You are on page 1of 5

J Neurosurg (Pediatrics 5) 100:437–441, 2004

Initial endoscopic management of pineal region tumors and associated hydrocephalus: clinical series and literature review
BAKHTIAR YAMINI, M.D., DANIEL REFAI, M.D., CHARLES M. RUBIN, M.D., AND DAVID M. FRIM, M.D., PH.D.
Sections of Pediatric Neurosurgery and Pediatric Hematology–Oncology, University of Chicago Children’s Hospital, University of Chicago, Illinois
Object. The authors report their experience in six patients with pineal tumors and associated hydrocephalus who underwent an endoscopic biopsy procedure and third ventriculocisternostomy (ETVC) in a single sitting. Methods. The ETVC was successfully performed without complication in all patients; however, a ventriculoperitoneal shunt was eventually required in four. Histological diagnosis was successfully established in four patients. The authors also reviewed the literature to assess reports involving ETVC and tumor biopsy sampling in patients with pineal tumors and hydrocephalus. A total of 54 cases, including those in this study, have been reported. Fifteen percent of the patients eventually required placement of a ventricular shunt. The transient complication rate was 15% with no death. A positive tissue diagnosis was established in 89% of the cases overall. Conclusions. The authors conclude that the endoscopic management of patients with pineal region masses and hydrocephalus may be a preferred initial strategy.

KEY WORDS • pineal region tumor • endoscopy hydrocephalus

• third ventriculocisternostomy •

anagement of pineal region tumors has changed considerably during the past quarter century. Conservative treatment involving shunt therapy and irradiation in the early 1970s evolved into a more aggressive approach with the advent of microneurosurgery.2,3 More recently, stereotactic and endoscopic technology has added a new dimension to the management of these tumors.1,20 The broad range of treatment modalities for these lesions is due to the diversity of tumor types found in the pineal region as well as the unique location of this structure (Fig. 1).21 The most common presenting signs and symptoms are related to raised ICP with hydrocephalus being present in approximately 90% of cases.6,21 Optimal treatment for a pineal region tumor may be aggressive resection, radiotherapy, chemotherapy, or a combination based on tumor histology.6,15,17 Obtaining an adequate tissue sample for histological diagnosis is an important aspect of treatment planning. Open craniotomy and microsurgical biopsy sampling can achieve this; however, the risk of permanent associated morbidity approaches 10%.6 Stereotactic biopsy sampling is a less invasive technique, but sampling error, due to the hetero-

M

geneous nature of tumors in the region, has been noted to be a significant problem. Additionally, this procedure can carry significant morbidity because of the numerous vascular structures in the vicinity of the pineal gland. Neither of these procedures, however, specifically addresses the hydrocephalus associated with masses in this area. For these reasons, endoscopy is emerging as the preferred initial management of these lesions.20 Patients presenting with hydrocephalus and a posterior third ventricular mass can undergo ETVC and an endoscopic tumor biopsy procedure in one sitting. Several case reports and short series involving this combined approach to pineal region tumors have been reported.4,5,7,8,11,13,14,18–20 We present our experience in treating eight patients presenting with hydrocephalus and a pineal mass. We specifically discuss the use of endoscopy as an initial treatment modality and elaborate on several aspects evident in our study and in the literature. Clinical Material and Methods Patient Population Between 1997 and 2001, 11 patients with pineal region tumors and associated hydrocephalus presented for surgical treatment at the University of Chicago Children’s Hospital. In one patient a VP shunt had been placed at an outside facility and two patients had previously undergone
437

Abbreviations used in this paper: CSF = cerebrospinal fluid; ETVC = endoscopic third ventriculocisternostomy; ICP = intracranial pressure; MR = magnetic resonance; VP = ventriculoperitoneal.

J. Neurosurg: Pediatrics / Volume 100 / May, 2004

In one patient (Case 8) a diagnosis of low-grade astrocytoma was made after endoscopic biopsy sampling several years previously at an outside institution. the scope and sheath are removed. thalamostriate and septal veins. There were five male and three female patients who ranged in age between 1 and 20 years (mean 8. Histological diagnosis was successfully es- TABLE 1 Summary of treatment in eight patients with pineal region masses associated with hydrocephalus* Case No. craniotomy before treatment of the hydrocephalus. The decision to proceed with the endoscopic biopsy procedure as initial treatment was based solely on the presence of a posterior third ventricular mass with dilated ventricles. six underwent ETVC and biopsy sampling and two underwent ETVC alone. The ventricular landmarks including the choroid plexus. The scope is then directed toward the posterior aspect of the third ventricle. 16 yes. Results Of the eight patients treated endoscopically. either an external ventricular drain or a ventricular catheter connected to a subcutaneous reservoir was left in place. 16 no yes. and the tumor is visualized (Fig.5. 11 yes. and foramen of Monro. Endoscopic Technique FIG. 1. Electrocautery is not used during this part of the procedure. et al. Using biting forceps. Eight of the patients with hydrocephalus were initially treated endoscopically.B. Cerebrospinal fluid samples for ␣-fetoprotein and ␤–human chorionic A right-sided precoronal incision and burr hole are initially made at the midpupillary line. Only one patient required emergency ventriculostomy prior to the endoscopic procedure. and the endoscope is advanced into the third ventricle. Sagittal T1-weighted MR image obtained in a patient with a pineal tumor and hydrocephalus. Two patients were lost to follow up after 1 month.6-mm flexible endoscope or a 7-mm rigid endoscope is inserted through the introducer into the lateral ventricle.5 months) (Table 1). 3 French Fogarty balloon catheter. somnolence headache somnolence. EOM palsy headache somnolence headache headache pineoblastoma pineoblastoma pineoblastoma embryonal/ rhabdomyosarcoma teratoma astrocytoma pineoblastoma astrocytoma ETVC/biopsy ETVC/biopsy ETVC/biopsy ETVC ETVC/biopsy ETVC/biopsy ETVC/biopsy ETVC actual dx actual dx astrocytoma aborted normal ependymal actual dx actual dx none no no yes. 2). Spinal MR imaging was performed between 1 and 10 days postoperatively in all patients except two in whom low-grade astrocytoma was diagnosed. The ventriculocisternostomy is made using a blunt probe in the usual location anterior to the mammillary bodies and is then widened using a No.75 years). 19 no 39 1 51 1 3 21 68 20 stable stable stable stable died stable stable stable none none none none none none none none * Dx = diagnosis. Bleeding is controlled using copious irrigation or monopolar cautery. Preoperative Data The presenting signs and symptoms are summarized in Table 1. 438 J. The floor of the third ventricle can often be visualized through the dilated foramen of Monro. and in another patient (Case 4) the biopsy procedure was aborted because of excessive bleeding when the tumor surface was coagulated. When hemostasis is achieved. The followup period ranged from 1 to 68 months (mean 25. In all but three cases. prior to closure of the wound. and one patient died at 3 months. In two patients biopsy specimens were not acquired (Table 1). 1). in six of whom a biopsy sample was obtained in the same sitting. EOM = extraocular muscle. gonadotropin were obtained at the time of initial intraoperative ventricle puncture. two or more biopsy specimens are then obtained from different regions of the mass. Yamini. A 4. 2004 . Each patient underwent preoperative computerized tomography and MR imaging with and without infusion of contrast material (Fig. and an introducer is used to puncture the lateral ventricle. Neurosurg: Pediatrics / Volume 100 / May. The standard landmarks in this region are identified.or 4. are identified. Age (yrs) Presenting Findings Final Pathological Diagnosis VP Shunt (postop day) Last Follow Up (mos) Neurological Deficit Procedure Biopsy Results Condition 1 2 3 4 5 6 7 8 14 3 20 3 8 1 5 16 obtunded headache.

performed 5 years previously. it is unusual to reverse the hydrocephalus microsurgically.18. Prior to this. determined after open craniotomy. and the ventriculoscope has been used for various neurosurgical conditions including hydrocephalus. with approximately a 60 to 80% long-term success rate overall (that is. In 125 patients with hydrocephalus followed for a mean of 28 months. Although ETVC was successful in all patients. This most likely converted the initial obstructive hydrocephalus to an absorptive hydrocephalus due to the release of protein and blood into the CSF. in this and other reports involving large series of ETVC-treated patients. In our series. In Case 3 the endoscopic and final pathological diagnoses differed. in 10 smaller series of patients with pineal tumors and hydrocephalus who underwent endoscopic biopsy sampling and ETVC. in one of our shunt-treated patients.. patients who present with pineal tumors and hydrocephalus are initially managed endoscopically.20 As documented. shunt independence).5 days) after the procedure. examination of the endoscopically obtained tissue showed a low-grade astrocytoma. four of whom harbored pineal region masses. it is generally our policy to monitor ICP with an external ventricular drain or a ventricular catheter and a noninvasive telemonitor. Since then. however.19 In addition to sampling CSF. In the early 1970s. The two patients lost to follow up were seen for 1 month postoperatively and were discharged without hydrocephalus. Discussion In 1973. the results are very good.20 Additionally. although our ETVC technique is very similar to others. only four of 48 patients subsequently required insertion of a VP shunt (Table 2). however.19 We found that the histological diagnosis was correct in four of six patients. 2. Pathological diagnosis. In Case 5. four of the six patients in whom a biopsy specimen was obtained subsequently required early shunt therapy. the incidence of shunt malfunction in this population is as high as 20%. the endoscope was mainly used to coagulate the choroid plexus in the treatment of hydrocephalus. Nevertheless. This 439 FIG. cysts. a subgroup of 33 with triventricular hydrocephalus due to tumor-induced aqueductal compression was associated with a patency rate of almost 85% after third ventriculostomy.11. Neurosurg: Pediatrics / Volume 100 / May.13. Five patients required subsequent craniotomy for tumor resection. two of our shunt-treated patients required placement of the shunt after having undergone craniotomy. endoscopic biopsy sampling of pineal lesions was unpopular because surgeons feared inducing uncontrollable bleeding. and tumors.12 There were nine patients with pineal tumors. was pineoblastoma. biopsy samples were not obtained at the same time. 2004 .12 Currently.Endoscopy in pineal region tumors literature in showing the efficacy of initial endoscopic management in cases in which pineal region masses are associated with hydrocephalus. and clinical outcome at last follow up are summarized in Table 1. however. The second goal in the endoscopic management of these patients is tissue procurement for diagnosis. Diagram of the ETVC and biopsy trajectory. approximately 90% present with hydrocephalus and require placement of a shunt for CSF diversion. There were no deaths directly attributable to the endoscopic procedures and no significant new postoperative neurological deficits were seen. In the series reported by Fukushima. The cause of this high failure rate in our series is unknown but could be due to the occlusion of the ventriculostomy by intraventricular debris formed when these tumors were sampled. in more recent studies. subsequent treatment. in many centers with neurosurgeons who are skilled in endoscopy. The two patients who did not undergo a biopsy procedure did not require placement of a shunt. Subsequent care was rendered at an outside institution. This is very sensitive to changes in ICP and may have encouraged us into a more aggressive approach with respect to subsequent shunt therapy. and in general. In addition. Fukushima. et al. The patient in Case 8 did not undergo a biopsy procedure. but the final pathological diagnosis. Finally. In one of these patients. craniospinal seeding of tumor along the meninges was demonstrated on MR imaging. Third ventriculocisternostomy in patients with aqueductal stenosis may avoid the need for a shunt. unless complete resection is achieved. evaluation of the biopsy sample was not diagnostic. and the ETVC was a repeated procedure because the initial ETVC.9 he obtained biopsy specimens in 21 patients. tablished in four of the six patients (Table 1). a VP shunt was placed in four patients between 11 and 19 days (mean 15. As noted previously. was made in only one of the four patients. adequate techniques have been developed for hemostasis. The first and primary objective is the diversion of CSF.10 provided the first modern description of the use of an endoscope in the treatment of pineal tumors. A correct diagnosis. Our findings are consistent with the J. Additionally. tumor diagnosis has been successful in almost all cases (Table 2). the goal in the endoscopic treatment of pineal tumors is twofold. Nevertheless. had become occluded. As has been demonstrated in a large series of patients with pineal tumors. one attempted biopsy procedure was aborted because excessive bleeding obscured the view after monopolar cautery had been used to coagulate the surface of the tumor.4.

the mortality rate can be as high as 2%. Blond S.16 Although some authors have reported a good overall correct diagnosis rate. it may be best to perform the biopsy procedure by using a combination of endoscopy and frameless stereotaxy. we have refrained from using cautery and instead use copious warm irrigation for hemostasis. 2004 . The small sample size may well have contributed to the misdiagnosis.13. however.] Neurochirurgie 40:3–9. of VP Shunts (%) Complications (%) Ellenbogen & Moores. In reviewing the literature. There was a 15% transient morbidity rate involving either hemorrhage or other transient neurological deficit (Table 2). Although the endoscopic biopsy procedure showed only ependymal wall.. 1997 Robinson & Cohen. 2000 Gangemi. Comments on their risk and implication apropos of 370 cases. Minim Invasive Neurosurg 40: 13–16. This approach has now evolved to the point where it should be considered in the initial management of patients presenting with pineal region masses and associated hydrocephalus.6 The ability to obtain multiple biopsy specimens from different regions of the tumor gives the endoscopic technique a theoretical advantage over the stereotactic technique. subsequent open craniotomy revealed a diagnosis of teratoma. Linggood RM: The management of pineal area tumors: a recent reappraisal. Sakka L. One intraoperative hemorrhage occurred during monopolar coagulation of the tumor surface prior to the biopsy procedure. et al. 1998 Gaab & Schroeder.. as previously noted.5-mm biting forceps. As can be seen from this review. et al: [Endoscopic surgery of third ventricle lesions. Neurosurg: Pediatrics / Volume 100 / May. was one of our earliest cases. The Southampton experience. Our results are not as encouraging as those in the literature. This most likely is due to the small sample size and does not reflect a special referral pattern. the permanent morbidity rate associated with this approach can be as high as 10%. Wilson CB. Ellenbogen RG. Benabid A. References 1. this will be minimized as endoscopic techniques and tools are refined. Hudgins RJ.4. 2001 Pople. where in 44 of the 48 cases in which endoscopic biopsy procedures were performed a histological diagnosis was achieved (Table 2). de Divitiis O: Provision of a neuroendoscopy service. Even in experienced hands.16 others have noted poor results when performing stereotactic biopsy procedures with significant sampling error. 2001 present series total 1 4 4 4 1 4 6 5 1 18 6 54 1 (100) of 1 3 (75) of 4 3 (75) of 4 3 (75) of 4 1 (100) of 1 4 (100) of 4 6 (100) of 6 5 (100) of 5 1 (100) of 1 17 (94) of 18 4 (67) of 6 48 (89) of 54 0 1 1 0 0 0 1 0 0 1 4/6 8 (15) of 54 0 3 (hem. 1997 Ferrer. 2000 Oi. the problem of misdiagnosis also occurs with endoscopic biopsy sampling.6 Additionally. mem = memory. TABLE 2 Summary of data obtained in review of reported cases involving endoscopic biopsy sampling and ETVC for pineal region masses* Authors & Year No.. et al. This most likely is due to the aforementioned reasons.22 There is no report of any permanent morbidity in the literature. Chapman PH. appears to be safe. et al. temp amnesia) 0 8 (15) of 54 temp 0 of 54 permanent * Hem = hemorrhage. however. 1998 Decq. 2000 (Fr) 6. Decq P. the tumor did not penetrate the ependymal wall and could not be seen during the endoscopic procedure. et al: [Stereotaxic biopsies of pineal tumors. 1998 5. in which the biopsy sample was obtained using a 1.] Neurochirurgie 46:286–294.13. Acta Neurochir 134:130–135.21 none was demonstrated in our series. in this case. some tumor types may not require microsurgical resection. Stereotactic biopsy sampling is the other minimally invasive method for establishing diagnosis. There were no significant deficits associated with our endoscopic biopsy procedures. Barat JL. In the other patient.7. increased mem loss & hemiparesis temp) 0 1 (hem) 0 0 0 1 (transient dysphagia & rt hemiparesis) 0 2 (hem. Stein BM: Surgical management of pineal region tumors. Moores LE: Endoscopic management of a pineal and suprasellar germinoma with associated hydrocephalus: technical case report.18–20.14. Bruce JN. Hopefully. J Neurosurg 68:689–697. of Cases Biopsy Definitive for Diagnosis (%) No.5.14. et al. 1997 J. In cases such as this in which there is no clear exophytic mass. Although it is quite likely that the actual correct diagnosis rate is closer to 90%.11.8. although germinomas are one of the most common tumor types found in this region.. In conclusion. evaluation of the tissue was nondiagnostic.B. et al: Pineal region tumors in children. 1980 4. 2001 Haw & Steinbok. 1988 7. Cancer 46:1253–1257.18. et al. Le Guerinel C. 1995 3. Yamini. et al. Of 440 particular note. there were no deaths attributable to the endoscopic procedures. the most recently published data are very encouraging—only one misdiagnosis has been reported since 2000.5. endoscopic biopsy sampling with ETVC has been reported in 54 patients to date and. 1997 de Divitiis. Since then. 1994 (Fr) 2.. temp = temporary. however. J Neurosurg Sci 42:137–143. combined. This problem may be an unavoidable consequence of the nature of the tumor types found in the region of the pineal gland.19 The gold standard for establishing a diagnosis is open craniotomy. Edwards MS.

2001 15. 1983 16. 2003. Chicago. J Neurosurg 93: 245–253. M. 1996 17. Childs Nerv Syst 8:332–336. 1992 18. Pediatr Neurosurg 34:215–217. Clin Neurosurg 39:509–532. Maiuri F. Br J Neurosurg 15:305–311. Frim DM: Endoscopic approach to noncommunicating fluid spaces in the shunted patient. Accepted in final form November 3. Hirakawa K. Fukushima T: Endoscopic biopsy of intraventricular tumors with the use of a ventriculofiberscope. Matsumoto S: Controversy pertaining to therapeutic modalities for tumors of the pineal region: a worldwide survey of different patient populations. 1997 21. Section of Neurosurgery. Minim Invasive Neurosurg 42:128–132. Address reprint requests to: Bakhtiar Yamini. J Neurosurg 38:251–256. Minim Invasive Neurosurg 44:70–73. Stein BM. Neurosurg: Pediatrics / Volume 100 / May. Goumnerova LC..uchicago. J Neurosurg 58:654–665. 2001 20. Tominaga J. Pagenstecher A. email: byamini@surgery. Fukushima T. et al: The role of endoscopic biopsy and third ventriculostomy in the management of pineal region tumours.D. Shibata M. 2001 14. et al: Endoscopic third ventriculostomy for hydrocephalus. et al: Stereotactic management of lesions of the pineal region. Pediatr Neurosurg 31:237–241. et al: Efficacy of neuroendoscopic procedures in minimally invasive preferential management of pineal region tumors: a prospective study. Neurosurgery 2: 110–113. Gangemi M. Athanasiou TC. Schatz CR. Robinson S. Sandeman DR. 1973 11. 1999 Manuscript received October 16. 1997 9. Gaab MR. Pople IK. Donati P. Oi S. Garcia-Fructuoso G. Technical note. MC 4066. Neurosurgery 39: 280–291. et al: Neuroendoscopic management of pineal region tumours. 1992 22. Acta Neurochir 139:12–21. Oi S. Colella G. Steinbok P: Ventriculoscope tract recurrence after endoscopic biopsy of pineal germinoma. Ferrer E. University of Chicago Children’s Hospital. Gangemi M. Maiuri F. Kendall BE: Diagnosis and management of pineal tumors. 1998 12. Santamarta D. Haw C. 2003. et al: Ventriculofiberscope: a new technique for endoscopic diagnosis and operation. J. 2004 441 . Jooma R.edu. Surg Neurol 48:360–367. 1999 13. 2000 19. Bruce JN: Surgical management of pineal region tumors (honored guest lecture). Cohen AR: The role of neuroendoscopy in the treatment of pineal region tumors. et al: Endoscopic surgery for pineal region tumors. Illinois 60637. 1978 10. Yamini B. Schroeder HW: Neuroendoscopic approach to intraventricular lesions. Ishijima B. 5841 South Maryland Avenue. Kreth FW. J Neurosurg 88:496–505.bsd.Endoscopy in pineal region tumors 8.