A rare case of primitive right atrium angio-sarcoma with favorable outcome, in a young female: case report and literature review / Angiosarcoma primitivo de la aurícula derecha en una mujer joven, con respuesta favorable al tratamiento: reporte de un caso y revisión de la literatura

Primary malignant cardiac tumors are extremely rare, but their associated mortality is very high. The clinical presentation is oñen variable and nonspecific; by the time symptoms appear, the tumor has usually evolved to a large intracardiac and paracardiac mass causing considerable hemodynamic involvement, regional invasión and distant dissemination. We report a 26 year-old female with a right atrium angiosarcoma with a surprisingly favorable outcome at 2 years follow-up after treatment with a combination of neoadjuvant chemotherapy and surgical resection. She presented with chest tightness, shortness of breath at effort, atypical chest pain, palpitations, asthenia, weight loss and profuse perspiration. Physical examination and irnaging diagnostic procedures, identified a pericardial effusion and a pathologic cardiac mass. The diagnosis of a malignant angiosarcoma was suggested afier computed tomography transthoracic and transesophageal echocardiography It was confirmed at surgery by a biopsy followed by histopathology and immunohistochemistry.

Los tumores cardíacos malignos son extremadamente raros y su mortalidad es muy alta. El cuadro clínico es variable e inespecífico: frecuentemente cuando aparecen síntomas ya existe una gran masa intracardíacayparacardíaca que causa compromiso hemodinámico grave, además de invasión locorregionaly diseminación a distancia. Comunicamos un caso especial de una mujerjoven que tuvo una evolución sorprendentemente favorable después de un tratamiento combinado con quimioterapia neoadyuvante y resección quirúrgica. La paciente presentó opresión precordial, disnea de esfuerzo, dolor torácico atípico, palpitaciones, astenia, baja de peso y sudoración profusa. El examen físico y exámenes complementarios, especialmente de imágenes, identificaron un derrame pericárdico y una masa cardíaca patológica. La tomografía computada y la ecocardiografía, particularmente la transesofágica, sugirieron el diagnóstico de angiosarcoma maligno, que se confirmó en una intervención quirúrgica que incluyó una biopsia con estudios histopatológicos e inmunohistoquímicos. Se discuten las alternativas del diagnóstico y del tratamiento, y se revisan la epidemiología y los recursos terapéuticos actuales en la literatura. Este caso ilustra la utilidad de la ecocardiografía transesofágica, para el diagnóstico de estos tumores y se plantea que el uso de nuevas alternativas quimioterapias asociadas a la extirpación quirúrgica pueden mejorar la sobrevida que, en nuestra paciente, alcanza dos años libre de síntomas.

Left ventricular diastolic performance in breast cancer survivors treated with anthracyclines.

OBJECTIVE: The aim of our study was to evaluate left ventricular diastolic and systolic performance in women diagnosed with breast cancer and treated with low doses of anthracyclines (epirubicin). METHODS AND RESULTS: Thirty-four female patients with breast cancer treated with epirubicin up to 450 mg/m2 (study group), and a matched control group of 34 women diagnosed with breast tumours, who had not started chemotherapy, were assessed by echocardiography. Left ventricular diastolic function was evaluated by measuring Doppler transmitral flow: the maximal velocity of the E and A waves (rapid filling and atrial filling), the Emax/Amax ratio, the pressure half time (PHT) of the E wave and the isovolumic relaxation time (IVRT). The left ventricular ejection fraction was calculated to assess systolic performance. We documented a significant decrease of Emax, whereas the A wave was significantly increased in the study group compared to the control group. The mitral E/A ratio was below 1 in the study group. Prolonged PHT and IVRT were also detected in the epirubicin-treated group when compared with the control group. Left ventricular systolic performance was not significantly altered in the study group in comparison with the control group. Although the ejection fraction was lower in the study group the difference did not reach statistical significance. CONCLUSION: Our study certified impaired left ventricular diastolic performance in patients with breast cancer treated with low total doses of epirubicin (< or = 450 mg/m2). We concluded that diastolic impairment is due to poor left ventricular compliance being an early marker of epirubicin cardiotoxicity that precedes systolic function alterations.

Conventional radiology and right heart catheterization in estimating primary pulmonary hypertension and pulmonary hypertension secondary to left-sided valvular disease.

AIM: to estimate the comparative value of conventional radiology and cardiac catheterization in establishing the diagnosis and severity of primary and secondary pulmonary hypertension. We also tried to achieve some correlations between the radiological and invasive parameters. METHODS: we performed a retrospective study over an 11-years time period, analyzing data from a group of 14 patients diagnosed with primary pulmonary hypertension compared to a matched group of 20 patients suffering from pulmonary hypertension secondary to mitral and aortic valvular disease. All the patients had undergone conventional radiology (chest X-ray) and catheterization of the right heart cavities and pulmonary artery. RESULTS: we detected significantly elevated pulmonary artery pressures and resistances in the primary pulmonary hypertension group compared to secondary hypertension patients. Cardiac output values were much lower in the primary pulmonary hypertension individuals compared with secondary pulmonary hypertension in left-sided valvular disease. The pulmonary artery arch diameter, the diameter of the right descending pulmonary artery and the value of the arterio-bronchial ratio were similar in the two groups. CONCLUSION: in both groups, we found a statistically significant positive correlation between the values of the pulmonary artery vascular resistance and the diameter of the right descending pulmonary artery, which are very specific parameters in the diagnosis of pulmonary hypertension. In the primary pulmonary hypertension group we identified a significant inverse correlation between the diameter of the right descending pulmonary artery and the values of cardiac output. This finding was not confirmed in the secondary group.

A rare case of primitive right atrium angio-sarcoma with favorable outcome, in a young female. Case report and literature review.

Primary malignant cardiac tumors are extremely rare, but their associated mortality is very high. The clinical presentation is oñen variable and nonspecific; by the time symptoms appear, the tumor has usually evolved to a large intracardiac and paracardiac mass causing considerable hemodynamic involvement, regional invasión and distant dissemination. We report a 26 year-old female with a right atrium angiosarcoma with a surprisingly favorable outcome at 2 years follow-up after treatment with a combination of neoadjuvant chemotherapy and surgical resection. She presented with chest tightness, shortness of breath at effort, atypical chest pain, palpitations, asthenia, weight loss and profuse perspiration. Physical examination and irnaging diagnostic procedures, identified a pericardial effusion and a pathologic cardiac mass. The diagnosis of a malignant angiosarcoma was suggested afier computed tomography transthoracic and transesophageal echocardiography It was confirmed at surgery by a biopsy followed by histopathology and immunohistochemistry.

Malignant primary pulmonary tumor with hemangiopericytoma-like features: conventional hemangiopericytoma versus solitary fibrous tumor.

Although extremely rare, the primitive lung hemangiopericytoma in adults is similar to hemangiopericytomas with other soft tissue localizations. Although generally benign and curable after radical surgery, it might also have a malignant clinical course with dissemination in both lungs, infiltration of vital organs (heart, pulmonary artery), extension to the adjacent tissues, and even pulmonary metastases. The treatment of choice is the complete tumor resection with negative surgical margins after excision. Certain histologic features might indicate a malignant potential. The clinical outcome of patients is variable: some are cured after radical surgery and others might present relapse and recurrences that necessitate a second intervention, radiation therapy, and/or chemotherapy. Over the years, the conventionally-defined hemangiopericytoma concept has evolved because of the nonspecific histologic growth pattern (characteristic monotonous appearance, moderate or high cellularity, and a well-developed branching vascular pattern) shared by numerous, unrelated benign or malignant lesions. Hemangiopericytoma is no longer considered a specific entity but rather as a characteristic growth pattern; in the heterogeneous group of hemangiopericytoma-like neoplasms, many lesion categories have been individualized and defined. We report an uncommon case of primitive lung tumor exhibiting hemangiopericytoma-like features, with an aggressive, fatal clinical course. Because of the major histologic overlap between solitary fibrous tumor and hemangiopericytoma and lack of clear classification criteria, we encountered difficulty in including this case in a known clinical entity; primitive solitary fibrous tumor of the lung, which mimics lung hemangiopericytoma, seemed to be the most plausible diagnosis. We discuss the case particularities and the radiologic and pathologic correlations.

Altered left ventricular diastolic performance in oncologic patients treated with epirubicin.

Anthracyclines confer an increased risk of cardiotoxicity. The authors evaluated left ventricular (LV) performance in patients treated with epirubicin. Sixty-eight patients with malignancies (study group), treated with epirubicin < or = 450 mg/m2, and a matched control group of 68 cancer patients who had not started chemotherapy were evaluated by Doppler echocardiography. The authors assessed LV diastolic function by measuring the transmitral flow: the maximal velocity of the E and A waves, the Emax/Amax ratio, the pressure half time of E wave (PHT), and the isovolumic relaxation time (IVRT). Global LV ejection fraction (LVEF) was estimated to determine the systolic performance. The authors documented alterations of the LV diastolic performance in the study group by finding a significant decrease in Emax, whereas Amax was significantly increased. Prolonged PHT and IVRT were also certified in the epirubicin-treated group compared with controls. No significant variation in LVEF between the 2 groups was detected.

Severe paraneoplastic hyponatremia and hypoosmolality in a patient with small-cell lung carcinoma: syndrome of inappropriate antidiuretic hormone secretion versus atrial natriuretic peptide or both?

It is well documented in literature that a majority of small-cell lung cancers are associated with paraneoplastic phenomena. We report the case of a 63-year-old man diagnosed with small-cell lung carcinoma, in whom a severe hyponatremia and renal sodium loss with inappropriate antidiuresis were also found during a routine laboratory testing. Syndrome of inappropriate antidiuretic hormone secretion was first suspected in this patient, but another complex pathogenetic mechanism involving atrial natriuretic peptides could be associated, potentiating the deflation of the plasma sodium level. In our patient, the plasma-atrial natriuretic peptide base level, determined with a sensitive radioimmunoassay, was above the normal range (183 pg/mL; normal range, 50 pg/mL, +/- 10 pg/mL), and the antidiuretic hormone plasma level had an oscillatory pattern, varying between 5.5 pg/mL and 7 pg/mL (normal range, 0-4.7 pg/mL). We discuss the pathogenesis and clinical aspects of this association and the therapeutic options for these types of patients.