Insuficiencia cardíaca como presentación de sarcoma cardíaco: caso clínico y revisión de la literatura / Cardiac sarcoma associated to heart failure: case report and review

RESUMEN: Los tumores cardíacos primarios malignos son infrecuentes en la práctica clínica. Suelen ser oligosintomáticos y de diagnóstico tardío lo que tiene como consecuencia una alta mortalidad. El caso clínico que se presenta a continuación es un ejemplo de esta patología, que debe ser especialmente considerada cuando la insuficiencia cardíaca no tiene una etiología clara. Se describen los hallazgos clínicos y de laboratorio, como también los resultados de estudios de imágenes y la histología. Se ilustra los hallazgos en la operación y la evolución clínica del paciente. Se incluye una revisión de la literatura.

ABSTRACT: Malignant primary cardiac tumors are infrequent in clinical practice. They are usually oligosymptomatic and a late diagnosis leads to a high mortality rate. The clinical case of a patient presenting with heart failure with unclear etiology is presented. Clinical and laboratory findings are described along with the results of imaging studies. Findings at the time of surgery and histopathological characteristics are illustrated. A review of the literature is included.

Primary pulmonary undifferentiated pleomorphic sarcoma (PPUPS)

Undifferentiated pleomorphic sarcoma (UPS) is a high-grade pleomorphic neoplasm with no identifiable line(s) of differentiation using currently available diagnostic techniques. Therefore, it is essentially a diagnosis of exclusion, which requires generous tissue sampling, adequate contextually interpreted immunohistochemistry, and relevant molecular studies. UPS is a common soft tissue sarcoma (historically one of the entities referred to as malignant fibrous histiocytoma (MFH)), which can develop in various organs, but lung involvement is usually due to metastasis. Primary Pulmonary UPS (PPUPS) is exceptionally rare and here we present a 66-year-old man who presented with anemia and weight loss, found to have a 17 cm right lung mass with invasion to the chest wall and diaphragm. Extensive sampling and immunohistochemistry studies failed to reveal any line of differentiation. Upon exclusion of a possible extrapulmonary origin, a diagnosis of PPUPS was rendered. In addition, we reviewed all 84 previously reported cases of PPUPS/PPMFH in the literature since 1979 and summarized the clinical information.

Oligodendroglioma with Sarcomatous Transformation: Case Report and Literature Review

Oligodendrogliomas are infiltrative tumors of the central nervous systemconsidered to be morphologically stable and to offer a better prognosis. Here, we describe the case of a 36- year-old man with an initial diagnosis of oligodendroglioma, World Health Organization (WHO) grade II, who presented transformation to a sarcomatous form, while maintaining the oligodendroglial component as well as the genetic characteristics of the initial tumor without having undergone any complementary treatments previously. Despite the favorable genetic characteristics, the tumor presented poor response to complementary treatments, and rapid progression, including spinal metastasis.

Epithelioid sarcoma with spontaneous pneumothorax and massive pleural effusion

No abstract available.

A massagem na criança com câncer: eficácia de um protocolo / Massage in children with cancer: effectiveness of a protocol

OBJETIVOS: a massagem é uma intervenção que pode contribuir para o alívio da dor, embora as evidências empíricas sejam escassas e contraditórias. O objetivo deste trabalho foi avaliar a eficácia de um protocolo de massagem no alívio da dor na criança internada, com patologia oncológica. MÉTODOS: através de um estudo randomizado e controlado com cegamento simples, foram estudadas 52 crianças, com idades compreendidas entre os 10 e os 18 anos, internadas num serviço de oncologia pediátrica. A intervenção consistiu na aplicação de um protocolo de massagem de três sessões com duração entre 20 a 30 minutos em dias alternados durante uma semana. A eficácia do protocolo foi medida através da avaliação da dor com a aplicação do Inventário Resumido de Dor e a eficácia de cada sessão de massagem pela Escala Visual Analógica (EVA). RESULTADOS: o protocolo de massagem apenas se revelou eficaz na diminuição da interferência da dor no andar (p < 0,05), apesar de ter contribuído para o alívio da dor e sua interferência nas atividades da criança. Após cada sessão de massagem a intensidade da dor sentida pela criança diminuiu (p < 0,001). CONCLUSÕES: apesar da reduzida dimensão da amostra, a massagem parece ser uma intervenção útil no alívio da dor da criança que sofre de patologia oncológica, embora permaneçam dúvidas quanto à eficácia deste protocolo de massagem. Todavia, os autores recomendam a sua utilização pela sua contribuição na promoção do bem-estar e qualidade de vida da criança.

OBJECTIVES: massage can help relieve pain, although empirical evidence is scarce and contradictory. This study aims to assess the effectiveness of a massage protocol in relieving pain in children hospitalized with cancer. METHODS: a randomized, controlled, and single-blind trial was performed in a sample of 52 children aged between 10 and 18 years who were hospitalized in a pediatric cancer ward. The intervention consisted of the implementation of a massage protocol with three sessions of 20 to 30 minutes on alternate days over a one-week period. The effectiveness of the protocol was evaluated by assessing pain using the Brief Pain Inventory (BPI), while the effectiveness of each massage session was measured using the Visual Analogue Scale (VAS). RESULTS: the massage protocol was only effective in reducing the interference of pain in walking (p < 0.05), although it also contributed to relieve pain and its impact on the children's activities. After each massage session, the intensity of the pain experienced by the child decreased (p < 0.001). CONCLUSIONS: despite the small sample size, massage therapy appears to be a useful intervention in reducing pain in children with cancer. However, there are still questions regarding the effectiveness of this massage protocol. The authors recommend its use due to its contribution to the promotion of the child's well-being and quality of life.

[Lung metastasis due to limb osteogenic and soft tissue sarcoma in the hospitalized patients at Al-Zahra hospital in Isfahan between 1995 and 2008]

Soft tissue sarcoma is a relatively rare disease from mesenchymal cells . Lung metastases occur in many of the patients with limb sarcoma . The aim of this study was the evaluation of lung metastasis due to limb osteogenic and soft tissue sarcoma in the hospitalized patients at Al-Zahra hospital in Isfahan between 1995 and 2008. This descriptive cross sectional study was done on the hospitalized patients at Al-Zahra hospital in Isfahan between 1995 and 2008. Lung metastases were evaluated by lung CT scan. The data were collected from the patients' dossier and their laboratory file. We used chi-square, fisher and T test, and SPSS software [v. 11] for analyzing the collected data. We evaluated 65 patients with limb sarcoma. Lung metastases were detected in 43.3% of the patients with osteogenic sarcoma and 11.4% of the patients with soft tissue sarcoma [p=0.005]. There were no relations between occurance of lung metastases and genders, the size of the primary tumor, the side of the stricken limb, and recurrence of the tumor. The incidence of lung metastasis in the patients with osteogenic sarcoma is more than the patients with soft tissue sarcoma. In view of the fact that the incidence of lung metastasis in the patients with osteogenic sarcoma is high, we suggest that these patients be evaluated for lung metastasis

Spermatocytic seminoma associated with undifferentiated sarcoma: A rare case report.

The presence of sarcomatous element transforms the usually innocuous spermatocytic seminoma into a highly aggressive neoplasm. We report a case of spermatocytic seminoma with undifferentiated sarcomatous component in a 43-year-old male, presented with testicular mass since two and a half years. Orchidectomy was performed and after 9 months, the patient presented with recurrent scrotal mass with bilateral pulmonary metastases.

Prolapsed uterine sarcoma causing non-puerperal uterine inversion in a post menopausal woman.

A rare case of non-puerperal uterine inversion caused by a large fundal sarcoma in a 57 year old menopausal woman who presented with profuse vaginal bleeding is reported. After vaginal excision of the fundal myoma, reduction of the uterine inversion combined approach both abdominal as well as vaginal successfully was then followed by total abdominal hysterectomy and bilateral salphingoopherectomy.

Derivação arterial extra-anatômica associada à leiomiossarcoma de coxa / Extra-anatomical arterial bypass with leiomyosarcoma of the thigh

As derivações extra-anatômicas são opções técnicas em situações em que as vias convencionais de revascularização são de alto risco cirúrgico ou anatomicamente impraticáveis. Neste relato, apresentamos o caso de uma paciente portadora de sarcoma de partes moles do terço proximal da coxa esquerda, com invasão tumoral dos vasos femorais. A paciente foi submetida à revascularização do membro inferior com enxerto extra-anatômico ilíaco externo para artéria poplítea acima do joelho com PTFF. Posteriormente, foi submetida à ressecção em monobloco do tumor, músculo quadríceps e vasos femorais, com boa evolução pós-operatória, patência do enxerto e sem recidiva local no seguimento de 24 meses.

Estudo da morbimortalidade cirúrgica, da sobrevida e dos fatores prognósticos de sarcomas primários do retroperitônio

Tendo como objetivo avaliar a morbimortalidade, sobrevida e os fatores prognósticos dos sarcomas primários do retroperitônio, analisamos retrospectivamente 59 prontuários de pacientes operados na Seção de Cirurgia Abdomino-Pélvica do Instituto Nacional de Câncer no período de junho de 1992 a julho de 2003. Vinte e três pacientes eram homens e 36 mulheres; 38 brancos, 6 negros e 15 pardos, com idade mediana de 50 anos (6-72). História familiar de câncer esteve presente em 21 casos (35,59%). As queixas mais comuns foram dor abdominal (31 pac.) e massa abdominal (17 pac.). TC foi o principal exame realizado para o diagnóstico (53 pac.). A taxa de ressecabilidade foi de 74,57% (44 pac.) e a de radicalidade entre os ressecados de 48,88% (22 pac.). Houve 2 óbitos pós-operatórios (3,38%) e 12 complicações pós-operatórias (20,33%). Os leiomiossarcomas (16 pac.) e os lipossarcomas (15 pac.) foram os mais incidentes. O grau de diferenciação tumoral mais freqüente foi o G3 (23 pac. [38,98%]) e o diâmetro tumoral médio, de 20,4 cm. A re-ressecção no total de ressecados (45 pac.) aconteceu em 21 pacientes (46,66%), sendo única em 16, dupla em 3 e tripla em 2 pacientes. Em 10 dos 21 pacientes, a re-ressecção foi radical. A sobrevida global foi de 49% em 2 anos e 20% em 5 anos, e a mediana de sobrevida livre de doença foi de 23 meses. A análise univariada, o diâmetro do tumor (> ou <= 12 cm), o grau de diferenciação tumoral ([Gl + G2] X [G3 + G4]), a ressecção radical (R0) ou paliativa (R1+R2) a hemotranfusão no ato operatório e a re-ressecção, mesmo que paliativa, nos casos de recidiva ou persistência de doença (n = 52), foram significativos para sobrevida (p = 0,0267, 0,048, 0,0001, 0,022 e 0,0003, respectivamente). Na análise multivariada, não houve fator independente. Essas análises sugerem que, no momento, somente o diagnóstico precoce, a cirurgia radical R0, a ausência de hemotransfusão intra-operatória e a re-ressecção nos casos de recidiva ou persistência de doença possibilitarão a sobrevida a longo prazo

To evaluate the morbjmortality, survival and the prognostics factors of the retroperitonial sarcomas, we analyzed retrospectively 59 records of patients operated by the Abdomino-pelvic Department of Surgery at the National Cancer Institute of Brazil in the period between June 1992 and July 2003. Twenty three were men and 36 women, 38 white, 6 black and 15 mixed. Mean age was 50 years old (6-72). Family history of cancer was present in 21 cases (35.59%). The most common complaints were abdominal pain (31 pts) and abdominal mass (17 pts). TC was the main exam for diagnosis (53 pats). Resectability rate was 76.27% (45 pts), andradicality 48.88% (22pts). There were 2 postoperative deaths (3.38%). There were 8 technical complications (13.55%), besides 4 clinical complications (6.77%). Liposarcomas (16 pts) and the leiomyosarcomas (15 pts) were the most common types. The most frequent tumor grade was G3 (3 pts [38.98%J) and the tumor median diameter was 20.4 cm. Re-resection rate was (46.66%), being single in 16, double in 3 and triple in 2 patient. The overall survival rate was 49% for 2 years and 20% for 5 years and the median disease free survival was 23 months. In the univariate analysis, the diameter of the tumor (> or <= 12 em), grade of tumor differentiation ([G1 + G2] X [G3 + G4J), radical R0) or palliative resection(R1+R2), operative blood transfusion, and re-resection, even if palliative, in the cases of recurrence or persistence of disease (n=52) were significant (p = 0.0267, 0.048, 0.0001, 0.022 and 0.0003, respectively). Multivariate analysis did not show any independent factor. These analyses suggest that, in the moment, only early diagnosis, radical surgery, absence of intra-operative blood transfusion and the re-resection in cases of recurrence or persistence of disease will provide greater chances of a long term survival

Nephrotic syndrome cured by removal of malignant gastric stromal tumor.

Malignant tumors have been associated with the development of nephrotic syndrome. We report a 73-year-old man with nephrotic syndrome who had a malignant stromal gastric tumor. Curative resection of the sarcoma resulted in remission of the nephrotic syndrome.

Virus de la hepatitis C en un grupo de pacientes hematológicos y oncohematológicos / Hepatitis C virus in a group of hematological and oncohematological patients

Background: Little information is available in Chile about hepatitis C virus (HCV) in hematological and oncohematological patients. Aim: To evaluate the prevalence of hepatitis C virus markers in a group of hematological and oncohematological pediatric patients seen at Valdivia Regional Hospital. Patients and methods: Antibodies against virus C, determined by ELISA and viral RNA, determined using RT-polymerase chain reaction, were measured in 54 blood samples from children with hematological diseases (34 with Acute Lymphoblastic Leukaemia, 4 with Hodgkin Diseases, 4 with Haemolytic Anemia, 5 with Sarcomas, 2 with Non-Hodgkin Lymphoma, 2 with Thrombocytopenic Purpura, 1 with an Ependimoma, one with a Wilms Tumor and 1 with a Von Willebrand Disease). Results: All samples were negative for antibodies against hepatitis C virus. Viral RNA was found in four children, all with a diagnosis of acute lymphoblastic leukemia and who received chemotherapy and multiple transfusions. Conclusions: The prevalence of Viral RNA for hepatitis C virus in oncohematological patients in our study is high and associated with the use of chemotherapy and multiple transfusions

Transformação sarcomatosa em meningeoma / Sarcoma arising in meningioma

Os autores relatam o caso de paciente de 40 anos, raça amarela, sexo masculino, que apresentou história arrastada de déficit de força em hemicorpo direito com predomínio em membro inferior direito, cujo diagnóstico de imagem (tomografia computadorizada de crânio e ressonância magnética) revelou tratar-se de neoplasia parassagital esquerda com características semelhantes às de um meningeoma. Foi submetido à cirurgia com ressecção radical, incluindo o seio sagitalinvadido, com boa evolução no pós-operatório. O diagnóstico anatomopatológico foi de meningeoma com transformação sarcomatosa (meningeossarcoma/sarcoma meníngeo). Foi submetido à radioterapia e o seguimento de 8 meses não evidenciou recidiva. A transformação sarcomatosa é rara e infere um prognóstico reservado. Os autores discutem a doença e procuram identificar semelhanças e diferenças clínicas e radiológicas com as principais neoplasias do sistema nervoso central derivadas de células meningoteliais e de hemangiopericitos.

Malignancy in meningiomas is not frequent, however on rare occasions a benign meningioma may change into a tumor with features of malignancy or even sarcomatous cells. A case of sarcoma arising in a meningioma in a 40 year-old man is reported. Despite of the poor prognosis after thesurgical and radiotherapic treatment, this patient goes on without recurrence for eight months after surgery and radiotherapy. The pathological and radiological features as well as a differentialdiagnosis of this disease are discussed by the authors based on literature review.

Sarcoma estromal de esófago / Stromal sarcoma of the esophagus

El sarcoma estromal de esófago es una patología poco frecuente. El objetivo de este trabajo es la comunicación de un caso tratado en nuestro Servicio de Cirugía. Se trata de un paciente de 52 años, sexo masculino con antecedentes de úlcera péptica y tabaquismo. Consultó por historia de seis meses de disfagia, baja de peso de seis kilogramos y hematemesis. Al examen físico no había hallazgos de importancia. Se realizó un estudio endoscópico alto, que mostró una lesión ulcerada de tercio distal de esófago. La radiografía de tórax mostró una imagen retrocardíaca de aproximadamente 8 cm de diámetro, que se correlacionó con un TAC tóraco-abdominal que mostró una lesión esofágica tumoral de ocho centímetros de diámetro. Se operó con abordaje por tóraco-freno-laparotomía. El hallazgo fue de un gran tumor de esófago distal, y de una metástasis única en el segmento seis del hígado. Se realizó una esofagectomía con ascenso gástrico y anastomosis esofagogástrica cervical, colecistectomía y resección de la lesión hepática. La histología mostró un tumor gastrointestinal estromal. Los sarcomas estromales del tubo digestivo son poco frecuentes, sus características histológicas y ultraestructurales sólo se han aclarado recientemente. La cirugía sigue ocupando un rol central en su tratamiento

Sarcomas de la pared torácica. Resección y reconstrucción / Chest wall neoplasm. Resection and reconstruction

Los sarcomas de la pared torácica corresponden al 5 por ciento de este tipo de neoplasias. La resección y reconstrucción de la pared torácica se ha desarrollado mucho en los últimos años debido a la evolución de las diferentes técnicas de reconstrucción, principalmente de los colgajos miocutáneos. En este artículo presentamos la experiencia obtenida durante 1984 a 1994 en el Instituto Nacional de Cancerología. Se llevaron a cabo 59 resecciones de la pared torácica; la indicación más frecuente la representaron los sarconas óseos en 16 pacientes (27.2 por ciento) y los sarcomas de partes blandas en 15 (25.4 por ciento). La indicación quirúrgica tuvo una finalidad paliativa en 26 casos 844.1 por ciento) y curativa en 33 (54.9 por ciento). A todos los pacientes se les realizó resección ósea. El promedio de costillas resecadas fue de 3.8. Después de la resección, el defecto esquelético y la estabilidad de la pared torácica se reconstruyó en 36 sujetos 861 por ciento); de éstos, se realizó con fascia lata en 17 (47.4 por ciento) y con malla sintética en 18 (50 por ciento). La reconstrucción de partes blandas fue necesaria en 21 casos y se llevó a cabo con colgajos miocutáneos; el más utilizado fue el músculo dorsal ancho en 11 pacientes. El tiempo quirúrgico promedio fue de cuatro horas 15 minutos y el de hospitalización de 8.4 días. La morbilidad fue del 20.3 por ciento y la mortalidad operatoria del 5.0 por ciento

Pre leukemic granulocytic sarcoma of vagina. A case report with review of literature.

Granulocytic sarcoma is an extramedullary tumor of malignant granulocytic progenitor cells, that may precede the onset of acute myeloid leukemia or appear during the leukemic manifestation or blastic crisis of chronic myeloproliferative disorders. We describe a case of granulocytic sarcoma of vagina in a 27 year old woman treated with local radiotherapy. After seven months of follow up she developed acute myeloid leukemia. The case has been presented in view of its rarity and discussed in light of the available literature.