Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0034065 (pulmonary embolism)
 14,979 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A single institution's experience of three patients with nephroblastoma extending via the inferior vena cava to the right heart is described. The case reports and the pertinent literature illustrate that preoperative failure to detect the intracardial extension increases the risk of right outflow obstruction and pulmonary embolism during or after surgery. Simultaneous thoraco-abdominal surgery with total circulatory arrest is advocated to remove the cardiac and caval tumor together with the primary tumor in order to prevent sudden hemodynamic and embolic complications. Noninvasive diagnostic methods usually suffice to detect this rare complication of nephroblastoma and to plan an interdisciplinary surgical procedure. The presence of this complication should not worsen the stage- and histology-related prognosis of the patient.
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PMID:Management of nephroblastoma with intracardiac extension: three case reports and review of the literature. 166 30

A 3 1/2-year-old girl developed thrombosis of the inferior caval and renal veins several weeks after complete resection of a nephroblastoma. Her mother had suffered from pulmonary embolism at the age of 18 years. Familial antithrombin III deficiency and persistently lowered free protein S levels in the proposita were found. It is assumed that the combination of these two regulatory defects of hemostasis contributed to the early occurrence of this severe thrombotic event.
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PMID:Inferior vena cava thrombosis in a child with nephroblastoma and combined deficiency of antithrombin III and free protein S. 217 1

In the management of Wilms tumor (nephroblastoma) with intracardiac extension (ICE) an interdisciplinary approach is mandatory. The three cases reported here reflect the impressive improvements of both diagnostic facilities and surgical strategies over the last 16 years as evidenced in the literature. Nowadays, prerequisite for a well-planned, safe, and successful operation is the exact delineation of the intravasal tumor extension and the use of the combined techniques of cardiopulmonary bypass (CPB) and deep hypothermic circulatory arrest (DHCA). In the first child (1976) only tumor nephrectomy was performed. Under chemotherapy fatal massive pulmonary embolism occurred. In the second child (1980) ICE was removed after tumor nephrectomy on an emergency basis using CPB. The third child (1987) was operated on electively using the concepts of CPB+DHCA. Now, both children are doing well without evidence of disease 13 years (case 2: stage III) and 6 years (case 3: stage IV) after an aggressive adjuvant-therapy regimen of multiagent chemotherapy and radiation. In conclusion, in children with nephroblastoma and ICE an aggressive surgical approach and subsequent multiagent chemotherapy are advocated.
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PMID:Surgical management of Wilms tumor with intracardiac neoplastic extension. 801 24

Wilms' tumour (nephroblastoma) is one of the most common solid malignant tumours in infancy and childhood. In about 40% of cases, invasion of the renal vein is present. Rarely, these tumours extend into the inferior vena cava and right atrium. We discuss a 7-week-old girl who presented with acute massive pulmonary embolism, and was found to have a large tumour of the left kidney. Later on, the diagnosis of Wilms' tumour was confirmed. Wilms' tumour presenting as massive pulmonary embolism is extremely rare; to our knowledge this is the fourth case described in literature. We review the cases previously described, and comment on the diagnostic and therapeutic features of this clinical entity.
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PMID:Wilms' tumour presenting as a pulmonary embolism. 922 22

A 4-year-old girl developed right metachronous Wilms tumor 2 years after completing treatment for a left-sided stage I Wilms tumor. The original treatment included 7 weeks of chemotherapy, delayed nephrectomy, and another 3 weeks of chemotherapy. The metachronous tumor on the right side extended into the inferior vena cava and right atrium. She developed pulmonary embolism as a result. She received chemotherapy and developed liquifaction of the tumor and toxic shock. She also had surgery. The patient is alive 3 years after the original diagnosis and 10 months after the relapse. The authors report this unusual case and discuss whether these cases can be identified early.
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PMID:Metachronous Wilms tumor associated with pulmonary embolism: how can we detect these cases early? A case report and literature review. 1268 54

Moderate to severe pulmonary embolism (PE) can cause pulmonary arterial hypertension and right ventricular (RV) heart damage. Previous studies from our laboratory indicate that the basal outflow tract of the RV is injured and has acute inflammation followed by tissue remodeling while the apex appears normal. The present studies examine transcription responses to chronic PE in RV apex and outflow tracts using DNA microarrays to identify transcription responses by region. Changes predominated in the RV outflow tract (8,575 genes showed >/=1.5-fold expression change). Gene ontology and KEGG analyses indicated a significant decrease in genes involved in cellular respiration and energy metabolism and increases in inflammatory cell adhesion molecules and extracellular matrix proteins. Signal pathways for wound healing such as fibroblast growth factor, collagen synthesis, and CCN proteins (named for the first three members of the family: cysteine-rich protein 61, connective tissue growth factor, and nephroblastoma overexpressed gene) were strongly upregulated. In comparison, few genes (422) showed significant change in the RV apex tissue. Apex-selective genes included two genes affecting metabolism and a stretch-sensitive transcription factor (ankyrin repeat domain 1). We conclude that the RV outflow tract is subject to strong proinflammatory and profibrotic remodeling transcriptional responses in chronic PE. Severe loss of genes involved in cellular respiration is consistent with previous histology indicating a shift in cell types present within the outflow tract tissue away from highly energy-dependant cardiomyocytes to less metabolically active cells during remodeling. The apex region of the RV had few compensating adaptations.
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PMID:Transcriptional changes in right ventricular tissues are enriched in the outflow tract compared with the apex during chronic pulmonary embolism in rats. 1960 18

Only 4-6% of children with renal tumor show inferior vena caval or right atrial infiltration at presentation. Tumor emboli are even rarer, and so far, sudden death as the presenting symptom has only been described at presentation in Wilms' tumor in eight cases. We report a unique case of Wilms' tumor that presented with small pulmonary emboli and immediately after as that was diagnosed before death by detection of small emboli and immediately after sudden death as massive pulmonary embolism. It seems that in cases of invasive vascular infiltration with a stable hemodynamic condition at diagnosis immediate surgery may be necessary.
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PMID:Recurrent pulmonary tumoral embolism and sudden death as the presenting symptom of Wilms' tumor. 2208 25

Wilms tumor is found in 1 in 10,000 children and most commonly presents in asymptomatic toddlers whose care givers notice a nontender abdominal mass in the right upper quadrant. This case of Wilms tumor presented as a critically ill eleven-year old with significant tachypnea, dyspnea, vague abdominal pain, intermittent emesis, new onset seizure, metabolic acidosis, and hypoxemia. This is the first case in the literature of Wilms Tumor with cavoatrial involvement and seizure and pulmonary embolism resulting in aggressive resuscitation and treatment. Treatment included anticoagulation, chemotherapy, nephrectomy, and surgical resection of thrombi, followed by adjunctive chemotherapy with pulmonary radiation.
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PMID:Dyspnea, tachycardia, and new onset seizure as a presentation of wilms tumor: a case report. 2485 Nov 88

Paraneoplastic syndromes are rare disorders that are triggered by an altered immune system response to neoplasm. Paraneoplastic syndromes may be the first or the most prominent manifestations of cancer. Wilm's tumor is the most frequent pediatric renal malignancy and usually presents with abdominal mass. Unusual presentations like acquired von Willebrand disease, sudden death due to pulmonary embolism and Cushing syndrome have been described in the literature. Cushing syndrome, as the presenting symptom of a malignant renal tumor in children, is a very rare entity. Few case reports are available in the literature exploring the option of preoperative chemotherapy as well as upfront nephrectomy. We report a rare case of paraneoplastic Cushing syndrome due to a Wilm's tumor. Based on gradual decrease of postoperative weight, blood pressure, serum adrenocorticotropic hormone, and plasma cortisol levels, along with histological confirmation of Wilm's tumor, paraneoplastic Cushing syndrome due to Wilm's tumor was confirmed.
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PMID:Paraneoplastic Cushing Syndrome Due To Wilm's Tumor. 2859 97

Wilms tumor is the most frequent pediatric renal malignancy, and its usual presentation is an abdominal mass or hematuria. Unusual presentations have also been reported, such as paraneoplastic syndromes (acquired von Willebrand disease, sudden death due to pulmonary embolism, and Cushing syndrome). These conditions can precede, occur concomitantly, or present in a later phase of tumor development. Precocious puberty, as paraneoplastic endocrine syndrome, has already been described in children with malignant tumors (brain, gonadal, adrenal tumors, and hepatoblastoma). However, little is known about central precocious puberty, as paraneoplastic manifestation of nephroblastoma or secondary to its specific chemotherapy. Here, we report a case of Wilms tumor and simultaneous precocious puberty in a 5-year-old girl. The initial diagnosis was premature telarche, but the clinical and biological pubertal progression changed our diagnosis to idiopathic central precocious puberty. Chemotherapy and nephrectomy were well tolerated, and we began treatment with a gonadotropin-releasing hormone agonist which showed favorable outcomes over the short term. We highlight the need for early diagnosis and work-up in all patients of precocious puberty, in order to institute timely management.
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PMID:Coincidental Central Precocious Puberty and Wilms Tumor in a 5-Year-Old Girl. 3158 53


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