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)

The authors report their experience with 70 pediatric patients with spinal cord compression (SCC) due to malignancies identified among 898 patients with solid tumors. An extradural tumor was the most frequent cause of SCC (71%); 54% of these were soft tissue sarcomas and neuroblastoma. Most intradural tumors (70%) were outside the spinal cord, 9/12 being metastatic medulloblastomas. The SCC localized mainly to the dorsal and lumbosacral regions (42% each). Pain was the most common symptom (94%). MRI proved diagnostic in all cases in which it was used, while myelography was diagnostic in 85% of 26 patients. CT scan demonstrated the lesion in 83% of the patients. Laminectomy was provided for patients with paraplegia of less than 96 h evolution; isolated recurrence of the main tumor; a primary spinal cord tumor; progression of neurologic symptoms after chemotherapy/radiotherapy; chemotherapy and radiotherapy-resistant tumor (when known); resection of a paraspinal tumor. Surgery was avoided when prognosis of primary disease was poor or risks exceeded possible benefits. Twelve/twenty-one (57%) patients with paraplegia were able to walk after laminectomy only, while 14% (2/14) improved after chemotherapy and radiotherapy. Survival rates were 38% for the former and 36% for the latter. Overall survival was related to the original malignancy. All patients (12) admitted without paraplegia and submitted to laminectomy were able to walk, and of these, 6 presented a primary spinal cord tumor. The remaining had paraspinal tumors that extended to the spinal canal. Almost 87% (20/23) of the patients without paraplegia who submitted to medical treatment were able to walk, while only one progressed to paraplegia. Patients with SCC may entertain radio- and chemotherapy when harboring tumors responding to such therapies and present no evidence of neurologic damage progression. The latter manifestation is a strong indication for laminectomy without delay.
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PMID:Spinal cord compression: a review of 70 pediatric patients. 1463 20

Neuroblastoma stands out among pediatric solid tumors because of its relative frequency, intriguing natural history, prognostic biologic features, and therapeutic challenges. It is the most common extracranial pediatric solid tumor and the most common neoplasm in infancy; >90% of the approximately 600 cases diagnosed annually in the United States are in children < or = 5 y old. Screening programs of infants show that many cases escape detection because of spontaneous regression or maturation into benign lesions. Origin from precursors of the sympathetic nervous system accounts for (a) primary sites in adrenal glands and in paraspinal locations from neck to pelvis and (b) high urinary levels of catecholamines in >90% of cases. This embryonal neoplasm often encases vascular structures and, unlike most solid cancers, usually presents with substantial metastatic disease (bone, bone marrow, lymph nodes, liver; spread to lung or brain is rare). Hence, defining disease status requires CT (or MRI), bone scan, metaiodobenzylguanidine (MIBG) scan, bone marrow tests, and urine catecholamine measurements. The natural history is strikingly variable but is largely predictable from clinical and biologic features. The latter are critical for distinguishing low-risk (90% survival) from high-risk ( approximately 25%-30% survival) subsets, allowing identification of patients who, despite a favorable clinical profile (e.g., localized tumor), are likely to develop lethal metastatic disease, versus patients who have an ominous clinical profile (e.g., widespread disease) but are likely to survive, sometimes with little or no cytotoxic therapy.
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PMID:Neuroblastoma: a disease requiring a multitude of imaging studies. 1523 64

The notion of emergency with regards to pediatric neuroimaging requires a strong knowledge of clinical indications. In children under 2 years of age, head trauma requires a CT scan in case of repeated or prolonged or rapidly increasing vomiting, focal signs, loss of consciousness, unusual behavior, seizures, clinical signs of skull fracture or polytrauma. The "shaken baby syndrome" is usually suspected in case of loss of consciousness or seizures before 8 months of age. The hematomas that are observed are subdural in location, diffuse and deeply located. Imaging is only mandatory for headache suggesting underlying space occupying lesion: permanent or increasing pain, nocturnal headache, headache during postural changes or efforts, associated to seizures or abnormal neurological examination. No imaging is indicated in case of first epileptic seizure associated to normal neurological examination and without any particular context. The presence of trauma, intracranial hypertension, persisting disturbances of consciousness or associated focal sign necessitates urgent neuroimaging. No imaging is indicated in case of typical febrile seizures, i.e. generalized, brief and occurring between 1 and 5 years of age. Spinal cord symptoms require immediate MRI evaluation. The most frequent tumor is neuroblastoma. In the absence of spinal tumor, brain abnormalities must be excluded (inflammatory disease). In neonates, CT scan or MRI must be readily performed in case of seizures or loss of consciousness to exclude ischemic, traumatic or infectious lesions.
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PMID:[Pediatric neuroimaging emergencies]. 1554 39

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 2 year old boy presented with features of opsoclonus, myoclonus and ataxia. Routine investigations of blood, urine, x-ray chest, bone scan, EEG and MRI of brain, were normal. Urine for VMA was negative. A right suprarenal mass was detected at MRI of abdomen. The mass was resected completely and was found histologically to be of differentiating type of neuroblastoma. The child was treated initially with prednisolone for 6 weeks along with sodium valproate. He is still on sodium valproate for his neurological symptoms. His symptoms still persist though they have decreased in intensity.
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PMID:A case of opsoclonus-myoclonus-ataxia with neuroblastoma. 1586 78

The ganglioneuroma is a benign neuroblastal tumor. All neuroblastomas and ganglioneuromas derive from immature cells of the sympathetic nerve system. The ganglioneuroma is a very rare disease and effects newborns and infants more often than adolescents and adults. The benign tumors are relatively difficult to diagnose since they usually are asymptomatic. A 5-year old girl with persistent bladder wall thickening and prominent course of the prevesical ureter presents for diagnosis and therapy. Sonography demonstrated a space-occupying lesion of the left kidney. Subsequent MRI raises the suspicion of a retroperitoneal neuroblastoma. The tumor could be removed in two surgical sessions. The ganglioneuroma is a benign tumor with symptoms depending on location and with a relatively good prognosis. Its low incidence and resultant limited experience often leads to delayed diagnosis, potentially determining therapy and diagnosis.
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PMID:[Retroperitoneal ganglioneuroma]. 1611 Apr 9

Magnetic resonance (MR) plays a unique role in the diagnosis and management of pediatric abdominal masses. The "as low as reasonably achievable" (ALARA) radiation dose of CT is zero when substituted by MR. Whole body MR may also compete with PET imaging to stage abdominal tumors. Specific advantages of MR include determination of resectability of hepatic tumors using MRI and MRA; staging of neuroblastoma in the bone marrow, lymph nodes, liver, and spinal canal; response of bilateral Wilms tumor and nephroblastomatosis; detection of pelvic tumors with sagittal sectioning, and peritoneal tumors with contrast enhancement.
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PMID:Magnetic resonance imaging of abdominal masses in the pediatric patient. 1615 36

A 39-year-old woman who presented with typical Cushingoid appearance (moon facies, central obesity, purpura) was admitted to our hospital because of pulmonary infection. She was found to have hypertension, severe hypokalemia, and metabolic alkalosis. Endocrine data revealed elevated plasma levels of ACTH and cortisol with lack of circadian rhythm, non-suppressibility to high-dose dexamethasone, and hyperresponsiveness to CRH stimulation. Although no pituitary mass was detected by MRI of the brain, inferior petrosal sinus sampling showed a step-up of central to peripheral ACTH levels; these data are consistent with the diagnosis of Cushing's disease. She was successfully treated with metyrapone to control hypercortisolemia. Ten months later, a mass was detected in the ethmoid sinus, which was surgically removed. After resection of the ethmoid sinus tumor, her Cushingoid features and hypercortisolemia disappeared, but recurred after enlargement of a second mass in the maxillary sinus. After resection of the maxillary sinus tumor, her hypercortisolemia subsided. Histologically, the tumor tissues from both the ethmoid and maxillary sinus were identical and consistent with the diagnosis of olfactory neuroblastoma. Immunohistochemically, the immunoreactivities of ACTH and POMC were positive in the cytoplasm of tumor cells, and immunoreactive ACTH was demonstrated in both tumor tissues. Thus, this is the second rare case with ectopic ACTH syndrome caused by olfactory neuroblastoma thus far reported.
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PMID:Olfactory neuroblastoma causing ectopic ACTH syndrome. 1641 Jun 58

A 3-year-old male, diagnosed with stage 4 neuroblastoma, developed recurrent leptomeningeal metastasis after multi-modality treatment including multi-agent chemotherapy, surgery, high dose chemotherapy plus stem cell rescue, cis-retinoic acid and intravenous (IV) topotecan. He then received intraommaya (IO) topotecan three times weekly (maximum dose; 0.4 mg). A complete response was achieved by a resolution of malignant cells in cerebrospinal fluid and resolution leptomeningeal enhancement by brain MRI. Treatment toxicities included low-grade fever and minimal headache. The duration of treatment response from IO topotecan was 18 weeks. The survival time from CNS recurrence in this patient was 13 months. We suggest IO topotecan be considered for neoplastic meningitis of tumors with known sensitivity to topotecan.
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PMID:Favorable response of intraommaya topotecan for leptomeningeal metastasis of neuroblastoma after intravenous route failure. 1657 4

Olfactory neuroblastoma is a rare tumor of the nasal cavity. It is a locally aggressive tumor with local recurrence, and distant metastasis occurs in 22-40% of patients. We report a case of olfactory neuroblastoma with cauda equina metastases. A 49-year old male had undergone surgery twice previously; the first for olfactory neuroblastoma in October, 1990, and the second for its intracranial and orbital metastasis in September, 1999. He complained of lumbago in autumn, 2005 and MRI showed two enhanced lesions in the cauda equina. The mass was partially removed and histologically diagnosed as olfactory neuroblastoma metastasis. Whole-spine irradiation of 32 Gy and lumber-spine irradiation of 10 Gy were performed. The mass at L2/3 was disappeared after the irradiation although the mass at L4/5 was not changed in size. The patient was discharged without neurological deficit and is now kept under observation as an outpatient. Olfactory neuroblastoma with spinal metastasis is rare and only 11 cases have been reported in the literature. A very poor prognosis was observed in the patients of olfactory neuroblastoma with spinal metastasis. Olfactory neuroblastoma is a radiosensitive tumor, and radiotherapy for spinal metastasis was reported to be effective. However, effectiveness of chemotherapy was still uncertain. The patient with olfactory neuroblastoma should be observed carefully even though no local recurrence had been detected over 10 years. Radiotherapy and further treatment including chemotherapy should be considered in case of spinal metastasis.
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PMID:[Olfactory neuroblastoma with spinal metastasis: case report]. 1749 47


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