Serum neuron‐specific enolase in children with neuroblastoma. Crelationship to stage and disease course

Abstract

Serum neuron-specific enolase (NSE) was measured in 61 children at diagnosis with all stages of neuroblastoma. The median serum values for Stages I, II, III, IV, and IV-S were 13, 23, 40, 214, and 40 ng/ml, respectively. Mean serum levels were different between groups I versus IV, (P = 0.0004) II versus IV (P = 0.0001) and IV-S versus IV (P = 0.004). The prognostic value of serum NSE for disease-free survival was determined in 54 patients at risk for relapse 2 or more years after diagnosis. The disease-free survival rate of all patients with levels of less than 100 ng/ml was 27/34 (79%), whereas it was 2/20 (10%) for those with higher levels. In 28 patients with lower stage disease and a good prognosis (Stages I, II, and IV-S) NSE levels were not predictive of relapse. Only 1 of these 28 patients had a raised level (greater than 100 ng/ml) and survived without relapse, whereas 4 patients who relapsed had serum NSE less than 100 ng/ml at diagnosis. In patients with Stages III and IV disease, a raised serum NSE level was associated with poor outcome: only 1/19 (5%) survived with NSE levels greater than 100 ng/ml, whereas survival was 5/8 (63%) with values below 100 ng/ml. Serial samples were analyzed on 17 patients; all 8 patients with initial NSE levels greater than 100 ng/ml achieved near normal levels during remission (median, 21 ng/ml). However, in only 4/10 patients studied at time of relapse, did the levels rise coincident with relapse. The sera of 47 patients with other forms of cancer and 19 siblings of cancer patients were at or near the normal limits (0-15 ng/ml), with three exceptions: acute lymphoblastic leukemia (286 ng/ml), hepatoblastoma (176 ng/ml), and primitive neuroectodermal tumor (105 ng/ml). Serum NSE is a useful marker for patients with advanced neuroblastoma in whom elevated levels were associated with a poor outcome; the raised NSE levels returned to near normal after therapy. In patients with Stage IV-S disease serum NSE levels were significantly lower than those in Stage IV despite their extensive tumor burden. Serum NSE estimation may confirm Stage IV-S status and suggest a more benign clinical course.

Treatment of localised resectable neuroblastoma. Results of the LNESG1 study by the SIOP Europe Neuroblastoma Group

“…At present, only abnormal MYCN gene status is believed to correlate with worse outcome in these patients, and postoperative treatment is therefore deemed necessary only in such cases (Rubie et al, 1997). A number of other factors, including regional lymph node involvement (Ninane et al, 1982;Rosen et al, 1984), tumour rupture (De Bernardi et al, 1995), unfavourable histology (Perez et al, 2000;Shimada et al, 2001), DNA index (Bagatell et al, 2005), and some biochemical markers (Hann et al, 1985;Zeltzer et al, 1986;Shuster et al, 1992), have been associated with greater risk of relapse and death.…”

Serum neuron-specific enolase in children's cancer

“…By contrast, focal staining for NSE has been observed in Ewing's sarcoma, rhabdomyosarcoma and lymphoma (Triche et al, 1985) and >25 ng ml' The level of > 100 ng ml1 was chosen as a in several tumours in adults including renal cell carcinoma second discriminant because it has been shown to have (Vinores et al, 1984). Biochemical analysis has shown that prognostic significance in stage IV under 1 year NSE accounts for 28% to 62.5% of the enolase activity of neuroblastoma; levels > 100 ng ml1 carry a worse prognosis neuroblastoma, but only 1% to 4.5% of that in Wilms' (Zeltzer et al, 1986).…”

“…neuroendocrine cells, and thought at one time to be specific Immunoreactive NSE is stable at least up to 4 years at this for these cell types, subsequently its demonstration in many temperature (Zeltzer et al, 1986), the distribution of NSE other cell types has indicated it lacks the specificity that the values from samples stored >2 years being similar to that of name implies (Schmechel, 1985). NSE is one of several samples analysed soon after collection.…”

“…Serum NSE levels can be distant lymph nodes and stage IVs defined as patients with raised in neuroblastoma and have prognostic significance localised tumour who would otherwise be stage I and II but (Ishiguro et al, 1983;Notomi et al, 1985; who have remote disease confined to one or more of the 1983; 1986 (1980). the serum NSE level (Zeltzer et al, 1986).…”

Ativação plaquetária na esclerose sistêmica e alternativas metodológicas para sua avaliação

“…Segundo Day e Thompsom (87) , a NSE ocorre também em plaquetas em níveis equivalentes à sua concentração no cérebro, e em eritrócitos em níveis aproximadamente 10% dessa. É considerada marcadora de câncer de pequenas células de pulmão (88)(89)(90) e neuroblastoma (91)(92)(93) .…”