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Ewing sarcoma (ES) and primitive neuroectodermal tumor (PNET) belong to the group of neoplasms called small round cell tumors. PNETs have been divided into central and peripheral. ES and peripheral PNETs arise from bones, soft tissues, or peripheral nerves. We present a case of hepatic ES/PNET in a healthy man that began four months before consultation with abdominal symptoms and weight loss. Upper gastrointestinal endoscopy and laboratory tests revealed no notable findings. The abdominal tomography revealed an enlarged liver due to a solid lesion that involved all its segments with intravenous contrast enhancement and large areas of necrosis. It compressed and displaced neighboring structures. Core needle biopsy of the liver lesion was performed: small round cell neoplasm. Immunohistochemistry revealed negativity for CD45, CKA1/A3, chromogranin, synaptophysin, and cytokeratins CK7 and CK20. Dim CD56 expression and CD99, FLI-1, and NKX2 positivity. He underwent chemotherapy treatment with carboplatin and etoposide for 6 cycles with clinical improvement and tolerance. Control images showed reduction of the mass with involvement of the right hepatic lobe, involvement of the inferior vena cava, infiltration of the right adrenal gland and upper pole of the right kidney. He was referred to hepatobiliary surgery for surgical resection of the residual lesion. The patient rejected the proposed surgical procedure. Our objective is to highlight the clinical and histological diagnostic challenge of this entity that requires ruling out other clinical entities.

El sarcoma de Ewing (ES) y el tumor neuroectodérmico primitivo (PNET) pertenecen al grupo de neoplasias denominadas tumores de células pequeñas y redondas. Los PNET se dividen en centrales y periféricos. El ES y los PNET periféricos surgen del tejido óseo, de los tejidos blandos o nervios periféricos. Presentamos un caso de ES/PNET hepático en un hombre sano que inició cuatro meses antes de la consulta con síntomas abdominales y pérdida de peso. La endoscopia digestiva alta y la analítica no revelaron hallazgos relevantes. En la tomografía de abdomen se evidenció hígado aumentado de tamaño a expensas de lesión sólida que comprometía todos sus segmentos con realce al contraste endovenoso y grandes áreas de necrosis. Comprimía y desplazaba estructuras vecinas. Se realizó biopsia con aguja gruesa de la lesión hepática: neoplasia de células pequeñas y redondas. La inmunohistoquímica reveló negatividad para CD45, CKA1/A3, cromogranina, sinaptofisina y citoqueratinas CK7 y CK20. Expresión tenue de CD56 y positividad de CD99, FLI-1 y NKX2. Realizó tratamiento quimioterápico con carboplatino y etopósido por 6 ciclos con mejoría clínica y tolerancia al mismo. En imágenes de control se evidenció reducción de la masa con afección del lóbulo hepático derecho, compromiso de la vena cava inferior, infiltración de la glándula suprarrenal y polo superior del riñón derechos. Se remitió a cirugía hepatobiliar para resección quirúrgica de la lesión residual. El paciente rechazó el procedimiento quirúrgico. Nuestro objetivo es destacar el desafío diagnóstico clínico e histológico de esta entidad que obliga a descartar otras entidades clínicas.

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Bat-mediated human rabies is a viral zoonotic disease that poses a serious threat to the public health of traditional peoples, especially indigenous populations that maintain primitive cultural and social habits, such as the Maxakali ethnic group, located in the southeastern region of Brazil. The sociocultural habit of this population led to the emergence between April and May 2022 of the viral spillover of rabies transmitted by bats, which decimated the lives of four children from this population who maintained contact with this animal as a recreational practice. Because the vampire bats Desmodus rotundus have exceptional ecology and social characteristics that can have important effects on the dynamics of viral dispersion in this indigenous population, I present the dynamics of contact between native children and the bat and the meaning of this relationship, which involves ritualistic and recreational significance. As important as knowing the reasons for this practice is discussing some intrinsic and extrinsic factors that imply risks that intensify the vulnerability of this population to the transmission of the rabies virus at any time. In view of this, I warn of the need to adopt efficient strategies to mitigate the risks of a new emergency in this region. Although emergency containment measures were carried out during the critical period of the outbreak, such animal and environmental control actions must become routine programmatic and structuring interventions. Essential for rabies surveillance in this population is to develop culturally adapted interethnic health education campaigns to guarantee the accessibility of the Maxakali indigenous people to the content taught, so that any attempt at domestication, captivity and recreational practices with bats of any species is discouraged, thus avoiding a possible re-emergence of this anthropozoonosis that has impacted not only the epidemiological scenario in this region, but throughout Brazil, and also throughout Latin America.

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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.

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Early cell specification in mammalian preimplantation embryos is an intricate cellular process that leads to coordinated spatial and temporal expression of specific genes. Proper segregation into the first two cell lineages, the inner cell mass (ICM) and the trophectoderm (TE), is imperative for developing the embryo proper and the placenta, respectively. Somatic cell nuclear transfer (SCNT) allows the formation of a blastocyst containing both ICM and TE from a differentiated cell nucleus, which means that this differentiated genome must be reprogrammed to a totipotent state. Although blastocysts can be generated efficiently through SCNT, the full-term development of SCNT embryos is impaired mostly due to placental defects. In this review, we examine the early cell fate decisions in fertilized embryos and compare them to observations in SCNT-derived embryos, in order to understand if these processes are affected by SCNT and could be responsible for the low success of reproductive cloning.

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Background: Persistent hyperplastic primary vitreous is a rare ocular condition that is usually unilateral, formed when normal regression of the hyaloid vascular system does not occur. Diagnosis is possible through ultrasonography, by obtaining images that provide information and also serve as a differential diagnosis. Clinically the condition presents with signs of leukocoria, microphthalmia, and cataracts, and it can be further classified into 6 degrees according to the evolution. The objective of this work is to describe the case and treatment of a canine of the Pointer breed that presented opacity in both eyes, with diagnosis confirmed through ocular ultrasonography. Case: A 6-year-old male pointer dog, weighing 27 kg, was referred to the Veterinary Medical Teaching Hospital of the Federal University of Santa Maria (UFRGS), Santa Maria, RS - Brazil with a history of blindness. According to the owner, the animal's right eye has always been "white", since it was a puppy and the left eye began to present the same characteristic a short time ago. On ophthalmological examination, neither eye showed signs of ocular discomfort, the Schirmer's tear test was within the range expected for the species, fluorescein and threat tests were negative, and a positive pupillary reflex was observed in the left eye. The right eye had total corneal opacity associated with a chronic lesion and the presence of pigment cells in the medial portion of the eyeball, the opacity making it impossible to visualize the other internal structures of the eyeball. The left eye presented lens opacity. In the ultrasound of the right eye, there were several alterations, the presence of a triangular-shaped hyperechogenic structure in the vitreous chamber, a decrease in the depth of the anterior chamber, and the presence of abnormality in the development of the lens. The findings are compatible with and confirmed the diagnosis of PHPV. In the left eye, the ultrasound images indicated hyperechogenicity of the posterior and anterior poles of the lens, findings compatible with cataracts. Through the animal's history, complete ophthalmic examination, and use of ocular ultrasonography, it was possible to diagnose and differentiate the causes of the white eye in the patient. The preoperative screening was continued with an electroretinography examination, which showed a satisfactory electrical response for the retina of the left eye and an unsatisfactory electrical response for the retina of the right eye. Phacoemulsification surgery was performed to remove the cataract in the left eye with intraocular lens implantation. The positive threat reflex returned in this eye, with a return of visual acuity. Discussion: Persistent hyperplastic tunica lentis and persistent hyperplastic primary vitreous (PHTVL/PHPV) are congenital anomalies caused by a failure to regress the fetal hyaloid vasculature. These conditions are not common, but occur sporadically in dogs. The PHPV classification can be classified into degrees, based on the morphological aspect of the lesion. Following this classification scheme, the PHPV of the animal in question refers to the 6th degree of evolution. The total opacity of the cornea in the right eye was associated with the presence of a hyperplastic primary vitreous and the leukocoria in the left eye was due to the presence of a mature cataract. The conclusion of the diagnosis was only possible through ocular ultrasonography, which is very useful to differentiate the causes of ocular opacity and leukocoria, in addition to allowing complete evaluation of the intraocular structures and being considered an essential exam to confirm the diagnosis of PHPV.

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La sensorialité tient plusieurs fonctions dans l'élaboration psychique de l'individu autiste et dans son évolution psychique au cours de sa prise en charge thérapeutique. Nous suivons dans cette recherche l'idée de la transformation d'un usage défensif de la sensorialité en un processus mutatif, d'une construction du moi corporel, ainsi qu'un travail de symbolisation primaire, d'inscription, de représentation et de figurabilité.Avec la psychothérapie d'un enfant autiste en institution, nous explorons les fonctions de la sensorialité sur la base de l'attention particulière portée aux éprouvés corporels dans la dimension transférentielle et contre-transférentielle. Celle-ci permet au thérapeute d'éprouver les angoisses en lien avec des expériences sensorielles primaires. En s'appuyant sur la résonnance contre-transférentielle des différentes angoisses du patient, une sensorialité partagée aide à la métabolisation des éprouvés de catastrophe primitive ayant entravé l'organisation d'un sentiment continu d'exister. Cette dynamique thérapeutique ouvre sur de nouvelles perspectives psychiques et relationnelles.

Resumos A sensorialidade assegura várias funções na elaboração psíquica do indivíduo autista e em seu desenvolvimento psíquico durante os seus cuidados terapêuticos. Nesta pesquisa perseguimos a ideia da transformação de um uso defensivo da sensorialidade em um processo mutativo, de uma construção do eu corporal, bem como de um trabalho de simbolização primária, de inscrição, representação e figurabilidade. Na psicoterapia de uma criança autista em instituição, exploramos as funções da sensorialidade se concentrando mais particularmente nas experiências corporais a partir da dimensão transferencial e contra-transferencial. Esse trabalho permite que o terapeuta experimente angústias ligadas a experiências sensoriais primárias. Com base na ressonância contra-transferencial das diferentes angústias do paciente, uma sensorialidade partilhada ajuda na metabolização das experiências de catástrofe primitiva que impedem a organização de um sentimento contínuo de existência. Esta dinâmica terapêutica abre novas perspectivas psíquicas e relacionais.

Sensoriality has several functions in the psychic elaboration of the autistic individual and in his psychic evolution during his therapeutic care. We follow in this research the idea of the transformation of a defensive use of sensoriality into a mutative process, of a construction of the bodily self, as well as a work of primary symbolization, inscription, representation and figurability. With the psychotherapy of an autistic child in an institution, we explore the functions of sensoriality based on the particular attention paid to bodily experiences in the transference and counter-transference dimension. This allows the therapist to experience anxieties related to primary sensory experiences. By relying on the counter-transferential resonance of the patient's various anxieties, a shared sensoriality helps to metabolize the experiences of primitive catastrophe that have hindered the organization of a continuous feeling of existing. This therapeutic dynamic opens up new psychic and relational perspectives.

La sensorialidad tiene varias funciones en la elaboración psíquica del individuo autista y en su evolución psíquica durante la asistencia terapéutica. Seguimos en esta investigación la idea de la transformación de un uso defensivo de la sensorialidad en un proceso mutativo, de una construcción del yo corporal, así como de un trabajo de simbolización primaria, inscripción, representación y figurabilidad. Con la psicoterapia de un niño autista en una institución, exploramos las funciones de la sensorialidad a partir de la particular atención prestada a las experiencias corporales en la dimensión transferencial y contratransferencial. Este trabajo le permite al terapeuta experimentar ansiedades relacionadas con las experiencias sensoriales primarias. Apoyándose en la resonancia contratransferencial de las diversas angustias del paciente, una sensorialidad compartida ayuda a metabolizar las experiencias de catástrofe primitiva que han impedido la organización de un sentimiento continuo de existir. Esta dinámica terapéutica abre nuevas perspectivas psíquicas y relacionales.

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Ewing sarcoma (ES) is a malignant neoplasm that affects bones and soft tissues, usually in young patients. Currently, ES is grouped with other tumors that share the same histological and genotypic characteristics, forming the Ewing Sarcoma Family of Tumors (ESFT), which includes ES of bone, extraosseous ES (peripheral neuroepithelioma), Askin tumor, and peripheral primitive neuroectodermal tumor (PNET). Its origin in peripheral nerves is extremely rare, making its diagnosis and treatment very challenging. We describe a case of a 27-year-old male with extraosseous ES originating in the sciatic nerve, which was surgically removed, and discuss the difficulties encountered in the management of this patient.

O sarcoma de Ewing (SE) é uma neoplasia maligna que acomete ossos e partes moles, geralmente em pacientes jovens. Atualmente, o SE é agrupado com outros tumores que compartilham as mesmas características histológicas e genotípicas, formando os Tumores da Família do Sarcoma de Ewing (TFSE), que incluem o SE ósseo, o SE extraósseo (neuroepitelioma periférico), o tumor de Askin, e o tumor neuroectodérmico primitivo (TNEP) periférico. Sua origem em nervos periféricos é extremamente rara, o que torna o seu diagnóstico e tratamento um grande desafio. Descrevemos o caso de um homem de 27 anos com SE extraósseo originário no nervo ciático, que foi removido cirurgicamente, e discutimos as dificuldades encontradas no manejo desse paciente.

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Introducción: El tumor neuroectodérmico primitivo periférico de riñón (PNETk) es una enfermedad rara muy agresivo que afecta mayormente a varones jóvenes. Reporte de caso: paciente varón de 19 años con cuadro clínico dolor abdominal, hematuria y masa palpable, en la tomografía se evidencia una enorme tumoración renal izquierda, Se le realiza nefrectomía radical convencional y se envía a estudio patológico más histoquímica resultando de PNETk. Luego paciente siguió su manejo por oncología para quimiterapia inicialmente. Conclusión: El PNETk que describimos representa el primer caso reportado en nuestro país, constituye una entidad clínica única por su rareza siendo un reto hacer diagnóstico y su comportamiento y manejo se sigue basando a reportes de casos debido a su poca frecuencia.

Introduction: Peripheral primitive neuroectodermal tumor of the kidney (PNETk) is a very aggressive rare disease that mainly affects young men. Case report: A 19-year-old male patient with symptoms of abdominal pain, hematuria and a palpable mass, the tomography shows a large left renal tumor. Conventional radical nephrectomy was performed and sent for pathology study plus histochemistry, resulting in PNETk. The patient then continued his oncology management for chemotherapy initially. Conclusion: The PNETk that we describe represents the first case reported in our country, it constitutes a unique clinical entity due to its rarity, being a challenge to make a diagnosis and its behavior and management is still based on case reports due to its infrequency.

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The origin of life was a cosmic event happened on primitive Earth. A critical problem to better understand the origins of life in Earth is the search for chemical scenarios on which the basic building blocks of biological molecules could be produced. Classic works in pre-biotic chemistry frequently considered early Earth as an homogeneous atmosphere constituted by chemical elements such as methane (CH4), ammonia (NH3), water (H2O), hydrogen (H2) and hydrogen sulfide (H2S). Under that scenario, Stanley Miller was capable to produce amino acids and solved the question about the abiotic origin of proteins. Conversely, the origin of nucleic acids has tricked scientists for decades once nucleotides are complex, though necessary molecules to allow the existence of life. Here we review possible chemical scenarios that allowed not only the formation of nucleotides but also other significant biomolecules. We aim to provide a theoretical solution for the origin of biomolecules at specific sites named "Prebiotic Chemical Refugia." Prebiotic chemical refugium should therefore be understood as a geographic site in prebiotic Earth on which certain chemical elements were accumulated in higher proportion than expected, facilitating the production of basic building blocks for biomolecules. This higher proportion should not be understood as static, but dynamic; once the physicochemical conditions of our planet changed periodically. These different concentration of elements, together with geochemical and astronomical changes along days, synodic months and years provided somewhat periodic changes in temperature, pressure, electromagnetic fields, and conditions of humidity, among other features. Recent and classic works suggesting most likely prebiotic refugia on which the main building blocks for biological molecules might be accumulated are reviewed and discussed.

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BACKGROUND: Embryonal tumor with multilayered rosettes (ETMR), NOS/C19MC- altered, is a rare and recently classified highly aggressive malignant brain tumor in the 2021 World Health Organization (WHO) classification of tumors of the central nervous system 5th edition. They are mostly diagnosed in children before the age of three years. Most of them are located in the supratentorial region. Prior to the reclassification of ETMR as a single entity, three distinct tumors, namely, embryonal tumor with abundant neuropil and true rosettes (ETANTR), ependymoblastoma (EBL) and medulloepithelioma (MEPL) were recognized. Recent studies showed that all the three entities have multilayered rosettes on morphology, sharing a common amplification of the C19MC locus at the chromosome 19q13.42 by fluorescence in situ hybridization, and highly specific immunohistochemical staining for LIN28A rendered their reclassification as a single entity. REPORT: A 13-year-old girl was rushed to the emergency room unconscious, with no return of spontaneous circulation after cardiopulmonary resuscitation. Autopsy revealed a left cerebellar hemisphere hemorrhagic tumor which histopathological examination revealed a multilayered ependymoblastic rosettes with abundant neuropil. The multilayered rosettes showed reactivity for vimentin but non-reactivity for pan-cytokeratin, the zones with abundant neuropil were reactive for synaptophysin consistent with a diagnosis of embryonal tumor with abundant neuropil and true rosettes now ETMR, NOS (WHO Grade 4) due to the lack of genetic testing for amplification of C19MC. CONCLUSION: ETMR is a highly aggressive CNS embryonal tumor with extremely poor prognosis. It should be considered in the differential diagnosis of pediatric brain tumors. Multilayered rosettes are a useful clue to histologic diagnosis.

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El tumor neuroectodérmico primitivo del ovario es un sarcoma de tejido blando de células redondas pequeñas, raro y agresivo, de origen neural que generalmente se asocia con una alta morbilidad y mortalidad. La inmunohistoquímica es un complemento útil en el diagnóstico diferencial. Se describe un caso de tumor neuroectodérmico primitivo del ovario en paciente nulípara de 21 años que refería dolor y aumento de la circunferencia abdominal. La ecografía mostró tumoración de contenido heterogéneo sólido-quística que aparentemente se originaba del anexo izquierdo. La resonancia magnética confirmó la presencia de tumoración que se extendía hacia la fosa iliaca izquierda sin afectación de órganos locales ni metástasis regionales o a distancia. Los marcadores tumorales estaban todos dentro del rango normal. Durante la laparotomía se observó tumoración de ovario izquierdo con ovario derecho normal. Se realizó salpingoforectomía izquierda debido al tamaño del tumor, resección en cuña de ovario derecho, linfadenectomía pélvica y omentectomía. El examen histopatológico reveló tumor compuesto por láminas de células redondas. Las células tumorales fueron positivas para cromogranina A, sinatrofisina, vimentina y enolasa específica de neuronas, lo que confirmó el diagnóstico de tumor neuroendocrino primitivo de ovario izquierdo, que se originaba de teratoma quístico inmaduro. La paciente rechazó la quimioterapia postoperatoria.

Primitive neuroectodermal tumor of the ovary is a rare and aggressive small round cell soft tissue sarcoma of neural origin that is usually associated with high morbidity and mortality. Immunohistochemistry is a useful adjunct in the differential diagnosis. We describe a case of a primitive neuroectodermal tumor of the ovary in a 21-yearold nulliparous patient who reported pain and increased abdominal circumference. Ultrasonography showed a solid-cystic heterogeneous tumor apparently originating from the left adnexa. Magnetic resonance imaging confirmed the presence of a tumor extending into the left iliac fossa without local organ involvement or regional or distant metastases. Tumor markers were all within the normal range. During laparotomy, a left ovarian tumor was observed with a normal right ovary. Left salpingo-oophorectomy was performed due the size of the tumor, right ovarian wedge resection, pelvic lymphadenectomy and omentectomy. Histopathologic examination revealed tumor composed of sheets of round cells. The tumor cells were positive for chromogranin A, synaptophysin, vimentin and neuron-specific enolase, which confirmed the diagnosis of a primitive neuroendocrine tumor of the left ovary originating from immature cystic teratoma. The patient refused postoperative chemotherapy.

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PURPOSE: Pineal anlage tumors (PAT) are extremely rare malignant tumors in the pediatric population considered to be a variant of pineoblastomas. These tumors are characterized by a combination of neuroectodermal and heterologous ectomesenchymal components. PAT can be clinically aggressive and has a propensity to cerebrospinal fluid (CSF) dissemination. So far, only 13 cases of these tumors have been reported in the literature. CASE ARTICLE: In this article, we report the case of an anlage tumor in a 3-year-old girl with a rapidly aggressive course.

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Ewing's sarcoma/Primitive Neuro-Ectodermic Tumor of the kidney accounts for less than 1% of all kidney tumors. We present the case of a 37-year-old male with abdominal pain secondary to a right renal tumor of 7 × 7 × 5 cm. A robotic right radical nephrectomy was performed. In the histopathological and immunohistochemistry was reported NKX2.2(+), CD99(+), synaptophysin(+), and FLI-1(+). Extraosseous Ewing's Sarcoma was diagnosed. The patient was treated with: Doxorubicin, Vincristine, Mesna, and Isophosphamide.

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ABSTRACT Background Embryonal tumor with multilayered rosettes (ETMR), NOS/C19MC- altered, is a rare and recently classified highly aggressive malignant brain tumor in the 2021 World Health Organization (WHO) classification of tumors of the central nervous system 5th edition. They are mostly diagnosed in children before the age of three years. Most of them are located in the supratentorial region. Prior to the reclassification of ETMR as a single entity, three distinct tumors, namely, embryonal tumor with abundant neuropil and true rosettes (ETANTR), ependymoblastoma (EBL) and medulloepithelioma (MEPL) were recognized. Recent studies showed that all the three entities have multilayered rosettes on morphology, sharing a common amplification of the C19MC locus at the chromosome 19q13.42 by fluorescence in situ hybridization, and highly specific immunohistochemical staining for LIN28A rendered their reclassification as a single entity. Report: A 13-year-old girl was rushed to the emergency room unconscious, with no return of spontaneous circulation after cardiopulmonary resuscitation. Autopsy revealed a left cerebellar hemisphere hemorrhagic tumor which histopathological examination revealed a multilayered ependymoblastic rosettes with abundant neuropil. The multilayered rosettes showed reactivity for vimentin but non-reactivity for pan-cytokeratin, the zones with abundant neuropil were reactive for synaptophysin consistent with a diagnosis of embryonal tumor with abundant neuropil and true rosettes now ETMR, NOS (WHO Grade 4) due to the lack of genetic testing for amplification of C19MC. Conclusion: ETMR is a highly aggressive CNS embryonal tumor with extremely poor prognosis. It should be considered in the differential diagnosis of pediatric brain tumors. Multilayered rosettes are a useful clue to histologic diagnosis.

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Introduction Primitive neuroectodermal tumors are rare neoplasms of the central nervous system that occur in children, with few reports in adults. These tumors are found most often in the cerebral hemispheres, with spinal cord disorders being rare. Case Report A 71-year-old man with motor and sensory deficits in the upper limbs, cervical pain, and urinary incontinence presented to the Neurosurgery Service. The physical examination revealed grade-III motor strength on the right side, grade IV- on the left upper limb, and grade IVþ on the left lower limb. A magnetic resonance imaging scan showed an expansive intramedullary lesion with a C3-C4 epicenter. Spinal decompression, lesional biopsy, and adjuvant radiotherapy were performed. The anatomopathological report showed a primitive neuroectodermal tumor. After a new treatment with adjuvant radiotherapy (20 1.8 Gy in the skull and neuroaxis and 5 1.8 Gy in tumor boost), the patient progressed without recurrence. Conclusion Since the characteristics of the tumor are similar to those of medulloblastoma, it is necessary to expand the studies on these lesions, to better understand their pathophysiology and list better diagnostic and therapeutic methods, in addition to those already available.

Introdução Os tumores neuroectodérmicos primitivos são neoplasias raras do sistema nervoso central que ocorrem em crianças, com escassos relatos em adultos. Esses tumores são encontrados mais frequentemente nos hemisférios cerebrais, sendo raros os acometimentos medulares. Relato de Caso Um homem de 71 anos com déficits motor e sensitivo em membros superiores, algia cervical e incontinência urinária apresentou-se ao Serviço de Neurocirurgia. O exame físico revelou força de grau III à direta, de grau IV- no membro superior esquerdo, e de grau IVþ no membro inferior esquerdo. Um exame de ressonância magnética denotou lesão expansiva intramedular com epicentro em C3- C4. Foram realizadas descompressão medular, biópsia lesional e radioterapia adjuvante. O laudo anatomopatológico evidenciou tumor neuroectodérmico primitivo. Após novo tratamento com radioterapia adjuvante (20 1,8 Gy no crânio e neuroeixo e 5 1,8 Gy em boost tumoral), o paciente seguiu sem recidiva. Conclusões Uma vez que as características do tumor se assemelham às do meduloblastoma, torna-se necessário ampliar os estudos acerca dessas lesões, a fim de compreender melhor sua fisiopatologia e elencar melhores métodos diagnósticos e terapêuticos, além dos já disponíveis.

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BACKGROUND: In vitro methods for hematopoietic differentiation of human pluripotent stem cells (hPSC) are a matter of priority for the in-depth research into the mechanisms of early embryogenesis. So-far, published results regarding the generation of hematopoietic cells come from studies using either 2D or 3D culture formats, hence, it is difficult to discern their particular contribution to the development of the concept of a unique in vitro model in close resemblance to in vivo hematopoiesis. AIM OF THE STUDY: To assess using the same culture conditions and the same time course, the potential of each of these two formats to support differentiation of human pluripotent stem cells to primitive hematopoiesis without exogenous activation of Wnt signaling. METHODS: We used in parallel 2D and 3D formats, the same culture environment and assay methods (flow cytometry, IF, qPCR) to investigate stages of commitment and specification of mesodermal, and hemogenic endothelial cells to CD34 hematopoietic cells and evaluated their clonogenic capacity in a CFU system. RESULTS: We show an adequate formation of mesoderm, an efficient commitment to hemogenic endothelium, a higher number of CD34 hematopoietic cells, and colony-forming capacity potential only in the 3D format-supported differentiation. CONCLUSIONS: This study shows that the 3D but not the 2D format ensures the induction and realization by endogenous mechanisms of human pluripotent stem cells' intrinsic differentiation program to primitive hematopoietic cells. We propose that the 3D format provides an adequate level of upregulation of the endogenous Wnt/ß-catenin signaling.

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Primitive neuroectodermal tumors (PNETs) belong to the Ewing sarcoma family of tumors. These lesions are highly aggressive and are usually found in paravertebral regions, lower limbs, and thorax. However, abdominal PNETs are extremely rare, and only 3 cases of pediatric PNET of the liver have been previously reported. Most patients exhibit symptoms associated with mass effect, due to rapid tumor growth and dissemination. Therefore, an appropriate differential diagnosis is of pivotal importance in order to initiate the corresponding treatment. Here we report the case of a 4-year-old female patient who was diagnosed with PNET of the liver, and we discuss the analysis of focal liver lesions and differential diagnosis in pediatric patients.

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Na clínica psicanalítica contemporânea temos nos defrontado, cada vez mais, com impasses técnicos que nos exigem repensar a teoria sobre a técnica psicanalítica. Essa revisão teórica se justifica a partir de uma maior incidência de quadros clínicos cujo dinamismo psíquico não se encontra balizado hegemonicamente pela lógica do recalque, mas pela predominância de outros mecanismos defensivos, como a clivagem/splitting do eu, e formas de expressão não verbais como passagens ao ato e acting outs. Diante da necessidade de reconfigurar alguns parâmetros da técnica psicanalítica, delimitamos como objetivo deste artigo a investigação do tema do manejo clínico das regressões em análise a partir das contribuições teórico-clínicas de Sándor Ferenczi e de seus desdobramentos e avanços propostos Michael Balint. Assim, pretendemos extrair das modificações no dispositivo analítico propostas por esses autores, elementos que nos ajudem a pensar o manejo clínico com pacientes cujo sofrimento remete às falhas traumáticas dos primórdios da constituição subjetiva.

In contemporary psychoanalytic clinic we have faced increasingly technical deadlocks that require to rethink the psychoanalytic technique theory. This situation requires a revision of psychoanalysis technical theory. This theoretical revision is justified by a higher incidence of clinical conditions whose psychic dynamic is not hegemonic marked by the logic of repression, but the predominance of other defensive mechanisms, e.g. splitting, and non-verbal forms of expression such as acting out. Given the need to reconfigure some parameters of the psychoanalytic technique, we set as the objective of this article the investigation of clinical management of regressions under analysis from the theoretical and clinical contributions of Sándor Ferenczi and from developments and advances by Michael Balint. Therefore, from the changes proposed by these authors, we will attempt to extract elements that will help us think different clinical management of patients whose suffering refers to traumatic failures in the early stages of development, in an analytical setting.

En la clínica psicoanalítica contemporánea nos hemos enfrentado, cada vez más, con impases técnicos que nos obligan a repensar la teoría sobre la técnica psicoanalítica. Esta revisión teórica se justifica por una mayor incidencia de cuadros clínicos cuyo dinamismo psíquico no responde a la lógica de la represión, sino por el predominio de otros mecanismos defensivos, como el clivaje / escisión del yo, y formas de expresión no verbal como pasajes al acto y "acting outs". Ante la necesidad de reconfigurar algunos parámetros de la técnica psicoanalítica, el objetivo de este artículo es delinear la investigación del tema del manejo clínico de las regresiones en análisis, a partir de las contribuciones teórico-clínicas de Sándor Ferenczi y sus desdoblamientos y avances en la teoría de Michael Balint. Así, pretendemos extraer, de las modificaciones en el dispositivo analítico propuestas por esos autores, elementos que nos ayuden a pensar sobre el manejo clínico con pacientes cuyo sufrimiento remite a los fracasos traumáticas de los primordios de la constitución subjetiva.

Assuntos

RESUMO

Primitive neuroectodermal tumor (PNET) is part of the Ewing sarcoma family of tumors. The present case reports a primitive neuroectodermal tumor (PNET) of rare location in the bladder in a newborn. It was evaluated with prenatal ultrasound and postnatal tomography that revealed a mass in the posterior wall of the bladder. The patient underwent partial cystectomy with subsequent analysis of the surgical piece removed, the histopathological study indicated a tumor of mesenchymal origin, and immunohistochemical staining confirmed the diagnosis of PNET of the bladder. Satisfactory result and short-term follow-up.

RESUMO

RESUMEN Introducción: el Linfoma cutáneo primario del centro folicular es indolente; la supervivencia es de 95% a cinco años; es el más común de los LCCB (35%). Tiene predilección por varones adultos mayores. Se manifiesta con pápulas, placas y tumores solitarios o agrupados principalmente en la piel cabelluda, la frente y el tronco. El objetivo de este trabajo es la presentación del caso por lo inusual de aparición en esta localización y ser el 1ero en nuestro servicio y provincia. Caso clínico: paciente JVL de 58 años de edad, que se interconsulta con los médicos del servicio de Dermatología por presentar lesiones en cuero cabelludo de 3 meses de evolución. Examen físico: Presenta lesiones localizadas en el cuero cabelludo, tumorales en número de 4 redondeadas eritemato violáceas de centro ulcerado con secreción serohemáticas y telangiectasias en su superficie las cuales eran dolorosas. Adenopatías múltiples, pequeñas, movibles en región cervical bilaterales. Se le realizó estudio histopatológico de los tumores para confirmar el diagnóstico clínico planteado. Discusión: los linfomas son tumores malignos del sistema linforreticular, representan un 25% del total de los LCP. Esta neoplasia aparece fundamentalmente entre los 40 y 60 años y es 2,2 veces más frecuente en el sexo masculino que en el femenino, en nuestro caso la paciente tenía 58 años de edad. Si bien es cierto que esta patología es infrecuente, en nuestro caso aparece en mujer con lesiones en cuero cabelludo en una de las localizaciones menos reportada, pero con las lesiones clásicas de los linfomas cutáneos primarios de células B, la tendencia a las ulceraciones se pueden encontrar en la forma centro folicular y en los linfomas difuso de células B como en nuestro caso. Conclusiones: realizamos esta presentación de linfoma cutáneo primario de células B enfermedad infrecuente que se ve mayormente en sexo masculino sin embargo en su variedad difuso se observa más frecuente en mujer en cuero cabelludo de forma ulcerada como apareció en nuestro caso y por ser el primero diagnosticado nuestra provincia.

ABSTRACT Introduction: the primary cutaneous lymphoma of the follicular center is indolent; 95% survival over five years; is the most common LCCB (35%). They have a predilection for larger adult males. It manifests with papules, plaques and solitary tumors or grouped mainly in the skinned hair, in front of the trunk. The purpose of this work is the presentation of the case due to the unusual appearance in this location and to be the first in our service and province. Clinical case: JVL patient of 58 years of age, who is consulted with doctors at the Dermatology service for presenting injuries in the hair of 3 months of evolution. Physical examination: There are lesions located in the hair, tumors in the number of 4 rounded violet erythema of the ulcerated center with serohemic secretion and telangiectasias on their surface that are painful. Multiple, small adenopathies, movable in bilateral cervical region. The histopathological study of the tumors was carried out to confirm the planted clinical diagnosis. Discussion: lymphomas are malignant tumors of the lymphorecticular system, representing 25% of the total PCL. This neoplasm appears fundamentally between 40 and 60 years and 2.2 times more often in men than in women, in case the patient had 58 years of age. If I believe that this pathology is unheard of, in one case it appears in many with lesions in the hair in one of the least reported locations, but with the classic lesions of primary cutaneous B-cell lymphomas, the tendency to ulcers is reduced. find in the form of follicular center and diffuse B-cell lymphomas as in this case. Conclusions: we present this presentation of primary cutaneous B-cell lymphoma that is infrequently ill, seen mostly in men, but in a diffuse variety, it is observed more frequently in the hair in an ulcerated form as it appears in our case and because it is the first diagnosed in our province.

RESUMO Introdução: o linfoma cutâneo primário do centro folicular é indolente; a sobrevivência é de 95% em 5 anos; é o mais comum dos LCCBs (35%). Ele tem predileção por homens adultos mais velhos. Manifesta-se na forma de pápulas, placas e tumores, solitários ou agrupados, principalmente no couro cabeludo, testa e tronco. O objetivo deste trabalho é apresentar o caso devido à sua peculiaridade neste local e ser o primeiro em nosso serviço e província. Caso clínico: paciente JVL, 58 anos, que é consultado pelos médicos do serviço de Dermatologia por apresentar lesões no couro cabeludo com 3 meses de evolução. Exame físico: Apresenta lesões localizadas no couro cabeludo, tumores em número de 4 eritematosos violáceos arredondados com centro ulcerado com secreções cerimônicas e telangiectasias dolorosas na superfície. Linfadenopatia múltipla, pequena e móvel na região cervical, bilateral. O estudo histopatológico dos tumores foi realizado para confirmação do diagnóstico clínico. Discussão: linfomas são tumores malignos do sistema reticular linfático, correspondendo a 25% de todos os LCP. Essa neoplasia surge principalmente entre 40 e 60 anos e é 2,2 vezes mais frequente em homens do que em mulheres, em nosso caso o paciente tinha 58 anos. Embora seja verdade que essa patologia não seja frequente, em nosso caso ela aparece em mulheres com lesões no couro cabeludo em uma das localizações menos relatadas, mas com as lesões clássicas dos linfomas cutâneos primários de células B, a tendência à ulceração é pode ser encontrado no centro folicular e nos linfomas difusos de células B, como em nosso caso. Conclusões: apresentamos esta apresentação do linfoma cutâneo primário de células B, uma doença rara observada principalmente no sexo masculino, mas em sua variedade difusa é observada mais frequentemente em mulheres no couro cabeludo de forma ulcerada como apareceu em nosso caso e por seja o primeiro diagnóstico em nossa província.