Stereotactic radiosurgery for pineal region tumors.

Our present treatment strategy for treating pineal region tumors is shown in Figure 5. We believe that stereotactic biopsy should be the first procedure in pineal region tumors so that a histologic diagnosis can be obtained. Based on the biopsy findings, the appropriate subsequent therapy, whether microsurgery, fractionated irradiation, or stereotactic radiosurgery, can be administered. Although our experience currently is limited to nine patients, we have found that stereotactic radiosurgery is a valuable alternative to microsurgery in the treatment of selected pineal region tumors.     

Endoscopic surgery for pineal region tumors.

Endoscopic surgery may play an important role in most patients with pineal region tumors. We report our experience with 5 patients treated by a burr hole endoscopic technique. The procedure included in all cases third ventriculostomy for the correction of hydrocephalus, CSF sample for cytology and tumor markers, and tumor biopsy for histological diagnosis. Endoscopic biopsies showed a pineocytoma in two cases, a germinoma in 2 and a low-grade astrocytoma in one. We agree that endoscopic surgery may allow us to select cases requiring a microsurgical approach (medium-sized or large non-germ-cell tumors) from cases to be treated only by irradiation and chemotherapy (germinomas and other non-germ-cell tumors). Then, in some patients with pineal region tumors the endoscopic procedure remains the only surgical treatment. When a direct microsurgical approach is indicated, it may be performed in a non-emergency situation and after correction of the hydrocephalus by endoscopic third ventriculostomy.     

Surgical management of pineal region tumors

Summary The authors advocate an aggressive surgical approach to pineal region tumors to provide a definitive histological diagnosis and to facilitate extensive tumor removal. This strategy is based on their surgical experience in 160 operations for pineal region tumors in which operative mortality was 4% with 3% permanent major morbidity. One-third of pineal tumors were benign for which surgery alone was usually curative. A gross total removal was possible in 31 of 107 malignant tumors. The supracerebellar infratentorial approach was preferred in 86% of patients.The tumors displayed considerable histological diversity with germ cell tumors most common (37%), followd by glial cell tumors (28%) and pineal cell tumors (23%). Mixed tumors occurred 15% of the time. Spinal metastases were rare, occurring in less than 10% of patients with malignant tumors.These results with a large series of pineal region tumors demonstrate the safety and efficacy of aggressive pineal tumor surgery.     

Stereotactic radiosurgery for pineal region tumors

The role of radiosurgery in the management of pineal region tumors is still in its incipient stages, although over the past few years its use has expanded, both as a primary treatment modality and as an adjunct to conventional therapies. This article gives a detailed overview of the recent literature regarding the merits of stereotactic radiosurgery to pineal region tumors, and offers guidelines for the practicing neurosurgeon and neuro-oncologist for the incorporation of radiosurgery into the clinical management of these difficult lesions.     

Management and outcome of pineal region tumors

The management and outcome of 45 patients with tumors in the pineal region are reviewed. The overall male to female ratio was 2:1, and over one-half of the patients presented during the 2nd decade of life. Sixteen of the 21 survivors are presently gainfully employed and suffer only minor deficits. Two of 3 survivors in whom the lesion was biopsied harbored germinomas, while the third had an epidermoid. Among the 18 nonsurvivors, all of whom died of their tumors, 15 succumbed within 2 1/2 years of hospital admission. Twelve of the 13 nonsurvivors in whom histological confirmation was obtained by operation or autopsy had malignant tumors. Computed axial tomography was helpful in making the diagnosis of tumor type in 7 of the 11 cases with confirmed pathology. Cerebrospinal fluid cytology disclosed malignant cells in 4 of 11 patients with proven pineal malignancy. Irradiation and shunting were performed in nearly all survivors and in a little over one-half of the nonsurvivors. Where noninvasive studies are equivocal for malignancy, biopsy may be indicated.     

Principles of treatment of the pineal region tumors

BACKGROUND: A pineal region tumor is an uncommon deep-seated, heterogeneous group of mass lesions of the brain, and the management strategy of any types of these tumors remains controversial. It is the purpose of this communication to present results of our experience in treating patients with these neoplasms. METHODS: From 1976 to 1999 about 700 patients with tumors of the pineal region and posterior third ventricle were managed at the Burdenko Neurosurgery Institute. In more than 330 cases the tumor was removed. In this paper we present results of 287 patients with histologically verified pineal region tumors for the period from 1976 to 1999. All of them had verified tumor histology, excluding only five cases in which stereotactic biopsy procedures were uninformative. There are four main groups of tumors: the germ cell tumors-87 (31%); the pineal parenchymal tumors-75 (27%); the glial tumors-77 (27%); and miscellaneous-43 (15%). There were 255 surgical procedures for tumor removal performed in 244 and stereotactically guided biopsies in 61 patients, 168 (58%) with obstructive hydrocephalus who underwent cerebrospinal fluid shunting. Radiation therapy was administered in 145 (51%) and chemotherapy in 16 patients. Follow-up data were collected in 209 cases (80%). Survival was calculated with the Kaplan-Meier product limit method. RESULTS: The occipital transtentorial and infratentorial supracerebellar approaches were the most commonly used on our series-138 (54%) and 87 (34%), respectively. In the remaining cases, subchoroidal approach, approach through the fourth ventricle, and the combined approaches were used. A total tumor removal was achieved in 148 operations (58%), subtotal in 74 (29%) and partial in 33 (13%). The projected 5-year and 10-year survival rates for patients with malignant pineal tumors, who received irradiation after tumor resection or underwent radiation therapy alone, were: 95% and 88% for pure germinomas, 80% and 50% for high grade gliomas, 44% and 0% for malignant pineal parenchymal tumors, and 20% and 0% for malignant germ cell tumors, respectively. CONCLUSIONS: Benign pineal tumors should be cured with surgery alone. Malignant tumors should be treated with aggressive resection followed with irradiation and chemotherapy. Pure germinomas, which are exquisitely radiosensitive, can be cured by conventional radiation therapy alone.     

Malignant tumors of the anus. A study of 106 cases

This study of 106 cases of malignant tumors of the anus has shown that the diagnosis was often made late. In 15 percent of the patients the tumor was at the anal margin; in 61 percent, in the anal canal, and in 24 percent, the tumor was so extensive that its origin could not be determined clinically. In nearly half of the cases, the cancers were advanced and showed perirectal metastases. Surgical treatment was the rule, with local excision for carcinomas of the anal margin and abdominoperineal resection for tumors of the canal. The general prognosis over a period of several decades remains poor. We found a 20 percent 5 year survival rate for epidermoid carcinoma of the anal canal, excluding deaths from causes other than cancer during the follow-up period.     

幕下小脑上锁孔入路显微手术切除松果体区肿瘤

目的探讨幕下小脑上锁孔入路显微手术切除松果体区肿瘤的可行性和安全性。方法依据术前神经影像学检查结果,制定个体化手术方案．采用经幕下小脑上锁孔入路,显微手术切除病变7例。骨窗大小约2．0 cm×2．5 cm,上缘达横窦和窦汇下缘。结果7例中,胚生殖细胞瘤2例；松果体细胞瘤2例；松果体母细胞瘤1例；胶质瘤1例；胆脂瘤1例。术后均行MRI检查,肿瘤全切除6例,1例次全切除。并发术后小脑肿胀、脑积水1例,再次行手术减压；1例术后出现一过性缄默及凝视；无死亡、感染及术后出血病例。近期随访效果良好。结论应用幕下小脑上锁孔入路显微手术切除松果体区肿瘤不仅可取得满意的切除率,而且手术创伤小、疗效好。     

Management of pineal region tumors in a pediatric case series

Neurosurgical Review - Pineal region tumors commonly present with non-communicating hydrocephalus. These heterogeneous histological entities require different therapeutic regimens. We evaluated our...     

Supracerebellar-infratentorial approach to pineal tumors.

Utilizing the Krause exposure, we have operated on 21 cases of tumors of the pineal region encountering a wide variety of histology with an overall 30% incidence of benign lesions. There has been no significant morbidity in the post-operative management of these patients and one surgical death in a patient with a highly malignant glioma who developed a delayed hemorrhage into the tumor following the operation. In those tumors of recognized malignancy, post-operative radiotherapy has been utilized. In tumors of the germinoma, variety radiation of the entire neural axis is advocated. Since many of the patients have undergone radiotherapy prior to our exposure of the pineal tumor, we have had an opportunity to witness the effects of radiation on this region. In those cases of benign tumors we have found dense adhesions between the tumor and the brain, making definitive surgical treatment of the tumor more difficult.     

幕下小脑上入路显微切除松果体区肿瘤

目的 探讨经幕下小脑上入路(Krause入路)显微外科切除松果体区肿瘤的方法和治疗效果。方法 分析1998年3月至2002年4月收治的松果体区肿瘤8例，其中生殖细胞瘤4例，胶质瘤2例。松果体瘤和松果体囊肿各1例。所有病人均采用坐位、气管内插管全麻、经Krause入路显微肿瘤切除，其中2例术中辅助影像导航定位。结果 除1例胶质瘤与大脑大静脉粘连，残存少许肿瘤外。其余病例均达解剖切除，脑脊液循环通畅，无后遗症状。结论 坐位Krause入路显示三脑室后部病变解剖结构清晰，病变暴露良好，基本能达到病灶全切。     

Novel application of computer-assisted cisternal endoscopy for the biopsy of pineal region tumors: cadaveric study

Summary Background. Long-standing debate continues about the management and biopsy of pineal tumors because of their complex microsurgical anatomy and deep location. Inspired by the concept of biopsy under direct visualization in the absence of hydrocephalus, we explored the effectiveness of neuroendoscope outside of its traditional territory using a new minimally invasive technique, computer-assisted cisternal endoscopy (CACE), for the biopsy of pineal tumors. Method. Five cadaver heads were dissected to expose the pineal region through the posterior fossa. In the other 5 heads, a rigid endoscope-wand combination was introduced in the supracerebellar space lateral to the arachnoid of the superior cerebellar cistern in midline. Endoscopic exposure of the pineal gland was correlated with the real-time image of the localizing wand. After the wand was removed, arachnoid was further dissected from the deep veins and the pineal gland, and a four-quadrant biopsy was obtained. Findings. The combination of technologies of frameless guided stereotaxy and neuroendoscopy enhanced our ability to navigate the ventriculoscope in narrow spaces (e.g., posterior fossa cisterns). Compared with transventricular and conventional stereotactic trajectories, application of CACE in supracerebellar infratentorial trajectory offered the shortest route to the pineal region, anatomical orientation, no violation of eloquent neurovascular structures, and adequate visibility to deep veins and arteries. Conclusions. CACE may be used to approach pineal lesions outside the cerebral ventricular system for biopsy or debulking. Continuous computer updates on the endoscope position allows its safe navigation in narrow spaces (e.g., cerebrospinal fluid cistern). Its success will await future surgical trials.     

Neuro-ophthalmological function of patients with pineal region tumors approached transtentorially in the semisitting position.

To optimize orientation and operative exposure for aggressive resection, the authors approached pineal region tumors transtentorially with the patient in a semisitting position. In the current report, 12 consecutive patients were evaluated to document operative ocular morbidity referable to the brain stem as well as visual deficits secondary to occipital lobe retraction. Before craniotomy, ophthalmological findings related to dorsal midbrain dysfunction were evident in four of the 10 patients who had previously undergone ventricular shunting. The other patients developed a partial or complete Parinaud's syndrome in the early postoperative period and some suffered additional deficits such as cranial nerve palsies. These deficits improved to varying degrees in all patients. Although each had full visual fields preoperatively, an absolute or incomplete left homonymous hemianopsia developed in the immediate postoperative period. Such visual field deficits fully resolved over a variable period of time in 10 of the 12 patients. One patient has a permanent left homonymous hemianopsia, while another has a left homonymous paracentral scotoma. Eight patients were able to return to preoperative pursuits. While ocular abnormalities related to dorsal midbrain dysfunction are most probably independent of operative approach, visual field deficits attributable to occipital lobe retraction were common in patients after a occipital transtentorial approach performed in the semisitting position. Reading difficulties associated with ocular motor dysfunction due to dorsal midbrain involvement represent the principal long-term neuro-ophthalmological complaint of patients who have undergone pineal region surgery.     

Efficacy of neuroendoscopic procedures in minimally invasive preferential management of pineal region tumors: a prospective study

OBJECT: This prospective study is based on a consecutive series of 20 patients with pineal region tumors who underwent minimally invasive preferential management. The purpose of this report is to discuss the role of neuroendoscopic procedures in the management of pineal region tumors. METHODS: If the tumor markers alpha-fetoprotein and human chorionic gonadotropin were not detected in serum and there was significant ventricular dilation visualized on neuroimages, neuroendoscopic surgery was first applied for tumor debulking with tissue diagnosis and gross morphological analysis of the tumor and the intraventricular structures, followed by third ventriculostomy. Subsequent procedures were determined on the basis of verified individual tumors. For treatment of germinomas and pineoblastomas, if no tumor dissemination was confirmed by pre-, intra-, or postoperative findings, stereotactic radiotherapy or radiosurgery was performed after one course of chemotherapy with the ICE regimen (isofomid, cisplatin, and etoposide) and followed by two additional courses of chemotherapy. For treatment of malignant germ cell tumors, after extensive surgery, adjuvant chemotherapy with the ICE regimen was performed in three courses in all cases. Then radiotherapy was started using various methods, depending on the evidence of tumor dissemination. For treatment of teratomatous and neuroectodermal tumors other than pineoblastomas, extensive surgical removal was performed. As for adjuvant therapy, if the tumor was a low-grade glioma or if the patient was younger than 5 years of age, postoperative treatment did not include radiotherapy. If the tumor was a malignant teratoma or high-grade glioma, conventional focal radiotherapy was performed, followed by chemotherapy with ICE for 1 year. All but two treated patients had ventriculomegaly. Neuroendoscopic procedures were performed in six of 15 treated patients. Neuroendoscopic biopsy with tumor debulking offered enough material for tissue diagnosis, including immunohistochemical analysis and, in one case, revealed evidence of tumor dissemination undetectable on neuroimaging. With one exception, no shunt was required in any patient undergoing endoscopic third ventriculostomy. Stereotactic radiotherapy was performed in indicated cases. Favorable therapeutic outcomes were obtained in all cases of germinoma and pineoblastoma, with follow-up periods ranging from 24 months to 6.5 years. CONCLUSIONS: Our minimally invasive preferential regimen clarified the precise indication for neuroendoscopic procedures, and the majority of our patients with dilated ventricles and no evidence of tumor markers were treated satisfactorily with effective neuroendoscopic procedures as the initial procedure, avoiding unnecessary craniotomy and radiotherapy and promising excellent therapeutic outcomes. The treatment for malignant pineal region tumors remains a subject for further study.     

Venous malformation of the pineal region.

A single and perhaps unique case of a venous malformation over the quadrigeminal plate causing acute obstructive hydrocephalus is reported. The lesion was well demonstrated by means of CAT scan but cerebral angiography failed to reveal the vascular nature of this lesion. Complete thrombosis of the lesion was achieved by surgical intervention and the resolution of the lesion was demonstrated by CAT scan. The patient remains asymptomatic and neurologically intact six months after the operation.