Le carcinome adénoïde kystique des glandes salivaires accessoires: Particularités cliniques et modalités thérapeutiques / Adenoid cystic carcinoma of accessory salivary glands: clinical features and therapeutic modalities

Etudier les particularités cliniques des carcinomes adénoïdes kystiques des glandes salivaires accessoires et discuter les modalités thérapeutiques de ce type tumoral. Patients et méthodes: Il s'agit d'une étude rétrospective descriptive ayant colligé 11 patients présentant un carcinome adénoïde kystique (CAK) des glandes salivaires accessoires (GSA) pris en charge dans notre département sur une période de 20 ans. Résultats: L'âge moyen de nos patients était de 51 ans avec un sex ratio à 0,83. Le délai moyen de consultation était de 20 mois. Le site tumoral était: la cavité buccale dans 3 cas, le massif facial et le cavum dans 6 cas, la trachée et le larynx dans 2 cas. Les motifs de consultation étaient variables selon le site tumoral. L'extension péri neurale était objectivée dans 3 cas. Le traitement était chirurgical suivi de radiothérapie chez 5 patients. Une radiothérapie exclusive a été indiquée chez 3 patients et une radio chimiothérapie concomitante dans 3 cas. Un recul moyen de 42 mois a pu être obtenu pour 10 patients. Une patiente a été perdue de vue. L'évolution était favorable avec absence de récidive locale chez 8 patients. Conclusion: Le traitement de référence reste une chirurgie d'exérèse large, suivie de radiothérapie adjuvante. L'imagerie est indispensable pour le bilan d'extension locorégional et à distance qui conditionne l'attitude thérapeutique.

Co-Occurrence of Pineal-Region and Pituitary-Stalk Hemangioblastomas in a Patient Presenting with Von Hippel-Lindau Disease ­ A Case Report

Introduction Hemangioblastomas of the pineal region or pituitary stalk are extremely rare. Only two cases of hemangioblastomas involving the pineal region have been reported, and four involving the pituitary stalk. The purpose of the present manuscript is to describe an unusual case of supposed hemangioblastoma found concomitantly in the pineal region and pituitary stalk of a patient diagnosed with Von Hippel-Lindau (VHL) disease. Case Report A 35-year-old female patient with a previous diagnosis of VHL complaining of occipital headaches and balance disturbances for three weeks, who previously had a cerebellar hemangioblastoma resected. The visual characteristics of the tumor suggested a friable vascular lesion with a reddish-brown surface, and an incisional biopsy was performed. The tumor consisted of a dense vascular network surrounded by fibrous stroma abundant in reticulin and composed by both fusiform and dispersed xanthomatous cells; the immunohistochemistry was immunopositive for neuronspecific enolase and immunonegative for epithelial membranous antigen. The patient has been monitored closely for 2 years, and the supratentorial masses have not presented any volume alteration. Conclusion This rare association must be taken into account in patients with VHL disease, or at least be suspected in patients who present a thickening of the pituitary stalk and a pineal-region mass. We believe a biopsy of our asymptomatic patient could have been dangerous due to inherent complications like intraoperative bleeding. We recommend close observation of asymptomatic lesions with MRIs every six months or until the lesions become symptomatic. If the pineal-region tumor does become symptomatic, gross resection via a transcallosal approach would be ideal.

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.

Tumor pineal resuelto por Abordaje Infratentorial Supracerebeloso Endoscópico. Reporte de un caso y revisión de la literatura / Pineal tumor resolved by Endoscopic Supracerebellar Infratentorial Approach. Case report and literature review

Introducción: Los tumores de la región pineal constituyen un grupo heterogéneo de lesiones localizadas en la porción posterior del tercer ventrículo bajo el esplenio del cuerpo calloso y encima de la lámina cuadrigémina. Dentro de estos, los tumores del parénquima pineal constituyen un grupo frecuente. En estas lesiones no existe actualmente un protocolo establecido debido a la ausencia de grandes series. Se sugiere una combinación de cirugía y radioterapia con o sin quimioterapia. El tratamiento de elección es la resección total, sin embargo, muchas veces no es posible por dificultades técnicas. En este contexto, la cirugía con visualización endoscópica puede contribuir a lograr este objetivo. Descripción del caso: Se presenta un paciente masculino de 22 años de edad con lesión de región pineal e hidrocefalia obstructiva triventricular al que se le practicó una tercerventriculostomía endoscópica y un abordaje infratentorial supracerebeloso con total visualización endoscópica. Se describe la técnica quirúrgica y se realiza un análisis crítico de la literatura actualizada. Conclusiones: Los tumores de la región pineal constituyen un reto terapéutico. La resección total es el principal objetivo en lesiones de grado intermedio o bajo de malignidad. El abordaje infratentorial supracerebeloso con total visualización endoscópica es efectivo en la resección quirúrgica de estas lesiones al mejorar la visualización y minimizar la retracción cerebelosa

Introduction: Pineal region tumors are a variable group of lesions located in the posterior wall of the third ventricle under the corpus callous splenium and above the tectal plate. Pineal gland tumors are frequent. There is not a standard protocol in these lesions due the lack of large series. A combination of surgery and chemo therapy or radiotherapy are recommended. The complete surgical resection is the treatment of choice. However, usually it is not possible to accomplished this goal due to technical limitations. In this scenario, the endoscopic visualization could contribute to archive the goal. Patient characteristics: A 22 years old young male patient with a pineal region lesion and obstructive hydrocephalus is presented. A third ventriculostomy was performed and an infratentorial supracerebelous approach with fully endoscopic visualization. The surgical technique is described and a critical review of literature is performed. Conclusions: Pineal region tumors represents a therapeutic challenge. Total removal is the most important objective in intermediate or low-grade lesions. The infratentorial supracerebelous approach with full endoscopic visualization is effective and improve the visualization while reducing the cerebellar retraction.

Quiste benigno de la glándula pineal en paciente con retinoblastoma bilateral. Reporte de caso y revisión de la literatura / Benign pineal gland cyst in a patient with bilateral retinoblastoma. Case report and literature review

Introducción: Los pacientes con Retinoblastoma (RB) hereditario se encuentran en riesgo de desarrollar otros tipos de tumores malignos primarios extraoculares durante la vida. Dentro de estos tumores se encuentra el pinealoblastoma, un tipo de neoplasia maligna que aparece en la glándula pineal y que se puede desarrollar en cualquier momento del diagnóstico del retinoblastoma bilateral. Objetivo: Presentar un caso inusual de retinoblastoma (RB) hereditario tratado y en remisión quien desarrolló un quiste pineal benigno. Diseño del estudio: Reporte de caso y revisión de literatura. Resumen del caso: Presentamos un paciente con diagnóstico de RB hereditario tratado y en remisión quien desarrolló un quiste pineal benigno durante el seguimiento clínico. Las lesiones quísticas de la glándula pineal reportadas por resonancia magnética son infrecuentes en edad pediátrica, sin embargo, su incidencia se ve aumentada en pacientes con RB, lo que genera incertidumbre sobre la posibilidad de presentar una neoplasia maligna. Aún se desconoce el mecanismo fisiopatológico de la aparición de quistes de la glándula pineal en pacientes con RB hereditario, pero podría tener relación con la alteración genética o con el tratamiento quimioterápico que reciben los pacientes para el tumor primario intraocular. Conclusión: Las características imagenológicas son fundamentales para diferenciar entre lesiones benignas y malignas de la glándula pineal en pacientes con retinoblastoma hereditario y para hacer el estrecho seguimiento junto con el examen clínico.

Background: Patients with hereditary Retinoblastoma (RB) are at risk of developing other types of extraocular primary malignancies throughout life. Among these tumors, pinealoblastoma is a type of malignancy that appears in the pineal gland and can develop at any time from the diagnosis of bilateral retinoblastoma. Objective: To present an unusual case of a patient with a diagnosis of hereditary BR who developed a pineal cyst. Study design: Case report. Case summary: We present in this article the case of a patient with a diagnosis of hereditary BR with remission who developed a pineal cyst during clinical follow-up. The cystic lesions of the pineal gland reported by magnetic resonance are infrequent in pediatric age, however its incidence is increased in patients with RB, which generates uncertainty about the possibility of the development of a primary pineal gland malignancy. The pathophysiological mechanism of pineal gland cysts in patients with hereditary RB is still unknown, but it could be related to a genetic alteration or to chemotherapy treatment that these patients receive for the primary intraocular tumor. Conclusion: The imaging characteristics are fundamental to differentiate between benign and malignant lesions of the pineal gland in patients with Hereditary Retinoblastoma and to make a close follow up.

"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

@#<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.

Five-year review of pineal region tumors at the Philippine General Hospital

@#<p style="text-align: justify;"><strong>RATIONALE:</strong> Pineal region tumors are rare neoplasms with a reportedly higher incidence in Asian countries; however, local Philippine data is lacking.<br /><strong>METHODS:</strong> A retrospective chart review was conducted on all newly diagnosed adult and pediatric patients with pineal region tumors admitted at the Philippine General Hospital between 2011 and 2015. Data  about  demographic  profile,  biochemical  markers,  imaging findings, histopathology, and treatment were collected.<br /><strong>RESULTS:</strong> Forty-two patients (36 males, 6 females; Sex Ratio = 6:1) were included in the study, with a mean age of 16.5 years. On imaging, solitary pineal area tumors were seen in 34 (81%) patients, while 8 (19%)  presented  with  synchronous  tumors  in  the  pineal  and suprasellar areas. Hydrocephalus was present in 41 (98%). Tumor marker (serum +/- CSF ?FP and ?hCG) determination was performed in 33(79%)  patients. Thirty-eight (90%) patients underwent surgical intervention for tumor biopsy and/or CSF diversion. Combining the tumor marker levels and histopathology results, there were 20(48%) germ  cell  tumors,  4(9%)  pineal  parenchymal  tumors,  1(2%) meningioma, 1(2%) epidermoid tumor, and 16(38%) tumors with incomplete  diagnosis.  Regarding  adjuvant  treatment,  5  patients underwent chemotherapy, 6 underwent radiotherapy, and 1 patient received both. Follow-up data were available in only 16 patients, with a mean follow-up of 12 months (range: 1-33 months).<br /><strong>CONCLUSION:</strong> The demographic profile and histologic subtypes of patients with pineal region tumors in this series were comparable with other series in the literature. However, due to limited resources leading to suboptimal medical care and poor follow-up, a reliable treatment outcome could not be determined</p>

A Case of Nongerminomatous Germ Cell Tumor with Fulminant Course Concomitant Leptomeningeal Metastasis

We present the case of a 9-year-old boy with a non-germinomatous germ cell tumor (NGGCT) in the pineal gland that exhibited a fulminant course following chemo- and radiotherapy. After the detection of the tiny cerebellar enhancing nodule at the end of chemo- and radiotherapy, tumor seeding progressed rapidly into the entire cisternal space. We herein report a rare case of NGGCT with fulminant clinical course of concomitant cerebellar seeding, with review of literature.

A Case of Nongerminomatous Germ Cell Tumor with Fulminant Course Concomitant Leptomeningeal Metastasis

We present the case of a 9-year-old boy with a non-germinomatous germ cell tumor (NGGCT) in the pineal gland that exhibited a fulminant course following chemo- and radiotherapy. After the detection of the tiny cerebellar enhancing nodule at the end of chemo- and radiotherapy, tumor seeding progressed rapidly into the entire cisternal space. We herein report a rare case of NGGCT with fulminant clinical course of concomitant cerebellar seeding, with review of literature.

Trilateral retinoblastoma with unilateral eye involvement / Retinoblastoma trilateral com acometimento ocular universal

SummaryRetinoblastomas (RB) are the main forms of intraocular tumor in childhood, with a worldwide incidence of 1 case per 15,000 to 20,000 live births. Trilateral RB (RBT) is a rare combination of unilateral or bilateral RB with a midline intracranial neoplasm of neuroblastic origin, usually found in the pineal region or the suprasellar region, presenting variable incidence of 0.5% up to 6% among patients with RB. The article reports a case of unilateral RBT in a patient treated at Hospital A.C.Camargo.

ResumoO retinoblastoma (RB) é a principal forma de tumor intraocular na infância, apresentando uma incidência mundial de 1 caso em cada 15 mil a 20 mil nascidos vivos. O RB trilateral (RBT) é uma rara combinação de RB unilateral ou bilateral com uma neoplasia da linha média intracraniana de origem neuroblástica, geralmente na região da glândula pineal ou na região suprasselar, apresentando incidência variável de 0,5 a 6% entre pacientes com RB. O artigo relata o caso de uma paciente com RBT com acometimento unilateral atendida no Hospital A.C.Camargo.

An Unusual Variant of Anlage Tumor of Pineal Region in an Infant

A 9-month-old male child was brought with complaints of increasing head size for 2 months, increasing lethargy and vomiting for the last 2 days. Radiology revealed a heterogeneously enhancing, globular lesion in the pineal region with hydrocephalus. Near total excision of the tumor was carried out. The histopathological examination of the lesion showed heterogenous elements in the form of mature neuroepithelial and ectomesenchymal tissue. The pathology and radiology of this unusual lesion is discussed with relevant review of literature.

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.

Experience of treatment for accumulation of extra-axil cerebrospinal fluid after skull surgery / 中南大学学报(医学版)

OBJECTIVE@#To explore the treatment and pathophysiology of extra-axil cerebrospinal fluid accumulation aft er skull surgery.@*METHODS@#The treatment of 46 cases of pineal regional tumor was retrospectively studied.@*RESULTS@#The CT showed that all patients had postoperative extra-axil cerebrospinal fluid accumulation in 6 hours after operation. 5 cases displayed symptomatic accumulation of extra-axil cerebrospinal fluid. 1 died 30 days aft er discharge, 4 performed S-P shunt and 3 of them switched to V-P shunt after S-P shunt failed.@*CONCLUSION@#Much more attention should be paid to postoperative accumulation of extra-axil cerebrospinal fluid. Both V-P and S-P are the effective strategies of therapy.

Upfront Stereotactic Radiosurgery for Pineal Parenchymal Tumors in Adults

OBJECTIVE: Pineal parenchymal tumors (PPTs) in adults are rare, and knowledge regarding their optimal management and treatment outcome is limited. Herein, we present the clinical results of our series of PPTs other than pineoblastomas managed by stereotactic radiosurgery (SRS) at upfront setting. METHODS: Between 1997 and 2014, nine consecutive adult patients with the diagnosis of PPTs, either pineocytoma or pineal parenchymal tumor of intermediate differentiation, were treated with SRS. There were 6 men and 3 women. The median age was 39 years (range, 31-53 years). All of the patients presented with symptoms of hydrocephalus. Endoscopic third ventriculostomy and biopsy was done for initial management. After histologic diagnosis, patients were treated with Gamma Knife with the mean dose of 13.3 Gy (n=3) or fractionated Cyberknife with 32 Gy (n=6). RESULTS: After a mean follow-up of 78.6 months (range, 14-223 months), all patients were alive and all of their tumors were locally controlled except for one instance of cerebrospinal fluid seeding metastasis. On magnetic resonance images, tumor size decreased in all patients, resulting in complete response in 3 patients and partial response in 6. One patient had experienced temporary memory impairment after SRS, which improved spontaneously. CONCLUSION: SRS is effective and safe for PPTs in adults and can be considered as a useful alternative to surgical resection at upfront setting.

A Case of Radiation-Induced Osteosarcoma after the Treatment of Pineoblastoma

Radiation therapy has an important role in postoperative treatment of neoplasms originated from central nervous system, but may induce secondary malignancies like as sarcomas, gliomas, and meningiomas. The prognosis of radiation-induced osteosarcomas is known as poor, because they has aggressive nature invasive locally and intractable to multiple treatment strategies like as surgical resection, chemotherapy, and so on. We report a case of radiation-induced osteosarcoma developed from skull after 7 years of craniospinal radiotherapy for pineoblastoma.

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.

Analysis of the surgical strategy for the treatment of pineal region tumors / 中华外科杂志

<p><b>OBJECTIVE</b>To evaluate and explore the optimal surgical strategy for the normalized treatment of pineal region tumors.</p><p><b>METHODS</b>From September 2007 to February 2012, 43 patients were treated in Chinese People's Liberation Army General Hospital, including 30 male and 14 female patients, with pineal region tumors and non-communicating hydrocephalus were enrolled, who were 1-52 years old, mean age was (27 ± 4) years. The clinical records, treatment strategy, and prognosis were retrospectively analyzed. All the patients routinely underwent endoscopic third ventriculostomy (ETV) and tumor biopsy as the initial treatment. Twenty-seven cases (62.8%, pure endoscopic group) with histological diagnosis of germinoma (23 cases) or pineoblastoma (4 cases) were treated with chemotherapy with/without radiation therapy after ETV. The rest 16 cases (37.2%, craniotomy group) with histological diagnosis of non-germinoma and non-pineoblastoma (5 astrocytomas, 4 pineocytomas, 4 teratomas, 2 ependymomas, and 1 pineopappiloma) had craniotomy and tumor resection after ETV. All the cases had routine follow-up at 1, 3, and 6 months after the final surgery. The clinical, imaging, and tumor markers analysis were routinely examined at follow-up.</p><p><b>RESULTS</b>In the pure endoscopic group, 1 case had intra-ventricular hemorrhage after ETV, followed by external ventricular drainage and recovered after 1 week. Endoscopic procedure related short-term ( < 3 months) complication rate was 2.3% (1/43), while long-term morbidity was 0. All cases in the pure endoscopic group had chemotherapy with/without radiation therapy. Long-term follow-up results showed that all cases were cured or had progression free survival (PFS). In the craniotomy group, 2 cases (2/16) developed intra-cranial hemorrhage after surgery, and had to be operated again for hematoma evacuation. In the craniotomy group, the short-term ( < 3 months) morbidity rate was 6/16. At 3 months follow-up, 1 case still had homonymous hemianopia, which made the long-term morbidity rate was 1/16.</p><p><b>CONCLUSIONS</b>For pineal region tumors with non-communicating hydrocephalus, simultaneous ETV with tumor biopsy can be the most favorable initial diagnostic and therapeutic treatment. Second-stage treatment (chemotherapy, radiation therapy, or craniotomy with tumor resection) can be selected according to the histological diagnosis.</p>

Clinicopathologic features of papillary tumors of the pineal region / 中华病理学杂志

<p><b>OBJECTIVE</b>To study the clinicopathologic features of papillary tumor of the pineal region (PTPR).</p><p><b>METHOD</b>Three hundred and eighty six cases of pineal region and posterior third ventricle tumors, two newborn and two adult pineal glands were analyzed by HE, PAS and immunohistochemistry of 16 antibodies (EnVision method).</p><p><b>RESULTS</b>Five cases of PTPR were diagnosed with mixed papillary features and densely cellular areas, and included one recurrent case. In the papillary areas, the vessels were lined by one or several layers of cuboidal/columnar cells; the vessel wall was hyalinized. In the densely cellular areas, sheets or nests of tumor cells were seen. The tumor cells of these five cases were immunoreactive to CK, CK8/18, synaptophysin, MAP2, nestin, S-100, and vimentin. Four cases were immunoreactive to NSE and CgA; and 2 cases were immunoreactive to NF. All five cases were negative for EMA, CK5/6, CEA, and NeuN. Ki-67 labeling index ranged from 1% to 6%.Three patients were alive, and the recurrent one died.</p><p><b>CONCLUSIONS</b>PTPR occurs in patients with over a wide age range, from children to adults, and is more commonly found in male than female. PTPR is composed of both papillary and solid areas, characterized by epithelial cytology, and needs to be differentiated from ependymoma. PTPR may originate from the specialized ependymocytes of the subcommissural organ. The prognostic factors are early diagnosis, complete surgical resection and radiotherapy.</p>