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)

Bone scintigraphy with 99mTc-polyphosphate or 99mTc-pyrophosphate was carried out in 54 children suspected of bone disease. Signs of skeletal metastases were recognized in 13 children by scintigraphy whereas X-ray examination showed lesions in only 10 of these. In 5 children with primary osteosarcoma, three cases of fibrous dysplasia, and 4 cases of osteomyelitis, the lesions were clearly demonstrated by scintigraphy. Abnormal accumulation of radioactivity in soft tissue lesions was observed in primary adrenal neuroblastoma, Hodgkin's granuloma, and metastatic Burkitt's lymphoma. Several cases are reported, and the value of bone scintigraphy in children is discussed.
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PMID:Bone scintigraphy in children. 18 23

An acutely ill 6-month-old female infant presented with massive hepatomegaly, accompanied by severe anemia with peripheral normoblastemia and thrombocytopenia. Bone marrow examination revealed erythroid hyperplasia with gross erythroid dysplasia, reduced granulocytic precursors, and virtually absent megakaryocytes. The bone marrow also contained completely necrotic cells occurring in clumps as well as singly. The appearances suggested bone marrow involvement by neuroblastoma. Accordingly, combination chemotherapy was instituted and laparotomy was performed as soon as her clinical condition had improved. Left adrenalectomy was carried out, because a small adrenal nodule of ganglioneuroma was present. Liver biopsy showed expansion of portal tracts by loose fibrous connective tissue containing hemosiderin deposits and some degenerate cellular debris, consistent with areas of involuted metastatic neuroblastoma. Complete recovery followed, and subsequent bone marrow examination was entirely normal. It is thought that the dyserythropoiesis probably resulted from the release of toxic metabolites from regressing neuroblastoma.
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PMID:Transient dyserythropoiesis occurring during the involutionary phase of stage IV-S neuroblastoma. 271 38

Using the NIH two-phase microlymphocytotoxic test, lymphocytes of patients and control subjects were typed for HLA antigens of A and B loci: A1, 2, 3, 9, 10, 11, 28 (or Aw 19, A30, 31), B5, 7, 8, 12, 13, 14, 15, 17, 18, 21, w22, 27, 35, 40. Patients tested: 1. 75 patients with Wilms' tumour, thereof 35 had their whole families tested, 2. 20 patients with neuroblastoma, 3. 26 patients with neurofibromatosis, thereof 21 had their whole families tested, 4. 166 patients with testicular germinal tumours and 41 children with germinal tumours of diverse localization, 5. 48 individuals with haemangioma, 6. 64 women with breast cancer and 50 with dysplasia. We investigated 490 patients and, with the addition of family studies, another 193 individuals, altogether 683 persons. The results were compared with a group of 301 healthy non-related persons or with a group of 116 healthy non-related men, or with 100 healthy women and, in the family studies, with members of 47 healthy three-member families with healthy children. The chi 2 test with a Yates correction or also Fisher's exact test were used for the purpose. The resultant p was corrected using multiplication by the number of the antigens typed. In some cases we used the relative risk (RR) value. The results can be summed up in the following seven points: 1. Wilms' tumour was found to have no significant association either in our population or family studies. The latter seem to testify rather against this tumour's linkage with HLA. Our study was inconclusive as to the significance of the more frequent incidence of HLA-A1 and/or A9 in the diseased children of those families where one of the parents had at least one of those antigens. It cannot be ruled out as a sign of better prognosis. We regards as indispensable the typing of HLA antigens in patients with Wilms' tumour coincident with an inborn anomaly, as well as in members of those patients' families, and also a conclusive elucidation of the possible association with HLA-A1 and/or A9. No other centre has as yet undertaken any family studies. Consequently our possibilities of comparison with other teams' results were meagre. 2. We point to the possible conceivable relationship between neuroblastoma and HLA-B13. We found this association, albeit non-significant after correction, potentially important, especially after comparisons with the results of an only other existing study.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:HLA system and some neoplastic diseases. 307 Nov 20

Ten cases of hydrops fetalis not associated with serologic incompatibility between mother and infant were studied by autopsy. Classic trisomy 21 was present in two, and in six others the postmortem examination revealed major congenital abnormalities (hydrometrocolpos-polydactyly syndrome, achondrogenesis type 2, congenital adenomatoid malformation of the lung, and aortic valvular dysplasia with left ventricular endocardial fibroelastosis). In two instances, anasarca was associated with a congenital tumor: a sacrococcygeal teratoma and an adrenal neuroblastoma. In six fetuses, prenatal diagnosis of hydrops fetalis or severe congenital malformations was established by ultrasonography.
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PMID:Nonimmunologic hydrops fetalis. A study of ten cases. 689 72

This article reviews the pathophysiology of congenital defects and tumors that are of interest to the pediatric surgeon. Reliable information has been obtained through prenatal diagnosis of fetal anomalies and careful studies of fetal animal models. The topics covered include fetal diaphragmatic hernia, oligohydramnios-induced pulmonary hypoplasia, renal dysplasia, prune belly, gastrointestinal obstruction, biliary atresia, Wilms' tumor, and neuroblastoma. In addition, some recent experimental studies delineating the ability of the fetus to heal without scarring may have implications for all surgeons.
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PMID:The developmental pathophysiology of surgical disease. 806 34

Dysembryoplastic neuroepithelial tumor (DNT) is a clinicopathologically unique group of tumors, mostly located in the temporal lobe, associated with intractable complex partial seizure in young patients. We report two unusual cases with multifocal involvement of diverse sites in the central nervous system. Case 1 is that of a 50-year-old man with 9-year history of grand mal seizures, who died of acute myocardial infarction. Case 2 is that of a 10-year-old girl with intractable complex partial seizures and behavioral disorder. Postmortem examination in case 1 showed multifocal tumor in the left temporal lobe, third ventricle, and basal ganglia. Magnetic resonance imaging in case 2 showed tumor in the right temporal lobe, both thalami, right cerebellar hemisphere, and pons. Histologically, both tumors were characterized by a multinodular appearance with a predominant component of alveolar arrangement of oligodendroglial-like cells around delicate capillaries, with mucoid matrix containing floating ganglion cells. There were also astrocytic nodules resembling pilocytic astrocytoma in case 1, and a gangliocytoma-like area merging with surrounding cortical dysplasia in case 2. Ultrastructural examination showed ganglionic differentiation in the oligodendroglial-like cells in case 2. They possessed dense core neurosecretory granules and many slender neuritic processes with microtubules arranged in parallel and terminating in synaptic junctions. The periventricularly located tumor with nodular extension to the periphery suggests an origin from subependymal germinal matrix with nests of primitive neuroblasts arrested in their embryonal migration. DNTs are related to ganglioglioma based on their common location and clinical behavior and on the presence of both ganglionic and astrocytic cells. They are also related to pilocytic astrocytoma by morphological and behavioral similarity. Together with cerebral neuroblastoma and central neurocytoma, they form a spectrum of tumors harboring small neuronal cells. The differentiation of DNT from oligodendroglioma is important so as to avoid unnecessarily aggressive therapy.
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PMID:Dysembryoplastic neuroepithelial tumor. A tumor with small neuronal cells resembling oligodendroglioma. 817 75

Improving survival rates following pediatric bone marrow transplantation (BMT) will likely result in greater numbers of children progressing to end-stage renal disease (ESRD) because of prior chemotherapy, irradiation, sepsis, and exposure to nephrotoxic agents. Renal transplantation remains the treatment of choice for ESRD; however, the safety of renal transplantation in this unique population is not well established. We report our experience with living related renal transplantation in three pediatric patients with ESRD following prior BMT. Two patients with neuroblastoma and ESRD because of BMT nephropathy, and one patient with Schimke immuno-osseous dysplasia and ESRD because of immune complex mediated glomerulonephritis and nephrotic syndrome. Age at time of BMT ranged from 2 to 7 yr. All patients had stable bone marrow function prior to renal transplantation. Age at renal transplant ranged from 8 to 14 yr. All three patients have been managed with conventional immunosuppression, as no patient received a kidney and BMT from the same donor source. These patients are currently 7 months to 6 yr status post-living related transplant. All have functioning bone marrow and kidney transplants, with serum creatinine levels ranging 0.6-1.2 mg/dL. There have been no episodes of rejection. One patient with a history of grade III skin and grade IV gastrointestinal-graft-vs.-host disease (GI-GVHD) prior to transplantation, had a mild flare of GI-GVHD (grade I) post-renal transplant and is currently asymptomatic. The incidence of opportunistic infection has been comparable with our pediatric renal transplant population without prior BMT. One patient was treated for basal cell carcinoma via wide local excision. Renal transplantation is an excellent option for the treatment of pediatric patients with ESRD following BMT. Short-term results in this small population show promising patient and graft survival, however long-term follow-up is needed. Pre-existing immune system impairment and bone marrow function should be taken into consideration when weighing different immunosuppressive agents for renal transplantation. Patients who have undergone renal transplantation following BMT are at high risk for opportunistic infections and malignancy, and need life-long medical surveillance.
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PMID:Successful renal transplantation following prior bone marrow transplantation in pediatric patients. 1536 89

Five cases of sirenomelia presented with rare manifestations are discussed. Three neonates were born alive and died within 2 to 12 hours after birth. One case was the offspring of a triple in vitro fertilization pregnancy with history of early intrauterine death of one of the triplets. The main features included fusion of lower extremities (five of five), renal agenesis (three of five), polycystic renal dysplasia (two of five), anal atresia with large bowel hypoplasia (three of five), pulmonary hypoplasia (four of five), and single umbilical artery (five of five). Other features that have only rarely been associated with sirenomelia included concurrence of congenital heart disease and neuroblastoma, gallbladder agenesis, and upper extremity defects.
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PMID:Rare manifestations of sirenomelia syndrome: a report of five cases. 1547 30

The authors report the case of a male neonate presenting with a heterogeneous cystic mass in the right renal area with no identifiable renal unit. The diagnosis of right perirenal extracapsular uro-haematoma secondary to posterior urethral valve was proposed on the basis of the antenatal clinical history. The late discovery at 39 weeks of amenorrhoea (pregnancy not followed) of a foetus with trabeculated bladder associated with left ureteropelvic dilatation, and a cystic mass occupying the right lumbar fossa was highly suggestive of right urinoma secondary an infravesical obstacle. In this case, vaginal delivery was probably responsible for intracystic bleeding of the urinoma. The postnatal morphological assessment including ultrasound, retrograde cystourethrography and MRI confirmed the presence of a posterior urethral valve complicated by right uro-haematoma displacing the right kidney and left ureteropelvic dilatation. This lesions corresponds to a collection of urine and blood in the right perirenal space due to caliceal or parenchymal perforation secondary to raised urinary tract pressure above an obstacle. Subcapsular or extracapsular perirenal urinoma, sometimes detected in neonates with posterior urethral valve, reflects protective decompression of the urinary tract (safety valve effect), as in our case. The neonatal diagnosis of a heterogeneous renal cystic mass is sometimes difficult (polycystic renal dysplasia, cystic lymphangioma, cystic neuroblastoma, adrenal haematoma). In some cases, the antenatal history may suggest the diagnosis of perirenal urohaematoma secondary to infravesical obstruction.
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PMID:[Neonatal uro-haematoma secondary to posterior urethral valve]. 1582 95

A significant diversity of tissue types interface at the base of the skull and contribute to the diagnostic challenges of skull base surgical pathology. Advances in surgical technique now permit biopsy and resection of lesions previously termed "inoperable." Retrospective review was made of all pathology specimens from skull base surgeries performed at the University of California Davis Medical Center from 1990 to 1996. Surgical biopsies and resections were performed on 186 patients who had 33 distinctive diagnoses. Any preoperative biopsy or tissue from referring institutions was reviewed prior to skull base surgery. One hundred eighteen patients had benign lesions, the most frequent of which were pituitary adenoma (55) and acoustic neuroma (27). Other benign lesions included angiofibroma, meningioma, fibrous dysplasia, and paraganglioma. Sixty-eight patients had malignant tumors, 32 of which were squamous cell earcinoma. Other malignancies included salivary carcinomas, basal cell carcinoma, neuroblastoma, melanoma, and several sarcomas. Unexpected findings were two metastatic carcinomas and five inflammatory lesions. Nearly 1500 intraoperative consultations were performed to establish resection margins and less commonly to confirm the diagnosis. The discrepancy rate between the intraoperative and final diagnosis was 1.8%. Immunohistochemistry and/or electron microscopy was utilized in 44% of the specimens to confirm the diagnosis. Surgical pathology is an essential ingredient to a successful skull base surgery program. Pathologists are involved in both pre- and intraoperative decisions. The diversity of lesions that arise from the skull base often has overlapping histologies that require careful attention to morphology and the use of ancillary studies for accurate diagnosis. The need for frequent intraoperative interpretations contributes to the significant challenge for the surgical pathologist.
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PMID:Surgical pathology of the skull base: a 7-year experience. 1717 Oct 65


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