Cytomegalovirus-associated stage 4S neuroblastoma relapsed stage 4.
Neuroblastoma is one of the most frequent solid tumors in childhood, rarely recurrent after five years from diagnosis. Cytomegalovirus (CMV), a major pathogen causing congenital birth defects and severe opportunistic diseases, has been shown to have teratogenic, immunodepressive and oncogenic properties. The case of a girl with stage 4S neuroblastoma diagnosed at three months and relapsed as stage 4 five years later is reported. In both circumstances, active CMV infection was revealed by positive CMV-specific IgM and IgA antibodies, CMV-DNAemia and CMV culture. At three months, the patient presented with subcutaneous nodules, hepatosplenomegaly and increased aminotransferase levels, and the opsolonus-myoclonus syndrome. Mental retardation developed later on. At 5 years, relapsed neuroblastoma was preceded by a mononucleosis-like syndrome concomitant with active CMV infection and decreased levels of immune cells and natural killer activity. Clinical, virologic, and immunologic findings suggest an immune-mediated pathogenic role for CMV in this tumor.
Department of Pediatrics, La Sapienza University, Rome, Italy.
G
G
Nigro
A
A
Schiavetti
J C
JC
Booth
A
A
Clerico
C
C
Dominici
A
A
Krzysztofiak
M
M
Castello
D000008:Abdominal Neoplasms / Q000276:immunology / Q000473:pathology / Q000821:virology; D001483:Base Sequence; D003586:Cytomegalovirus Infections / Q000276:immunology / Q000473:pathology / Q000821:virology*; D005260:Female; D006258:Head and Neck Neoplasms / Q000276:immunology / Q000473:pathology / Q000821:virology; D006801:Humans; D007223:Infant; D007694:Killer Cells, Natural / Q000276:immunology; D008969:Molecular Sequence Data; D009447:Neuroblastoma / Q000276:immunology / Q000473:pathology / Q000821:virology*; D012008:Recurrence; D014412:Tumor Virus Infections / Q000276:immunology / Q000473:pathology / Q000821:virology*