Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0029713 (immaturity)
 4,335 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Clinical and biological significance of increased urinary excretion of dopamine in Japanese children with neuroblastoma was investigated. There was an increase in dopamine excretion in 19 of 29 patients (66%) and 15 of 19 in stages III and IV (79%). When the ratio of noradrenaline and dopamine was divided into two at the value of 3.5 x 10(-2), the disease-free survival rate was four of 16 (25%) in the low ratio group and nine of 19 (69%) in the high ratio group. In five patients, the urinary analysis revealed that only the level of dopamine was elevated before initiation of the therapy. The common features of these patients were as follows: (1) the age at diagnosis was 1 to 4 years; (2) all originated from the suprarenal region; (3) stages were advanced III or IV; and (4) the prognosis was poor. N-myc oncogene of the primary tumor was evident in three, and all were amplified to 32, 37, and 112 copies. These observations suggested that the immaturity of catecholamine metabolism may correlate to the poor prognosis and that "dopaminergic neuroblastoma" may be a clinical subentity of poor prognostic neuroblastoma.
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PMID:Dopaminergic neuroblastoma as a poor prognostic subgroup. 338 88

In mature and immature teratoma the treatment is surgical. The risk of recurrence can be estimated from the parameters primary site (with the coccygeal tumors being most at risk), histological grade of immaturity and completeness of the primary resection including the adjacent organ of origin (coccyx, ovary, testis etc.). In case of a microscopically complete tumor resection there is no role for adjuvant chemo- or radiotherapy irrespective of the histological grade of immaturity. Malignant germ-cell tumors (GCT) account for 2.9% of all malignant tumors of children younger than 15 years of age. More than half of the tumors occur at extragonadal sites such as the ovaries (26%), the coccygeal region (24%), the testes (18%) and the brain (18%) represent then primary sites. In patients with extensive tumor growth, metastatic disease or secreting intracranial tumors a delayed tumor resection after preoperative chemotherapy is preferable. In these patients malignant non-seminomatous GCT may be diagnosed clinically due to the increased serum or cerebrospinal fluid levels of the tumor markers AFP and/or beta-HCG. Current risk adapted treatment protocols containing cisplatinum allow long-term remissions in about 80% including patients with bulky or metastatic tumors. In the cisplatinum era the prognostic factors like histology, primary site of the tumor and initial tumor stage have partly lost their former impressive significance in infants and children. On the other hand the completeness of the primary tumor resection according to oncological standards has been established as the most powerful prognostic parameter superior to tumor marker levels or primary site of the tumor.
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PMID:Germ-cell tumors in childhood and adolescence. GPOH MAKEI and the MAHO study groups. 1081 91