RESUMEN
In this study, we investigate the hypothesis that IgG4-related autoimmune reaction is involved in the formation of inflammatory aortic aneurysms (IAA). We obtained 23 cases of IAA and 11 cases of atherosclerotic aortic aneurysms (AAA) as control group. We evaluated the expression of IgG4 in both IAA study cases and AAA control cases. In addition, immunohistochemical expression of C-Kit, CD21, CD34, S-100 protein, SMA, vimentin, p53, beta-catenin, and ALK-1, and EBV-LMP1 expression by in situ hybridization were performed only in IAA cases. Of the 23 patients, 20 were males and 3 were females (M: F ratio 6.7:1); age ranged from 43 to 81 years (average 64.3 years). Histologically, all 23 cases of IAA formed a mass that displayed inflammatory myofibroblastic tumor-like features. All lesions stained strongly and diffusely for vimentin and SMA (100%); 17 stained strongly and focally for CD34 (74%); and all were negative for C-Kit, CD21, S-100 protein, p53, beta-catenin, EBV-LMP1, and ALK-1. The numbers of infiltrating IgG4-positive plasma cells in IAA cases exceed that of AAA cases. Score 3 (>50 plasma cells/one 40X field) of IgG4-positive plasma cells was only seen in IAA cases (13/23, 57%), whereas none of the 11 cases of AAA showed score 3 IgG4-positive plasma cells (P=0.0018, Fischer's exact test). Our findings suggest that IAA may be an aortic manifestation of the IgG4-related sclerosing disease. The high number of positive plasma cells, >50 plasma cells/one 40X field is more specific for the IAA than for AAA; however, lesser number can be seen in both IAA and AAA patients.
Asunto(s)
Aneurisma de la Aorta/patología , Enfermedades Autoinmunes/patología , Hipergammaglobulinemia/patología , Esclerosis/patología , Adulto , Anciano , Anciano de 80 o más Años , Aneurisma de la Aorta/inmunología , Aneurisma de la Aorta/metabolismo , Enfermedades Autoinmunes/inmunología , Enfermedades Autoinmunes/metabolismo , Biomarcadores/metabolismo , Recuento de Células , Femenino , Humanos , Hipergammaglobulinemia/inmunología , Hipergammaglobulinemia/metabolismo , Inmunoglobulina G/sangre , Inmunoglobulina G/inmunología , Masculino , Persona de Mediana Edad , Células Plasmáticas/inmunología , Células Plasmáticas/metabolismo , Células Plasmáticas/patología , Esclerosis/inmunología , Esclerosis/metabolismoRESUMEN
BACKGROUND: In patients with primary cardiac sarcoma, the tumor's location is more important than cell type in determining patient presentation, therapy options, and outcomes. The purpose of the current study was to investigate the outcomes after right-side heart sarcoma resection. METHODS: Clinicopathologic data from patients who underwent right-side heart sarcoma resection at our institution and patients identified in a literature search were examined. Morbidity and the 30-day mortality rate and survival were determined. We used univariate and multivariate analyses to identify independent predictors of overall survival. RESULTS: We identified 57 patients who underwent right-side heart sarcoma resection. Right-side heart failure was the most common complication (4 patients, 19%), and the 30-day mortality was 14% (3 patients). The overall 5-year survival rate was 17%, and the median overall survival duration was 9 months. Multivariate analyses revealed that surgical margin status was the only independent predictor of survival. Patients with negative surgical margins had a longer median overall survival duration (27 months versus 4 months) and a significantly higher overall 5-year survival rate (36% versus 0%; p = 0.0003) than patients with positive surgical margins. CONCLUSIONS: The patients with right-side heart sarcoma resection had worse survival after resection than that reported for our patients who underwent resection for left-side heart sarcoma or pulmonary artery sarcoma. Because positive surgical margin status is an independent predictor of reduced survival, induction chemotherapy should considered to enhance resectability in right-side heart sarcoma patients, thus maximizing the possibility of obtaining negative surgical margins.
Asunto(s)
Procedimientos Quirúrgicos Cardíacos , Neoplasias Cardíacas/cirugía , Sarcoma/cirugía , Adulto , Femenino , Estudios de Seguimiento , Neoplasias Cardíacas/mortalidad , Humanos , Masculino , Estudios Retrospectivos , Sarcoma/mortalidad , Tasa de Supervivencia/tendencias , Texas/epidemiología , Resultado del TratamientoRESUMEN
BACKGROUND: We present a large, single institution experience with adult cardiac tumors and address factors affecting outcome. METHODS: A retrospective review was made of all patients who underwent surgery for primary cardiac tumors from April 1975 through August 2002. RESULTS: Eighty-five patients (33 male and 52 female) with a mean age of 54 years were identified with follow-up available for 80 (94%) patients. There were 68 (80%) benign tumors and 17 (20%) malignant tumors. Three tumors recurred and were resected giving a total of 88 surgeries. All benign tumors were grossly resected and the extent of resection for malignant disease ranged from 14 (78%) gross resections and 3 (17%) debulkings to 1 (5%) biopsy. There were 4 (5%) early hospital deaths. Median survival was 9.6 months and 322 months for patients with malignant and benign diseases, respectively. Significant predictors of long-term mortality were malignant disease (P <0.0001) and New York Heart Association class (P <0.03). CONCLUSIONS: Surgical resection provides excellent outcome in patients with benign cardiac tumors. Malignant tumors continue to pose a challenge with good local tumor control but limited survival owing to metastatic disease.
Asunto(s)
Neoplasias Cardíacas/cirugía , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Femenino , Neoplasias Cardíacas/diagnóstico , Neoplasias Cardíacas/mortalidad , Humanos , Masculino , Persona de Mediana Edad , Recurrencia Local de Neoplasia , Complicaciones Posoperatorias , Estudios Retrospectivos , Tasa de SupervivenciaRESUMEN
Twenty-seven fractured human vertebral bodies and 24 unfractured human vertebrae from adjacent levels were studied postmortem using histologic and high-resolution radiographic techniques. The findings were compared with those in the vertebral bodies of individuals without spinal fracture. Forty-six human thoracolumbar spines were obtained from individuals at autopsy. Standard radiographs were made of all specimens. Twelve of the 46 individuals had a total of 27 fractured vertebral bodies by plain radiographic criteria. Attention was focused on these fractured vertebrae as well as on 24 unfractured vertebral bodies that were harvested from a level immediately adjacent to the fractured vertebral bodies. Twelve vertebral bodies from four individuals with no evidence of fracture or inflammatory spondyloarthropathy were also studied for comparison. The vertebral bodies were graded by their mineral density as measured by dual-energy x-ray absorptiometry and sectioned into 3-mm sagittal cuts. High-resolution contact radiographs were prepared for each section prior to decalcification and tissue sectioning on a large format microtome. Mid-and parasagittal tissue sections of each vertebra were prepared for standard hematoxylin and eosin stains. A total of 126 sections were studied. The histologic characteristics of the fractured vertebrae (n = 27) were compared with those of adjacent unfractured levels (n = 24) and with vertebrae from individuals without fracture (n = 12). Vertebral bodies with fractures secondary to osteoporosis were consistently characterized histologically by focal areas of endochondral new bone formation adjacent to avascular necrotic bone and unreactive marrow. Such ongoing new bone and new vessel formation adjacent to nonhealing areas were also documented in radiographically unfractured vertebral bodies from individuals with osteoporotic fractures at adjacent levels. No areas of endochondral new bone formation or areas of focal necrosis were found in vertebral bodies from individuals without radiographic evidence of osteoporosis. A vascular necrosis of the vertebral body is a common histologic finding in individuals with osteoporosis. Indeed, our histologic observations suggest subclinical fractures and microfractures of the vertebral body may be the underlying pathologic process leading to avascular necrosis in individuals with osteoporosis. Microtrabecular fractures and endplate fractures were commonly seen in osteoporotic vertebral bodies, often in vertebrae that appeared to be uninvolved on specimen radiographs.