Enkephalins and ACTH in the mammalian nervous system.

The pentapeptides methionine-enkephalin and leucine-enkephalin belong to the opioid family of peptides, and the non-opiate peptide adrenocorticotropin hormone (ACTH) to the melanocortin peptide family. Enkephalins/ACTH are derived from pro-enkephalin, pro-dynorphin or pro-opiomelanocortin precursors and, via opioid and melanocortin receptors, are responsible for many biological activities. Enkephalins exhibit the highest affinity for the δ receptor, followed by the µ and κ receptors, whereas ACTH binds to the five subtypes of melanocortin receptor, and is the only member of the melanocortin family of peptides that binds to the melanocortin-receptor 2 (ACTH receptor). Enkephalins/ACTH and their receptors exhibit a widespread anatomical distribution. Enkephalins are involved in analgesia, angiogenesis, blood pressure, embryonic development, emotional behavior, feeding, hypoxia, limbic system modulation, neuroprotection, peristalsis, and wound repair; as well as in hepatoprotective, motor, neuroendocrine and respiratory mechanisms. ACTH plays a role in acetylcholine release, aggressive behavior, blood pressure, bone maintenance, hyperalgesia, feeding, fever, grooming, learning, lipolysis, memory, nerve injury repair, neuroprotection, sexual behavior, sleep, social behavior, tissue growth and stimulates the synthesis and secretion of glucocorticoids. Enkephalins/ACTH are also involved in many pathologies. Enkephalins are implicated in alcoholism, cancer, colitis, depression, heart failure, Huntington's disease, influenza A virus infection, ischemia, multiple sclerosis, and stress. ACTH plays a role in Addison's disease, alcoholism, cancer, Cushing's disease, dermatitis, encephalitis, epilepsy, Graves' disease, Guillain-Barré syndrome, multiple sclerosis, podocytopathies, and stress. In this review, we provide an updated description of the enkephalinergic and ACTH systems.

An unusual tumour: Hamartoma of mature cardiac myocytes / Hamartoma de miocitos cardiacos maduros: un tumor inusual

Heart neoplasms are uncommon and usually benign. Hamartoma of mature cardiac myocytes is an unusual lesion with only a few reported cases. It is a heterogeneous mixture of well-differentiated myocytes, fibroblasts, adipocytes and blood vessels. We present a case of hamartoma of mature cardiac myocytes and a concise review of the pertinent literature. A multi-lobulated polypoid tumour attached to the wall of the right atrium was found during an autopsy of a young woman. Microscopy revealed cardiomyocytes, fibrous connective tissue and well-differentiated adipocytes. The immunohistochemical study had a positive immunoreactivity for desmin, muscle-specific actin (HHF-35) and CD34 markers, showing the different types of mesenchymal cells involved. This combination of markers has not been previously used. Other tumours, such as cardiac rhabdomyoma and cardiac myxoma were ruled out due to the differences in histological characteristics and clinical presentation

Las neoplasias cardiacas son inusuales, y la mayoría benignas. El hamartoma de miocitos cardiacos maduros es una lesión inusual con pocos casos reportados en la literatura; está conformado por una mezcla heterogénea de miocitos, fibroblastos, adipocitos y vasos sanguíneos bien diferenciados. El objetivo de este artículo es describir un caso de hamartoma de miocitos cardiacos maduros y hacer una breve revisión de la literatura. Se trata de una mujer de 23 años en cuya autopsia se encontró una lesión tumoral polipoide multilobulada fija a la pared del atrio derecho. Microscópicamente se observaron miocitos cardiacos, tejido fibroconectivo y adipocitos bien diferenciados. El estudio de inmunohistoquímica confirmó la histogénesis de la lesión, ya que hubo inmunoreactividad positiva para los marcadores desmina, actina músculo específica/HHF-35 y CD34, marcadores que no habían sido usados en conjunto en la misma muestra en publicaciones previas. Se descartaron otros tumores como el rabdomioma cardiaco y el mixoma cardiaco por las características histológicas y la presentación clínica de estos que difieren con nuestro caso

An unusual tumour: Hamartoma of mature cardiac myocytes.

Heart neoplasms are uncommon and usually benign. Hamartoma of mature cardiac myocytes is an unusual lesion with only a few reported cases. It is a heterogeneous mixture of well-differentiated myocytes, fibroblasts, adipocytes and blood vessels. We present a case of hamartoma of mature cardiac myocytes and a concise review of the pertinent literature. A multi-lobulated polypoid tumour attached to the wall of the right atrium was found during an autopsy of a young woman. Microscopy revealed cardiomyocytes, fibrous connective tissue and well-differentiated adipocytes. The immunohistochemical study had a positive immunoreactivity for desmin, muscle-specific actin (HHF-35) and CD34 markers, showing the different types of mesenchymal cells involved. This combination of markers has not been previously used. Other tumours, such as cardiac rhabdomyoma and cardiac myxoma were ruled out due to the differences in histological characteristics and clinical presentation.

Trombosis antenatal de aneurisma espontáneo de conducto arterial / Antenatal thrombosis of arterial ductus spontaneous aneurysm

Los aneurismas espontáneos del conducto arterial se detectan inusualmente de forma prenatal. Su incidencia varía entre 1,5 por ciento y 8 por ciento de los embarazos. Usualmente se solucionan espontáneamente; sin embargo, pueden presentarse complicaciones letales como trombosis, embolia y ruptura. Los casos fatales descritos son escasos, la mayoría después del nacimiento. El objetivo es presentar el reporte de autopsia de un mortinato cuya causa de muerte fue la trombosis de aneurisma del conducto arterial. La madre fue una primigesta de 22 años de edad, con embarazo de 40 semanas sin antecedentes de importancia, atendida en un hospital de tercer nivel de Bucaramanga, Santander. Le realizamos una cesárea emergente por perfil biofísico fetal alterado y bradicardia fetal. Se obtuvo un mortinato masculino sin esfuerzo respiratorio, hipotónico, cianótico, que no respondió a maniobras de reanimación. Los hallazgos de autopsia relevantes fueron: dilatación auricular derecha y dilatación preductal con trombosis del conducto cuya luz estaba completamente ocluida por un coágulo. Los aneurismas ductales prenatales son una entidad que merece más estudio para determinar estrategias de diagnóstico precoz y seguimiento, y así disminuir el riesgo de desenlaces fatales(AU)

Introduction: Spontaneous ductus arteriosus aneurysm is a condition rarely diagnosed on prenatal imaging. Literature reveals 1,5 to 8 percent incidence on pregnancies. Most cases have spontaneous resolution, nonetheless, life-threatening complications such thrombosis, embolism and rupture can occur. Fatal cases reports are scarce, most of them presenting on newborns, days to months after birth. Objective: To present the autopsy report of a stillbirth whose cause of death was thrombosis of the arterial duct aneurysm. Methods: A 22-year healthy prime mother with 40-week pregnancy was assisted at a tertiary hospital in Bucaramanga, Santander. She required emergency cesarean section due to low biophysical profile and fetal bradycardia. The newborn had no respiratory effort, was floppy, cyanotic and did not respond to resuscitation maneuvers. The relevant autopsy findings were right atrial dilatation and preductal dilation with thrombosis of the duct whose lumen was completely occluded by a clot. Conclusions: The prenatal ductal aneurysm is an entity that deserves more study to determine strategies for early diagnosis and follow-up thus decrease the risk of fatal outcomes(AU)

Carcinogénesis de los tumores serosos del ovario: implicaciones quirúrgicas, avances recientes y futuros retos para su diagnóstico y tratamiento / Carcinogenesis of ovarian serous tumors: Surgical implications, recent advances and future challenges for its diagnosis and treatment

Resumen ANTECEDENTES El cáncer de ovario es una importante causa de muerte de mujeres, responsable de 150,000 fallecimientos anuales en el mundo, con una supervivencia a 5 años inferior a 45%. OBJETIVO Exponer los avances en diagnóstico y tratamiento de la carcinogénesis de los tumores serosos ováricos y las repercusiones de los nuevos conceptos en el tratamiento clínico y quirúrgico. METODOLOGÍA Estudio retrospectivo basado en la búsqueda sistemática de artículos con las palabras clave (MeSH): neoplasmas serosos de ovario, carcinogénesis, carcinoma seroso de bajo grado y carcinoma seroso de alto grado. La selección de los artículos estuvo a cargo, de forma independiente, de dos investigadores. De la misma forma dos patólogos evaluaron los artículos preseleccionados. RESULTADOS Se seleccionaron y analizaron 66 artículos. CONCLUSIONES Para la creación y adopción de estrategias en prevención primaria, diagnóstico y tratamiento de esta patología se requiere mayor comprensión de la fisiopatología y de los hallazgos encontrados en la última década, sobre todo los relacionados con las mutaciones en el gen BRCA, antecedentes familiares de tumores serosos del ovario, cáncer de mama o peritoneal de tipo epitelial, entre otras. La atención multidisciplinaria y actualizada repercutirá, significativamente, en la supervivencia de las pacientes.

Abstract BACKGROUND Globally, ovarian cancer is the seventh neoplasm and the eight oncologic cause of death in women, responsible of 150,000 deaths per year, with a 5 year survival under the 45%, mainly represented by high-grade serous carcinomas. Findings such as serous intraepithelial carcinomas and genetic predisposition have become a constant for the vast majority of cases. OBJECTIVE To summarize and review the most recent advances about the carcinogenesis of the ovarian serous tumors, its implications on the pathological diagnosis and the consequences of these new concepts for the clinical and surgical therapies. MATERIALS AND METHODS We performed a systematic search to select all kinds of languages articles, using the keywords "serous ovarian neoplasms" AND "carcinogenesis" AND "low-grade serous carcinoma" OR "high-grade serous carcinoma". Two investigators independently extracted characteristics and results to select the articles. Two pathologists independently did the assessment of the preselected articles selecting the most appropriate ones, based on their utility for this review. RESULTS 66 articles were selected and included in the bibliography for this review. CONCLUSIONS A better understanding of the physiopathology and many other new findings that have been done during the last decade are essential for the treatment of this patients, for the creation and application of strategies for primary prevention and screening for the risk, and the medical and surgical interventions, mainly directed to patients with mutations on BRCA, family history of breast, ovarian or peritoneal cancer, among other conditions. A multidisciplinary and updated approach will affect significantly the long-term survival.

Metaplasia ósea endometrial / Endometrial osseous metaplasia

La metaplasia ósea endometrial es una patología que se presenta con poca frecuencia y causa una gran variedad de síntomas como: sangrado uterino, dolor pélvico e infertilidad. Esta última, generalmente es secundaria y junto con el antecedente previo de aborto, constituyen una pieza clave en la sospecha diagnóstica. En el estudio de esta enfermedad, es necesario el uso de ecografía transvaginal y radiografía simple abdominal. El tratamiento definitivo se realiza mediante la extracción histeroscópica del material óseo, el cual es enviado a patología para corroborar el diagnóstico. La infertilidad revierte con la extracción del tejido óseo y no se han demostrado complicaciones en gestaciones futuras. Presentamos el caso de una paciente con metaplasia ósea endometrial por su rareza e importancia en el diagnóstico diferencial de sangrado uterino(AU)

Endometrial osseous metaplasia is a rare pathology that causes a variety of symptoms such as uterine hemorrhage, pelvic pain and infertility. This last consequence is generally secondary, and along with a history of abortions, represents a key element in suspected diagnosis. It is necessary to use transvaginal ultrasound and simple abdominal radiography for the study of the disease. The final treatment is performed by hysteroscopic removal of the bone material, which is sent to the pathology laboratory to confirm diagnosis. The infertility may be eliminated with the removal of the bone material and no further complications in future pregnancies have been observed. This is the case of a patient with endometrial osseous metaplasia, which is reported because of its infrequency and importance in the differential diagnosis of uterine bleeding(AU)

Embarazo molar de 25 semanas con feto vivo: reporte de autopsia / Molar pregnancy with 25-week fetus

Antecedentes: La mola hidatidiforme (MH) se caracteriza por la degeneración hidrópica de las vellosidades coriales, hiperplasia del trofoblasto y se clasifica en completa (MC) y parcial (MP), y difieren en su cariotipo, histopatología, clínica y riesgo de malignidad. Constituye el 1% de las pérdidas fetales en menores de 17 semanas. El objetivo de esta comunicación es exponer un caso de MP con feto de 25 semanas, al cual se le realizó autopsia en el 2014. Caso clínico: Primigesta de 21 años con pobre control prenatal y embarazo de 25 semanas con diagnóstico de preeclampsia y óbito fetal de sexo masculino de 615 g, al que se le solicitó autopsia demostrándose malformaciones múltiples con agenesia del cuerpo calloso, hidrocefalia supratentorial, hipoplasia cerebelosa, meningocele sacro, micropene, hipospadias y retardo del crecimiento intrauterino, la placenta de 750 g demostró hallazgos de enfermedad trofoblástica gestacional de tipo MP. Discusión: La MP presenta vellosidades coriónicas con hiperplasia trofoblástica y feto que tiende a fallecer en una temprana edad gestacional. El feto puede presentar anomalías congénitas secundarias a la aneuploidia y la distinción se basa en histopatología de la placenta, en casos difíciles se emplea citogenética o citometría de flujo. En el presente caso se encontraron características placentarias (degeneración hidrópica vellositaria, inclusiones e hiperplasia trofoblástica) y las malformaciones fetales relacionadas. Conclusión: El estudio histopatológico de la placenta y del producto de la gestación permiten el diagnóstico definitivo para poder determinar el seguimiento de la paciente y disminuir así las complicaciones.

Background: Hydatidiform moles (HM) are characterized by hydropic degeneration of chorionic villi, hyperplasia of the trophoblast and is classified in complete (CM) and partial (PM), and they are different in their karyotype, histopathology, clinical and risk of malignancy. It constitutes 1% of abortion in under 17 weeks. The purpose of this communication is to present a case of PM with 25-week fetus, which fetal autopsy in 2014. Clinical case: Primigravid of 21 years old, with a pregnancy of 25 weeks diagnosed with preeclampsia and stillbirth of 615 g, autopsy demonstrating multiple malformations with agenesis of corpus callosum, hydrocephalus, hypoplasia of the cerebellum, myeloschisis in the sacral region, micropenis, hypospadias and intrauterine growth retardation, the placenta (750 g) has findings of gestational trophoblastic disease type PM. Discussion: The PM has villous tissue with trophoblastic proliferation and fetus tends to die at an early gestational age. The fetus has congenital abnormalities because the aneuploidy, diagnoses is based on histopathology of the placenta, in difficult cases cytogenetic or flow cytometry is used. In this case, placental characteristics (hydropic villus, trophoblastic proliferation and "pseudoinclusions") and related fetal malformations were found. Conclusion: The histopathological study of the placenta and the fetus allow a definitive diagnosis to determine the monitoring of the patient and thus reduce complications.