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.

Actualización sobre los tumores de la región pineal / Update on pineal region tumors

Introducción: Los tumores de la región pineal constituyen un grupo heterogéneo de lesiones agrupadas más por su localización común que por su relación histológica. Pueden clasificarse en tumores de células germinales, del parénquima pineal, embrional y de estructuras adyacentes. Objetivo: Caracterizar la fisiopatología de los tumores de la región pineal y algunos aspectos de su tratamiento. Métodos: Se realizó una revisión bibliográfica, se seleccionaron estudios relacionados con la fisiopatología de los tumores de la región pineal y su manejo. Las fuentes de información consultada fueron Google Scholar, PubMed, SciElo y Dialnet; se utilizaron los descriptores: neoplasias pineales, pinealoma, neoplasias encefálicas. Se seleccionaron 23 artículos, más del 75 por ciento son de los últimos 5 años. Desarrollo: La tasa y la velocidad de crecimiento de estas neoplasias determinan la rapidez con la que se instauran los diversos síntomas, de ahí que la presentación más común es la hidrocefalia triventricular, acompañada de dolor de cabeza, náuseas, vómitos, disminución de la actividad, somnolencia o letargo y alteraciones visuales. También se pueden observar paresias y síndromes endocrinos. El diagnóstico se basa en la exploración de marcadores tumorales en suero, líquido cefalorraquídeo, tomografía y resonancia magnética. Finalmente, la quimioterapia y la intervención quirúrgica constituyen las principales alternativas terapéuticas tras el diagnóstico de la enfermedad. Conclusiones: Este tipo de neoplasia es poco frecuente, sus manifestaciones clínicas dependen de la localización, tamaño y tipo de tumor; además, el tratamiento suele ser quirúrgico y posterior a la quimioterapia, si las características de la neoplasia lo permiten(AU)

Introduction: Tumors of the pineal region constitute a heterogeneous group of lesions better grouped by their common location than by their histological relationship. They can be classified into tumors of germ cells, pineal parenchyma, embryonal and adjacent structures. Objective: To characterize the pathophysiology of pineal region tumors and some aspects of their treatment. Methods: A literature review was carried out, we selected studies related to the pathophysiology of pineal region tumors and their management. The information sources consulted were Google Scholar, PubMed, SciELO and Dialnet; the descriptors were used pineal neoplasms, pinealoma, brain neoplasms. Twenty three articles were selected, more than 75percent are from the last 5 years. Results: The rate and speed of growth of these neoplasms determine the speed with which the various symptoms are established, hence the most common presentation is triventricular hydrocephalus, accompanied by headache, nausea, vomiting, decreased activity, drowsiness or lethargy and visual disturbances. Paresis and endocrine syndromes may also be seen. The diagnosis is based on the examination of tumor markers in serum, cerebrospinal fluid, tomography and magnetic resonance imaging. Finally, chemotherapy and surgery are the main therapeutic alternatives after diagnosis of the disease. Conclusions: This type of neoplasm is rare, the clinical manifestations depend on the location, size and type of tumor; in addition, the treatment is usually surgical and then chemotherapy, if the characteristics of the neoplasm allow so(AU)

Pubertad precoz asociada a tumor: a propósito de un caso / Precocious puberty associated with a tumor: Report of a case

La pubertad precoz es una patología poco común en la práctica médica cotidiana, sin embargo, hay factores independientes que pueden afectar el inicio de la misma, como son: la raza, el bajo peso al nacer y la obesidad. Los cambios físicos tempranos, característicos en el comienzo de la pubertad aparecen como resultado de la producción de hormonas sexuales, en relación con la actividad del eje hipotálamo-hipófisis-gonadal, en nuestro paciente aparecen a los 7 años de edad. El inicio de la pubertad, comienza con el desarrollo de las mamas en las niñas y con el aumento del tamaño testicular en los niños. La pubertad precoz se clasifica en: central, también llamada dependiente de la hormona liberadora de gonadotrofinas (GnRH) y periférica, conocida como independiente de GnRH. El primer paso en la evaluación del paciente con sospecha de pubertad precoz, consiste en obtener una cuidadosa y detallada historia familiar y del paciente, seguida de un examen físico completo. El diagnóstico de pubertad precoz se basa en estudios de imagen y en pruebas de laboratorio. Los picos de LH o de FSH y el pico de la relación LH/FSH, pueden ser utilizados para determinar el diagnóstico.

Precocious puberty is a rare pathology in everyday medical practice, however there are independent factors can affect the onset of puberty, such a race, low birth weight and obesity. The activation of the hypothalamic-pituitary-gonadal axis results in a production of sex hormones with development of breasts in girls and increase testicular size in boys. It's classified as central also called gonadotrophin releasing hormone (GnRH)-dependent, and peripheral. The first step in the evaluation of the suspected precocious puberty is to obtain a careful and detail family and patient clinical history, follow by a complete physical examination, it's diagnostic approach is based on imaging and laboratory tests. The LH or FSH levels and a peak LH/FSH ratio can be used to determine the diagnostic.

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.

Successful Treatment by Chemotherapy of Pineal Parenchymal Tumor with Intermediate Differentiation: A Case Report / Journal of the Korean Cancer Association, 대한암학회지

A 37-year-old male presented with a mass measuring 2.5 cm in size in the midbrain and obstructive hydrocephalus, which had manifested as a headache and dizziness. Magnetic resonance (MR) imaging of the brain showed intermediate enhancement on T1-weighted MR imaging and a high intensity of enhancement on T2-weighted MR. Neurosurgeons performed an occipital craniotomy with partial removal of the tumor and the postoperative diagnosis was a pineal parenchymal tumor with intermediate differentiation. He had undergone irradiation with 54 Gy of radiation on 27 fractions for removal of the remaining tumor approximately one month after surgery. However, in follow-up imaging performed four months after radiotherapy, a remnant mass in the superoposterior aspect of the midbrain was found to have extended to the hypothalamus and the third ventricle. He was treated with six cycles of procarbazine, lomustine, vincristine chemotherapy. At five months since the completion of chemotherapy, the brain MR imaging showed no evidence of any remaining tumor and he no longer displayed any of his initial symptoms.

A case of pineal germioma presenting with diabetes insipidus / 대한내과학회지

Central diabetes insipidus caused by pineal gland neoplasm is rare. Here, we describe a case of central diabetes insipidus and pineal germinoma with seeding to pituitary stalk along CSF pathway. A 27 year-old male patient was admitted due to polyuria, polydipsia, headache, vomiting, diplopia, and decreased visual acuity for recent two months. Urine osmolality measured after water deprivation was below 100mOsm/kg. However, urine osmolality increasd above 300 mOsm/kg with pitressin administration. Brain MRI showed a 2cm sized mass with seeding into pituitary stalk along CSF in pineal region. Surgical biopsy revealed pineal germinoma. Two cycles of chemotherapy with etoposide and cis-platin were done followed by brain irradiation(2,500cGy). Follow up brain MRI after second chemotherapy showed complete remission. The patient had no neurologic and endocrinologic deficit after the treatment.

Clinical Report of 46 Intracranial Tumors with LINAC Based Stereotactic Radiosurgery / 대한치료방사선과학회지

Between July 1988 and December 1992, we treated 45 patients who had deep seated inoperable or residual and/or recurrent intracranial tumors using LINAC based stereotactic radiosurgery at the Department of Therapeutic Radiology, Kangnam St. Mary's Hospital, Catholic University Medical College. Treated intracranial tumors included pituitary tumors(n=15), acoustic neurinomas(n=8), meningiomas(n=7), gliomas(n=6), craniopharyngiomas(n=4), pinealomas(n=3), hemangioblastomas(n=2), and solitary metastatic tumor from lung cancer (n=1). The dimension of treatment field varied from 0.23 to 42.88 cm3(mean ; 7.26 cm3). The maximum tumor doses ranging from 5 to 35.5 Gy (mean; 29.9 Gy) were given, and depended on patients' age, target volume, location of lesion and previous history of irradiation. There were 22 male and 23 female patients. The age was varied from 5 to 74 years of age(a median age; 43 years). The mean duration of follow-up was 35 months (2~55 months). To date, 18(35.1%) of 46 intracranial tumors treated with SRS showed absent or decrease of the tumor by serial follow-up CT and/or MRI and 16(34.8%) were stationary, e.g. growth arrest. From the view point of the clinical aspects, 34(73.9%) of 46 tumors were considered improved status, that is, alive with no evidence of active tumor and 8(17.4%) of them were stable, alive with disease but no deterioration as compared with before SRS. Although there showed slight increase of the tumor in size according to follow-up imagings of 4 cases(pituitary tumor 1, acoustic neurinomas 2, pinealoma 1), they still represented clinically stable status. Clinically, two(4.4%) patients who were anaplastic astrocytoma(n=1) and metastatic brain tumor(n=1) were worsened following SRS treatment. So far, no serious complications were found after treatment. The minor degree headache which could be relieved by steroid or analgesics and transient focal hair loss were observed in a few cases. There should be meticulous long term follow-up in all cases.

Clinical analysis of 3rd Decade Male 47 Cases with Intracranial Tumors in Korea

The authors represented a clinical analysis of 3 rd decade male 47 patients with intracranial tumors who had been histologically confirmed after operation and biopsy at the department of neurosurgery of Capital Armed Forces General Hospital From Feb. 1985 to Jan, 1988. We classified the intracranial tumors according to Russell and Rubinstein's classification. The results were as follows: 1) Among the intracranial tumors, gliomas were found most frequently(40.4%), and followed by pituitary adenomas(19.2%), pinealomas(10.6%), osteomas(6.4%), medulloblastomas(4.3%), craniopharyngiomas(4.3%), blood vessel tumors(4.3%). Pituitary adenomas occupied 19.2% of all intracranial tumors and as a single entity these were the highest incidence. 2) Tumors of the pineal region constitutes 10.6% of intracranial tumors. It was relatively high incidence compared with other reports. 3) Intracranial tumors occurred more frequently in supratentorial region(85.1%). The most frequent location was sellar and parasellare region(23.4%), and followed by frontal(14.9%), parietal(12.7%), pineal gland(10.6%), temporal(8.5%), cerebellar region(8.5%) in order. 4) The most common duration of symptoms were within 3 months(42.6%). The main clinical symptoms and signs were headache(80.9%), nausea or/and vomiting(55.3%), papilledema(44.7%), so called symptoms triad of the brain tumor, and other ophthalmic symptoms, gait disturbance, motor dysfunction,cerebellar sign in order.

A Clinical Analysis on the Pineal Region Tumors

The authors analyzed 24 cases of the pineal region tumors which were treated at the Department of Neurosurgery, Yonsei University during the last eight years. Tumors of the pineal region constitutes 2.5% of all brain tumors in authors' series. Most(79.1%) of the patients were younger than 20. Of these 24 cases, 19 were male and 5 were female. Presenting symptoms were headache, vomitting and clouding of sonsciousness due to obstructive hydrocephalus, followed by eye signs, such as Parinaud's syndrome, abducens palsy, and papilledema. Other signs are ataxia and hemiplegia. Plain skull films showed abnormal calcifications and findings of increased intracranial pressure. Location and size of the tumors were most accurately visualized by the brain CT scan. Pathological types of the pineal tumors could be differentiated by the brain CT scan from the patterns of contrast enhancement and tumor density. Among the 24 patients received VP shunt surgery for hydrocephalus. Eighteen patients had the radiation therapy either after the surgery or radiation alone. Germinoma was the most frequent type of pineal region tumors in this series, accounting for half of the patients who had the surgery. There was no immediate postoperative mortality, but one patient with pineoblastoma died from multiple spinal seeding nine months after the surgery. In conclusion, it is most important to identify the pathological type of the tumors in pineal region, because the prognosis of the patient as well as the mode of treatment varies according to the pathological diagnosis. Stereotaxic biopsy of the pineal region tumors is the most accurate diagnostic methods in authors' series among such other methods as brain CT scan, CSF cytology and/or tumor marker study.