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
La afección metastásica de la mama de una neoplasia extra mamaria maligna es un hecho infrecuente y constituye aproximadamente el 1,7% de todos los procesos malignos de la mama. Por otra parte, los sarcomas de tejidos blandos constituyen tan solo el 0,7% de todos los tumores malignos del organismo, por lo que es un hecho infrecuente encontrar en la mama una metástasis procedente de un sarcoma de partes blandas extra mamario, Se hace la referencia de un caso visto en el Hospital Coromoto de Maracaibo, paciente de 14 años con rabdomiosarcoma metastásico en mama izquierda, sin evidencia de enfermedad generalizada a quien se le practica mastectomía radical modificada, radioterapia y quimioterapia dándole así el tratamiento adecuado para este tipo de tumor
The condition of metastatic breast extra mammary malignant neoplasm is rare event and is about of 1.7% of all malignancies of the breast. On the other hand the soft tissue sarcomas constitute only 0.7% of incidence in relation to the all malignant tumors in the body, making it a rare event found in the breast, found metastasis from an extra mammary soft tissue sarcoma, reference is made a case view and treated in the Coromoto Hospital in Maracaibo city, is a patient 14 years old with metastatic rhabdomyosarcoma in the left breast with no evidence of widespread disease, who underwent modified radical mastectomy, giving chemotherapy and radiation therapy after surgery how a appropriate form of treatment for these kind of tumors
Subject(s)
Female , Neoplasm Metastasis/diagnosis , Neoplasm Metastasis/therapy , Breast Neoplasms/therapy , Rhabdomyosarcoma, Alveolar/pathology , Rhabdomyosarcoma, Alveolar/therapy , Breast Neoplasms/surgery , Breast Neoplasms/pathology , Medical OncologyABSTRACT
O rabdomiossarcoma é o tumor de partes moles mais comum na infância, sendo raro o acometimento exclusivamente cutâneo. Apresenta-se caso de criança com nódulo doloroso na face, cuja análise histopatológica e imunoistoquímica confirmou tratar-se de rabdomiossarcoma, o qual foi conduzido por equipe multidisciplinar. Os tumores de partes moles são responsáveis por 6 por cento de todos os tumores infantis; destes, 53 por cento são rabdomiossarcomas, que podem acometer qualquer sítio. A manifestação como nódulo dérmico é incomum, representando um desafio diagnóstico, já que não possui características clínicas que o diferenciem de outras patologias.
Rhabdomyosarcoma is the most common soft tissue tumor in childhood; however, it rarely affects only the skin. This case report describes a child with a painful nodule on her face. Histopathology and immunohistochemistry confirmed the diagnosis of rhabdomyosarcoma, and a multidisciplinary team then followed up the patient. Soft tissue tumors are responsible for 6 percent of all childhood tumors, and 53 percent of these are rhabdomyosarcomas, which may affect any part of the body. Presentation in the form of skin nodules is rare and represents a diagnostic challenge, since there are no clinical characteristics that differentiate this condition from other pathologies.
Subject(s)
Child, Preschool , Female , Humans , Facial Neoplasms/pathology , Rhabdomyosarcoma, Alveolar/pathology , Skin Neoplasms/pathology , Chemotherapy, Adjuvant , Facial Neoplasms/therapy , Neoplasm Staging , Rhabdomyosarcoma, Alveolar/therapy , Skin Neoplasms/therapySubject(s)
Humans , Adolescent , Female , Skin Neoplasms/secondary , Rhabdomyosarcoma, Alveolar/pathology , ThighABSTRACT
PURPOSE: A case study and literature review of alveolar rhabdomyosarcoma (RMS) in an adult. METHODS: A 48-year-old male patient presented at our clinic complaining of proptosis that had persisted for 2 weeks in his left eye. A computed tomography (CT) scan revealed a destructive soft-tissue mass in the left ethmoid sinus with invasion of the left orbit and compression of the medial rectus muscle. Endoscopic intranasal biopsy revealed alveolar RMS. Conservative debulking and orbital wall decompression were performed. RESULTS: Immunohistochemical testing was positive for desmin, S-100, and smooth muscle actin (SMA), supporting the diagnosis of RMS. Since ipsilateral cervical and spinal metastasis was detected, systemic treatment was administered simultaneously. CONCLUSIONS: Although rarely found in adults, RMS should be considered in the differential diagnosis of orbital tumors. Immunohistochemical analysis plays an important role in the definitive diagnosis of RMS.
Subject(s)
Middle Aged , Male , Humans , Tomography, X-Ray Computed , Rhabdomyosarcoma, Alveolar/pathology , Paranasal Sinus Neoplasms/pathology , Orbital Neoplasms/pathology , Neoplasm Invasiveness , Magnetic Resonance Imaging , Ethmoid Sinus , Diagnosis, Differential , BiopsyABSTRACT
Se informan cinco casos de rabdomiosarcoma alveolar con presentación clínica atípica. Tres pacientes presentaron linfadenomegalias como primera manifestación clínica, imitando el comportamiento de un linfoma o de un tumor primario no identificado con metástasis ganglionares. Un sujeto presentó enfermedad tumoral sistémica, y dos presentaron el tumor primario en localizaciones atípicas, como el mediastino y el retroperitoneo. Todos los enfermos fallecieron y cuatro fueron autopsiados. El diagnóstico histopatológico fue confirmado por medio de estudios de inmunohistoquímica en cuatro casos. El rabdomiosarcoma alveolar tiene mal pronóstico y puede presentar un comportamiento clínico y una morfología variable, simulando linfomas, leucemias y enfermedad metastásica sistémica sin tumor primario conocido, como en los casos que aquí se informan