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      Desarticulación de cadera por sarcoma sinovial monofásico: Caso clínico Translated title: Hip disarticulation by monophasic synovial sarcoma: Case report

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          Abstract

          El sarcoma sinovial es una entidad morfológica, clínica y genéticamente distinta y puede tener cualquier otra ubicación. No existe una terapia estandarizada. Generalmente el tratamiento es quirúrgico, con resección radical de la lesión, aplicándose en algunos casos radioterapia adyuvante. Los sarcomas sinoviales han mostrado quimiosensibilidad a la ifosfamida con una respuesta aproximada del 24%. Se encuentran presentes más frecuentemente en las extremidades, siendo su diseminación metastásica a ganglios linfáticos regionales y/o pulmón, hígado, hueso y cerebro. Se trata de un caso clínico: paciente femenino de 40 años de edad, portadora de tumor en miembro inferior derecho, en el tercio medio del muslo, de 15x10 cm de diámetro, que compromete el vasto anterior, lateral y posterior del cuádriceps derecho, con irrupción de la aponeurosis y extensión al tejido celular subcutáneo. Por medio de la inmunohistoquímica se observó inmunomarcaje de las células neoplásicas con EMA, QAE1/AE3, vimentina, CD99, CD34 y Bcl2, como diagnóstico de sarcoma sinovial monofásico. Para el tratamiento se procedió a desarticulación de cadera, postoperatorio sin complicaciones y evolución satisfactoria. Se refiere al servicio de oncología médica para tratamiento coadyuvante. Se concluye que los procedimientos quirúrgicos en el tratamiento de tumores en extremidades se divide en resección amplia del tumor con preservación del miembro mediante endoprotesis, y la desarticulación, no existiendo evidencias clínicas que soporten la ventaja sobre cualquier otra opción terapéutica, sin embargo, el objetivo principal del procedimiento oncológico es controlar el tumor para disminuir la recurrencia y mejorar la supervivencia del paciente.

          Translated abstract

          Synovial sarcoma is a clinically and genetically distinct morphological entity, and can be found in any location. There is no standard therapy. Treatment is usually surgical; with radical resection of the lesion, applying, in some cases, adjuvant radiotherapy. Synovial sarcomas have shown chemosensitivity to ifosfamide with an approximate 24% response. They are most often present in the extremities causing metastatic spread to regional lymph nodes and/or lung, liver, bone and brain. It is a clinical case: a female 40 year old patient with a tumor in right leg, on the middle third of the thigh, 15x10 cm in diameter, that involves the anterior, lateral and posterior vastus of the right quadriceps, with emergence of aponeurosis and extension to the subcutaneous tissue. Through immunohistochemical test, immuno-labeling in neoplastic cells with EMA, QAE1/AE3, vimentin, CD99, CD34 and Bcl2, as diagnosis of monophasic synovial sarcoma, was observed. For treatment, hip disarticulation was carried out, no postoperative complications and satisfactory outcome. The patient is referred to medical oncology service for adjuvant treatment. We conclude that surgical procedure in the treatment of tumors in extremities is divided into wide resection of the tumor with preservation of the member by endoprosthesis, and disarticulation amputees, with no clinical evidence to support the benefit of any other treatment option, however, the main purpose of the oncologic procedure is to control the tumor recurrence and improve patient survival.

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          Most cited references38

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          Soft-tissue sarcomas in adults.

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            Evidence for human lung stem cells.

            Although progenitor cells have been described in distinct anatomical regions of the lung, description of resident stem cells has remained elusive. Surgical lung-tissue specimens were studied in situ to identify and characterize human lung stem cells. We defined their phenotype and functional properties in vitro and in vivo. Human lungs contain undifferentiated human lung stem cells nested in niches in the distal airways. These cells are self-renewing, clonogenic, and multipotent in vitro. After injection into damaged mouse lung in vivo, human lung stem cells form human bronchioles, alveoli, and pulmonary vessels integrated structurally and functionally with the damaged organ. The formation of a chimeric lung was confirmed by detection of human transcripts for epithelial and vascular genes. In addition, the self-renewal and long-term proliferation of human lung stem cells was shown in serial-transplantation assays. Human lungs contain identifiable stem cells. In animal models, these cells participate in tissue homeostasis and regeneration. They have the undemonstrated potential to promote tissue restoration in patients with lung disease. (Funded by the National Institutes of Health.).
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              Chemotherapy is associated with improved survival in adult patients with primary extremity synovial sarcoma.

              To determine if ifosfamide-based chemotherapy (IF) offers a survival benefit to adult patients with primary extremity synovial sarcoma. Prospectively collected patient data from 2 institutions was used to identify all adult patients (>or=16 years) with >or=5 cm, deep, primary, extremity, synovial sarcoma that underwent surgical treatment of cure from 1990 to 2002. A total of 101 patients were identified and the median follow-up for survivors was 58 months. Clinical, pathologic, and treatment variables were analyzed for disease-specific survival (DSS), distant recurrence-free survival (DRFS), and local recurrence-free survival (LRFS). Sixty-eight (67%) patients were treated with IF and 33 (33%) patients received no chemotherapy (NoC) for the primary tumor. The characteristics of the IF-treated patients [median tumor size = 7.2 cm; monophasic n = 46 (68%)] were similar to NoC patients [median tumor size = 7 cm; monophasic n = 23 (70%)]. The 4-year DSS of the IF-treated patients was 88% compared with 67% for the NoC patients (P = 0.01). Smaller size (HR = 0.3 per 5-cm decrease, P < 0.0001) and treatment with IF (HR = 0.3 compared with NoC, P = 0.007) were independently associated with an improved DSS. Treatment with IF was independently associated with an improved DRFS (HR = 0.4, P = 0.03) but not associated with an improved LRFS (P = 0.39). Ifosfamide-based chemotherapy was associated with an improved DSS in adult patients with high-risk, primary, extremity, synovial sarcoma and should be considered in the treatment of such patients.
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                Author and article information

                Journal
                s
                Salus
                Salus
                Universidad de Carabobo (Valencia, Carabobo, Venezuela )
                1316-7138
                August 2014
                : 18
                : 2
                : 13-17
                Affiliations
                [01] Valencia orgnameUniversidad de Carabobo orgdiv1Facultad de Ciencias de la Salud orgdiv2Escuela de Medicina Venezuela
                Article
                S1316-71382014000200004 S1316-7138(14)01800204
                bddbfa8e-bfff-4572-aaed-fcaf0f6daaae

                http://creativecommons.org/licenses/by/4.0/

                History
                : December 2013
                : July 2014
                Page count
                Figures: 0, Tables: 0, Equations: 0, References: 30, Pages: 5
                Product

                SciELO Venezuela

                Self URI: Texto completo solamente en formato PDF (ES)
                Categories
                Casos Clínicos

                tumor,Sarcoma sinovial,desarticulación,muslo,cirugía de cadera,Synovial sarcoma,disarticulation,thigh,hip surgery

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