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)

An Unusual Dermoid Cyst of the Pineal Region: Case Report in a Child

Introduction The pineal gland is a common location for intracranial germ cells, but dermoids are not commonly observed in this area. In the present paper, we discuss the clinical and radiological features as well as the treatment and outcome of this cyst in a 6-year- old child with a pineal dermoid cyst. Case Presentation The patient presented with chronic headache 6 months before admission in 2018. On the first admission, an enhanced lesion with a small cyst was detected in brain imaging. Magnetic resonance imaging (MRI) of the brain at follow-up (2 months after the first presentation) showed enlargement of the cyst size with compression on the adjacent structures. Radical excision of the tumor was performed after the endoscopic biopsy due to pressure exerted on the adjacent structures. Conclusion Dermoid cyst should be considered as a differential diagnosis for enhanced lesions of the pineal region.

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.

DESCARTES and his project of a fantasized brain / DESCARTES e seu projeto de um cérebro fantasioso

ABSTRACT. Interest in anatomy dates from the earliest times. Such knowledge was acquired through dissections of animals and human corpses by many researchers. The macroscopic anatomy of the varied structures of the brain were identified over the centuries, and the predominating solid substance was seen as amorphous, and devoid of any specific function, until the Renaissance. René Descartes, a personage with a brilliant and creative mind, conceived the brain, its structure and function, in a distinct manner to what was known at his time. He valued the solid matter and gave it, for the first time, a theoretical minute structure, related to a presumptive function based on the presence of the pineal gland and the animal spirits, underlying cognitive, sensory and motor activities. Such structural view was endorsed, in a given sense, by the microscopic findings of Marcello Malpighi, which begun to change the understanding of the nervous system.

RESUMO. O interesse pela anatomia data desde a mais remota antiguidade. Esse conhecimento foi adquirido por meio de dissecções em animais e cadáveres humanos, por muitos pesquisadores. A anatomia macroscópica de variadas estruturas do cérebro foi identificada com o passar dos séculos, e a substância sólida predominante foi vista como amorfa e destituída de qualquer função específica até o período do Renascimento. René Descartes, um personagem com uma mente brilhante e criativa, concebeu o cérebro, sua estrutura e função, de um modo distinto ao que era conhecido no seu tempo. Ele valorizou e deu à substância sólida, pela primeira vez, uma estrutura teórica formada por elementos minúsculos, relacionada a uma função presuntiva, baseada na presença da glândula pineal e dos espíritos animais, subjacentes às atividades cognitiva, sensorial e motora. Essa vista estrutural foi, de certa maneira, endossada pelos achados microscópicos de Marcello Malpighi, o que começou a modificar a compreensão do sistema nervoso.

Prevalence of pineal gland and choroid plexus calcification among iraqi patients attending CT scan units / Prevalencia de calcificación de la glándula pineal y del plexo coroideo entre pacientes iraquíes que asisten a las unidades de tomografía computarizada

SUMMARY: Pineal gland calcification is the most common physiological intracranial calcification followed by the choroid plexus calcification. The objective of the study was to determine the prevalence of the pineal gland and choroid plexus calcification among the Iraqi population attending computed tomography scan units in Baghdad, estimate the mean diameters of the pineal gland calcification, and to detect any correlation between these calcifications with age and sex. This multi-centric cross-sectional study examined 485 CT scans of Iraqi patients between the ages of 1 and 100 years attending CT scan units in the period 1 December 2018 to 1 April 2019. Descriptive and inferential statistics were used. The prevalence of pineal gland calcification was found to be 68 % with the 30-39 age group and male sex predominance. It was found to increase after the first decade of life without real consistency. The mean for pineal gland calcification anterior-posterior diameter was 4.55±2.13 and the mean of the right-left diameter was 3.95±1.54. These diameters were found to differ according to sex and age. Choroid plexus calcification was found to have a prevalence of 53.6 %. In most cases, choroid plexus calcification was found bilaterally (77.3 %). There was no difference in sex, but choroid plexus. In conclusion, calcification was noticed to increase gradually according to age. Both pineal gland and choroid plexus calcification have a relatively high prevalence. While pineal gland calcification formation was demonstrated to have a close relation to age and sex, choroid plexus calcification formation was noticed to relate only to age.

RESUMEN: La calcificación de la glándula pineal es la calcificación intracraneal fisiológica más común después de la calcificación del plexo coroideo. El objetivo del estudio fue determinar la prevalencia de calcificación de la glándula pineal y del plexo coroideo entre la población iraquí que asiste a las unidades de tomografía computarizada en Bagdad, estimar los diámetros medios de la calcificación de la glándula pineal y detectar la posible correlación entre estas calcificaciones con la edad y el sexo. Este estudio transversal multicéntrico examinó 485 tomografías computarizadas de pacientes iraquíes entre 1 y 100 años de edad que asistieron a unidades de tomografía computarizada en el período del 1 de diciembre de 2018 al 1 de abril de 2019. Se utilizaron estadísticas descriptivas e inferenciales. Se encontró una prevalencia de calcificación de la glándula pineal del 68 % con predominio del sexo masculino en el grupo de 30 a 39 años. Se observó que aumentaba después de la primera década de vida sin una coherencia real. La media del diámetro anteroposterior de la calcificación de la glándula pineal fue de 4,55 ± 2,13 y la media del DIÁ- METRO derecho-izquierdo fue de 3,95 ± 1,54; estos diámetros difieren según el sexo y la edad. La calcificación del plexo coroideo tiene una prevalencia del 53,6 %. En la mayoría de los casos, la calcificación del plexo coroideo se encontró de forma bilateral (77,3%). No hubo diferencia de sexo, no obstante en el plexo coroideo se observó que la calcificación aumentaba gradualmente según la edad. Tanto la calcificación de la glándula pineal como del plexo coroideo tienen una prevalencia relativamente alta. Si bien se demostró que la formación de calcificación de la glándula pineal está relacionada con la edad y el sexo, se observó que la formación de calcificación del plexo coroideo se relaciona solo con la edad.

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.

Uso da melatonina como curativo endodõntico em reimplante dentário de ratos pinealectomizados: estudo histométrico / Late dental replantation after the use of melatonia as an endodontic dressing: histological and morphometric study in pinealectomized rats

Dentre os vários tipos de traumas, a avulsão dentária representa uma condição crítica por deslocar o dente de seu ambiente natural, expondo-o a um meio externo seco e contaminado, resultando na reabsorção da raiz que pode ser parcial ou total. Pouco tem sido estudado sobre os fatores sistêmicos no processo de reparo em traumatismo dentário. Uma dessas condições é a deficiência da melatonina, que pode ocorrer em pessoas com hábitos noturnos. A sua deficiência tem sido relacionada a prejuízo no reparo de diversos tipos de tecidos, mas não existem estudos desta condição nos casos de traumas dentários. Assim o objetivo deste trabalho foi avaliar a influência da melatonina no processo de reparo em reimplante dentário. Para isso estudou-se o emprego de um curativo endodôntico à base de melatonina (M) ou corticosteroide e antibiótico (CA) no reimplante tardio de incisivos de ratos, pinealectomizados (PNX) ou não. Foram utilizados 40 ratos divididos em 4 grupos de 10 animais. Em 2 desses grupos (PNX-M e PNX-CA), a glândula pineal foi removida cirurgicamente. Trinta dias depois, o incisivo superior direito desses animais foi extraído, a polpa radicular foi removida, os canais foram limpos, irrigados e secos. Na sequência, nos Grupos PNX-M e M, os canais foram preenchidos com curativo a base de melatonina a 3%. Nos grupos PNX-CA e CA os canais foram preenchidos com curativo à base de Doxiciclina e Triancinolona. O ápice foi selado com um plug de MTA e os dentes foram reimplantados nos alvéolos de origem. O período extrabucal em meio seco foi de 20 minutos. Os animais receberam dose única de penicilina G Benzatina por via intramuscular. A eutanásia foi realizada 45 dias depois e as peças contendo os dentes foram processadas para obtenção de lâminas que foram coradas em hematoxilina e eosina para análise histológica e morfométrica. Os resultados demonstraram que o grupo PNX-M foi mais comprometido pela reabsorção radicular do que os grupos PNX-CA e CA. Quando analisado somente o tipo de reabsorção, a reabsorção inflamatória foi a que predominou (p< 0,05). Pode-se concluir que o curativo endodôntico com melatonina a 3% proporcionou reparo semelhante ao do curativo com corticosteroide antibiótico nos dentes reimplantados em animais não pinealectomizados. Nos animais pinealectomizados o curativo com a melatonina a 3% foi menos efetivo no controle da reabsorção radicular, principalmente a reabsorção inflamatória(AU)

Among the various types of trauma, tooth avulsion is a critical condition for displacing the tooth from its natural environment, exposing it to a dry and contaminated external environment, resulting in partial or total root resorption. Little has been studied about the systemic factors in the process of dental trauma repair. One such condition is melatonin deficiency, which can occur in people with nocturnal habits. Its deficiency has been related to impairment in the repair of various tissue types, but there are no studies of this condition in cases of dental trauma. Thus, the objective of this study was to evaluate the influence of melatonin on the repair process in dental replantation. For this purpose, we studied the use of a melatonin-based endodontic dressing in late reimplantation of rat incisors, pinealectomized or not. Forty rats divided into 4 groups of 10 animals were used. In 2 of these groups (PNX-M and PNX-CA), the pineal gland was surgically removed. Thirty days later, the right upper incisor of these animals was extracted, the root pulp was removed, the canals were cleaned, irrigated and dried. Then, in the PNX-M and M Groups, the channels were filled with a 3% melatonin-based dressing. In the PNX-CA and CA groups, the channels were filled with Doxycycline and Triamcinolone dressing. The apex was sealed with an MTA plug and the teeth were reimplanted into the original alveoli. The extraoral period in dry medium was 20 minutes. The animals received a single dose of intramuscular penicillin G benzathine. Euthanasia was performed 45 days later and the teeth containing parts were processed to obtain slides that were stained with hematoxylin and eosin for histological and morphometric analysis. The results showed that the PNX-M group was more affected by root resorption than the PNX-CA and CA groups. When analyzing only the type of resorption, inflammatory resorption predominated (p < 0.05). It can be concluded that the 3% melatonin endodontic dressing provided similar repair to the antibiotic corticosteroid dressing on the reimplanted teeth in non-pinealectomized animals. In pinealectomized animals the dressing with 3% melatonin was less effective in controlling root resorption, especially inflammatory resorption(AU)

Embryonal Carcinoma of the Pineal Gland Developed in an Adolescent Boy with Klinefelter Syndrome / 임상소아혈액종양

Klinefelter syndrome (KS) is characterized by small testes, gynecomastia, tall stature, and hypergonadotropic hypogonadism. This condition is associated with extra X chromosomes. It is well known that these aneuploidies predispose individuals to the development of several cancers. Moreover, there are many case reports that show KS patients to have a higher relative risk for the development of malignancy. However, incracranial germ cell tumor (ICGCT) associated with KS is very uncommon. Herein, we report delayed diagnosis of KS in a 15-year-old boy with ICGCT, embryonal carcinoma of the pineal gland, after multimodality treatment in Korea.

Changes of the Melatonergic System in Bipolar Disorder: A Literature Review / 우울ㆍ조울병

Recently, melatonergic agents have been gaining much interest in the treatment of mood disorders. The elucidation of the underlying biological mechanisms related to the melatonergic system in mood disorders is warranted to ensure the proper use of melatonergic agents. Changes of the melatonergic system have been investigated in several studies of patients with bipolar disorder (BP) and depression. Accumulating evidence has indicated that patients with BP might exhibit abnormal melatonin secretion patterns, increased light-induced melatonin suppression, altered pineal gland volume, genetically abnormal melatonin synthesis enzyme, and modified melatonin receptors. In this review, the findings of studies performed to explore the association between the melatonergic system and BP are discussed. Moreover, the interpretations and limitations of these findings are described.

Effect of melatonin on the onset of puberty in male juvenile rats / 대한해부학회지

Melatonin or N-acetyl-5-methoxytryptamine, the fascinating molecule secreted by the pineal gland. Melatonin has a close interaction with hypothalamic-pituitary-gonadal axis. In non-seasonal breeders like rat its exact role in reproduction is controvertible. So it is worth to explore the possible role of melatonin on the onset of puberty in male albino rats. Two groups of male rats aged 5 and 10 days were used for the study. In each group, there were three subgroups, each receiving melatonin for 5 days, 10 days or till the day of descent of testes. Similar subgroups were used as controls. Without handling, animals were observed daily for the onset of puberty. On the day of descent of testes, body weight of the animal was noted, blood was collected, serum was separated and used for radio immunoassay. For histomorphometric analysis, all morphometric measurements were done using an occular micrometer. Volume fraction of seminiferous tubules, intertubular connective tissue of testes, cortex and medulla of thymus were estimated by point count method. In both the age groups melatonin advanced the age on descent of testes, increased the body weight, organ weight. It also increased the serum hormone levels. So, in conclusion this study indicates that exogenous melatonin advances the onset of puberty in male albino wistar rats and this effect is more pronounced in the younger animals.

Meningioma of the Pineal Region ­ Differential Radiologic Aspects of Pineal Region Tumors Based on a Clinical Case

Pineal region tumors are uncommon among neoplasm of the central nervous system, with this region being the most heterogeneous in terms of histological types. Meningiomas are rarer still, but can be found at this site, with origins in either the velum interpositum or falcotentorial junction. Neuroimaging exams can distinguish malignant from benign lesions besides helping to define the origin of the lesion as the pineal parenchymal or surrounding structures. We report the case of a woman with a pineal region tumor in which differential diagnoses included meningioma and germinoma, with confirmation of the former based on radiological characteristics and histopathology. In addition, a brief review of differential diagnoses and approaches for cases of lesions in this region is provided.

Os tumores da região da pineal apresentam uma baixa frequência entre as neoplasias do sistema nervoso central, sendo esta região a mais heterogênea em termos de tipos histológicos possíveis. Meningiomas são lesões ainda mais raras, porém possíveis de advirem desta localização, sejam originados do velum interpositum ou da junção falcotentorial. Os exames de neuroimagem permitem distinguir lesões malignas de benignas além de auxiliar na definição entre origem do parênquima pineal ou de estruturas adjacentes. Apresentamos o caso de uma mulher adulta com uma neoplasia da região da pineal cujos diagnósticos diferenciais incluíram meningioma e germinoma, evidenciando-se pelas características radiológicas e resultados histopatológicos tratar-se do primeiro. Além disso, fazemos uma breve revisão a respeito dos diagnósticos diferencias e condutas frente a uma lesão desta região.

Pineal and Suprasellar Germinoma Cooccurence with Vertebra Plana: A Case Report

Germinoma is the most common type of intracranial germ cell tumors (GCTs). Pineal gland and suprasellar region are the most frequent sites of central nervous system (CNS) involvement. Intracranial masses caused by Langerhans cell histiocytosis (LCH) mimics features of CNS GCTs. LCH frequently involve spine and is the most common cause of vertebra plana in children. A 15-year-old boy presented with progressing symptoms of polydipsia, polyuria, general headache, nausea and severe back pain. Brain MRI showed brain tumor with simultaneous involvement of suprasellar region and pineal gland. An excisional biopsy of suprasellar mass was done. The pathologic assessment confirmed the diagnosis of germinoma. Patient's treatment continued accordingly. A spine MRI, done due to persistent backache, showed a vertebra plana. We reevaluated the primary diagnosis suspecting LCH. Germinoma of CNS was confirmed and a biopsy of vertebral lesion resulted in hemangioma. Thus we report a case of CNS germinoma with co-occurrence of vertebra plana. We emphasized the importance of histopathologic diagnosis of pineal/suprasellar masses and primary investigation of other CNS regions including spine for possible metastasis or comorbidities.

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.

Papillary Tumor of the Pineal Region: Case Report and Literature Review

Papillary tumor of the pineal region (PTPR) is a neuroectodermal tumor thought to originate from cells of the subcommissural organ. Its oncologic properties are still under investigation, as well as the most suitable therapeutic measures for this type of neoplasm.We report the case of a 36-year-old woman with a 1-year history of headache and intermittent diplopia. The magnetic resonance imaging (MRI) scan showed a heterogeneously enhancing mass in the pineal region that caused an acute hydrocephalus, and an emergency shunt derivation was necessary. One week later, the patient was submitted to subtotal tumor resection, and remained asymptomatic in the post-operative period. In the follow-up, the patient remained asymptomatic; in the imaging control 3.5 years after the surgical resection, local recurrence was identified, and the patient was submitted to a local radiation protocol. Our literature review showed an early clinical onset due to intracranial hypertension signs. Definitive clinical onset might be reached only through a histopathological examination. Gross total resection followed by radiotherapy is the current standard of care. Local recurrence is often observed, with rare dissemination to the cerebral spinal fluid. The natural history of the PTPR remains unknown, as well as the best treatment strategy. Large case series with longer follow-ups are necessary for further conclusions.

Daily melatonin administration improves osseointegration in pinealectomized rats

Abstract The hypothesis of this study was that the peri-implant bone healing of the group of pinealectomized rats would differ from the control group. The samples were subjected to immunohistochemical, microtomographic (total porosity and connectivity density), and fluorochrome (mineralized surface) analyses. Objectives The goal of this study was to investigate the cellular changes and bone remodeling dynamics along the bone/implant interface in pinealectomized rats. Material and Methods The total of 18 adult male rats (Rattus norvegicus albinus, Wistar) was divided into three groups (n=6): control (CO), pinealectomized without melatonin (PNX) and pinealectomized with melatonin (PNXm). All animals were submitted to the first surgery (pinealectomy), except the CO group. Thirty days after the pinealectomy without melatonin, the second surgery was conducted, in which all animals received an implant in each tibia (36 titanium implants with surface treatment were installed - Implalife® São Paulo, SP, Brazil). By gavage, the rats of the PNX group received the vehicle solution, and the procedure. Results Immunohistochemical analysis for runt-related transcription factor 2 (RUNX2), alkaline phosphatase (ALP), osteopontin (OP) and osteocalcin (OC) showed that the bone repair process in the PNXm group was similar to that of the CO group, whereas the PNX group showed a delay. The microtomographic parameters of total porosity [Po(tot)] and bone surface (BS) showed no statistically significant differences, whereas for the connective density (Conn.Dn) a statistical difference was found between the CO and PNXm groups. Fluorochrome analysis of the active mineralized surface showed statistically significant difference between the CO and PNX and between the CO and PNXm groups. Conclusion The absence of the pineal gland impaired the bone repair process during osseointegration, however the daily melatonin replacement was able to restore this response.

Basal cell carcinoma, odontogenic cysts, brain and skeletal abnormalities (Gorlin Goltz Syndrome) in a 46-year-old woman

@#<p style="text-align: justify;"><strong>OBJECTIVE:</strong> To present the case of a 46-year-old woman with basal cell carcinoma, odontogenic cysts, brain anomalies and skeletal abnormalities.<br /><strong>METHODS:</strong><br /><strong>Design:</strong> Case Report<br /><strong>Setting:</strong> Tertiary National University Hospital<br /><strong>Patient:</strong> One<br /><strong>RESULTS:</strong> A 46-year-old woman consulted for a non-healing, necrotic left orbital ulcer that started as a skin-colored, papilla-like lesion on the upper eyelid. There were also hyperpigmented lesions with ill-defined borders over both paranasal areas. Tissue biopsies revealed basal cell carcinoma. Radiologic imaging showed cystic lesions in the mandible, straightening of cervical vertebrae and calcifications of the falx cerebri, tentorium cerebelli, pineal gland and choroid plexus. Based on established major and minor clinical and radiologic criteria, we arrived at a diagnosis of Gorlin Goltz Syndrome or Nevoid Basal Cell Carcinoma Syndrome (NBCCS). She underwent wide excision of the left orbital mass with exenteration, excision of left and right paranasal masses, left total parotidectomy with facial nerve preservation, enucleation of mandibular cyst and cervicofacial reconstruction with skin grafts of the left orbital area and ala.<br /><strong>CONCLUSIONS:</strong> NBCCS is a rare autosomal dominant disorder with a high tendency for neoplasms and developmental anomalies. Diagnosis can easily be missed if the physician is unaware of its classic but bizarre presentation. Early recognition and prompt specialist referral is very important in order to prevent complications and provide better prognosis. Patients should be reminded of the importance of follow-up as other presentations of the syndrome may manifest later in life and family genetic screening and counseling should be undertaken.</p>

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>

Basal cell carcinoma, odontogenic cysts, brain and skeletal abnormalities (Gorlin Goltz Syndrome) in a 46-year-old woman

OBJECTIVE: To present the case of a 46-year-old woman with basal cell carcinoma, odontogenic cysts, brain anomalies and skeletal abnormalities.METHODS:Design: Case ReportSetting: Tertiary National University HospitalPatient: OneRESULTS: A 46-year-old woman consulted for a non-healing, necrotic left orbital ulcer that started as a skin-colored, papilla-like lesion on the upper eyelid. There were also hyperpigmented lesions with ill-defined borders over both paranasal areas. Tissue biopsies revealed basal cell carcinoma. Radiologic imaging showed cystic lesions in the mandible, straightening of cervical vertebrae and calcifications of the falx cerebri, tentorium cerebelli, pineal gland and choroid plexus. Based on established major and minor clinical and radiologic criteria, we arrived at a diagnosis of Gorlin Goltz Syndrome or Nevoid Basal Cell Carcinoma Syndrome (NBCCS). She underwent wide excision of the left orbital mass with exenteration, excision of left and right paranasal masses, left total parotidectomy with facial nerve preservation, enucleation of mandibular cyst and cervicofacial reconstruction with skin grafts of the left orbital area and ala.CONCLUSIONS: NBCCS is a rare autosomal dominant disorder with a high tendency for neoplasms and developmental anomalies. Diagnosis can easily be missed if the physician is unaware of its classic but bizarre presentation. Early recognition and prompt specialist referral is very important in order to prevent complications and provide better prognosis. Patients should be reminded of the importance of follow-up as other presentations of the syndrome may manifest later in life and family genetic screening and counseling should be undertaken.

Melatonin Rescues Human Dental Pulp Cells from Premature Senescence Induced by H₂O₂

Although anti-aging activities of melatonin, a hormone secreted by the pineal gland, have been reported in senescence-accelerated mouse models and several types of cells, its impact and mechanism on the senescence of human dental pulp cells (HDPCs) remains unknown. In this study, we examined the impact of melatonin on cellular premature senescence of HDPCs. Here, we found that melatonin markedly inhibited senescent characteristics of HDPCs after exposure to hydrogen peroxide (H₂O₂), including the increase in senescence-associated β-galactosidase (SA-β-gal)-positive HDPCs and the upregulation of p21 protein, an indicator for senescence. In addition, as melatonin attenuated H₂O₂-stimulated phosphorylation of c-Jun N-terminal kinase (JNK), while selective inhibition of JNK activity with SP600125 significantly attenuated H₂O₂-induced increase in SA-beta-gal activity. Results reveal that melatonin antagonizes premature senescence of HDPCs via JNK pathway. Thus, melatonin may have therapeutic potential to prevent stress-induced premature senescence, possibly correlated with development of dental pulp diseases, and to maintain oral health across the life span.

Isolated Trochlear Palsy Due to Pilocytic Astrocytoma Involving the Pineal Gland

No abstract available.