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)

A case of meningeal carcinomatosis associated with cerebral metastases from an adrenal neuroblastoma is described. The clinical picture was ushered-in by bilateral sciatic pain in a 50 years old female and was followed by rapidly progressive sensory-motor deficits of the arms and legs, leading to flaccid quadriplegia associated with paralysis of cranial nerves and episodes of mental confusion. Death occurred 4 months alter, in cardiac failure. At autopsy, a bilateral tumor of the adrenal glands was found. No metastases were detected anywhere except in the central nervous system. Histology identified the tumor as a neuroblastoma; meningeal carcinomatosis, radicular infiltration by tumor cells and parenchimal metastases were found in the central nervous system. Neuroblastoma is typically a tumor of childhood, only 13% of them being found in adult's according to Russell and Rubinstein. Meningeal metastases from adrenal neuroblastoma have not hitherto been reported in the literature. In our opinion, the most likely mode of spread of tumor cells to the central nervous system was hematogenous because of the presence of small multiple intraparenchimal metastases; however, possible spread through the perineural lymphatics, as proposed by others, cannot be excluded, due to the prominent localization of tumor cells at spinal roots level. The main differential diagnostic problems (paraneoplastic neuropathy (Wyburn-Mason) and infectious subacute or chronic meningitis) are discussed. The authors stress the emportance of complete cerebro-spinal fluid examination including a careful search for tumor cells.
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PMID:[Meningeal carcinomatosis: clinical and anatomical study of a case of suprarenal neuroblastoma (author's transl)]. 6

Olfactory neuroblastoma is an uncommon malignant tumor originating in the olfactory epithelium in the roof of the nose. It usually produces nasal obstruction and secondary sinus disease and is often mistaken by the physician for a nasal polyp. Olfactory neuroblastoma has been observed to cause death by distant metastasis or by invasion through the cribriform plate and secondary meningitis in most instances, and is generally associated with a low cure rate. During the past eight years, we have treated three patients with olfactory neuroblastoma by a combination of extensive local surgery using the lateral rhinotomy approach with ethmoidectomy and medial partial maxillectomy, combined with either preoperative or postoperative irradiation therapy. From a review of the literature we have tried to draw meaningful conclusions regarding treatment.
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PMID:Olfactory neuroblastoma. Management and prognosis. 80 2

This report evaluates the efficacy of extensive chest wall resection and prosthetic reconstruction in 15 children with chest wall malignancies. There were nine boys and six girls, with a mean age of 9.6 years. Eleven patients had primary chest wall tumors including Ewing's sarcoma (ES), six; rhabdomyosarcoma (RH), two; chondrosarcoma (CS), one; Askin's malignant neuroectodermal tumor, one; and mesenchymal sarcoma, one. Four children had metastases to chest wall and lung from Wilms' tumor (WT), two; osteogenic sarcoma (OS), one; and neuroblastoma (NB), one. Chest wall resection of two to six ribs and reconstruction with Marlex mesh (seven), lattisimus flap (two), prolene mesh (one), and more recently, a Gortex patch (five), was performed. Eight of the patients required concomitant en-bloc pulmonary resection (wedge, five; lobectomy, two; pneumonectomy, one) and two required resection of diaphragm. Fourteen received adjunctive therapy (chemotherapy, 14; irradiation, eight [preoperative, five; postoperative, three]. Six patients had second-look resections after chemotherapy. There was no operative mortality. Early pulmonary function was normal; however, pulmonary restrictive disease and scoliosis occurred with growth. One ES patient developed a radiation-induced second malignant tumor at age 10 and one ES child died at age 6 (no evidence of disease) of meningitis. Average survival length for ES patients was 77 months (range, 18 to 132 months.) Currently, eight patients are alive and five are free of disease. Extensive chest wall resection and reconstruction is useful in the treatment of primary chest wall tumors, but is palliative in metastatic cases. The Gortex patch is the current prosthetic of choice.
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PMID:Chest wall resection and reconstruction for malignant conditions in childhood. 320 68

Monoclonal antibodies have been proposed as targeting agents for cytotoxic compounds in vivo. We have undertaken a phase 1 study of 131I UJ13A in patients with neuroblastoma as well as a pilot study for the intrathecal use of radiolabelled conjugates for diffuse malignant meningitis. Access to solid tumour deposits appears to be a major problem in targeting sufficient isotope to elicit a therapeutic response. This, however, is overcome in the intrathecal use of antibodies for diffuse disease. Problems have also been identified in changes in the pharmacokinetics of antibody handling that occur on repeated administration of conjugate to patient. This is presumably due to the generation of an anti-mouse Ig response. These studies indicate some of the limitations in using murine antibodies as targeting agents and point to areas where their use may be of maximal effect.
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PMID:Diagnosis and therapy of neuroectodermally associated tumours using targeted radiation. 362 19

Unexpected sudden cardiac death among children with a history of Kawasaki disease has come to be reported in Japan. Death occurred between 2 months and 8 years after complete recovery from Kawasaki disease according to our study material. To study the lesions of Kawasaki disease sequelae we examined 61 cases of Kawasaki disease which came to autopsy. In 17 of these the deaths apparently to be due to sequelae of this disease, were characterized by cardiac insufficiency caused by ancient coronary aneurysm with organized thrombotic occlusion and superimposed acute ischemic myocardial degeneration and/or necrosis. The age of the lesions appears to correlate with the interval period between complete recovery from this disease and death. Six cases succumbed incidentally of other causes: one traffic accident, one hemophilus meningitis, one chronic myeloid leukemia, one neuroblastoma, one meningeal hemorrhage due to rupture of basilar arterial aneurysm, and one acute lymphatic leukemia. Even in these cases, definite sequelae of arteritis were detected. A surgically resected coronary aneurysm and a ventricular aneurysm were also examined. It was disclosed by mass physical check-up of school children that 0.1% had a history of Kawasaki disease, among which 5-6% showed cardiac and/or coronary abnormality including aneurysms. The high incidence of cardiac involvement in Kawasaki disease has been proved in this study and this kind of lesion has the possibility of resulting in unexpected cardiac death of children.
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PMID:Pathological study of sequelae of Kawasaki disease (MCLS). With special reference to the heart and coronary arterial lesions. 379 88

During a 4-year period, 26 children with systemic malignancies suffered cerebrovascular accidents. These occurred in 17 patients with lymphoreticular malignancy and nine patients with solid tumors. They were the presenting signs of malignancy in three patients and were the direct cause of death in six. Cerebrovascular accidents were directly related to disseminated intravascular coagulation in eight patients, to chemotherapy in eight patients, to metastatic tumor in three patients, to thrombocytopenia in three patients, and to fungal meningitis in one patient. All patients with disseminated intravascular coagulation had leukemia and at times, cerebrovascular thrombosis predated systemic or laboratory evidence of disseminated intravascular coagulation. This review indicates that four major syndromes are apparent in children with cancer: vascular thrombosis associated with disseminated intravascular coagulation, acute arterial or sagittal sinus thrombosis secondary to L-asparaginase in children with leukemia, acute neurologic dysfunction in patients with osteogenic sarcoma treated with high-dose methotrexate, and obtundation, seizures, and focal neurologic deficits in patients with neuroblastoma metastatic to the torcular region. Although elevated WBC counts and thrombocytopenia occur frequently in children with cancer, in themselves they uncommonly result in strokes. It is concluded that cerebrovascular accidents are a relatively frequent cause of acute neurologic compromise in children with cancer and that certain types of malignancies and their treatment predispose patients to this complication.
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PMID:Cerebrovascular accidents in children with cancer. 386 Jul 96

A case of olfactory neuroblastoma in a 9 month-old girl is reported. Blindness and purulent meningitis were the first symptoms. Radiological examinations including CT scan revealed a sphenoidal tumor with penetration of the meninges. Surgery revealed an ethmoido-sphenoidal tumor mass extending into the pituitary fossea which consisted of malignant cells with the histological aspect of esthesioneuroblastoma. The rarity of this tumor in early childhood and its clinical polymorphism are emphasized.
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PMID:[Olfactory neuroblastoma disclosed by blindness in a 9-month-old girl]. 400 68

Cefotiam (CTM) was evaluated for its safety and efficacy in children. Twenty-six patients were treated with 40 to 200 mg/kg per day of CTM by intravenous administrations. The diagnosis of the patients were acute pharyngitis (2), acute bronchitis (1), pneumonia (4), empyema (2), urinary tract infection (2), typhoid fever (1), acute enterocolitis (2), partially-treated purulent meningitis (1), and suspected septicemia in neuroblastoma (1); and the remaining ten patients were considered to have nonbacterial infections. The pathogens recovered were Streptococcus pyogenes (1), Streptococcus pneumoniae (1), Staphylococcus aureus (4), Haemophilus influenzae (4), Escherichia coli (1), enteropathogenic Escherichia coli (1), Salmonella typhi (1), and Campylobacter jejuni (1). All but two patients of bacterial infections were cured after the CTM therapy, and the rate of efficacy was 87.5%. Diarrhea (3), urticaria (1), transient elevation of GOT and GPT (1), and transient eosinophilia (3) were found to be associated with the CTM therapy. However, no severe adverse reactions were encountered. Half life of the serum CTM level was 0.93 +/- 0.13 hours, and excretion into the urine was rapid. CSF concentration obtained 1 hour after an intravenous injection of 21 mg/kg of CTM in a case with inflamed meninges was 1.5 mcg/ml, and the CSF/serum ratio was 9.0%. From these data, CTM appears to be a safe and effective antibiotic when used in children with susceptible bacterial infections.
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PMID:[Clinical evaluation of cefotiam therapy in children (author's transl)]. 627 Apr 13

The therapeutic effects of cefmenoxime (CMX), a new synthetic cephalosporin antibiotic, were examined in the treatment of various pediatric infections. Patients treated were infants and children ranging from one-month-old to 13-year-old suffering from pharyngitis in 2 cases, bronchopneumonia in 3 cases, cervical lymphadenitis in 2 cases, urinary tract infections in 7 cases, tympanitis in 2 cases, suppurative meningitis, sepsis, subcutaneous apostem, acute enteritis, chest wall apostem, phlegmon, staphylococcal scalded skin syndrome in 1 case each, a total of 23 cases. As regards method of administration, CMX from a vial was dissolved in physiological saline or distilled water for injection, and the solution was administered by 3 to 5 minutes one short intravenous injection (14 cases), or CMX was diluted with large volume parenteral product and administered by 30 to 60 minutes drip infusion (9 cases). The dosage of the drug was 30 to 200 mg/kg/day; 103 mg/kg/day and under in 21 cases, 150 mg/kg/day and 200 mg/kg/day in 1 case each. The administration was continued for 3 to 27 days. As regards clinical efficacy, "good" or "excellent" results were obtained in all the cases except 2 cases, one was alpha-Streptococcus acute tympanitis supervening neuroblastoma, and the other was Pseudomonas urinary tract infection. The efficacy rate was 91.3% with excellent in 11 cases, good in 10 cases. As regards bacteriological effects, of 13 strains of Gram-positive bacteria, 10 strains were eliminated and 3 strains were not changed, while of 10 strains of Gram-negative bacteria, 8 strains were eliminated and 2 strains were reduced; thus CMX showed better results against Gram-negative bacteria rather than against Gram-positive ones. The antimicrobial activity of CMX against Gram-positive bacteria was inferior to those of CTM and CEZ, but CMX showed the highest antimicrobial activity against Gram-negative bacteria. No clinical side effects nor abnormal laboratory findings obviously attributable to CMX were observed.
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PMID:[Therapeutic effects of cefmenoxime in the treatment of various infections on infants and children]. 630 39

Transfacial approaches, traditionally used for malignant tumors of the paranasal sinuses, provide limited exposure when several sinuses are involved and are unsuitable for tumors that erode through the floor of the anterior cranial fossa. A transcranial approach may aid in the removal of such lesions. To better understand the risks and benefits of this surgical approach, we reviewed all patients (n = 76) who underwent a transcranial approach as part of the excision of paranasal sinus lesions between 1984 and 1993 at our institution. The spectrum of disease included adenocarcinoma (13 patients), squamous cell carcinoma and olfactory neuroblastoma (11 patients each), adenoid cystic carcinoma and poorly differentiated forms of carcinoma (6 patients each), melanoma (5 patients), and miscellaneous others (24 patients). Most patients had ethmoid sinus involvement; tumors were also commonly found in the cribriform plate, sphenoid sinus, and nasal fossa. In each patient, a bifrontal craniotomy was performed with extradural dissection to the floor of the anterior fossa and osteotomies for resection of involved elements. In 47 patients (62%), disease in the orbit, the anterior nasal cavity, or the soft tissues of the face required transfacial as well as transcranial resections. Bony defect in the anterior fossa floor was repaired with a pedicled pericranial flap. Patients with major complications included six patients with epipericranial and/or epidural hematomas requiring evacuation, three with transient cerebrospinal fluid leaks, two who developed bifrontal cerebral infarcts, and one who died soon after surgery. No meningitis was seen. To date, 26 patients (34%) have died; of those living (mean follow-up, 34 mo), 42 (84%) remain in full remission. The transcranial approach can achieve removal of erosive, invasive tumors from this area with predictable morbidity and may be considered whenever sinus tumors breach the anterior cranial base or extend beyond the reach of conventional transfacial approaches.
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PMID:Anterior transcranial (craniofacial) resection of tumors of the paranasal sinuses: surgical technique and results. 897 51


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