Pineoblastoma en el adulto. A propósito de un caso

Fundamento: Los pineoblastomas son tumores cerebrales infrecuentes dentro del grupo de los tumores primitivos neuroectodérmicos. La presentación clínica por lo general está relacionada con la obstrucción del acueducto de Silvio, lo que ocasiona hidrocefalia e hipertensión intracraneal. Objetivo: Describir las características clínicas e imagenológicas de un paciente adulto con diagnóstico de pineoblastoma. Presentación de caso: Masculino, 33 años, que comenzó con disminución de la agudeza visual que evolucionó a la amaurosis bilateral y trastorno de la marcha. Se diagnosticó hidrocefalia obstructiva triventricular, por lo cual se realizó tercer ventriculostomía endoscópica. Se decidió un abordaje supracerebeloso infratentorial a la lesión tumoral, con apoyo endoscópico en posición semisentado; se logró la resección parcial. En estudio inmunohistoquímico se encontró Ki-67 mayor de 50 % de las células, neurofilamento y sinaptofisina positiva. Se concluyó como pineoblastoma. Recibió radioterapia convencional con Co-60, fraccionada, con un total de 30 dosis diarias de 2 Gy. La evolución del paciente a los 6 meses de operado fue satisfactoria. Conclusiones: El tratamiento combinado de cirugía y radioterapia constituye un esquema terapéutico ideal en este tipo de lesiones tumorales. La endoscopia es una herramienta de gran valor en los abordajes quirúrgicos a la región pineal.

Background: Pineoblastomas are infrequent brain tumors among the neuroectodermal primitive tumors. Clinical presentation is usually related to Silvian aqueduct obstruction, which causes hydrocephalus and intracranial hypertension. Objective: To describe the clinical and imagenological characteristics in an adult patient diagnosed with pineoblastoma. Case presentation: Male, 33 years old, who started with visual acuity decline that evolved to bilateral amaurosis and gait disorder. Triventricular obstructive hydrocephalus was diagnosed, therefore, an endoscopic third ventriculostomy was realized. It was decided to use an infratentorial supracerebellar approach to the tumor lesion, with endoscopic support in semi-seated position; partial resection was achieved. In immunohistochemical study, Ki-67 was found to be higher than 50% of the cells, neurofilament and synaptophysin positive. It was concluded as pineoblastoma. He received fractionated Co-60 conventional radiotherapy, with a total of 30 daily doses, 2 Gy. The evolution of the patient 6 months after surgery was satisfactory. Conclusions: The combined treatment of surgery and radiotherapy constitutes an ideal therapeutic scheme in this type of tumor lesions. Endoscopy is a tool of great value in surgical approaches to the pineal region.

Papillary Tumor of the Pineal Region:Report of One Case / 中国医学科学院学报

We report a case of papillary tumor in the pineal region.The imaging findings mainly included:(1)slight high density on CT images;(2)short T1 signal,cystic changes,and evident enhancement on magnetic resonance imaging.

Papillary tumor in pineal region: Immunohistochemistry meeting the diagnostic challenge

Papillary tumor of pineal region (PTPR) is extremely rare and poses diagnostic challenge with other central nervous system tumors having papillary architecture. Immunohistochemistry is crucial for a definitive diagnosis of PTPR.

Resección microquirúrgica de meningioma de la región pineal / Microsurgical resection of meningioma of the pineal region

Introducción: Los meningiomas de la región pineal son lesiones poco frecuentes, representando el 2 al 8% de los tumores localizados en esta área. Estos tumores son lesiones con comportamiento biológicamente benigno y potencialmente curables con la resección quirúrgica radical. A su vez representan un desafío quirúrgico debido a su profunda localización y la cercanía de estructuras anatómicas vitales. El abordaje supracerebeloso infratentorial, en posición semisentado (cuando las condiciones generales del paciente lo permiten), provee un corredor natural a la región pineal mediante la retracción cerebelosa gravitatoria. Objetivo: Presentación de la exéresis microquirúrgica completa de un meningioma de la región pineal. Descripción del caso: Paciente de 52 años que consulta por inestabilidad de la marcha y diplopía de 2 semanas de evolución. La resonancia magnética cerebral mostró una lesión expansiva, que realza con contraste, de 43 x 30 mm en la región pineal, con hidrocefalia obstructiva acompañante. Se realiza la exéresis microquirúrgica mediante abordaje supracerebeloso infratentorial con el paciente en posición semisentado. Resultado: Se realizó la exéresis completa de la lesión. La paciente cursó el primer día postoperatorio en Unidad de Terapia Intensiva y luego pasó a sala de internación general. Fue dada de alta al tercer día, sin déficit neurológico. La anatomía patológica de la lesión informó meningioma transicional (OMS grado I). El control imagenológico postquirúrgico mostró la resección de la lesión sin remanente tumoral. Conclusión: Los meningiomas de la región pineal presentan un desafío quirúrgico debido a la relación con estructuras anatómicas vitales. El abordaje supracerebeloso infratentorial ofrece un corredor anatómico natural mediante la retracción cerebelosa por la gravedad.

Background: Meningiomas of the pineal region are rare lesions representing 2 to 8% of the tumors located in this area. These tumors are lesions with biologically benign behavior and potentially curable by radical surgical resection. As well, they are a surgical challenge due to their deep location and the proximity to vital anatomical structures. The supracerebellar infratentorial approach, in a semisitting position (when the patient's general conditions allow it), provides a natural corridor to the pineal region through gravitational cerebellar retraction. Objetive: Presentation of a complete microsurgical resection of a meningioma of the pineal region. Case description: A 52-year-old patient who consulted due to gait instability and diplopia of 2 weeks of evolution. Brain magnetic resonance showed an expansive lesion, with contrast enhancement, 43 x 30 mm in the pineal region, with obstructive hydrocephalus. The microsurgical resection was performed by supracerebellar infratentorial approach with the patient in a semisitting position. Result: The complete microsurgical resection was achieved. The patient spent the first postoperative day in Intensive Care Unit, and then went to general room. He was discharged on the third postoperative day, without neurological deficit. The histopathology assessment of the lesion reported transitional meningioma (WHO grade I). Post-surgical imaging showed the resection of the lesion without remaining tumor. Conclusion: Meningiomas of the pineal region present a surgical challenge due to the relationship with vital anatomical structures. The supracerebellar infratentorial approach offers a natural anatomical corridor through cerebellar retraction by gravity.

Mature teratoma of the pineal region in the paediatric age group: A case report and review of the literature

@#Introduction: Intracranial teratomas account for 0.5% of all intracranial tumours and 2-4% of intracranial tumours in children. However, in terms of tumours of the pineal area, the exact incidence is not ascertained. Although, it is noted that 50-60% of central nervous system (CNS) germ cell tumours are found in the pineal gland. The degree of difficulty in the sampling of lesions in the pineal gland during biopsy emphasizes the importance of correlating the imaging studies, histopathologic findings, and serum and cerebrospinal fluid (CSF) tumour markers. Case Report: This case report is that of a 9-year-old male who presented with frontal headache of eight days, with associated photophobia, nausea and vomiting, and diplopia. Biopsy with intraoperative navigation was done and the specimen was referred for histopathologic evaluation. The biopsy showed findings consistent with a mature teratoma with no histologic findings of an immature component or secondary somatic malignancy. Comparison of the pre-operative and post-operative multiaxial cranial CT scan showed findings that was consistent with a residual lesion. This was correlated with the pre-operative serum tumour markers which showed alpha-fetoprotein of 22.5 ng/mL and beta-HCG of 1.0 mIU/mL(IU/L), and the post-operative tumour markers of the cerebrospinal fluid that showed alpha-fetoprotein of 3.28 ng/mL and beta-HCG of 18.9 mIU/mL (IU/L). Conclusion: A review of the literature and comparison with current case in relation to the histopathologic, serum and CSF findings, and imaging studies was done to better understand the mechanism of this lesion.

Surgical Treatment of Cavernous Angiomas in the Pineal Gland ­ Case Report

Pineal cavernous angioma is a vascular malformation that has a prevalence lower than 1%. The etiology is debated. It is believed that it is originated from an autosomal dominant inheritance or from radiotherapeutic treatment. Complete resection enables the cure and prevents complications of the natural evolution of the lesion, mainly hemorrhagic events. A female patient, with 67 years of age, presented mental confusion and visual acuity deficit, which evolved to periods of psychomotor agitation. A magnetic resonance imaging (MRI) scan of the head showed a lobulated mass lesion in the pineal region with hypersignal on T1 and hyposignal on the susceptibility weighted imaging (SWI) sequence. Hydrocephalus was also noticed. The patient underwent a microsurgery for complete lesion resection though a supracerebellar/ supratentorial access. The anatomopathology revealed an arteriovenous malformation compatible with cavernous angioma. The pineal cavernous angioma is a rare malformation that should be considered in the differential diagnosis of expansive lesions of the pineal gland.

“Somewhere up there”: A case Of pineal gland tumor in a 20-year-old male

@#<p style="text-align: justify;"><strong>BACKGROUND:</strong> Pineal region tumor is a rare and reportable case. Incidence rate adults is 0.025 in 10,000 hence there is no established guidelines among adults  for diagnosis and management of this case.<br /><strong>CASE:</strong> A case of a 20-year-old male with a two-month history of  intermittent  headache,  occipital  area  with  VAS  5/10,increasing in severity. Until two days prior to admission with severe headache VAS 9-10/10, occipital, and nonradiating.Patient  noted  episodes  of  projectile  vomiting  hence,admitted. Patient presented with non-lateralizing symptoms but  noted  papilledema  and  parinaud  syndrome.Cranial  MRI with contrast revealed a 2.5cm pineal gland tumor with obstructive  hydrocephalus.  Serum  AFP  (alpha-fetoprotein  )  and  beta-HCG  (beta subunit of human chorionic gonadotropin) were requested and revealed elevated levels.The patient underwent endoscopic third ventriculostomy but no biopsy was done due to high risk of bleeding. Patient underwent series of radiotherapy and was advised to undergo chemotherapy but patient refused. Patient had improved upward gaze but with residuals, no recurrence of headache or vomiting, had normalization of the serum tumor markers but noted increase in size of the tumor despite radiotherapy.<br /><strong>CONCLUSION:</strong> Case  reports  of  pineal  region  tumors  will  help doctors  in  the  primary  hospitals  diagnose  such  cases  and differentiate it from benign causes of headache. This will aid in early referral to specialists and early intervention.</p>

"Somewhere up there": A case of pineal gland tumor in a 20-year-old male

BACKGROUND: Pineal region tumor is a rare and reportable case. Incidence rate adults is 0.025 in 10,000 hence there is no established guidelines among adults  for diagnosis and management of this case.CASE: A case of a 20-year-old male with a two-month history of  intermittent  headache,  occipital  area  with  VAS  5/10,increasing in severity. Until two days prior to admission with severe headache VAS 9-10/10, occipital, and nonradiating.Patient  noted  episodes  of  projectile  vomiting  hence,admitted. Patient presented with non-lateralizing symptoms but  noted  papilledema  and  parinaud  syndrome.Cranial  MRI with contrast revealed a 2.5cm pineal gland tumor with obstructive  hydrocephalus.  Serum  AFP  (alpha-fetoprotein  )  and  beta-HCG  (beta subunit of human chorionic gonadotropin) were requested and revealed elevated levels.The patient underwent endoscopic third ventriculostomy but no biopsy was done due to high risk of bleeding. Patient underwent series of radiotherapy and was advised to undergo chemotherapy but patient refused. Patient had improved upward gaze but with residuals, no recurrence of headache or vomiting, had normalization of the serum tumor markers but noted increase in size of the tumor despite radiotherapy.CONCLUSION: Case  reports  of  pineal  region  tumors  will  help doctors  in  the  primary  hospitals  diagnose  such  cases  and differentiate it from benign causes of headache. This will aid in early referral to specialists and early intervention.

"Somewhere up there": A case of pineal gland tumor in a 20-year-old male

BACKGROUND: Pineal region tumor is a rare and reportable case. Incidence rate adults is 0.025 in 10,000 hence there is no established guidelines among adults  for diagnosis and management of this case.CASE: A case of a 20-year-old male with a two-month history of  intermittent  headache,  occipital  area  with  VAS  5/10,increasing in severity. Until two days prior to admission with severe headache VAS 9-10/10, occipital, and nonradiating.Patient  noted  episodes  of  projectile  vomiting  hence,admitted. Patient presented with non-lateralizing symptoms but  noted  papilledema  and  parinaud  syndrome.Cranial  MRI with contrast revealed a 2.5cm pineal gland tumor with obstructive  hydrocephalus.  Serum  AFP  (alpha-fetoprotein  )  and  beta-HCG  (beta subunit of human chorionic gonadotropin) were requested and revealed elevated levels.The patient underwent endoscopic third ventriculostomy but no biopsy was done due to high risk of bleeding. Patient underwent series of radiotherapy and was advised to undergo chemotherapy but patient refused. Patient had improved upward gaze but with residuals, no recurrence of headache or vomiting, had normalization of the serum tumor markers but noted increase in size of the tumor despite radiotherapy.CONCLUSION: Case  reports  of  pineal  region  tumors  will  help doctors  in  the  primary  hospitals  diagnose  such  cases  and differentiate it from benign causes of headache. This will aid in early referral to specialists and early intervention.

Glioblastoma Multiforme in the Pineal Region with Leptomeningeal Dissemination and Lumbar Metastasis

We report a case of a 31-year-old woman with glioblastoma multiforme (GBM) in the pineal region with associated leptomeningeal dissemination and lumbar metastasis. The patient presented with severe headache and vomiting. Magnetic resonance imaging (MRI) of the brain showed a heterogeneously enhanced tumor in the pineal region with obstructive hydrocephalus. After an urgent ventricular-peritoneal shunt, she was treated by subtotal resection and chemotherapy concomitant with radiotherapy. Two months after surgery, MRI showed no changes in the residual tumor but leptomeningeal dissemination surrounding the brainstem. One month later, she exhibited severe lumbago and bilateral leg pain. Thoracico-lumbar MRI showed drop like metastasis in the lumbar region. Finally she died five months after the initial diagnosis. Neurosurgeons should pay attention to GBM in the pineal region, not only as an important differential diagnosis among the pineal tumors, but due to the aggressive features of leptomeningeal dissemination and spinal metastasis.

Abordaje supracerebeloso infratentorial a la región pineal. técnica, indicaciones y anatomía microquirúrgica en 3D / Supra-adventitious approach to the pineal región. technique, indications and 3D microsurgical anatomy

INTRODUCCIÓN: el abordaje supracerebeloso infratentorial fue descripto originalmente por Sir Victor Horsley, siendo posteriormente adaptado por Stein quien aplica la técnica microquirúrgica mejorando los resultados de las cirugías de la región pineal. OBJETIVOS: resaltar y sistematizar las indicaciones, detalles técnicos-anatómicos en el abordaje supracerebeloso infratentorial en base a nuestra experiencia quirúrgica y la revisión de la anatomía microquirúrgica de la región Pineal. MATERIAL Y MÉTODOS: se realizó un estudio descriptivo retrospectivo analizando las historias clínicas de 7 pacientes intervenidos quirúrgicamente a través de un abordaje supracerebeloso infratentorial, entre septiembre 2010 hasta septiembre 2013 en nuestro servicio. Las imágenes intraquirúrgicas y de preparados anatómicos fueron adquiridas en 3D. La revisión bibliográfica se realizó en Pub Med utilizando las palabras claves: Abordaje Supracerebeloso Infratentorial; Región Pineal; Craniectomía Suboccipital. RESULTADOS: durante el período 2010-2013 fueron intervenidos 7 pacientes. A todos se le practicó un abordaje supracerebeloso infratentorial. Fueron tratados 5 tumores de la región pineal, 1 glioma del culmen cerebeloso y 1 MAV de la fisura cerebelo mesencefálica. Se estandarizaron los siguientes pasos para la realización del abordaje supracerebeloso infratentorial de Stein: preparación prequirúrgica, posición sentado, posición de la cabeza, marcación y antisepsia cutánea, colocación de campos estériles, incisión de piel y tejido celular subcutáneo, disección de plano muscular, craneotomía, apertura dural, preparación del corredor supracerebeloso, disección intradural y anatomía microquirúrgica de la región pineal y consideraciones sobre elcierre. CONCLUSIONES: se sistematizó el abordaje supracerebeloso infratentorial con el que pudimos acceder a tumores pineales y también a lesiones vasculares en la región. En todos los casos la exposición anatómica fue suficiente para tratar en forma adecuada las patologías mencionadas, con mínima retracción de las estructuras del SNC

INTRODUCTION: even though Horsley was the first one to describe the supracerebellar infratentorial approach, it was Stein who adapted it to microsurgical techniques in an attempt to improve the results of surgical procedures on the pineal gland. OBJECTIVES: to enhance the indications and technical details of the supracerebellar infratentorial approach, based upon our experiences, we review the microsurgical anatomy of the pineal region that is exposed via this approach. MATERIALS AND METHODS: a retrospective descriptive study was conducted by analyzing the records of seven patients who had been operated on using this approach at our institution between September 2010 and September 2013. The images shown were obtained in 3D. RESULTS: the indications for surgery in these seven patients were a pineal gland tumor in five patients, and a culmen glioma and mesencephalic-fissure AVM in one patient each. The following steps were standardized according to Stein's description of the supracerebellar infratentorial approach: pre-surgical preparation, sitting position, head position, incision drawing and anti-sepsis, surgical field, collocation, skin and hypodermis incision, muscular dissection, hemostatic control, craniotomy, dural opening, supracerebellar corridor preparation, intradural dissection, microsurgical anatomy study, and considerations regarding closure. CONCLUSIONS: via this approach, we not only have been able to access pineal gland tumors but also vascular lesions in this region. The anatomical exposure achieved was enough to treat these lesions successfully, with minimal manipulation of the CNS

Gamma knife surgery for pineal region tumors: an alternative strategy for negative pathology / Cirurgia com gamma knife como tratamento experimental para tumores da região da pineal: uma estratégia alternativa quando os dados anatomopatológicos são negativos

Objective : Pineal region tumors (PRTs) are uncommon, and treatments vary among neoplasm types. The authors report their experience with gamma knife surgery (GKS) as an initial treatment in a series of PRT patients with unclear pathological diagnoses. Method : Seventeen PRT patients with negative pathology who underwent GKS were retrospectively studied. Nine patients had further whole-brain and spinal cord radiotherapy and chemotherapy 6–9 months after GKS. Results : Sixteen of 17 cases were followed up over a mean of 33.3 months. The total response rate was 75%, and the control rate was 81.3%. No obvious neurological deficits or complications were attributable to GKS. Conclusion : The findings indicate that GKS may be an alternative strategy in selected PRT patients who have negative pathological diagnoses, and that good outcomes and quality of life can be obtained with few complications. .

Tumores da região da pineal (TRP) são pouco frequentes e as propostas de tratamento são bastante variadas. Os autores relatam sua experiência em cirurgias com uso gamma knife (CGK) como tratamento experimental inicial em séries de TRP que não têm diagnóstico anatomopatológico ou nos quais o diagnóstico não ficou claro. Foram estudados retrospectivamente 17 pacientes com TRP nestas condições e que foram submetidos a CGK. Destes, 9 pacientes foram submetidos posteriormente a radioterapia de todo o encéfalo e medula espinhal entre 6 e 9 meses depois da CGK. Dezesseis dos 17 pacientes foram acompanhados por um período médio de 33,3 meses. A taxa total de resposta nos pacientes foi de 75% e a taxa dos controles, 81,3%. Não houve nenhum déficit neurológico evidente que pudesse ser atribuído à CGK. A CGK como tratamento experimental pode ser uma estratégia alternativa no grupo específico de pacientes com TRP em que não há diagnóstico anatomopatológico, podendo ser obtida uma boa qualidade de vida com poucas complicações para esse grupo de pacientes.

Correlación cito-histológica del tumor papilar de la glándula pineal. Presentación de un caso / Papillary pineal tumor: cyto-histological correlation. Case report

En la última revisión de la Organización Mundial de la Salud (OMS) en relación a los tumores del sistema nervioso central (SNC), se describieron nuevas entidades, como el Tumor Papilar de la Glándula Pineal. Esta lesión de rara aparición, se ha identificado en adultos jóvenes. El diagnóstico de estos tumores es complejo ya que depende de su ubicación, edad de aparición y el aspecto histológico; éste último tiene similitudes con otras lesiones como el ependimoma papilar o el papiloma/carcinoma de plexos coroides. Citológicamente presentan características claras que pueden ayudar al diagnóstico a través de la impronta en el estudio intraoperatorio; reconocer ciertos criterios con éste importante y sencillo método diagnóstico ha sido la motivación principal para el estudio de entidades poco frecuentes del SNC, además de corroborar el necesario trabajo de un equipo multidisciplinar.

In the latest revision of the central nervous system tumors (CNS) of the World Health Organization (WHO), new entities has been described, as papillary tumor of the pineal region. This rare lesion has been identified in young adults. The diagnosis of these tumors is complex, depends on the location, age of onset and histological appearance. Histological characteristics have similarities with other lesions such as papillary ependymoma, papiloma / choroid plexus carcinoma. Cytologically have clear characteristics that can aid in the diagnosis through the smears on the intraoperative study. Certain criteria for recognize this important and simple diagnostic method has been the main motivation for the study of CNS rare entities, as our case, in addition to corroborating the necessary work of a multidisciplinary team.

Microsurgical surgery options for tumors in pineal region / 中国综合临床

Objective To explore the significance of surgical resection for the pineal region tumor,the extent of tumor resection,the surgical approaches and treatment measures of hydrocephalus.Methods Twentythree patients diagnosed of pineal region cancer were recruited for this study.Thirteen patients received ventriculo-peritoneal shunt(V-P),during which 7 cases received intraoperative end-plate colostomy.Two of the 13 cases received a second V-P procedure.Another 13 cases received operation by corpus callosum- septum-Dome Room-the third ventricle approach to remove the tumor; Eight cases underwent the tumor resection by suboccipital supratentorial approach (Poppen approach )and 2 cases underwent the tumor resection by the infratentorial cerebellar approach( Krause approach).Results Total tumor resection was performed in 11 cases,subtotal or major total resection in 8 cases and partial resection in 4 cases.Nine cases underwent postoperative radiotherapy alone,8 receiving radiotherapy plus chemotherapy,and 6 cases receiving neither radiotherapy nor chemotherapy.Two cases died during treatment.Conclusion Though it is not practicable to completely remove the germ cell tumor in patients with relatively large tumors,cerebrospinal fluid circulation pathways should be reestablished,including ventriculo-peritoneal shunt,colostomy from the end of the third ventricle to the end plate.If tumors are not too large,they would be easy to be removed.The effects on the surrounding brain tissue would not be much significant.If the connection of Ⅲ,V ventricles were normal,the patient may not need shunt or colostomy.Radiotherapy and chemotherapy can be given to this kind of patients after the surgery.To improve the efficacy,other types of tumors,expecially the benign tumors,should be totally removed.

Contrasted study between thin coronal sectional anatomy of the pineal region and MRI image / 解剖学报

Objective To investigate the morphology and relationships with the adjacent structures in the pineal region on the thin sections and to provide anatomic data for imaging diagnosis and surgical treatment of diseases in this region. Methods By CT and MRI examination, one normal head specimen was selected for this study. Using the computerized freezing milling technique, the specimen was sliced from anterior to posterior. The in vivo MR images were obtained from ten normal Chinese male adult volunteers using a 3.0 T GE scanner. The base lines of the sectioning and the MR scan were perpendicular to the AC-PC line. Then primary sections were contrasted with the corresponding MR images. Results By the appearance of the pineal peduncle and the disappearance of the pineal gland, the pineal region could be divided into three parts from anterior to posterior, and the shape changed from an inverted triangle to a trapezoid and a triangle gradually. The first interspace was getting wider in the anterior and middle parts of the pineal region, while in the posterior part of the pineal region, it was getting narrower and disappeared finally. From anterior to posterior, the bilateral internal cerebral veins were always in the midline of the pineal region and descended gradually.Conclusion By the computerized freezing milling technique, the anatomic details and adjacent relationships of the pineal region could be exhibited clearly in the thin serial sections, which could help the imaging diagnosis and surgical treatments for minute diseases in this region.

Pineal Cavernous Malformations: Report of Two Cases

Pineal hemorrhage only occurs in rare cases, and this known to have several different causes such as germ cell tumors, pineal cysts and vascular malformations, including the cavernous malformations. Pineal cavernous malformations are extremely rare: to date only fifteen cases have been reported worldwide. Although the diagnosis of pineal cavernous malformation is not easy because of the extreme rareness of this condition, the presence of this lesion can be suspected based on its typical radiological findings. Case 1. A 42-year- old man presented with a limitation in his upward gazing. Radiologic examinations showed acute hemorrhage in the pineal region. He underwent ventriculo-peritoneal (VP) shunting but the patient's condition deteriorated after the shunting surgery. We operated and totally removed the tumor and the hemorrhages via an occipital-transtentorial approach. Case 2. A 37-year-old man presented with diplopia. Radiologic examinations showed acute hemorrhage in the third ventricle. He underwent VP shunting, and after this procedure the diplopia was aggravated. We operated and totally removed the tumor and the hemorrhages via an occipital-transtentorial approach. If there is no doubt about the pineal cavernous malformation on MR imaging, we strongly recommend early surgical intervention without performing a risky biopsy. In this study, we describe our experiences for the diagnosis of cavernous malformations in the pineal region with special emphasis on the radiological aspects and the clinical course of this disease.

To discuss on the surgical removal of the region of pineal gland

Pineal gland is structured by special morphology of neural cells. The tumor originates from the gland or its surrounding tissnes therefore these tumors have histologically different origins. They are in deep location with narrow path inward. Pineal tumor has not specific symptoms and usually diagnosed lately. The removal surgery comfuses of 2 stages - first: brain ventricular-abdomen drainage, second: tumor removal within 2-3 weeks after the first stage. There are 5 methods to approach the tumor, with own advantages and short comings

Roles of endoscopic biopsy and third ventriculostomy in the diagnosis and treatment of pineal region tumors in children / 第三军医大学学报

Objective To investigate the roles of endoscopic biopsy and third ventriculostomy in the diagnosis and treatment of pineal region tumors in children. Methods Endoscopic biopsy and third ventriculostomy were performed in 9 pediatric patients with pineal region tumors. Results Successful third ventriculostomy, confirmed by MRI, was performed in 9 cases of children with obstructive hydrocephalus. No complications were found in all patients. Conclusion Endoscopic biopsy and third ventriculostomy are effective neuroendoscopic procedures in minimally invasive preferential management of pineal region tumors.

A Role of Trial Radiation Therapy in the Pineal Region Tumors / 대한방사선종양학회지

PURPOSE: The aim of this retrospective study was to assess the treatment results of 30 patients with pineal region tumors who were underwent radiation therapy under the diagnosis by either CT or MRI. There was no histological verification. We analyzed the prognostic factors that have a significant effect on the overall survival (OS) and disease free survival (DFS) rates. MATERIALS AND METHODS: A total 30 patients with pineal region tumors were treated between March 1983 and August 1995. After a trial radiation therapy of 20~30 Gy/2~3 weeks, the patients were evaluated for their clinical response and radiological response by either CT or MRI and the final treatment direction was then decided. According to their response to the trial radiation therapy and the involved site, radiation treatment was given in various fields i.e., local, ventricle, whole brain and craniospinal field. The radiation dose ranged from 40.8 to 59.4 Gy (Median 50.4 Gy). The median follow up was 36.5 months (4~172 months). RESULTS: An improvement or stability in the clinical symptoms was observed in 28 patients (93.3%) after the trial RT. Nineteen patients (63.3%) showed a partial or complete response by CT or MRI. The two-year and five-year survival rates of the patients were 66.7% and 55.1%, respectively. No significant difference in the survival rates according to the degree of the radiological response was abserved after the trial RT. The results of univariate analysis showed that age, the primary site, the performance status (KPS>or=70), the degree of response after completing RT and the RT field were significant prognostic factors affecting the survival and disease free survival rates (p<0.05). CONCLUSION: The clinical and histological characteristics of pineal region tumors are quite complex and diverse. Therefore, it is difficult to predict the histological diagnosis and the possibility of radiocurability only with the initial response to RT. We think that the development of less invasive histological diagnostic techniques and tailored treatment to the histological type of each tumor are needed.

Endoscopic Management of Pineal Region Tumors with Associated Hydrocephalus

PURPOSE: In general, pineal region tumors are managed by using microsurgical approach or stereoctactic biopsy. However, in selected cases endoscopic approach to pineal lesions might prove to be as effective as microsurgery and less invasive. We report an alternative surgical strategy for managing certain patients with pineal neoplasms that allows treatment of the symptomatic hydrocephalus as well as tumor biopsy under direct vision in the same sitting. MATERIALS AND METHODS: Twenty-two patients with pineal region tumors with associated hydrocephalus were treated in one session by endoscopic third ventriculostomy and endoscopic tumor biopsy at our institution from October 1996 to January 2000. All patients were retrospectively evaluated. RESULTS: There was no operative mortality. There was one cause of significant bleeding during biopsy, but was controlled endoscopically, and the patient recovered completely without neurologic deficit resulting from intra-operative bleeding. The symptoms related to increased intracranial pressure(ICP) have resolved in all patients, and the need for a shunt is completely eliminated. Histological diagnosis was achieved in 21 of the 22 patients by this procedure. A biopsy was not obtained in one patient. Although this pineal region tumor was seen endoscopically, this could not be biopsied because of technical difficulties in working around an enlarged massa intermedia. The lesions included fourteen germinomas, three mixed germ cell tumors, and one each of the followings: pineocytoma, pineoblastoma, pineocytoma/pineoblastoma(intermediate type), meningioma, and low grade glioma. Five of the 22 patients subsequently underwent formal microsurgical tumor removal. Additional chemotherapy or radiotherapy could then be initiated according to the histological diagnosis. CONCLUSION: We consider that endoscopy affords a minimally invasive way of reaching three objectives by one-step surgery in the management of pineal region tumors with associated hydrocephalus: 1) cerebrospinal fluid(CSF) sample for analysis of tumour markers and cytology, 2) treatment of hydrocephalus by third ventriculostomy, and 3) several biopsy specimens can be obtained identifying tumors which will require further open surgery or adjuvant radiation and/or chemotherapy. However, complications and morbidities should be emphasized so as to be avoided with further technical experience.