ABSTRACT
Los tumores cardíacos malignos son neoplasias poco frecuentes que pueden presentarse de diversas formas, lo que dificulta su diagnóstico. La ecocardiografía y la resonancia magnética cardíaca son técnicas fundamentales para el diagnóstico, la caracterización y la evaluación de su extensión tumoral. La identificación de la línea tumoral es esencial al iniciar un tratamiento oncológico dirigido. Si bien el "estándar de oro" para este fin es el estudio anatomopatológico (obtenido por biopsia o resección quirúrgica), en los casos en que esto no es posible, la resonancia magnética cardíaca es la técnica no invasiva que proporciona un mejor abordaje diagnóstico. El tratamiento de elección es la resección quirúrgica y el pronóstico, en general, es malo. Presentamos el caso de una paciente con un tumor cardíaco de presentación clínica atípica, en la que un abordaje imagenológico multimodal aporta información clave y complementaria para el diagnóstico y la caracterización tisular.
Malignant cardiac tumors are rare neoplasms that can present in various forms, making their diagnosis difficult. Echocardiography and cardiac magnetic resonance imaging are fundamental techniques for the diagnosis, characterization, and evaluation of tumor extension. Identification of the tumor line is essential when initiating targeted cancer therapy. Although the "gold standard" for this purpose is the pathological study (obtained by biopsy or surgical resection), in cases where this is not possible, cardiac resonance is the non-invasive technique that provides a better diagnostic approach. The treatment of choice is surgical resection and the prognosis is generally poor. We present the case of a patient with an atypical clinical presentation, in which a multimodal approach provides key and complementary information for tumor diagnosis and tissue characterization.
Os tumores cardíacos malignos são neoplasias raras que podem se apresentar de várias formas, dificultando seu diagnóstico. A ecocardiografia e a ressonância magnética cardíaca são técnicas fundamentais para o diagnóstico, caracterização e avaliação da extensão tumoral. A identificação da linha do tumor é essencial ao iniciar a terapia direcionada do câncer. Embora o "padrão ouro" para esse fim seja o estudo patológico (obtido por biópsia ou ressecção cirúrgica), nos casos em que isso não seja possível, a ressonância cardíaca é a técnica não invasiva que proporciona melhor abordagem diagnóstica. O tratamento de escolha é a ressecção cirúrgica e o pronóstico geralmente é ruim. Apresentamos o caso de uma paciente com apresentação clínica atípica, em que a abordagem multimodal fornece informações essenciais e complementares para o diagnóstico do tumor e caracterização do tecido.
Subject(s)
Humans , Female , Middle Aged , Heart Neoplasms/diagnostic imaging , Magnetic Resonance Imaging , Coronary Angiography , Electrocardiography , Multimodal ImagingABSTRACT
Undifferentiated sarcomas are rarely identified in the intracranial region. A 23-year-old man was admitted with a chief complaint of headache. Initial magnetic resonance images showed signs of low-grade glioma in the frontal lobe. Stereotactic biopsy was performed, and a diagnosis of diffuse astrocytoma was confirmed. Three months later, the patient presented with a high-grade tumor as seen on imaging studies. He underwent total resection of the tumor and histopathological tests identified an undifferentiated sarcoma. The patient died eight months later due to massive tumor bleeding. To the best of our knowledge, this is the first report of undifferentiated sarcoma arising from low-grade glioma without any chemotherapy or radiotherapy.
Subject(s)
Humans , Young Adult , Astrocytoma , Biopsy , Diagnosis , Drug Therapy , Frontal Lobe , Glioma , Headache , Hemorrhage , Histiocytoma, Malignant Fibrous , Radiotherapy , SarcomaABSTRACT
We report a 48 years old women presenting with a painful abdominal mass and hepatomegaly. An abdominal CAT scan showed a focal lesion of 14 cm diameter in liver segment 4. The patient was subjected to a left hepatectomy with a normal postoperative evolution. The pathological diagnosis of the surgical piece was an undifferentiated sarcoma. Nine months later, a local relapse was detected and the patient died 22 months after the operation.
Introducción: Los sarcomas indiferenciados (embrionario) del hígado (SIEH) son neoplasias infrecuentes. Se presentan principalmente en edad pediátrica y son considerados de mal pronóstico. En adultos, existen 71 pacientes publicados en el mundo, y en nuestro país no hay casos descritos en este grupo etario. Objetivos: Presentar el caso de una paciente adulta portadora de un SIEH, que fue sometida a cirugía resectiva y realizar una revisión del tema. Caso clínico: Mujer de 48 años de edad, con historia de dolor abdominal, masa palpable en epigastrio y hepatomegalia. Marcadores tumorales negativos, tomografía abdominal revela lesión focal de 14 centímetros en segmento 4. Se aborda quirúrgicamente, biopsia rápida revela tumor sólido maligno indiferenciado, y se realiza hepatectomía izquierda. Evolución postoperatoria favorable. Mediante el análisis histopatológico, histoquímico e inmunohistoquímico se diagnostica un SIEH y se corrobora una resección R0. Al noveno mes de evolución se pesquisa foco de recidiva hepática, inicia progresivo compromiso del estado general, falleciendo 22 meses después de la cirugía. Discusión: Actualmente se recomienda resección completa del tumor seguido de quimioterapia coadyuvante, con lo cual se han logrado sobrevidas libres de enfermedad mayores a 5 años. El dar a conocer las experiencias de casos aislados en esta patología tan infrecuente, permitiría aumentar la casuística mundial, mejorar las técnicas de enfrentamiento, y evaluar el impacto de la quimioterapia en el pronóstico.
Subject(s)
Humans , Female , Adult , Hepatectomy , Liver Neoplasms/surgery , Neoplasms, Germ Cell and Embryonal/surgery , Chemotherapy, Adjuvant , Fatal Outcome , Neoplasm Recurrence, Local , Liver Neoplasms/pathology , Liver Neoplasms/drug therapy , Neoplasms, Germ Cell and Embryonal/pathology , Neoplasms, Germ Cell and Embryonal/drug therapy , SarcomaABSTRACT
An undifferentiated (embryonal) liver sarcoma (ULS) originates from a primitive mesenchymal cell, with a predilection for childhood and very rare occurrence in adults. We report a case of a ULS that was incidentally found in a 53-year-old female. Our case was initially interpreted as a large hydatid cyst, which was later suspected to be a neoplastic lesion because its size was increasing and a solid portion was newly detected after shrinkage of the cyst following drainage. The patient underwent successful right hepatic lobectomy with complete resection, and is currently disease-free without adjuvant therapy. Although it is difficult to diagnose a hepatic cyst as a ULS due to its rare occurrence in adulthood and lack of specific findings, its possibility should be considered, especially when its size is increasing, because early diagnosis and curative resection are necessary for a favorable outcome.
Subject(s)
Adult , Female , Humans , Middle Aged , Drainage , Early Diagnosis , Echinococcosis , Liver , SarcomaABSTRACT
The ovarian mucinous tumor associated with true sarcoma is very rare. Four cases of true sarcoma: fibrosarcoma, undifferentiated sarcoma, rhabdomyosarcoma, angiosarcoma in ovarian mucinous tumor have been reported. We present a case of mucinous borderline tumor associated with undifferentiated sarcoma with angiosarcoma-like appearance in 80-year-old woman.
Subject(s)
Aged, 80 and over , Female , Humans , Fibrosarcoma , Hemangiosarcoma , Mucins , Ovary , Rhabdomyosarcoma , SarcomaABSTRACT
Primary undifferentiated cardiac sarcomas are extremely rare, and typically undergo aggressive local spread. The prognosis of undifferentiated sarcoma is reported to be very poor even if surgical resection, with or without adjuvant chemotherapy and radiotherapy, is performed. Here we present our case of primary undifferentiated sarcoma of the left atrium. A 33-year-old woman presented with a month history of progressive breathlessness and exertional chest pain. Transthoracic echocardiography showed a large mobile mass obstructing much of the left atrium. At surgery a superseptal approach to the atria enabled removal of all the left heart tumor which was attached to the left upper pulmonary vein. Immunochemistry staining revealed the undifferentiated atrial sarcoma. She received three cycles of darcabazine and adriamycin and after three months there was evidence of tumor recurrence. Eleven months later from operation, she died with congestive heart failure.
Subject(s)
Adult , Female , Humans , Chemotherapy, Adjuvant , Chest Pain , Doxorubicin , Echocardiography , Heart Atria , Heart Failure , Heart Neoplasms , Immunochemistry , Prognosis , Pulmonary Veins , Radiotherapy , Recurrence , SarcomaABSTRACT
Primary pericardial sarcomas are extremely rare. The authors report a case of a 46-yr-old woman in whom a large mediastinal mass was discovered. The patient presented with cough, dyspnea, and orthopnea. Diagnostic investigations, such as echocardiography, computed tomography, and exploratory thoracotomy provided the evidence of a large mass in the mediastinum, attached by a broad base to the superior portion of the pericardium. A excisional biopsy was performed, and histologic examination of a biopsy specimen showed undifferentiated sarcoma. However, the complete removal of the mass was impossible due to adhesion to the adjacent great vessels. After the completion of the chemotherapy the patient was completely asymptomatic. However, follow-up transesophageal echocardiography showed a residual 3x4 cm-sized mass. The patient received the radiotherapy with a total dose of 55 Gy over 6 weeks. At present, there is no evidence of disease progression.
Subject(s)
Female , Humans , Middle Aged , Biopsy , Cough , Disease Progression , Dyspnea , Echocardiography , Mediastinal Neoplasms/diagnosis , Prognosis , Sarcoma/diagnosis , Tomography, X-Ray ComputedABSTRACT
Undifferentiated(embryonal) sarcoma of the liver developing predominantly in the late childhood is extremely rare in malignant hepatic tumors. It has been described by a variety of terms such as malignant mesenchymoma, embryonal sarcoma, fibromyxosarcoma, embryonal rhabdomyosarcoma or primary sarcoma of the liver. The tumor usually presents a huge mass containing solid and sometimes cystic components, and is cmposed of large stellate and spindle cells in an abundant myxoid matrix. The treatment of choice is surgical resection with or without adjuvant chemotherapy and/or radiotherapy, but the prognosis of this disease has been reported extremly poor. We experienced a case of undifferentiated(embryonal) sarcoma of the liver recently which was confirmed by pathological examinations in a 18-year-old boy. He visited our hospital for examination of the progressive growing RUQ abdominal mass detected incidentally. On physical examination, a firm, non-tender, hard, fixed, huge, abdominal mass was palpated in the right upper quadrant of abdomen. Abdominal ultrasonography, computerized tomography and magnetic resonance imaging showed a 10x15cm sized mass confined to the left lobe of the liver, and it was hypovascular mass on angiography. We performed left lobectomy including the tumor and it was confirmed as undifferentiated (embryonal) sarcoma of the liver by pathologic examination. We herein report this case including histologic, electronmicroscopic and flow cytometric results with the review of literatures.