case here reported is exceptional as it presents in an infant and, although some cases have been reported in the literature, this type of tumor is rare in early childhood. The most common locations include the ascending ramus and the angle of the jaw, although these tumors may also affect the upper maxilla, in which case they may involve the maxillary sinus. According to the review of the literature, these tumors show a recurrence rate of about 25%. Consequently, after the intraoperative clinical examination of the lesion and the assessment of the initial suspected diagnosis by means of intraoperative biopsy under general anesthesia, we carried out the resection of the tumor with wide margins at some points of the tumor location.     

Chondroid chordoma of the L5 spinous process and lamina: a case report

Chordoma is a rare bone tumor that originates from the remnants of the notochord. These tumors have axial distribution particularly at the upper and lower ends of the vertebral column. This paper reports a rare occurrence of a chordoma in the posterior elements of the L5 vertebra. A differential diagnosis of a benign tumor (giant cell tumor, aneurysmal bone cyst or osteoblastoma) was made initially. Other differential diagnoses included plasmacytoma and metastasis. The tumor was removed enbloc. Histopathological examination revealed the tumor mass to be chordoma. There were no clinical or radiological signs of recurrence at 21 months follow-up. Chordomas are tumors of the axial skeleton. However, they may occur in unusual sites in ectopic notochordal tissue. The case is being presented for its unusual site of occurrence.