A novel case of left atrial and right lung mass turned out to be unconventional metastasis of uterine leiomyosarcoma with a review of literature

Uterine leiomyosarcoma (ULMS) is a rare malignancy arising from the myometrial smooth muscle wall, and cardiac metastases are extremely rare. Metastasis to the heart is a very unusual finding, and atrial metastasis is even rarer. Here, we report a case of a 45 year old woman who presented with dyspnea and pleural effusion and had a significant history of hysterectomy done for ULMS. Magnetic resonance imaging revealed a left atrial mass, which was resected and revealed to be a metastasis of leiomyosarcoma on histopathology and immunohistochemistry. Metastatic ULMS may rarely present as a left atrial mass with acute clinical presentation. Detailed clinical history and accurate diagnosis are vital for further management.

A case of anomalous horizontal fissure and hypoplastic middle lobe of the right lung / Un caso de fisura horizontal anómala y lóbulo medio hipoplásico del pulmón derecho

During dissection practice for medical students at Nagasaki University, a horizontal fissure and hypoplastic middle lobe were encountered in the right lung of an 81-year-old Japanese female. In a normal right lung, the horizontal fissure is situated anterior to the oblique fissure. On the contrary, in the present case, the horizontal fissure was situated posterior to the oblique fissure. We identified a small, additional lobe on the medial surface of this lung. It was situated in the medial part of the oblique fissure, and was determined to be a small hypoplastic middle lobe. As a result of the hypoplasia of the middle lobe, the original horizontal fissure may have disappeared from the anterior surface. The horizontal fissure, in this case, may be a compensatory additional fissure that developed within the inferior lobe. This horizontal fissure was incomplete, both in length and depth. The left lung was normal. This case might be very rare; however, it may aid in the understanding of normal lung development. Therefore, we report this case, including its bronchial branching and vascular distribution.

Durante la práctica de disección para estudiantes de medicina en la Universidad de Nagasaki, se encontró una fisura horizontal y un lóbulo medio hipoplásico en el pulmón derecho de una mujer japonesa de 81 años de edad. En un pulmón derecho normal, la fisura horizontal está situada anterior a la fisura oblicua. Por el contrario, en el presente caso, la fisura horizontal estaba situada posterior a la fisura oblicua. Se identificó un pequeño lóbulo adicional en la superficie medial de este pulmón. Estaba situado en la parte medial de la fisura oblicua, y se determinó que era un pequeño lóbulo medio hipoplásico. Como resultado de la hipoplasia del lóbulo medio, la fisura horizontal original puede haber desaparecido de la superficie anterior. La fisura horizontal, en este caso, puede ser una fisura adicional compensatoria que se desarrolla dentro del lóbulo inferior. Esta fisura horizontal era incompleta, tanto en longitud como en profundidad. El pulmón izquierdo era normal. Este caso podría ser muy raro; Sin embargo, puede ayudar en la comprensión del desarrollo pulmonar normal. Por lo tanto, informamos este variación, incluyendo su ramificación bronquial y la distribución vascular.

Síndrome de la cimitarra. Correlación anatomoembriológica / Scimitar syndrome. Correlation anatomo-embryological

Resumen Objetivos Describir morfológicamente un bloque visceral toracoabdominal de un caso de síndrome de la cimitarra. Proponer una hipótesis patogenética que explique el desarrollo de las conexiones venosas pulmonares de este síndrome. Método El espécimen anatómico se describió con el sistema secuencial segmentario. Se determinó el situs, las conexiones entre los segmentos cardíacos y las anomalías agregadas. Se describió la anatomía de ambos pulmones, incluida la conexión venosa pulmonar. Se elaboró una hipótesis patogenética que explica la conexión venosa pulmonar a través de una correlación entre la patología del síndrome y el desarrollo normal de las venas pulmonares. Resultados El situs fue solitus, las conexiones entre los segmentos cardíacos fueron normales, existió hipoplasia y displasia del pulmón derecho con secuestro del lóbulo inferior; las venas pulmonares derechas drenaron a un colector curvo en la porción suprahepática de la vena cava inferior y las venas pulmonares izquierdas lo hicieron a la aurícula izquierda. El secuestro pulmonar recibió irrigación a través de una colateral aortopulmonar. Existió una comunicación interauricular. Conclusiones La hipótesis patogenética propone que las conexiones venosas pulmonares de este síndrome representan la persistencia del horizonte XIV de Streeter (28-30 días de desarrollo), período en el que el seno de las venas pulmonares presenta conexión venosa doble con la aurícula izquierda y con un colector de la conexión primitiva que desemboca en la vitelina derecha de la que se deriva la porción suprahepática de la vena cava inferior.

Abstract Objectives To describe morphologically a toracoabdominal visceral block of a scimitar's syndrome case. We propose a pathogenetic theory wich explains the development of the pulmonary venous connection in this syndrome. Method The anatomic specimen was described with the segmental sequential system. The situs was solitus, the connections between the cardiac segments and the associated anomalies were determined. The anatomy of both lungs, including the venous pulmonary connection, was described. A pathogenetic hypothesis was made, which explains the pulmonary venous connection throw a correlation between the pathology of this syndrome and the normal development of the pulmonary veins. Results The situs was solitus, the connections of the cardiac chambers were normal; there were hypoplasia and dysplasia of the right lung with sequestration of the inferior lobe; the right pulmonary veins were connected with a curved collector which drainaged into the suprahepatic segment of the inferior vena cava; the left pulmonary veins were open into the left atrium. The sequestered inferior lobe of the right lung received irrigation throw a collateral aortopulmonary vessel. There was an atrial septal defect. Conclusions The pathogenetic hypothesis propose that the pulmonary venous connection in this syndrome represent the persistent of the Streeter's horizon XIV (28-30 days of development), period in which the sinus of the pulmonary veins has double connection, with the left atrium and with a primitive collector into the right viteline vein which forms the suprahepatic segment of the inferior vena cava.

Síndrome da cimitarra: A propósito de três casos singulares / Scimitar syndrome: the purpose of three individual cases

Os autores relatam três casos de síndrome da cimitarra (SC), que em sua forma habitual cursa com drenagem venosa anômala no pulmão direito, de aspecto radiográfico característico, hipoplasia no referido pulmão e dextroposição cardíaca. Enfatizam sobre a radiografia simples do tórax, como principal exame na investigação diagnóstica, e o valor do estudo angiográfico, na elucidação de anomalias vasculares quando houver indicação cirúrgica. Outros exames complementares, como a tomografia computadorizada, são avaliados para a perfeita caracterização dessa síndrome e de suas variantes...

The authors write about three cases of scimitar syndrome which, commonly, releases as an atypical venous drainage of the right lung, with characteristic image, as well as hypoplasia of the same lung and dextropositioned heart. They emphasize the thoracic radiographic image as the most important complementary method of diagnosis and the importance of the angiographic study for the diagnose of vascular abnormalities with surgical indication. Other exams such as computerized tomography are of value in characterization of syndrome and its variants...

Síndrome de la cimitarra: un caso interesante / Scimitar syndrome: an interesting case

Introducción. El síndrome de la cimitarra es una anomalía congénita inusual caracterizada por un drenaje venoso anómalo, total o parcial, del pulmón derecho en la vena cava inferior, un suministro arterial sistémico parcial, hipoplasia del pulmón afectado, dextrocardia y anormalidades bronquiales y en la lobulación pulmonar. La alteración fundamental obligatoria es el retorno venoso pulmonar anormal derecho total o de sus dos tercios superiores a la vena cava inferior (imagen que semeja a una cimitarra, una espada turca, corta y curva). La orientación diagnóstica se lleva a cabo con exámenes de gabinete y con el cuadro clínico asociado a los hallazgos de la radiografía de tórax. Caso clínico. La imagen radiográfica demuestra la presencia del corazón hacia la derecha, asociado a una imagen pulmonar disminuida de tamaño. Estos hallazgos obligan a realizar un cateterismo cardiaco y/o una angiotomografía para confirmar el diagnóstico. La complicación de este padecimiento va de acuerdo al tiempo de aparición de los síntomas cuando se sospecha el cortocircuito de izquierda a derecha, los signos de hipertensión arterial pulmonar que se comprueban con un ecocardiograma y un cateterismo cardiaco y con presencia de anomalías congénitas como hipoplasia pulmonar derecha, secuestro pulmonar con presión de vena anómala y presencia de malformaciones cardiovasculares asociadas. Conclusiones. El pronóstico de estos pacientes, tanto en niños mayores como en adultos, es generalmente bueno. El tratamiento es conservador; el tratamiento quirúrgico sólo es considerado en pacientes sintomáticos y en aquéllos con anormalidades cardiacas asociadas.

Background. Scimitar syndrome is a rare congenital anomaly characterized by abnormal venous drainage of all or part of the right lung into the inferior vena cava due to partial systemic arterial supply, hypoplasia of the affected lung, or bronchial abnormalities and pulmonary lobulation. The fundamental change required is the right abnormal pulmonary venous return, total or upper two-thirds of the inferior vena cava (an image that resembles the scimitar or Turkish sword). The condition is diagnosed using laboratory analyses and clinical findings associated with chest x-ray. Case report. The radiographic image shows the presence of right heart, an image associated with decreased lung size. These findings require performing cardiac catheterization and/or angiotomography to confirm the diagnosis. The complication of this disease is in accordance with time of onset of symptoms of suspected short circuit from left to right. Signs of pulmonary hypertension are evidenced by echocardiography and the presence of congenital anomalies such as hypoplastic right lung, pulmonary sequestration, anomalous venous pressure and associated cardiovascular malformations. Conclusions. In older children and adults, the prognosis is good with conservative treatment. Surgical treatment is considered in symptomatic patients and in those patients with associated cardiac abnormalities.

A Case of Agenesis of the Right Lung Accompanied by Cleft Lip and Palate

This paper describes a case of agenesis of the right lung in a male neonate, who suffered respiratory difficulty. He was evaluated by plain X-ray, bronchography, lung perfusion scan, chest CT, and brain CT. After he died, autopsy was performed. The results revealed multiple anomalies which included cleft lip and palate, absence of left thumb and radius bone, hemivertebra between L1 and L2, arachnoid cyst in occipital lobe of brain. We report this case with brief review fo some related literature.