Tumor gigante de células germinales: un abordaje quirúrgico / Giant Mediastinal Germ Cell Tumor: a Surgical Approach

Los tumores de células germinales (TCGs) se forman a partir de células embrionarias y generalmente se presentan en pacientes de entre 11 y 30 años de edad. Los TCG pue-den presentarse como tumores extragonadales, siendo el mediastino anterior el sitio más común en el 50 a 70% de los casos. Presentamos a un paciente masculino de 21 años con un tumor sólido mediastinal de 17 x 15 cm que, de acuerdo a la tomografía de tórax (TC), ocupaba toda la cavidad torácica izquierda desplazando el corazón ha-cia la cavidad torácica derecha. El estudio patológico fue reportado por el patólogo co-mo un TCG.

Germ cell tumors (GCTs) are formed from embryonic cells and usually occur in patients between age 11 and 30 years. GCT can present as extra-gonadal tumors, with the an-terior mediastinum being the most common site in 50 to 70% of cases. We present a 21-year-old male patient with a solid mediastinal tumor of 17 x 15 cm that, according to the chest tomography (CT), it was occupying the entire left thoracic cavity moving the heart towards the right thoracic cavity. The pathological study was reported by the pathologist as a GCT tumor.

Encefalitis por anticuerpos contra el receptor N-metil-D-aspartato secundaria a teratoma ovárico en una paciente pediátrica / Encephalitis due to antibodies against the N-methyl-D-aspartate receptor secondary to ovarian teratoma in a pediatric patient

La encefalitis por anticuerpos contra el receptor N-metilD-aspartato es un proceso inmunomediado en el que autoanticuerpos se dirigen contra la subunidad GluN1 del receptor de glutamato del sistema nervioso central. Se caracteriza por la aparición aguda o subaguda de síntomas psiquiátricos, como confusión, pérdida de la memoria a corto plazo, cambios de conducta, catatonía, seguidos por manifestaciones neurológicas, tales como convulsiones, alteraciones del movimiento, disfunciones autonómicas, coma y depresión respiratoria. Es grave y potencialmente mortal. Su asociación con teratoma de ovario como síndrome paraneoplásico fue descrita en mujeres jóvenes. En la población pediátrica, es mucho menos frecuente y se reporta en comunicaciones de 1 o 2 pacientes y en series de pocos casos. Se presenta una paciente de 13 años con encefalitis paraneoplásica por anticuerpos contra el receptor N-metil-Daspartato, secundaria a un teratoma ovárico maduro.

The encephalitis due to antibodies against the N-methylD-aspartate receptor is a process immune-mediated in which antibodies are directed against the GluN1 subunit of the glutamate receptor in the central nervous system. It is characterized by an acute or subacute onset of psychiatric symptoms such as confusion, short-term memory loss, behavioral changes, catatonia followed by neurological manifestations such as seizures, movement disturbances, autonomic dysfunctions, coma, and respiratory depression. It is serious and life threatening. Its association with ovarian teratoma as a paraneoplastic syndrome was described in youngwomen. In the pediatric population it is much less frequent and is reported in publications of one or two patients and in series of few cases. We present a 13-year-old patient with encephalitis paraneoplastic due to antibodies against the N-methyl-Daspartate receptor, secondary to a mature ovarian teratoma.

Cistoadenoma volumoso associado a teratoma ovariano maduro, com evolução para pseudomixoma peritoneal: relato de caso / Giant cystadenoma associated with mature ovarian teratoma with progression to pseudomyxoma peritonei: a case report

Massas anexiais representam grande desafio diagnóstico, devido a seu caráter geralmente assintomático¹. Traz-se caso peculiar de paciente que evoluiu com sintomas exuberantes de rápida evolução, situação característica de cistoadenomas, tumores benignos de origem epitelial²,³. Realizada laparotomia, com achados macroscópicos que revelaram conteúdo mucinoso e presença de pelos e neovascularização em seu interior. O anatomopatológico confirmou o diagnóstico de cistoadenoma associado a teratoma ovariano, sendo assim considerado um tumor de colisão ovariana.

Adnexal masses represent a major diagnostic challenge due to its usually asymptomatic characteritcs¹. We present a peculiar case of a patient who evolved with exuberant symptoms of rapid evolution, characteristic of cystadenomas, benign tumors of epithelial origin²,³. Laparotomy was performed, with macroscopic findings that revealed mucinous content and the presence of hairs and neovascularization inside. Pathology confirmed the diagnosis of cystadenoma associated with ovarian teratoma, thus being considered an ovarian collision tumor.

Teratoma ovariano como causa de dor abdominal em criança na idade pré-escolar / Ovarian teratoma as a cause of abdominal pain in preschool children

Introdução: Os tumores ovarianos, raros na infância, apresentam maior incidência entre 8 e 9 anos, tendo pico aos 19 anos. A sintomatologia principal manifesta-se por dor abdominal, massa palpável, febre, constipação e mais raramente polaciúria e disúria. Os teratomas ovarianos maduros embora sejam neoplasias majoritariamente benignas, se malconduzidas, podem evoluir para emergências cirúrgicas. Objetivo: Analisar o teratoma ovariano maduro como possível etiologia de dor abdominal crônica em pacientes pediátricos. Relato de Caso: Criança, 2 anos e 9 meses, levada ao atendimento médico com relato de dor abdominal crônica, 3 meses de evolução, com períodos de agudização, de forte intensidade, associada à disúria e polaciúria conduzida como principal suspeita infecção do trato urinário (ITU). A partir da clínica compatível, associado a leucocitúrias, mesmo não havendo crescimento bacteriano nas culturas, devido à recorrência do quadro, seguiu-se a propedêutica adequada de investigação de ITU de repetição, através do rastreio de má formações das vias urinárias. O ultrassom abdominal total com dinâmica do trato urinário constatou presença de volumosa formação expansiva originada provavelmente em ovário esquerdo. Estendida a pesquisa diagnóstica foi realizada tomografia computadorizada do abdome e pelve com achados sugestivos de teratoma ovariano esquerdo, exercendo comportamento expansivo local com desvio e compressão de estruturas anatômicas circunvizinhas. Com a hipótese diagnóstica evidenciada foi encaminhada à cirurgia pediátrica e oncologia para tratamento adequado, com realização de ooforectomia e seguimento clínico. Conclusão: Importância da propedêutica adequada para casos de dor abdominal crônica em pacientes pediátricos devido à extensão de possíveis diagnósticos diferenciais.

Introduction: Ovarian tumors, rare in childhood, have a higher incidence between 8 and 9 years, peaking at 19 years. The main symptomatology is manifested by abdominal pain, palpable mass, fever, constipation, and more rarely polaciuria and dysuria. Mature ovarian teratomas although they are mostly benign neoplasms, if poorly conducted, they can progress to surgical emergencies. Objective: To analyze mature ovarian teratoma as a possible etiology of chronic abdominal pain in pediatric patients. Case Report: Child, 2 years and 9 months, taken to medical care with report of chronic abdominal pain, three months of evolution, with periods of sharpness, of strong intensity, associated with dysuria and polaciuria and primary suspected urinary tract infection (UTI). From the compatible clinic, associated with leukocytes, though there is no bacterial growth in the cultures, due to the recurrence of the condition, appropriate propaedeutic investigation of repeat UTI was followed by screening for urinary pathway malformations. Total abdominal ultrasound with urinary tract dynamics was requested, which that found the presence of a large expansive formation probably originating in the left ovary. Extended diagnostic research, the computed tomography of the abdomen and pelvis was performed with findings suggestive of left ovarian teratoma exerting local expansive behavior with deviation and compression of surrounding anatomical structures. With the diagnostic hypothesis evidenced, she was referred to pediatric surgery and oncology for appropriate treatment with oophorectomy and clinical follow-up. Conclusion: Importance of adequate propaedeutics for cases of chronic abdominal pain in pediatric patients due to the extent of possible differential diagnoses.

Teratomas del mediastino / Mediastinum Teratomas

RESUMEN Introducción: Los teratomas se definen como tumores de tejidos extraños al órgano o sitio anatómico en el cual se originan. Los teratomas mediastinales no son frecuentes, representan alrededor del 5 por ciento al 10 por ciento de todos los tumores mediastinales. Objetivo: Describir los resultados del tratamiento quirúrgico de pacientes con diagnóstico de teratomas mediastinales. Métodos: Se estudiaron 12 pacientes tratados entre enero de 2001 y diciembre de 2018. Las variables evaluadas fueron sexo, edad, tipo histológico: maduro o inmaduro, vía de acceso quirúrgico, accidentes quirúrgicos y evolución postoperatoria. Resultados: Correspondieron 9 al sexo femenino y 3 al masculino. El promedio de edad fue de 33,3 años (17-60 años). Hubo predominio absoluto del tipo maduro (11). La esternotomía media fue el acceso más frecuente. La toracotomía se realizó cuando el tumor, voluminoso, ocupaba la mayor parte de un hemitórax. Los accidentes quirúrgicos fueron un desgarro pulmonar y una apertura del pericardio. De dos pacientes tratados mediante cirugía torácica videoasistida, uno fue convertido por sangrado venoso molesto. Al año de seguimiento todos estaban vivos, sin evidencias de recidiva. Conclusiones: Contrariamente a lo esperado, hay predominio del sexo femenino, mientras que la edad y el tipo histológico coinciden con la literatura. La esternotomía, aún hoy, es comúnmente aceptada, a pesar del auge de la cirugía torácica videoasistida. La resección total produce resultados excelentes para los teratomas benignos(AU)

ABSTRACT Introduction: Teratomas are defined as tumors of tissues foreign to the organ or anatomical site in which they originate. Mediastinal teratomas are rare, accounting for about 5-10 percent of all mediastinal tumors. Objective: To describe the outcomes of the surgical treatment of patients diagnosed with mediastinal teratomas. Methods: Twelve patients treated between January 2001 and December 2018 were studied. The variables evaluated were sex, age, histological type (mature or immature), surgical access route, surgical accidents, and postoperative evolution. Results: Nine patients corresponded to the female sex and three, to the male. The average age was 33.3 years (17-60 years). There was an absolute predominance of the mature type (11). Median sternotomy was the most frequent access. Thoracotomy was performed when the bulky tumor occupied most of a hemithorax. The surgical accidents were lung tear and opening of the pericardium. Of two patients treated by video-assisted thoracic surgery, one was converted for bothersome venous bleeding. At one year of follow-up, all were alive, with no evidence of recurrence. Conclusions: Contrary to expectations, there is predominance of the female sex, while age and histological type coincide with the literature. Sternotomy, even today, is commonly accepted, despite the rise of video-assisted thoracic surgery. Total resection produces excellent outcomes in benign teratomas(AU)

Oral hairy polyp in a teenager: case report and literature review / Pólipo piloso oral em um adolescente: relato de caso e revisão de literatura

ABSTRACT Objective: To report an unusual case of oral hairy polyp (HP) and review the literature, providing epidemiological, clinical and histopathological information on this disease. Case description: A 12-year-old male patient was referred to a Stomatology department with a nodule in the posterior midline of the tongue. The patient did not know exactly when it arose or whether it had grown since then. Clinical exam revealed a bulky and mobile pedunculated mass lesion on the dorsum of the tongue, with a diameter of approximately 1 cm. The patient's mother reported no previous health problem. An excisional biopsy was performed, the surgical specimen was sent for anatomopathological analysis, and the findings were compatible with the diagnosis of HP. Comments: Hairy polyp is a rare lesion, especially in the oral region. The literature search revealed only 10 case reports of oral HP published between January 1999 and January 2019, and they revealed a predominance of the disease in female newborns. Two uncommon facts were presented in this case: the patient was male and diagnosis was made at 12 years old.

RESUMO Objetivo: Relatar um caso incomum de pólipo piloso (PP) oral e revisar a literatura para trazer informações epidemiológicas, clínicas e histopatológicas acerca da doença. Descrição do caso: Paciente do sexo masculino, 12 anos de idade, encaminhado ao Departamento de Estomatologia com nódulo na região posterior de linha média da língua. O paciente não soube relatar quando surgiu a lesão e se ela havia crescido desde então. O exame clínico revelou massa pedunculada, volumosa e móvel no dorso da língua, medindo aproximadamente 1,0 cm de diâmetro. A mãe do paciente relatou que ele nunca havia tido nenhum problema de saúde anterior. Foi realizada biópsia excisional e o material foi enviado para análise anatomopatológica, sendo os achados compatíveis com o diagnóstico de PP. Comentários: O pólipo piloso é uma lesão rara, especialmente na região oral. A pesquisa bibliográfica revelou apenas dez relatos de casos de PP oral, publicados entre janeiro de 1999 e janeiro de 2019, sendo observada predominância da doença em recém-nascidos do sexo feminino. Dois fatos incomuns ocorreram neste caso: tratava-se de um paciente do sexo masculino e o diagnóstico se deu aos 12 anos de idade.

Adolescente con teratoma mediastinal y compromiso pericárdico / Adolescente com teratoma mediastinal e envolvimento pericárdico / Adolescent with mediastinal teratoma and pericardial involvement

Se presentó un paciente de 14 años con antecedentes de salud que acudió a consulta por presentar dolor torácico de 6 días de evolución sin irradiación y con ausencia de fiebre, disnea u otros síntomas. En la radiografía de tórax se observó una masa mediastinal anterior y media que se confirmó con la tomografía computarizada y donde la localización y las características imagenológicas sugirieron el diagnóstico de teratoma mediastinal. Se realizó exéresis del tumor que comprometía también parte del pericardio, confirmándose histológicamente por anatomía patológica la presencia de un teratoma quístico maduro del mediastino(AU)

A 14-year-old patient with a history of health who attended the clinic due to chest pain that lasted six days without irradiation and with absence of fever, dyspnea or other symptoms. The chest radiograph showed an anterior and middle mediastinal mass that was confirmed with computed tomography and where the location and imaginal characteristics suggested the diagnosis of mediastinal teratoma. The excision of the tumor that also compromised part of the pericardium was performed, confirming histologically by pathological anatomy the presence of a mature cystic mediastinal teratoma(AU)

Um paciente de 14 anos de idade, com histórico médico que se apresentou na clínica devido a dor torácica de seis dias de evolução sem irradiação e sem febre, dispnéia ou outros sintomas, foi apresentado. A radiografia de tórax mostrou uma massa mediastinal anterior e média que foi confirmada com tomografia computadorizada e onde a localização e características de imagem sugeriram o diagnóstico de teratoma mediastinal. O tumor foi extirpado, o que também comprometeu parte do pericárdio, e a presença de um teratoma cístico mediastinal maduro foi confirmado histologicamente por anatomia patológica(AU)

Teratoma tímico productor de beta-HCG: una causa infrecuente de pubertad precoz periférica / Beta-hCG-producing thymic teratoma: an uncommon cause of peripheral precocious puberty

INTRODUCCIÓN: Entre las causas de pubertad precoz periférica en el varón están los tumores secretores de betagonadotrofina coriónica humana (Β-HCG), como hepatoblastomas, disgerminomas, corio-carcinomas y teratomas inmaduros. En pediatría los teratomas mediastínicos son raros, representan el 7-10% de los teratomas extragonadales. OBJETIVO: Describir caso de un paciente que cursa con pubertad precoz periférica debida a teratoma tímico secretor de Β-HCG. CASO CLÍNICO: Escolar masculino de 7 años 10 meses consultó por cuadro de 3 meses de cambios de la voz, ginecomastia, aparición de vello pubiano y aumento de volumen genital. En exámenes destacaba edad ósea de 9 años, testosterona total 9,33ng/ml (< 0,4ng/ml), dehidroepiandrosterona sulfato (DHEAS), 17-hidroxi-progesterona (17-OH-P) y prueba de hormona adrenocorticotrofina (ACTH) normales, hormona luteinizante (LH) y hormona folículo estimulante (FSH) basales bajas, Β-HCG 39,5mU/ml (< 2,5 mUI/ml), alfa fetoproteína (α-FP) 11,2ng/ml (0,6-2,0 ng/ml). Estudio de imágenes para determinar origen de secreción de Β-HCG incluye: ecografía testicular y tomografla axial computarizada (TAC) torácica, abdominal y pelviana normales; resonancia cerebral y selar sin hallazgos significativos. Tomografía por emisión de positrones/tomografía computada (PET SCAN) evidenció imagen de tumor en mediastino antero-superior. Se resecó el tumor, cuya biopsia evidenció teratoma quístico inmaduro en timo. Evolución postoperatoria fue satisfactoria, con normalización de niveles hormonales. CONCLUSIÓN: La presentación de un teratoma en paciente pediátrico es infrecuente, aún más, si es inmaduro, su localización es tímica y es secretor de Β-HCG. Es relevante considerarlo dentro de diagnósticos diferenciales frente a pubertad precoz, de modo que pueda efectuarse un manejo oportuno.

INTRODUCTION: Among the causes of peripheral precocious puberty in men are the beta- human cho rionic gonadotropin (Β-HCG)-secreting tumors, such as hepatoblastomas, dysgerminomas, chorio carcinomas, and immature teratomas. In pediatrics, the mediastinal teratomas are rare, representing the 7-10% of extragonadal teratomas. OBJECTIVE: To describe the case of a patient with peripheral precocious puberty due to a Β-HCG -secreting thymic teratoma. CLINICAL CASE: A seven-years-old schoolboy presents a three-months history of voice changes, gynecomastia, pubic hair appearance, and increased genital volume. In the exams, bone age of nine years, total testosterone 9.33ng/ml (< 0.4ng/ml), dehydroepiandrosterone sulfate (DHEAS), 17-hydroxyprogesterone (17-OHP), and normal adrenocorticotropic hormone (ACTH) test stand out; luteinizing hormone (LH) and follicle stimulating hormone (FSH) with low basal levels, P-HCG 39.5mU/ml (< 2.5 mUI/ml), alpha feto protein (Α-FP) 11,2ng/ml (0.6-2.0 ng/ml). Imaging study to determine the origin of P-HCG secretion shows normal testicular ultrasound and thoracic, abdominal, and pelvic computerized axial tomo graphy (CAT); brain and sellar resonance without significant findings. The positron emission tomography/computed scan (PET SCAN) shows a tumor image in the anterosuperior mediastinum. The tumor is resected, and the biopsy shows an immature cystic teratoma in the thymus. Post-operatory evolution was satisfactory, with normalization of hormonal levels. CONCLUSION: The appearance of a teratoma in a pediatric patient is rare, even more if it is immature, with thymic location and Β-HCG- secretor. It is important to consider it within the differential diagnosis facing precocious puberty, as a better way to handle appropriately.

Thymic teratoma presenting as non-immune hydrops fetalis

Teratomas are one of the most frequent tumors in the pediatric population. They occur anywhere along the midline of the body, following the course of the embryonic germ cell ridge. In the mediastinal location, they exert space occupying effects, leading to a myriad of complications, including non-immune hydrops fetalis. We describe a fatal case of an immature thymic teratoma in a neonate presenting with hydrops fetalis. This case emphasizes the importance of early diagnosis and surgical intervention in such cases.

Teratoma inmaduro (maligno) de ovario / Ovarian immature (malignant) teratoma

Resumen El teratoma inmaduro se describió por primera vez en 1960 y puede ser puro o estar mezclado con un componente maduro. Es la segunda neoplasia maligna más común de células germinales de todos los cánceres de ovario (representa <1%). Alrededor del 50% de los teratomas inmaduros puros del ovario ocurren en mujeres entre las edades de 10 y 20 años. Se debe considerar el tratamiento para preservar su fertilidad futura porque la mayoría de los tumores de células germinales de ovario son curables con la cirugía conservadora y la quimioterapia combinada de seguimiento. La mayoría de los pacientes diagnosticados con un teratoma inmaduro se curan de su enfermedad. Sin embargo en todas las pacientes, se recomienda un seguimiento cercano, particularmente en los primeros 2 años después del diagnóstico, donde existe una mayor probabilidad de recurrencia.

Abstract The immature teratoma was first described in 1960 and cay be pure or mixed with a mature component. It is the second most common malignant germ cell neoplasm of all ovarian cancers (representing <1%). About 50% of pure immature teratomas of the ovary occur in women between the ages of 10 and 20 years. Treatment should be considered to preserve future fertility because the majority of ovarian germ cell tumors are curable with conservative surgery and combined combination chemotherapy. Most patients diagnosed with an immature teratoma are cured of their disease. However, in all patients, we recommend close follow-up, particularly in the first 2 years after diagnosis, where there is a greater chance of recurrence.

Loss of expression of the Neural Cell Adhesion Molecule 1 (NCAM1) in atypical teratoid/rhabdoid tumors: a new diagnostic marker?

Background: Atypical teratoid/rhabdoid tumors (AT/RT) are aggressive embryonal tumors of the central nervous system. They are largely characterized by inactivating mutations of the SMARCB1 tumor suppressor gene. AT/RT patients have a very poor prognosis and no standard therapeutic protocol has been defined yet. Recently, multimodal therapy with multiple drug combinations has slightly improved the overall survival, however drug toxicity remains high. In this scenario, a better understanding of the pathophysiology of the disease is needed. Methods: We evaluated the gene expression profile of AT/RT samples to find new genetic factors contributing to the pathophysiology of the disease. We found target genes significantly differentially expressed between AT/RT and medulloblastoma (MB), the most common embryonal brain tumor. The mRNA expression was validated by quantitative real-time PCR and, at the protein level, expression was validated by immunohistochemistry in an independent set of tumors. Results: The Neural cell adhesion molecule 1 (NCAM1) gene was found to be consistently downregulated in AT/RT samples when compared to MB and normal brain tissue. Immunohistochemistry showed that the expression of NCAM1 in AT/RT was significantly lower than that of MB. Conclusion: NCAM1 is an important molecule involved in neuron-to-neuron and neuron-to-muscle adhesion during development. Downregulation of NCAM1 has been implicated in several human cancers suggesting that it might have a tumor repressor role. In this study we found a significantly reduced expression of NCAM1 in AT/RT when compared to MB and we suggest that this feature can be used as a diagnostic marker, along with demonstration of SMARCB1 (INI1) or SMARCA4 (BRG1) inactivation. The roles of NCAM1 in the pathophysiology of AT/RT are still to be determined (AU)

Teratoma sacrococcígeo: diagnóstico, evaluación y terapia prenatal / Sacrococcygeal teratoma: diagnosis, evaluation and prenatal therapy

El teratoma sacrococcígeo representa la lesión tumoral más frecuente durante la gestación. Si bien su frecuencia es baja, en asociación con hidrops puede determinar la aparición de insuficiencia cardiaca fetal y posterior muerte fetal in útero. La evaluación prenatal mediante ultrasonografía, debe orientarse hacia la selección de aquellos fetos candidatos a intervención prenatal para mejorar el resultado perinatal de fetos con compromiso hemodinámico que se encuentran bajo la viabilidad. El objetivo de la presente actualización es exponer las herramientas actualmente en uso para realizar dicha evaluación y los resultados de la cirugía prenatal. La puntuación del perfil cardiovascular fetal mediante la ecografía, se propone como una herramienta sensible y útil en la selección de fetos candidatos a intervención prenatal. Tanto la cirugía abierta como el abordaje mínimamente invasivo se exponen como alternativas en el manejo prenatal de fetos candidatos a intervención con sobrevida reportada en torno al 50 y 44 por ciento respectivamente.

The sacrococcygeal teratoma is the most common tumor during prenatal period. Although its frequency is low, together with hydrops could determine the occurrence of fetal heart failure, and subsequent fetal death in utero. Prenatal ultrasonographic evaluation, should be directed toward selection of those fetuses as candidates for prenatal therapy, to improve their perinatal outcome, when they present hemodynamic compromise while they are under fetal viability. The purpose of this update is to expose the tools currently used to conduct the assessment and the results of prenatal surgery. The fetal cardiovascular profile score by ultrasound is proposed as a sensible and useful tool in the selection of candidates for prenatal intervention. Both open surgery as minimally invasive approach are presented as alternatives in the prenatal management of fetus as candidates to intervention, has been reported a survival about 50 and 44 percent respectively.

Atypical Teratoid Rhabdoid Tumor in a Child: Case Report / Tumor teratóide rabdóide atípico emuma criança: relato de caso

Atypical teratoid/rhabdoid tumor (ATRT) is a rare and aggressive type of embryonal tumor of the central nervous system (CNS) occurring in childhood. The present study aims to describe the case of a 16-year-old girl who presented with an occipital mass lesion that was diagnosed as ATRT.We present a brief review of the current knowledge of the treatment of this rare neoplasm. A previously healthy 16-year-old girl was referred after two episodes of partial complex seizure 2 weeks before admission. MRI showed a right parieto-occipital lesion with homogeneous contrast-enhancing and significant surrounding brain edema. The patient underwent uneventful surgical resection of the lesion and was discharged home on the fifth postoperative day. Pathologic examination revealed it to be ATRT. The patient was referred to chemotherapy and radiotherapy. After 6 months of follow-up, the patient remains free of seizure and disease progression. ATRT is a rare and aggressive disease. Therefore, early diagnosis and treatment may improve the patient's prognosis and quality of life.

O tumor teratoide rabdoide atípico (TTRA) é um tipo de neoplasia rara, com comportamento maligno, que atinge o sistema nervoso central (SNC) de crianças. O objetivo do presente estudo é relatar o caso de uma paciente de 16 anos de idade admitida no nosso serviço com uma lesão occipital que revelou tratar-se de TTRA em estudo anatomopatológico. Uma paciente previamente hígida foi admitida no nosso serviço referindo ocorrência de dois episódios de crises convulsivas nas últimas 2 semanas. A ressonância magnética de crânio mostrou a presença de lesão occipital direita com captação homogênea do meio de contraste. A paciente foi submetida a cirurgia de ressecção da lesão expansiva sem intercorrências. A análise histopatológica e imuno-histoquímica revelou tratar-se de TTRA. A paciente foi encaminhada para a realização de quimioterapia e radioterapia complementares ao tratamento cirúrgico. Após 6 meses de seguimento ambulatorial, a paciente encontra-se assintomática, sem recorrência das crises convulsivas e/ou progressão radiológica da doença. TTRA é uma doença rara e agressiva. Com isso, diagnóstico e tratamento antecipados podem aprimorar prognóstico e qualidade de vida dos pacientes.

Correlation between C-Reactive Protein in Peripheral Vein and Coronary Sinus in Stable and Unstable Angina / Correlação entre Proteína C-Reativa em Veia Periférica e Seio Coronário na Angina Estável e Instável

Background: High sensitivity C-reactive protein (hs-CRP) is commonly used in clinical practice to assess cardiovascular risk. However, a correlation has not yet been established between the absolute levels of peripheral and central hs-CRP. Objective: To assess the correlation between serum hs-CRP levels (mg/L) in a peripheral vein in the left forearm (LFPV) with those in the coronary sinus (CS) of patients with coronary artery disease (CAD) and a diagnosis of stable angina (SA) or unstable angina (UA). Methods: This observational, descriptive, and cross-sectional study was conducted at the Instituto do Coração, Hospital das Clinicas, Faculdade de Medicina, Universidade de São Paulo, and at the Hospital Beneficência Portuguesa de Sao Paulo, where CAD patients referred to the hospital for coronary angiography were evaluated. Results: Forty patients with CAD (20 with SA and 20 with UA) were included in the study. Blood samples from LFPV and CS were collected before coronary angiography. Furthermore, analysis of the correlation between serum levels of hs-CRP in LFPV versus CS showed a strong linear correlation for both SA (r = 0.993, p < 0.001) and UA (r = 0.976, p < 0.001) and for the entire sample (r = 0.985, p < 0.001). Conclusion: Our data suggest a strong linear correlation between hs-CRP levels in LFPV versus CS in patients with SA and UA. .

Fundamento: A proteína C-reativa de alta sensibilidade (PCR-as) é comumente utilizada na prática clínica para avaliar o risco cardiovascular. Entretanto, a correlação entre os níveis séricos de PCR-as (valores absolutos) periférico versus central ainda não foi feita. Objetivo: Avaliar a correlação entre os níveis séricos de PCR-as (mg/L) em veia periférica do antebraço esquerdo (VPAE) versus seio coronário (SC), em pacientes portadores de doença arterial coronária (DAC) com diagnóstico de angina estável (AE) ou angina instável (AI). Métodos: Estudo observacional, descritivo, transversal, realizado no Instituto do Coração do Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo e no Hospital Beneficência Portuguesa de São Paulo, onde foram avaliados os pacientes encaminhados ao hospital com DAC para angiografia coronária. Resultados: Quarenta pacientes com DAC (20 AE e 20 AI) foram incluídos no estudo. Amostras de sangue na VPAE e SC foram coletadas simultaneamente antes da angiografia coronária. A análise de correlação entre os níveis séricos de PCR-as em VPAE versus SC mostrou forte correlação linear tanto para AE (r = 0,993, p < 0,001) como para AI (r = 0,976, p < 0,001) e em toda a amostra (r = 0,985, p < 0,001). Conclusão: Nossos dados sugeriram forte correlação linear entre os níveis de PCR-as em VPAE versus SC na AE e AI. .

Teratomas sacro coccígeos predominantemente quísticos: una revisión pronóstica / Review prognostic sacral coccygeal predominantly cystic teratomas

El teratoma sacro coccígeo es el tumor fetal más frecuente. Se suele diagnosticar en el 2do y/o 3er trimestre, raramente antes de las 14 semanas. La clasificación utilizada es la anatómica. Se presentan dos casos, uno tipo II y otro tipo III, cuyo factor común es la predominancia quística, con un resultado desfavorable en ambos. En los dos casos coinciden con la clasificación estructural de Bernachi, la cual infiere el pronóstico.

Ovarian immature teratoma during pregnancy: a case report

Germ cell tumors are derived from the primordial germ cells of the ovary and immature teratoma is the second most common germ cell malignancy. About 50% of pure immature teratomas of the ovary occur in women between the ages of 10 and 20 years, and they rarely occur in pregnancy. A 21-year-old woman, gravid 1, para 0, at 18 weeks of gestation, was incidentally diagnosed with a right ovarian mass 180 mm by 200 mm, 160 mm in diameter, during a prenatal ultrasound scanning. She underwent surgery by unilateral salpingo-oophorectomy and surgical staging. The result of pathology showed a stage 1a grade 1 immature teratoma of ovary. Her pregnancy continued until term. At 38 weeks she delivered with breech presentation a normal 2900 g male newborn by cesarean section. Although immature teratomas of ovary during pregnancy are rare, clinicians should consider their eventuality in younger pregnant women in asymptomatic cases

Teratoma inmaduro con gliomatosis peritoneal y embarazo / Immature teratoma with peritoneal gliomatosis in pregnancy

Presentamos el caso clínico de una paciente de 18 años sin antecedentes a destacar. Cursando las 30 semanas de embarazo se constata una tumoración abdominal que requirió tratamiento quirúrgico y se diagnosticó un teratoma inmaduro grado 3, asociado a gliomatosis peritoneal. Se realizó una cesárea al término y se instauró el tratamiento con quimioterapia presentando una recidiva a los siete meses que requirió tratamiento quirúrgico conservador y nuevo plan de quimioterapia estando actualmente libre de enfermedad y en seguimiento. Lo infrecuente de esta patología y asociada a embarazo, motivó su comunicación.

We report the case of a healthy 18-year old patient with adnexal mass diagnosed by her 30th week of pregnancy. It required surgical treatment and a grade 3 immature teratoma associated with peritoneal gliomatosis was diagnosed. A cesarean section was performed at term and subsequently chemotherapy was established. The patient presented a recurrence seven months later, which required fertility-sparing surgery and a new chemotherapy plan. The patient is currently disease-free and under surveillance. The infrequency of this disease and associated with pregnancy, led to their communication.

Intra-pulmonary teratoma: A rare case.

A 28-year-old female, home-maker presented to our department with a four-year history of intermittent episodes of cough and haemoptysis. She had no history of fever, weight loss or expectoration. Physical examination was unremarkable.