Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027819 (neuroblastoma)
 27,800 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

This is a brief case report of a patient who developed chylothorax subsequent to abdominal radiation for neuroblastoma, treated by aspirations and local chemotherapy, and mediastinal irradiation.
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PMID:Chylothorax in neuroblastoma. 89 52

Local control is vital for long-term survival for patients with stage III neuroblastoma, and although cure is difficult, ultimate success in stage IV neuroblastoma will necessitate control of the primary tumor as well as effective therapy of the metastases. The proper timing of surgical resection of the primary tumor is uncertain. Patients with stage III and IV neuroblastoma treated from 1977 to 1988 were retrospectively reviewed as to whether the resection was performed before or after chemotherapy. Complications assessed include significant blood loss, damage to adjacent organs, and delays before postsurgical chemotherapy could be given. Sixty patients were treated primarily at the authors' institution: 18 with stage III and 42 with stage IV disease. Chemotherapy consisted of combinations of nitrogen mustard, adriamycin, dacarbazine (DTIC), cisplatin, vincristine, and cyclophosphamide (MADDOC). Nine patients with stage III neuroblastoma underwent initial resection of the primary tumor before receiving chemotherapy. Three had complications, all with excessive blood loss (0.57, 2.0, and 3.0 times the estimated total blood volume [TBV]). One patient had renal infarction, and another had regrowth of the tumor before chemotherapy could be administered 35 days after surgery. There were no complications in the eight secondary explorations, four of which were complete resections. All had viable tumor in the resected specimen. Eleven of the 42 stage IV patients had primary resections, 5 of whom had complications: colocutaneous fistula, unilateral renal necrosis, chylothorax, and excessive blood loss (1.3 and 2 TBV). None of the 18 patients with delayed resection after 3 to 12 courses of chemotherapy had surgical complications with complete (14 patients), near complete (2 patients), or subtotal resections (2 patients).(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Surgical management of stage III and IV neuroblastoma: resection before or after chemotherapy? 194 91

This is the first reported association of congenital chylothorax and mediastinal neuroblastoma in a newborn infant. The infant exhibited features of both congenital and acquired chylothorax. Resolution of the chylothorax occurred following ligation of thoracic duct after failure of conservative management with multiple thoracentesis and chest tube drainage.
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PMID:Congenital chylothorax and mediastinal neuroblastoma. 200 37

The clinical presentation and surgical treatment of thoracic anomalies--developmental malformations of the respiratory tract, congenital chylothorax or mediastinal masses--in 15 infants are reported. The age range at operation was 2 weeks to 8 months. The diagnoses were lobar emphysema (3 cases), bronchogenic cyst (3), cystic adenomatoid malformation (1), enteric duplication (2), hyperplastic thymus (2), neuroblastoma (1), chylothorax (1), cystic lymphangiectasia (1) and tracheal stenosis (1). The most common symptom was respiratory embarrassment, with acute development in half of the cases. The diagnosis could be established or suspected from chest radiography in 14 of the 15 infants. All were submitted to thoracotomy. None died postoperatively, but three had major complications. At postoperative follow-up 13 of 14 patients were free from respiratory symptoms.
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PMID:Surgical management of thoracic anomalies in infants. Respiratory-tract malformations, congenital chylothorax and mediastinal masses. 338 51

Of the 2877 patients who underwent chest surgery at our department during the 20-year period between 1973 and 1992, 9 (0.3%) developed postoperative chylothorax. The underlying disease included primary lung cancer in 5 patients, pulmonary metastasis in 1, invasive thymoma in 2, and neuroblastoma of the posterior mediastinum in 1. For the treatment of chylothorax, the thoracic duct was ligated in 2 patients with a high volume of chylous leakage. In 6 patients treated conservatively, early pleurodesis was attained by injecting 1 to 5 doses (mean: 2.2 doses) of the streptoccal preparation OK-432 intrathoracically; favorable results were achieved. In 1 patient, the diagnosis of chylothorax was delayed because of postoperative pyothorax. This patient developed nutritional deficiency, compromised immunity, and disseminated intravascular coagulation (DIC), which led to death before the chylothorax could be treated. In principle, postoperative chylothorax should be treated conservatively. Favorable results can be expected with the intrathoracic injection of OK-432 beginning at the early postoperative period to achieve pleurodesis, combined with the prevention of nutritional deficiency, electrolyte imbalance, and infection.
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PMID:Treatment of postoperative chylothorax by pleurodesis with the streptococcal preparation OK-432. 782 62

Chylothorax and chylopericardium are rare conditions occurring in infants and children. Both may be traumatic or nontraumatic in origin. We reviewed our experiences with the management of sixteen pediatric cases (10 males, 6 females; 11-days to 14-years old) of chylothorax and chylopericardium from 1997 to 2003. There were fifteen cases of chylothorax (9 left, 2 right, 4 bilateral) and two cases of chylopericardium (1 isolated and 1 associated with chylothorax), and their incidences of occurrence after cardiothoracic surgery were 0.89% and 0.12%, respectively. Of the fifteen cases occurring after cardiothoracic surgery, thirteen patients had corrective or palliative surgery for complex congenital heart disease, and two patients had removal of thymolipoma and neuroblastoma, respectively. Included also in this review was an 11-day old preterm infant with hydrops fetalis and congenital heart disease who developed chylothorax. Characteristics of chylous effusion included a presence of whitish opaque fluid in the pleural cavity and the pericardial cavity, having a triglyceride content ranging from 59 to 1689 mg/dl which was higher than a plasma triglyceride, a protein content of 2.4 to 7.4 g/dl, and a presence of lymphocyte predominance. The average latent period for diagnosis of chylothorax or chylopericardium was 13 days (range 3-30). All patients were treated primarily with nutritional modification using medium-chain triglycerides (MCT) instead of long-chain triglycerides in their diet; and only a few cases needed bowel rest with total parenteral nutrition (TPN). Twelve patients completely responded to a MCT-rich diet; two cases resolved after switching to TPN and another case needed surgery for ligation of lymphatic vessels around the thymus gland. The mean duration of lymph drainage was 12.1 days (range 3-29) and the average length of time of continued conservative treatment (MCT-rich diet and TPN) was 29.8 days (range 18-47). Fourteen patients (87.25%) had good outcome, i.e. resolution from chylothorax or chylopericardium and return to normal diet. Two of the fourteen patients developed severe infections; one was diagnosed with suspected bacterial endocarditis and the other had candidemia. However, both responded well to antibacterial and antifungal drugs, respectively. One case succeeded after surgery. A case that had low compliance with dietary recommendations and required repeated placement of drainage devices died due to infection with enterococcal septicemia. Early and good compliance with MCT-rich diet is essential for achieving a favorable outcome in the management of chylothorax and chylopericardium in children.
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PMID:Management of chylothorax and chylopericardium in pediatric patients: experiences at Siriraj Hospital, Bangkok. 1592 38

Chylothorax is an uncommon complication in children. Although surgery and trauma are the most common causes encountered, hematological as well as solid malignancies can present with chylothorax. This study aimed to describe the presentation and management of malignant chylothorax in children. This is a case series from a pediatric hematology-oncology unit. Chylothorax was diagnosed by demonstrating high triglyceride content in the pleural fluid and a low cholesterol concentration in relation to the serum cholesterol. Cytology for malignant cells and investigations for tuberculosis were performed in all patients. Initial management included placement of an intercostal tube and administration of a fat-free diet with the addition of medium-chain triglycerides. Appropriate treatment of the underlying malignancy was initiated simultaneously. Three children with diagnoses of Stage IV neuroblastoma, lymphoblastic lymphoma, and Hodgkin lymphoma developed chylothorax. Malignant cytology was positive in the patient with T-NHL. All patients were found to have associated hypoproteinemia and hypoalbuminemia. The chylothorax resolved with conservative measures in two patients. It remained intractable in the child with T-NHL, in whom the lymphoma was refractory to chemotherapy. Chylothorax is a rare but challenging complication that can accompany childhood malignancies. Surgical interventions, radiotherapy, and pleurodesis are alternatives if the chylothorax is refractory to medical management.
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PMID:Chylothorax in children with cancer: A milky predicament. 3008 60

Neonatal chylothorax is a common cause of neonatal congenital pleural effusion and is often caused by the accumulation of chylous fluid in the thoracic cavity due to the rupture of the thoracic duct and its branched lymphatic vessels for a variety of reasons. Neonatal chylothorax caused by malignant tumors is extremely rare, and this is the first case of neonatal mediastinal neuroblastoma with chylothorax in China. The boy was found to have pleural effusion in the left thoracic cavity in the uterus, and experienced apnea at birth, as well as dyspnea and cyanosis as the main manifestations after birth. He was diagnosed with left chylothorax based on conventional biochemical analysis of pleural effusion. After the treatment including persistent chest drainage and symptomatic and supportive treatment, the drainage of the left thoracic cavity reached a volume of 90-180 mL per day. Neonatal refractory chylothorax was considered. Chest radiograph on day 13 after birth showed lesions in the upper left lung field, and contrast-enhanced plain CT scan of the chest suggested the possibility of posterior mediastinal neuroblastoma. The autopsy confirmed giant posterior mediastinal neuroblastoma (poorly differentiated), which involved the C7-T6 spinal canal and the nearby erector spinae, with a small amount of tumor tissue in the liver and both adrenal glands. Mediastinal tumor is considered the underlying cause of chylothorax in this case.
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PMID:[Fetal pleural effusion in the uterus and dyspnea after birth]. 3280 38