ABSTRACT
ABSTRACT: We described the clinical and anatomopathological findings observed in a case of nasal leiomyosarcoma in a five-year-old male Quarter Mile horse, whose main complaints were decreased sports performance and bilateral purulent nasal discharge. The nodule was observed in the nasal cavity, obstructing the left nostril and associated with purulent drainage. The nodule was of irregular shape and yellow color, measuring 19.4 cm × 6.9 cm × 4.3 cm in size, with coalescent multifocal areas that were brownish, friable, opaque, and fetid. When cut, the surface was compact, grayish-white, and smooth with yellow, friable, irregular multifocal areas, measuring 1-3.2 cm in diameter. Histopathological examination showed spindle-shaped neoplastic cells, which was negative on Masson's trichromic stain. A diagnosis of leiomyosarcoma was established based on the morphotintorial aspects of neoplastic cells and confirmed through immunohistochemistry, with positive immunostaining for antibodies 1A4, HHF35, desmin, and S100. Leiomyosarcoma primarily affects the nasal cavity of horses and should be included in the differential diagnosis of diseases that affect the nasal cavity and cause nasal obstruction associated with dyspnea.
RESUMO: Descreve-se os achados clínicos e anatomopatológicos observados em um caso de leiomiossarcoma nasal em um equino, Quarto de Milha, macho, de cinco anos de idade, com queixa principal de diminuição do rendimento esportivo e secreção nasal purulenta bilateral. Na cavidade nasal, observou-se um nódulo obstruindo a narina esquerda associada à secreção purulenta. O nódulo era de superfície irregular, amarelada, brilhante, fibroelástica, medindo 19,4 x 6,9 x 4,3cm de tamanho, com áreas multifocais a coalescente acastanhadas, friáveis, opacas e fétidas. Ao corte, a superfície era compacta, branco-acinzentada e lisa com áreas multifocais amarelas, friáveis e irregulares, medindo 1-3,2 cm de diâmetro. No exame histopatológico foi observado proliferação de células neoplásicas fusiformes, que foram negativas na coloração de Tricrômico de Masson. O diagnóstico de leiomiossarcoma foi estabelecido com base nos aspectos morfotintoriais das células neoplásicas e confirmado através da imuno-histoquímica, no qual houve imunomarcação positiva para os anticorpos 1A4, HHF35, Desmina e S100. O leiomiossarcoma pode afetar primariamente a cavidade nasal de equinos, devendo ser incluído no diagnóstico diferencial de doenças que afetam a cavidade nasal e que provocam quadros de obstrução nasal associado a dispneia.
ABSTRACT
We described the clinical and anatomopathological findings observed in a case of nasal leiomyosarcoma in a five-year-old male Quarter Mile horse, whose main complaints were decreased sports performance and bilateral purulent nasal discharge. The nodule was observed in the nasal cavity, obstructing the left nostril and associated with purulent drainage. The nodule was of irregular shape and yellow color, measuring 19.4 cm × 6.9 cm × 4.3 cm in size, with coalescent multifocal areas that were brownish, friable, opaque, and fetid. When cut, the surface was compact, grayish-white, and smooth with yellow, friable, irregular multifocal areas, measuring 1-3.2 cm in diameter. Histopathological examination showed spindle-shaped neoplastic cells, which was negative on Masson's trichromic stain. A diagnosis of leiomyosarcoma was established based on the morphotintorial aspects of neoplastic cells and confirmed through immunohistochemistry, with positive immunostaining for antibodies 1A4, HHF35, desmin, and S100. Leiomyosarcoma primarily affects the nasal cavity of horses and should be included in the differential diagnosis of diseases that affect the nasal cavity and cause nasal obstruction associated with dyspnea.
Descreve-se os achados clínicos e anatomopatológicos observados em um caso de leiomiossarcoma nasal em um equino, Quarto de Milha, macho, de cinco anos de idade, com queixa principal de diminuição do rendimento esportivo e secreção nasal purulenta bilateral. Na cavidade nasal, observou-se um nódulo obstruindo a narina esquerda associada à secreção purulenta. O nódulo era de superfície irregular, amarelada, brilhante, fibroelástica, medindo 19,4 x 6,9 x 4,3cm de tamanho, com áreas multifocais a coalescente acastanhadas, friáveis, opacas e fétidas. Ao corte, a superfície era compacta, branco-acinzentada e lisa com áreas multifocais amarelas, friáveis e irregulares, medindo 1-3,2 cm de diâmetro. No exame histopatológico foi observado proliferação de células neoplásicas fusiformes, que foram negativas na coloração de Tricrômico de Masson. O diagnóstico de leiomiossarcoma foi estabelecido com base nos aspectos morfotintoriais das células neoplásicas e confirmado através da imuno-histoquímica, no qual houve imunomarcação positiva para os anticorpos 1A4, HHF35, Desmina e S100. O leiomiossarcoma pode afetar primariamente a cavidade nasal de equinos, devendo ser incluído no diagnóstico diferencial de doenças que afetam a cavidade nasal e que provocam quadros de obstrução nasal associado a dispneia.
Subject(s)
Animals , Sarcoma/veterinary , Nasal Obstruction/veterinary , Dyspnea/veterinary , Horse Diseases , Leiomyosarcoma/veterinaryABSTRACT
Resumen ANTECEDENTES: Los leiomiomas son los tumores pélvicos más frecuentes en la mujer; sin embargo, su localización vaginal es excepcional. Suelen ser asintomáticos y encontrarse como un hallazgo clínico. En los últimos 20 años solo se han reportado 85 casos, y solo dos han sido recurrentes. OBJETIVO: Reportar un caso de miomatosis vaginal recurrente en una paciente histerectomizada y revisar la bibliografía al respecto. CASO CLÍNICO: Paciente de 58 años, histerectomizada, con una tumoración vaginal. El reporte histopatológico informó una proliferación fusocelular, debidamente delimitada, dispuesta en haces entrecruzados. Los núcleos eran alargados, monomorfos y de extremos romos. El estroma era escaso y colagénico. No se observaron atipias citonucleares ni necrosis. El estudio inmunohistoquímico de la lesión con actina de anticuerpos antimúsculo liso y desmina se reportó positivo. Se diagnosticó miomatosis vulvovaginal recurrente. Se trató mediante resección quirúrgica. CONCLUSIÓN: Los leiomiomas vulvovaginales son extremadamente raros y la bibliografía al respecto es poca; su recurrencia es verdaderamente excepcional. De ahí la importancia de la publicación de estos casos, que aporta información que pueden tomar en cuenta otros clínicos al momento del diagnóstico.
Abstract BACKGROUND: Leiomyomas are the most frequent pelvic tumors in women; however, their vaginal location is unusual. They are usually asymptomatic and present as a clinical finding. In the last 20 years only 85 cases have been reported, and only two have been recurrent. OBJECTIVE: To report a case of recurrent vaginal myomatosis in a hysterectomized patient and review the literature. CLINICAL CASE: A 58-year-old hysterectomized patient with a vaginal tumor. The histopathologic report reported a fusocellular proliferation, properly delimited, arranged in crisscross bundles. The nuclei were elongated, monomorphous and blunt ended. The stroma was sparse and collagenous. No cytonuclear atypia or necrosis were observed. Immunohistochemical study of the lesion with anti-smooth muscle antibody actin and desmin was reported positive. Recurrent vulvovaginal myomatosis was diagnosed. It was treated by surgical resection. CONCLUSION: Vulvovaginal leiomyomas are extremely rare, and the literature is sparse; their recurrence is truly exceptional. Hence the importance of publishing these cases, providing information to be considered by other clinicians at the time of diagnosis.
ABSTRACT
ABSTRACT A 43-years-old Brazilian woman, Caucasian, premenopausal, was attended with a history of lower abdominal pain, distension, and bleeding. Pelvic and transvaginal ultrasound revealed an enlarged uterus with a large, well-defined, uniformly hyperechoic lesion. The patient underwent total hysterectomy and the specimen was sent for anatomopathological evaluation. The histopathological analyses revealed a leiomyoma with extensive cystic degeneration and atypical characteristics, the immunohistochemical study confirmed the benignity of the case. The finding of atypical leiomyoma with cystic degeneration is rare and should be carefully evaluated to exclude malignant diseases.
RESUMEN Mujer brasileña de 43 años, caucásica, premenopáusica, fue atendida con antecedentes de dolor abdominal bajo, distensión y menorragias. La ecografía pélvica y transvaginal reveló un útero agrandado con una lesión grande, bien definida y uniformemente hiperecoica. La paciente fue sometida a histerectomía total y la pieza fue enviada para evaluación anatomo-patológica. Los análisis histopatológicos revelaron un leiomioma con degeneración quística extensa de características atípicas y la inmunohistoquímica confirmó la benignidad del caso. El hallazgo de un leiomioma atípico con degeneración quística es raro y debe evaluarse cuidadosamente para descartar enfermedades malignas.
RESUMO Mulher brasileira, 43 anos de idade, caucasiana, na pré-menopausa, foi atendida devido a história de dor em abdômen inferior, distensão e sangramento. A ultrassonografia pélvica e transvaginal revelou útero aumentado com grande lesão hiperecoica, bem definida e uniforme. A paciente foi submetida à histerectomia total, e a amostra foi enviada para avaliação anatomopatológica. A análise histopatológica revelou quadro de leiomioma com degeneração cística extensa e características atípicas; o estudo imuno-histoquímico confirmou a benignidade do caso. O achado de leiomioma atípico com degeneração cística é raro e deve ser cuidadosamente avaliado para excluir doenças malignas.
ABSTRACT
SUMMARY: The aim of this study was to evaluate the effects of stretching and therapeutic ultrasound (TUS) on desmin and laminin contents of rat muscle after contusion. Male Wistar rats (n = 35, 8-9 weeks of age, 271 ± 14g body weight) were divided into five groups: Control group (CG) (n= 03); Injured group (IG) (n= 8); Injured + ultrasound group (IUSG) (n= 8); Injured+stretching group (ISG) (n= 8); Injured +ultrasound + stretching group (IUSSG) (n= 8). The application of ultrasound started 72 hours after the contusion, using the 50 % pulsed mode, 0.5 W/cm2, 5 min, once a day, for five consecutive days. Passive manual stretching was started on the tenth day after injury, with four repetitions of 30 s each and 30 s rest between repetitions, once a day, five times per week, for a total of ten applications. After 22 days, the rats were euthanazied and the gastrocnemius of both limbs removed for desmin and laminin immunohistochemistry morphometric measurement. Analysis was conducted using ANOVA one way post-hoc Tukey to parametric data and Kruskall-Wallis for non-parametric data. The IUSSG animals showed a larger area of desmin than ISG (p<0.05). It was found a decrease in laminin comparing IUSG to IG. However, laminin area was higher in ISG than all groups (p<0.05). UST isolated or in combination with stretching influenced gastrocnemius regeneration in different manners. While stretching applied isolated enhanced gastrocnemius regeneration noticed by the increase in laminin area, in combination with TUS strengthened the muscle healing rising desmin area.
RESUMEN: El objetivo de este estudio fue evaluar los efectos del estiramiento y la ecografía en los contenidos de desmina y laminina del músculo de rata después de la lesión. Ratas Wistar macho (n = 35, 8-9 semanas de edad, 271 ± 14 g de peso corporal) se dividieron en cinco grupos: grupo de control (CG) (n = 03); Grupo lesionado (GL) (n = 8); Lesionado + grupo de ultrasonido (LGU) (n= 8); Lesionado + grupo de estiramiento (LGE) (n = 8); Lesionado + ultrasonido + grupo de estiramiento (LUGE) (n = 8). La aplicación de ultrasonido comenzó 72 horas después de la lesión, usando el modo pulsado al 50 %, 0,5W / cm2, 5 min, una vez al día, durante cinco días consecutivos. El estiramiento manual pasivo se inició el décimo día después de la lesión, con cuatro repeticiones de 30 seg cada una y 30 seg de descanso entre repeticiones, una vez al día, cinco veces por semana, para un total de diez aplicaciones. Las ratas fueron sacrificadas después de 22 días, y se extrajo el músculo gastrocnemio de ambos miembros para la medición morfométrica de desmina y laminina a través de inmunohistoquímica. El análisis se realizó utilizando ANOVA unidireccional Tukey post-hoc para datos paramétricos y Kruskall-Wallis para datos no paramétricos. Los animales LUGE mostraron un área mayor de desmina que LGE (p <0,05). Se encontró una disminución en la laminina comparando LGU con GL. Sin embargo, el área de laminina fue mayor en LGE que en todos los grupos (p <0,05). El tratamiento con ultrasonido aislado o en combinación con estiramiento influyó en la regeneración del músculo gastrocnemio de diferentes maneras. Si bien el estiramiento aplicado, en combinación con tratamiento de ultrasonido, fortaleció el área de desmina, la regeneración del músculo gastrocnemio mejoró por el aumento en el área de laminina aumentando la curación muscular.
Subject(s)
Animals , Male , Rats , Ultrasonic Therapy/methods , Muscle, Skeletal/pathology , Contusions/therapy , Muscle Stretching Exercises/methods , Immunohistochemistry , Analysis of Variance , Laminin/analysis , Rats, Wistar , Muscle, Skeletal/injuries , Desmin/analysisABSTRACT
Sarcomas são neoplasias mesenquimais malignas, raras, que acometem, principalmente, crianças e adolescentes. O rabdomiossarcoma, subtipo oriundo da musculatura esquelética, é condição incomum em adultos, acometendo sítios de localização não habitual, crescimento rápido e de difícil tratamento. Apresenta-se caso de adulto jovem com nodulação em lóbulo auricular esquerdo, cuja análise histopatológica e imuno-histoquímica confirmou tratar-se de rabdomiossarcoma alveolar, o qual foi conduzido em conjunto com a Oncologia.
Sarcomas are rare and malignant mesenchymal neoplasms that mainly affect children and adolescents. Rhabdomyosarcoma, a subtype originating from skeletal muscle, is an uncommon condition in adults. It affects sites of unusual location, presents fast growth, and is challenging to treat. We report a case of a young adult with nodules in the left auricular lobe. The histopathological and immunohistochemical analysis confirmed the alveolar rhabdomyosarcoma, and treatment was conducted in association with Oncology.
ABSTRACT
Knowing the ultrastructure of skeletal muscle is critical to understand how it works under normal situation and the disorders caused by extreme or pathological conditions. Sarcomere is the basic structural unit of striated muscle tissue. An important element of sarcomere architecture are the intermediate filaments, including the desmin protein. Desmin protein contributes to maintenance of cell integrity, efficient transmission of force and mechanochemical signaling within the myocyte. Because of this, desmin protein has constantly been a focus of research that investigates its alterations associated to damage and muscle atrophy under different conditions. The purpose of the following literature review is to describe the basic concepts of muscle ultrastructure, emphasizing the desmin protein role under conditions of muscle disuse atrophy and aging.
Conocer la ultraestructura del músculo esquelético es crítico para entender cómo trabaja bajo situaciones normales y en desórdenes causados por condiciones extremas o patológicas. La sarcómera es la unidad de estructura básica del tejido muscular estriado. Elementos importantes en la arquitectura de la sarcómera son los filamentos intermedios, incluyendo la proteína desmina. La proteína desmina contribuye en mantener la integridad celular, la transmisión eficiente de fuerza y la señalización mecanoquímica dentro del miocito. Debido a lo anterior, la proteína desmina ha sido constante foco de investigación en trabajos que estudian sus alteraciones asociadas a daño y atrofia muscular bajo diferentes condiciones. El propósito de la siguiente revisión de la literatura es describir los conceptos básicos de la ultraestructura muscular, enfatizando en el rol de la proteína desmina bajo condiciones de atrofia muscular por desuso y envejecimiento.
Subject(s)
Humans , Animals , Sarcomeres/ultrastructure , Aging , Muscle, Skeletal/ultrastructure , Desmin/ultrastructure , Intermediate Filaments/ultrastructureABSTRACT
Geralmente, a cardiomiopatia restritiva por deposição de desmina é caracterizada pela restrição ao enchimento diastólico ventricular e por diferentes graus de bloqueio atrioventricular (BAV). Neste relato, são descritas as alterações anatomopatológicas do sistema de condução cardíaco relacionadas ao BAV. O nó sinusal, o nó compacto e o feixe penetrante (feixe de His) não apresentavam anormalidades, entretanto, havia extensa fibrose das porções terminais do feixe ramificante e do início dos feixes esquerdo e direito, no topo do septo ventricular. A patogenia dessa substituição fibrosa é provavelmente a mesma que origina a extensa fibrose do miocárdio ventricular contrátil, e permanece por ser elucidada.
Generally, restrictive cardiomyopathy due to desmin deposition is characterized by restriction to ventricular diastolic filling and different degrees of atrioventricular block (AVB). In this report, we describe the pathological changes of the cardiac conduction system related to AVB. The sinus node, the compact node, and the penetrating bundle (bundle of His) had no abnormalities, however, there was extensive fibrosis of the terminal portions of the branching bundle and the beginning of the left and right bundles at the top of the ventricular septum. The pathogenesis of this fibrous replacement is probably the same that leads to extensive fibrosis of the working ventricular myocardium, and remains to be elucidated.
En general, la miocardiopatía restrictiva, debido a la deposición de desmina se caracteriza por la restricción de llenado diastólico ventricular y por los distintos grados de bloqueo auriculoventricular (BAV). En este informe, se describen los cambios anatómicos y patológicos del sistema de conducción cardiaco relacionado con BAV. El nodo sinusal, el nodo compacto y haz penetrante (haz de His) no tuvo alteraciones, sin embargo, había fibrosis extensa de las porciones terminales del haz en porción ramificante y del comienzo de los haces izquierda y derecha, en la parte superior del tabique ventricular. La patogenia de esta sustitución fibrosa es probablemente la misma que origina la fibrosis extensa del miocardio ventricular contráctil, y queda por dilucidar.
Subject(s)
Humans , Male , Adolescent , Adult , Atrioventricular Block/etiology , Cardiomyopathy, Restrictive/complications , Desmin/metabolism , Heart Conduction System/pathology , Atrioventricular Block/pathology , Cardiomyopathy, Restrictive/pathology , Fatal Outcome , FibrosisABSTRACT
Nemaline myopathy (NM) is a congenital disease that leads to hypotonia and feeding difficulties in neonates. Some cases have a more benign course, with skeletal abnormalities later in life. We analyzed a series of eight patients with NM obtained from a retrospective analysis of 4300 muscle biopsies. Patients were classified as having the typical form in five cases, intermediate form in two cases and severe form in one case. Histochemical analysis showed mixed rods distribution in all cases and predominance of type I fibers in five cases. Immunohistochemical analysis showed abnormal nebulin expression in all patients (four heterogeneous and four absent), homogeneous desmin expression in four cases, strongly positive in three and absent in one, fast myosin expression in a mosaic pattern in six cases and absent in two cases. There was no specific relation between these protein expression patterns and the clinical forms of NM.
Miopatia nemalínica (NM) é uma doença congênita que leva a hipotonia e dificuldade de sugar em neonatos. Alguns casos possuem uma evolução benigna, com deformidades ósseas tardias. Nós analisamos uma série de oito pacientes com NM obtidos da análise retrospectiva de 4300 biópsias musculares. Os pacientes foram classificados como forma típica em cinco casos, forma intermediária em dois casos e forma severa em um caso. Análise histoquímica mostrou distribuição mista dos rods em todos os casos e predominância de fibras tipo I em cinco casos. Análise imuno-histoquímica mostrou expressão anormal da nebulina em todos os pacientes (quatro heterogênea e quatro ausente), expressão homogenea da desmina em quatro casos, fortemente positiva em tres e ausente em um, expressão da miosina (rápida) com padrão em mosaico em seis casos e ausente em dois casos. Não há relação específica entre a expressão destas proteínas e as formas clínicas da NM.
Subject(s)
Child , Child, Preschool , Female , Humans , Infant , Male , Desmin/metabolism , Immunohistochemistry , Muscle Proteins/metabolism , Muscles/pathology , Myopathies, Nemaline/pathology , Myosins/metabolism , Biopsy , Electromyography , Myopathies, Nemaline/metabolism , Retrospective Studies , Severity of Illness IndexABSTRACT
Se presenta mujer de 46 años de edad con dolor lumbar y síndrome general a quien se le realizaron exámenes complementarios y Tomografía axial computarizada que arrojó la presencia de tumor en el espacio retroperitoneal. Posteriormente, la paciente es intervenida. Se envía el especimen quirúrgico al departamento de Anatomía Patológica. Nos motivamos en la presentación porque la poca frecuencia en esta localización ocasiona diagnóstico controvertido, pues las contrapartes malignas son las que toman la primacía. Unimos además, la sintomatología acompañante y el resultado de la imagenología practicada hace sospechar el diagnóstico de malignidad. Finalmente, queríamos referir que, a pesar del tamaño del tumor, los síntomas compresivos que ocasionó, y el resultado de Tomografía axial computarizada que hicieron pensar en la posibilidad de la malignidad desde el punto de vista clínico, nos sorprendió que se tratara de un tumor benigno de partes blandas.
We report a 46 years age, female with lumbar pain and general syndrome whom was achieved several tests and computed tomography scan that releaved the presence of tumour in the retroperitoneal space. Later ,she was submitted to surgical intervention. Surgical specimen was sent to the Pathological Anatomy department . We gave a reason for the presentation of that due to its controversial diagnosis and a few frequency in this site where the hightest frequency belongs to the malign counterpart neoplasms .We also takes into account the symptons and the result of the computed tomography scan that it thougth us of the malignant diagnosis from clinical point of view but we have very surprised that it was a benign tumour of soft parts.
ABSTRACT
Se presenta mujer de 46 años de edad con dolor lumbar y síndrome general a quien se le realizaron exámenes complementarios y Tomografía axial computarizada que arrojó la presencia de tumor en el espacio retroperitoneal. Posteriormente, la paciente es intervenida. Se envía el especimen quirúrgico al departamento de Anatomía Patológica. Nos motivamos en la presentación porque la poca frecuencia en esta localización ocasiona diagnóstico controvertido, pues las contrapartes malignas son las que toman la primacía. Unimos además, la sintomatología acompañante y el resultado de la imagenología practicada hace sospechar el diagnóstico de malignidad. Finalmente, queríamos referir que, a pesar del tamaño del tumor, los síntomas compresivos que ocasionó, y el resultado de Tomografía axial computarizada que hicieron pensar en la posibilidad de la malignidad desde el punto de vista clínico, nos sorprendió que se tratara de un tumor benigno de partes blandas(AU)
We report a 46 years age, female with lumbar pain and general syndrome whom was achieved several tests and computed tomography scan that releaved the presence of tumour in the retroperitoneal space. Later ,she was submitted to surgical intervention. Surgical specimen was sent to the Pathological Anatomy department . We gave a reason for the presentation of that due to its controversial diagnosis and a few frequency in this site where the hightest frequency belongs to the malign counterpart neoplasms .We also takes into account the symptons and the result of the computed tomography scan that it thougth us of the malignant diagnosis from clinical point of view but we have very surprised that it was a benign tumour of soft parts(AU)
Subject(s)
Humans , Female , Middle Aged , Leiomyoma/pathology , Leiomyoma/surgeryABSTRACT
As cardiomiopatias restritivas podem possuir diversas etiologias, entre elas destacam-se as doenças de depósito por diferentes materiais, como por desmina. As desminopatias são patologias incomuns, que evoluem com distúrbios de condução, miopatias periféricas e disfunção ventricular. O presente relato descreve paciente com bloqueio átrio ventricular total como evento inicial, sendo acometido posteriormente por alterações musculares esqueléticas e insuficiência cardíaca. Na investigação foi definido diagnóstico de cardiomiopatia restritiva por acúmulo de desmina.
Restrictive cardiomyopathies may have different etiologies, among which we can point out storage diseases by accumulation of different materials such as desmin. Desminopathies are uncommon diseases that progress with conduction abnormalities, peripheral myopathies, and ventricular dysfunction. The present report describes a patient with complete atrioventricular block as the initial event; he later developed skeletal muscle alterations and heart failure. The investigation led to the diagnosis of restrictive cardiomyopathy due to desmin accumulation.