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)

We describe two patients to illustrate that neonatal respiratory distress can be associated with airway compression from an intrathoracic neuroblastoma. The clinical features can be either life-threatening from the time of birth or so mild as to escape detection in the immediate newborn period.
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PMID:Neonatal neuroblastoma presenting with respiratory distress. 650 89

We report an unusual presentation of congenital intrathoracic neuroblastoma. Severe respiratory distress was present at birth and several salient clinical signs were suggestive of neonatal myocarditis. Excessive catecholamine secretions may have caused the observed cardiac dysfunction. Peculiar previously unreported cutaneous lesions were also noted in this case.
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PMID:Intrathoracic neuroblastoma presenting with neonatal cardiorespiratory distress. 672 96

Manifestations of pulmonary metastases were reviewed in a retrospective study of 30 patients with the autopsy diagnosis of neuroblastoma seen over a 25 year period. Seven patients with histologic evidence of pulmonary metastases are reported. Radiologic manifestations included examples of direct extension, hematogenous spread, and lymphangitic spread. Two patients with lymphangitic pulmonary metastases had clinically significant respiratory distress. Pulmonary spread in neuroblastoma represents widely disseminated metastatic diseases and is a grave prognostic sign.
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PMID:Pulmonary metastases in neuroblastoma. 697 16

Ninety-six patients with thoracic neuroblastoma were studied in a prospective fashion. Median age at presentation was 0.9 years. Forty-eight percent of the patients presented with stage A disease, 20% stage B, 13% stage C, 17% stage D, and 2% stage DS. Seventy-five patients have been followed for greater than 4 years. A posterior mediastinal mass was diagnosed on incidental chest roentgenograms performed for nontumor-related symptoms in 49% of the cases. Sixteen percent of the cases presented with neurological symptoms and 14% of the patients presented with acute respiratory distress. Urinary catecholamines were elevated in 76% of the cases. Complete surgical resection was carried out in 47% of the cases, while incomplete resection or biopsy was performed in 45%. No operation was performed in 3 patients. Minor surgical complications occurred in 20% of the patients, and 3% of the patients had significant perioperative complications. One patient died as a complication of therapy. Overall actuarial survival was 88% at 4 years. This study confirms the favorable outcome in children with mediastinal neuroblastoma. The basic biology of thoracic neuroblastomas seems to differ from that of other sites in that the majority of patients present at a younger age with localized disease or regional lymph node metastases, and have an improved survival even after correcting for age and stage. While complete excision is recommended, if possible, radical surgical procedures are not indicated since an excellent prognosis is associated with combined modality therapy.
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PMID:Thoracic neuroblastoma: a Pediatric Oncology Group study. 846 49

We report a patient with a congenital mediastinal dumbbell neuroblastoma (NB) presenting as respiratory distress at birth rather than a neurologic deficit. The tumor did not respond to low-dose chemotherapy, but radiotherapy and surgery were effective. The patient was observed postoperatively without further therapeutic intervention considering the favorable biologic factors despite the appearance of liver metastases, which ultimately regressed spontaneously. The authors believe that most congenital NBs have favorable outcomes with a possibility of spontaneous regression, and that an understanding of the biologic features of tumors is important to avoid overtreatment of lesions with a favorable prognosis. Keywords Congenital neuroblastoma. Dumbbell tumor. Liver metastasis. Spontaneous regression
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PMID:Congenital mediastinal dumbbell neuroblastoma with spontaneous regression of liver metastases. 988 Jul 6

A male infant presenting with respiratory distress was found to have primary thoracic neuroblastoma. This method of presentation in the newborn period is rare. His symptoms resolved spontaneously with conservative management. Full staging revealed a poorly differentiated stage 2 tumour with favourable cytogenetic markers.
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PMID:Neonatal neuroblastoma presenting with respiratory distress. 1132 82

Neuroblastic tumors are the third most common cause of solid tumors in early childhood. Cervical tumors account for only 5% of cases. In this report, we describe a series of four pediatric neuroblastic tumors of the neck. The histological diagnosis was ganglioneuroblastoma in three cases and neuroblastoma in one case. Presenting signs were solitary cervical mass in two cases and respiratory distress in association with Claude-Bernard Horner's syndrome in two cases. Mean age at presentation was 15 months. Cervical computed tomography scan and/or magnetic resonance imaging depicted calcifications within the tumor in 50% of cases and allowed accurate assessment of extension. Increased urine catecholamine levels were observed only in the patient with neuroblastoma. Scintigraphy with [131]iodine-methyliodobenzylguanidine demonstrated selective uptake by the tumor in two cases. Amplification of N-myc oncogene, a documented unfavorable prognostic sign, was not found in any case. Surgical treatment was performed in all patients. Neoadjuvant chemotherapy was performed in one case. All patients underwent regular surveillance. No evidence of recurrence has been observed with a mean follow-up period of 7 years.
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PMID:Primary pediatric neuroblastic tumors of the neck. 1151 94

Neuroblastoma is a common solid tumor of childhood that can involve the abdomen, thorax, pelvis, or the head and neck. The clinical manifestations are dependent on the widespread distribution of neural crest tissue and the length of the sympathetic chain involvement. Abdominal pain and hypertension may occur as a result of renal vasculature compression; respiratory distress may be evident in thoracic tumors; and Homer's syndrome or heterochromia of the iris may manifest from neuroblastoma of the head and neck. In addition, symptoms of cord compression and back pain may result from spinal cord compromise due to epidural invasion. Metastatic involvement of the liver, skin, periorbital regions, or bone may cause hepatomegaly, skin nodules, proptosis, or bone marrow failure, respectively. Clinical findings along with tumor metastasis may be studied by various imaging modalities to assess the nature and extent of the tumor. Diagnostic tests include plain radiography, ultrasonography, CT scanning, and MR imaging. Bone marrow studies, bone scans, and scintigraphy with 131I-metaiodobenzylmandelic may be utilized for metastatic evaluation. By using these imaging studies to detect the nature and behavior of neuroblastoma, early intervention may indeed improve patient survival.
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PMID:Imaging neuroblastoma in children. 1262 83

The case of a one-month-old patient admitted to the Department of Pediatrics (Medical and Health Science Center, Debrecen University) because of respiratory distress caused by a cervical mass compressing the upper respiratory pathways is presented. The mass could only be partially removed, the histological diagnosis proved to be neuroblastoma (SBCT: "small blue cell tumor"). Despite the fact that the DNA index of tumor cells (ploidy measurements) and the age of the patient suggested a favourable prognosis, the tumor continued to grow and metastases appeared. Because of symptoms of compression exerted on the respiratory system by the tumor, chemotherapy had to be applied. Since a standard OPEC/OJEC chemotherapeutic protocol proved to be not entirely effective and a residual tumor was still present, retinoic acid and interferon treatment was introduced. Presently, 4 years after the diagnosis, the patient is in complete remission and can be considered to be cured. The case presented here demonstrates that despite the favorable prognosis of the majority of infant neuroblastomas, in some cases the anatomic location of the tumor, leading to disturbance of vital functions, may serve as indication of chemotherapy. Our experience also proved the efficacy of retinoic acid and interferon treatment in relapsed neuroblastoma.
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PMID:[Cervical neuroblastoma in an infant]. 1510 2

Leukoerythroblastosis is a rarely observed disease characterized by the presence of leukocytosis, erythroid and myeloid blast cells in peripheral blood. To our knowledge, it had not been diagnosed in a premature newborn before the case we report have.A female baby weighing 1164 grams, who was born prematurely at the 29th week of gestation by Cesarean section was referred to our newborn intensive care unit due to prematurity and respiratory distress with no prenatal pathological findings. Physical examination revealed tachypnea and hepatosplenomegaly. Routine laboratory measurements showed significant leukocytosis (85,000/mm3) and anemia (Hb: 9.6 g/dL and Hct: 27.6%). The platelet count was normal. The peripheral blood smear suggested leukoerythroblastosis with the presence of nucleated erythrocytes, monocytosis, and 4% blasts. Bone marrow cytogenetic examination was normal. Parvovirus B19 Ig G and M serology were detected to be positive. The etiological factors observed in leukoerythroblastosis occurring during neonatal and early childhood period are congenital-postnatal viral infections, juvenile myelomonocytic leukemia and osteopetrosis. To our knowledge, no case of leukoerythroblastosis in such an early phase has been reported in the in literature. As a result, premature delivery and leukoerythroblastosis were thought to have developed secondary to intrauterine parvovirus B19 infection. Leukoerythroblastosis is a rarely observed disease characterized by the presence of leukocytosis, erythroid and myeloid blast cells in peripheral blood. It is reported that it can be observed following hematologic malignancies especially juvenile myelomonocytic leukemia, acute infections, hemolytic anemia, osteopetrosis, myelofibrosis, neuroblastoma and taking certain medicines. To our knowledge, it has not been diagnosed in a premature newborn before. Here we the case of a newborn who was referred to our intensive care unit due to being born prematurely at the 29th week of gestation and diagnosed with leukoerythroblastosis.
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PMID:Premature labor and leukoerythroblastosis in a newborn with parvovirus B19 infection. 1626 29


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