Favorable prognosis for survival in children with coincident opso-myoclonus and neuroblastoma

Abstract

Case reports of 28 neuroblastoma patients who had opso-myoclonus as their presenting feature are reviewed. As compared with the 30%-34% two-year survival rate for the overall population of patients with neuroblastoma, those who exhibited the opso-myoclonus/neuroblastoma combination had a tumor-free two-year survival rate of 89.3%. This excellent prognosis may be explained partially by earlier diagnosis and a higher percentage (71% vs. 33%) of patients with Stage I, II, and IV-S disease in the opso-myoclonus sub-group. However, these factors are not, of themselves, sufficient to explain totally the differences in survival rate since five of seven patients with Stage III-IV disease also exhibited long-term survival. This raises the question as to whether the neurologic dysfunction in these patients is pathogenetically related to an unknown factor (possibly autoimmune) which also controls growth and spread of the tumor.

Managing Paraneoplastic Neurological Disorders

“…Conversely, approximately 2%-3% of children with neuroblastoma have paraneoplastic opsoclonus-myoclonus [58,59]. Tumors in children with paraneoplastic opsoclonus-myoclonus apparently have a better prognosis than tumors in patients without this PNS.…”

Paraneoplastic limbic encephalitis in a patient with extensive disease small-cell lung cancer

“…Paraneoplastic limbic encephalitis (PLE) is a rare disorder infrequently accompanying cancer, coexisting with SCLC in ~50% of the cases (5). It was suggested that the prognosis of cancer patients with accompanying paraneoplastic syndromes may be better compared with that of patients with the same cancer but without associated paraneoplasia (6)(7)(8).…”

Frequency of SOX Group B (SOX1, 2, 3) and ZIC2 antibodies in Turkish patients with small cell lung carcinoma and their correlation with clinical parameters

“…14,25 Detailed analyses of such patients have shown PND to be associated with smaller tumor size, 26,27 limited-stage disease, 17,28 cellular infiltration of tumors, 29 -32 and better cancer prognosis. [33][34][35] Autoimmune PND is observed in Ͻ 1% of patients with SCLC, 25 yet antibodies against the PND-associated, neuroectodermal antigens HuD, ZIC4, recoverin, and VGCC(P/Q-type) have been reported in Յ 25.5%, 16%, 15%, and 5% of patients with SCLC, respectively.…”