ABSTRACT
El rabdomiosarcoma es el tumor maligno de partes blandas más frecuente en la edad pediátrica. Puede afectar cualquier localización anatómica. El subtipo histológico alveolar suele causar lesiones en las extremidades en niños de mayor edad. Los sitios metástasicos más frecuentes son el pulmón, la médula ósea, el hueso y los ganglios linfáticos. Describimos el caso de un paciente con rabdomiosarcoma alveolar (RA) con metástasis cardíaca, una presentación poco frecuente de la patología.
Rhabdomyosarcoma is the most common malignant soft tissue tumor in pediatric age. It can affect any anatomical location. Alveolar histological subtype usually presents lesions on the extremities in older children. The most common metastatic sites are the lung, bone marrow, bone and lymph node. We describe a case of alveolar rhabdomyosarcoma with cardiac metastasis in a pediatric patient, a rare presentation of the pathology.
Subject(s)
Humans , Male , Child , Soft Tissue Neoplasms/pathology , Rhabdomyosarcoma, Alveolar/pathology , Foot Diseases/pathology , Heart Neoplasms/secondary , Rhabdomyosarcoma, Alveolar/diagnosis , Rhabdomyosarcoma, Alveolar/therapy , Ventricular Septum , Foot Diseases/diagnosis , Foot Diseases/drug therapy , Heart Neoplasms/diagnosis , Heart Neoplasms/drug therapy , Neoplasm StagingABSTRACT
Los linfomas cardíacos primarios son un subtipo muy poco frecuente de tumor en los cuales la lesión primaria se encuentra en el corazón. Los tumores suelen ser infiltrantes y se localizan en la aurícula derecha, seguidos del pericardio. Su mortalidad es notablemente alta y el diagnóstico tardío es el principal factor para su mal pronóstico. Describimos el caso de un paciente que presentó shock obstructivo por derrame pericárdico profuso causado por un tipo raro de tumor cardíaco primario, un linfoma pericárdico de células T/NK.
Primary cardiac lymphomas are a rare subtype of lymphomas in which the primary lesion is in the heart. The tumors are usually located in the right atria, followed by the pericardium and are frequently infiltrative. Mortality is remarkably high in this group and the delayed diagnosis is the main factor for its poor prognosis. We describe the case of a patient that presented with obstructive shock due to profuse pericardial effuse caused by a rare kind of primary cardiac tumor, a T/NK cell pericardial lymphoma.
Os linfomas cardíacos primários são um subtipo de tumor muito raro, no qual a lesão primária está no coração. Os tumores geralmente são infiltrativos e localizam-se no átrio direito, seguidos pelo pericárdio. Sua mortalidade é notavelmente alta e o diagnóstico tardio é o principal fator que produz seu mau prognóstico. Descrevemos o caso de um paciente que apresentou choque obstrutivo devido a um derrame pericárdico profuso causado por um tipo raro de tumor cardíaco primário, um linfoma pericárdico de células T/NK.
Subject(s)
Humans , Female , Aged , Lymphoma, T-Cell/pathology , Lymphoma, T-Cell/drug therapy , Lymphoma, T-Cell/diagnostic imaging , Heart Neoplasms/pathology , Heart Neoplasms/drug therapy , Heart Neoplasms/diagnostic imaging , Pericardial Effusion/therapy , Pericardial Effusion/diagnostic imaging , Pericardium/pathology , Cardiac Tamponade/therapyABSTRACT
Primary cardiac lymphoma (PCL) is a rare and fatal disorder. It may often mimic other common cardiac tumors like cardiac myxoma because of similarities in the clinical presentation. We report a case of PCL of diffuse large B-cell type, in a 38-year-old, immunocompetent male who presented with superior vena cava syndrome that was excised as a myxoma. Histology revealed a large cell population diffusely and strongly expressing CD45, CD20, MUM1/IRF4 and FOXP1 hinting at an activated B-cell (ABC)-like phenotype. After four cycles of Rituximab with CHOP (cyclophosphamide, hydroxydaunorubicin, Oncovin, and prednisolone) the tumor regressed completely but the patient had a relapse and subsequently succumbed to the disease confirming the aggressive nature. The aggressive behavior of PCL may be possibly linked to its ABC-like origin.
Subject(s)
Adult , Antibodies, Monoclonal, Murine-Derived/administration & dosage , Antigens, CD20/biosynthesis , Leukocyte Common Antigens/biosynthesis , Antineoplastic Agents/administration & dosage , Antineoplastic Combined Chemotherapy Protocols/administration & dosage , B-Lymphocytes/immunology , Cyclophosphamide/administration & dosage , Doxorubicin/administration & dosage , Fatal Outcome , Forkhead Transcription Factors/biosynthesis , Gene Expression Profiling , Heart Neoplasms/complications , Heart Neoplasms/diagnosis , Heart Neoplasms/drug therapy , Heart Neoplasms/pathology , Histocytochemistry , Humans , Immunohistochemistry , Interferon Regulatory Factors/biosynthesis , Lymphoma, B-Cell/complications , Lymphoma, B-Cell/diagnosis , Lymphoma, B-Cell/drug therapy , Lymphoma, B-Cell/pathology , Male , Microscopy , Phenotype , Prednisone/administration & dosage , Radiography, Thoracic , Recurrence , Repressor Proteins/biosynthesis , Superior Vena Cava Syndrome/diagnosis , Superior Vena Cava Syndrome/pathology , Tomography, X-Ray Computed , Treatment Outcome , Vincristine/administration & dosageABSTRACT
We report the case of a 33-year-old male with primary seminoma of the anterior mediastinum with initial clinical manifestations suggestive of heart disease
Subject(s)
Humans , Male , Adult , Heart Neoplasms/secondary , Ventricular Outflow Obstruction/etiology , Seminoma/secondary , Testicular Neoplasms/pathology , Heart Neoplasms/drug therapy , Ventricular Outflow Obstruction/drug therapy , Seminoma/drug therapy , Heart VentriclesABSTRACT
Rabdomiosarcoma primário do coraçäo obstruindo veias cavas, foi diagnosticado em uma criança de 10 anos de idade, ocm quadro clínico recente de três meses, caracterizado por comprometimento do estado geral, febre persistente e acentuado emagrecimento. Säo enfatizados os aspectos clínicos e os exames complementares desta rara afecçäo, que permitem o diagnóstico em vida, bem como a conduta terapêutica realizada e sua evoluçäo
The authors report a case of a child, 10 years old with a primary rhabdomyosarcoma of the heart that obstructed both caval veins. The clinical picture that lastedjust three months before the admission was characterized by persistent fever, accentuated loss of weight and a very deteriorated general aspect. We emphazise the clinical aspectos and other subsidiary elements that easily allowed to make the precise diagnosis in life as well as the therapeutic measures andfollow up, besides a review ofthe literature, where it was confirmed the rarity of this pathological entity.