Result treatment of pineal region tumors on 45 patients with the infratentorial supracerebellar approach

Nội dung text: Result treatment of pineal region tumors on 45 patients with the infratentorial supracerebellar approach

1. Journal of military pharmaco-medicine n o2-2018 RESULT TREATMENT OF PINEAL REGION TUMORS ON 45 PATIENTS WITH THE INFRATENTORIAL SUPRACEREBELLAR APPROACH Nguyen Van Hung*; Dong Van He** SUMMARY Objectives: A pineal region tumor is an uncommon deepseated, heterogeneous group of mass lesions of the brain, the pineal region is the origin of lesions with a highly diverse histopathology. The purpose of this communication is to present results of our experience in treating patients with these neoplasms. Subjects and methods: From 2010 to 2016, 112 patients were operated at Department of Neurosurgery, Vietduc Hospital. The tumors were removed in 112 cases. In this paper, we present results of microsurgery on 45 patients with pineal region lesions who were operated by infratentorial supracerebellar (ITSC) approach. On the basis of the MRI and CT, we divided all pineal tumors into five groups: 1) Small tumors [less than 2.5 cm in diameter] mainly located in the quadrigeminal cistern; 2) Midsized tumors (4 cm in diameter) located in both the posterior part of the third ventricle and quadrigeminal cistern; 3) Large tumors (4 cm in diameter) occupied the pineal region and expanded into lateral ventricle in some cases; 4) Giant tumors (6 cm in diameter) made up nearly the whole third ventricle, expanding into the fourth and lateral ventricles. Results: In 45 patients, a significant obstructive hydrocephalus was diagnosed preoperatively and a ventriculoperitoneal (VP) shunt was seen in 12 patients (26.67%) and 43 patients (73.33%) with endoscopic third ventriculostomy were performed 1 month before. Total tumor removal was achieved in 18 patients (40%), subtotal removal in 23 patients (51.11%) and partial removal in 4 patients (8.89%). Pathohistological examination revealed 11 pineocytomas (24.44%), 9 germinomas (20%), 2 pineoblastomas (4.44%), 7 astrocytomas (15.56%) and 3 astrocytompilocytic, 3 epidermoid cysts, 3 yolk sack tumors. Conclusions: The infratentorial supracerebellar approach provides a direct midline view to the pineal region between the dorsal cerebellum and tentorium. These results confirm that surgery in the pineal region, especially using infratentorial supracecebellar approach, can be accomplished with relatively low morbidity and mortality. Benign pineal tumors should be cured with surgery alone. Malignant tumors should be treated with aggressive resection followed with irradiation and chemotherapy. * Keywords: Pineal region tumors; Infratentorial supracerebellar approach. INTRODUCTION brain tumors and these tumors are Pineal region lesions are rare and approximately 10 times more common in uncommon deepseated tumors of the children than in adults and constitute 3% brain occupying 0.4% to 1% of adult to 11% of childhood brain tumors [1, 2]. * 103 Military Hospital ** Vietduc Hospital Corresponding author: Nguyen Van Hung (hungpttk103vn@gmail.com) Date received: 01/09/2017 Date accepted: 18/01/2018 148
2. Journal of military pharmaco-medicine n o2-2018 Pineal tumors are a heterogeneous group SUBJECTS AND METHODS of mass lesions originating in or located 1. Subjects. adjacent to the pineal gland and represent From 2010 to 2016, 112 patients were a spectrum of neoplasms ranging from operated at Department of Neurosurgery, benign to malignant. The three major Vietduc Hospital. The tumor was removed categories include germ cell tumors, in 112 cases. In this paper, we present pineal parenchymal cell tumors and the treatment results of 45 patients with supporting tissue neoplasms [2]. pineal region lesions who were operated Before the microsurgical techniques, by infratentorial supracerebellar most authors advocated surgical approach. conservatism because of an operative 2. Methods. mortality from 30 to 70% and morbidity of * Study design: up to 65% after surgery [4, 5, 6, 7, 8]. Recent advances in neuroradiology CT, - A retrospective clinical evaluation of patients with pineal region tumors was MRI, microneurosurgical techniques, performed. modern neuroanesthesia and postoperative intensive care have led to - A prospective study with surgical more encouraging surgical results. Now procedure: Microsurgery of pineal region surgical tumors are removed with tumors was performed by infratentorial microsurgery and fractionated radiation supracerebellar approach. Infratentorial therapy, which allows an overall mortality supracerebellar approach is a midline of less than 5% and a 5-year survival rate incision made extending down to of 60 to 75%, the mortality of direct approximately C 2 and up to the region of the lambdoid suture, above the external surgery has been reduced to under 5% occipital protuberance. The craniotomy and morbidity rate down to a minimal level should extend just above the inferior [9, 10, 11]. Outcome analysis of surgical margin of lateral sinuses and the torcular. strategies for pineal lesions are hindered A bilateral symmetric craniotomy with a by the small number of patients and the free bone flap is performed above the retrospective design of nearly all studies foramen magnum rim. After semioval [4, 9]. incision of the dura below the lateral We report the surgical treatment outcome sinuses is made, some bridging veins for 45 patients with pineal region lesions between the cerebellum and the tentorium who have been applied infratentorial are cauterized and divided. With the supracerebellar (ITSC) approach alone. patient in the sitting position, the We have chosen an open surgical approach cerebellum drops down by gravity and in order to achieve the pathohistologically provides 1 to 1.5 cm of space between its exact diagnosis and possible total lesion surface and the tentorium. This allows removal. a relatively easy access over the 149
3. Journal of military pharmaco-medicine n o2-2018 superior surface of one or both cerebellar tumor compression or infiltration of the hemispheres to the pineal region midbrain, the third ventricle walls and the (figure 1 ). hypothalamus as impaired oculomotor function such as upward gaze palsy and other variations of Parinaud’s syndrome - Preoperative diagnostic studies: An analysis of the MRI and CT with pathologically proven pineal region tumors was performed, focusing on the relationship between the tumor and adjacent anatomic structures, the tumor size and its predominant expansion. On the basis of these data, we divided all pineal tumors into four groups: 1) Small tumors [less than 2.5 cm in diameter] was mainly located in the quadrigeminal cistern; 2) Midsized tumors (2.5 - 4.0 cm in diameter) was sited in both the posterior part of the third ventricle and quadrigeminal cistern; 3) Large tumors (4 - 6 cm in diameter) occupied the pineal region and expanded into lateral ventricle in some cases; 4) Giant tumors (diameter > 6 cm) made up nearly the whole third ventricle, expanding into the fourth and lateral ventricles. - Postoperative complications: Bleeding in the subdural or epidural effusions, inflammation (meningoencephalitis), significant risk of the oculomotor Figure 1: Pineal region tumor and dysfunction An assessment of the infratentorial supracerebellar approach. quality of life of all patient groups with a * Assessment of results: mean Karnofsky performance status score. - Preoperative clinical signs: The clinical * Statistical analysis: All statistical manifestations of the pineal tumors were analyses were performed using SPSS attributable to a number of phenomena, 16.0 software for Windows (SPSS, Inc., including increased intracranial pressure Chicago IL). A p-value < 0.05 was because of obstructive hydrocephalus, considered to be statistically significant. 150
4. Journal of military pharmaco-medicine n o2-2018 RESULTS AND DISCUSSION treated by endoscopic third ventriculostomy (ETV). 1. Demographic characteristics of the patients. 3. Preoperative diagnostic studies. There were 21 male and 24 female Preoperative diagnostic evaluation patients. Median age was 24.4 years consisted of a medical history, physical (5 - 65 years). Mean follow-up was 26 examination, detailed neurological months (4 - 48 months). examination and neuroradiological studies. Table 1: Types of pineal region tumors. CT and MRI of the brain revealed information about tumor localization and Tumor type Male Female Total extension. CT-scans were obtained for 26 Germinoma 8 1 9 out of the 45 patients. MRI scans were performed in 45 patients. We divided all Pineocytoma 4 7 11 pineal tumors into five groups: Small Pineoblastoma 2 0 2 tumors with diameter ≤ 2.5 cm: 1 case Astrocytoma 3 4 7 (2.22%); midsized tumors 2.5 - 4 cm in Pilocytic 1 2 3 diameter: 19 cases (42.22%); large tumors 4 - 6 cm in diameter: 22 cases Epidermoid cyst 0 3 3 (48.89%); giant tumors: > 6 cm in Yolk-sac tumor 2 1 3 diameter: 03 cases (6.67%). The Menengimas 3 4 7 maximum tumor size varied from 24 to 97 mm (median 42 mm). MRI showed the Total 21 24 45 exact diagnosis of the localization of 2. Preoperative clinical signs. tumors and differentiation among blood, CSF, fat and calcium within tumors. Also, The most important clinical symptoms MR angiography provided important were related to the effects of increased information about the very complex deep intracranial pressure. Obstructive venous system in pineal region [11]. hydrocephalus was detected in 45 patients (100%), headache was present in all 4. Surgical treatment. patients (100%), vomiting was encountered Several operative approaches to the in 40 patients (88.89%). Blurred vision pineal region are available. Two approaches was present in 21 patients (46.67%). are rarely used, which are Dandy's Parinaud's syndrome was observed in transcallosal approach and Van Wagenen's 12 patients (26.67%), seizure was present transcortical transventricular approach in 18 patients (40%), diplopia was seen in [10, 11]. Nowadays, two approaches 12 patients (26.67%). Glasgow coma to the pineal region are widely used. score (GCS) 7 - 9 was achieved in They are Poppen-Yasargil occipital 4 patients (8.69%) at admission due to interhemispheric approach and Krause- acute hydrocephalus. These patients Stein infratentorial supracerebellar approach came to hospital by emergency and were [12]. The optimal approach for a specific 151
5. Journal of military pharmaco-medicine n o2-2018 patient depends on the anatomical features 5. Pathohistology. of the tumor. Also, the surgeon’s level of According to the classification of World experience and comfort with each approach Health Organization, tumors in this series should be considered. are divided into four main groups: The surgical removal of the tumor and germ cell tumors, pineal parenchymal opening the posterior third ventricle can tumors, glial tumor, and miscellaneous. relieve the obstructive hydrocephalus. Pathohistological examination revealed The infratentorial supracerebellar 11 pineocytomas (24.44%), 9 germinomas approach was used in all patients in a (20%), 2 pineoblastomas (4.44%), sitting position, supine position. The surgical 7 astrocytomas (15.56%), and procedure was done as described [8, 10]. 3 astrocytompilocytic, 3 epidermoid cysts, Although it has been reported that the sitting position is no longer in use due to 3 yolk sack tumors. The histological the high incidence of venous air diagnosis was summarized in table 1. embolism, we have maximally reduced Patients with malignant pineal lesions the possibility of venous air embolism (germinomas, pineoblastomas, yolk sac following the standardized [8]. tumor ) underwent irradiation and/or In 45 patients, a significant obstructive chemotherapy according to standardized hydrocephalus was diagnosed preoperatively protocol [7, 9]. All benign lesions were and a ventriculoperitoneal shunt was completely removed. seen in 12 patients (26.67%) and 6. Surgical results, complications 33 patients (73.33%) were performed and outcome. endoscopic third ventriculostomy 1 month before. To date, performing the endoscopic The postoperative survival was 93.33%, third ventriculostomy seems to be the postoperative mortality accounted for best option in patients with obstructive 6.67%. After 3.6 months, KPS I + II of hydrocephalus [12]. survival patients was good. 46.32% of The extent of lesion removal was cases were completely cured by MRI or graded as total, subtotal and partial. Total CT-scan. There were 3 cases of tumor removal was achieved in 18 patients perioperative mortality (6.67%). (40%), subtotal in 23 patients (51.11%) Overall mortality was seen in and partial in 4 patients (8.89%). Totally 9 patients (20%) during follow-up care removed tumors in our series were from 38.7 ± 11.6 months. 9 patients died, pineocytomas and symptomatic pineal among which four patients with germinomas cyst. The number of benign pineal cysts is and two patients with pineoblastomas, two increasing due to improved neuroradiological diagnostic methods [8, 10]. In our study, patients with Yolk-sac tumor, and patients with pineal cysts who were developed astrocytomas. operated had a median cyst size of 20 x Three patients had postoperative seizures 15 mm and persistent symptoms. that were couped with antiepileptic drugs. 152
6. Journal of military pharmaco-medicine n o2-2018 Four patients experienced meningitis and 2. Chan H.S, Humpreys R.P, Hendrick E.B were treated with systemic antibiotic et al. Primary intracranial choriocarcinoma. A administration. There were three patients report of two cases and review of the literature. Neurosurgery. 1984, 15, pp.540-545. with a cerebrospinal fluid fistula who 3. Cumins F.M, Taveras J.M, Schlesinger needed wound revision and plastic of the E.B. Treatment of gliomas of the third ventricle dura. Other complications included and pinealomas. With special reference to the 8 hemiparesis and 3 diabetes insipidus. value of radiotherapy. Neurology. 1960, 10, Postoperative sequelae included vision pp.1031-1036. disorders (26 patients = 57.78%), double 4. Dandy W.E. Operative experience in vision (17 patients = 37.78%). cases of pineal tumor. Arch Surg. 1936, 33, pp.19-46. CONCLUSIONS 5. Poppen J.L, Marino R Jr. Pinealomas and tumors of the posterior portion of the third The infratentorial supracerebellar ventricle. J Neurosurg. 1968, 28, pp.357-364. approach provides a direct midline view to 6. Jenkin R.D.T, Simpson W.J.K, Keen the pineal region between the dorsal C.W. Pineal and suprasellar germinomas. cerebellum and tentorium. Its main Results of radiation treatment. J Neurosurg. disadvantage is hard-to-access to larger 1978, 48, pp.99-107. tumors and tumors with lateral extension. 7. Obrador S, Soto M, Gutierrez Diaz J.A. These results confirm other literature Surgical management of tumors of the pineal reports that surgery in the pineal region. Acta Neuro-Chir. 1976, 34, pp.159-171. region, especially using infratentorial 8. Torkildsen A. Should extirpation be supracecebellar approach, can be attempted in cases of neoplasm in or near the accomplished with relatively low morbidity third ventricle of the brain? Experience with a palliative method. J Neuro-Surg. 1948, 5, and mortality. pp.249-275. Benign pineal tumors should be cured 9. Wara W.M, Fellows C.F et al. Radiation with surgery alone. Malignant tumors therapy for pineal tumors and suprasellar should be treated with aggressive germinomas. Radiology. 1977, 124, pp.221-223. resection followed with irradiation and 10. Bruce, Stein. Neurosurgical Clinical. chemotherapy. Pure germinomas, which 1990, 1, pp.123-127. are exquisitely radiosensitive can be cured 11. Lupret V, Jeliai I. Approach problems in by conventional radiation therapy alone. anterior third ventricle tumors: Review of 33 Cases. Surgery in and around the brain stem REFERENCES and the third ventricle (Springer Verlag, Berlin 1. Abay E.O, Laws E.R, Grand G.L et al . Heidelberg. 1986. Pineal tumors in children and adolescents. J 12. Jadik S, Wissing H, Fried Rich K et Neurosurg. 1981, 55, pp.889-895. al,.Neurosurgery. 2009, 64, p.533. 153