ABSTRACT
We report a case of a mediastinal seminoma occurring 19 months after the resolution of a pineal germinoma. A 15-year-old boy with headaches and visual changes was diagnosed with a pineal germinoma by biopsy and mildly elevated beta-human chorionic gonadatropin (beta-HCG) in serum and cerebral spinal fluid. Radiation therapy leads to the resolution of his pineal germinoma and normalization of the beta-HCG. A mediastinal seminoma (germinoma) was diagnosed nearly 2 years later because of rising serum beta-HCG. There was no evidence of recurrent central nervous system disease. The patient underwent systemic chemotherapy with the complete resolution of the mediastinal seminoma.
Subject(s)
Germinoma/radiotherapy , Mediastinal Neoplasms/drug therapy , Neoplasms, Second Primary/drug therapy , Pinealoma/radiotherapy , Seminoma/drug therapy , Adolescent , Chorionic Gonadotropin, beta Subunit, Human/blood , Chorionic Gonadotropin, beta Subunit, Human/cerebrospinal fluid , Germinoma/blood , Germinoma/cerebrospinal fluid , Germinoma/pathology , Humans , Male , Mediastinal Neoplasms/blood , Mediastinal Neoplasms/cerebrospinal fluid , Mediastinal Neoplasms/pathology , Neoplasms, Second Primary/blood , Neoplasms, Second Primary/cerebrospinal fluid , Neoplasms, Second Primary/pathology , Pinealoma/blood , Pinealoma/cerebrospinal fluid , Pinealoma/pathology , Seminoma/blood , Seminoma/cerebrospinal fluid , Seminoma/pathology , Time FactorsABSTRACT
BACKGROUND: Nonrhythmic involuntary ocular oscillations and axial and segmentary myoclonia are associated in the opsoclonus-myoclonus syndrome. In adults, a paraneoplastic origin is generally found. We report the first of opsoclonus-myoclonus associated with non-Hodgkin's lymphoma. CASE REPORT: A 66-year-old woman rapidly developed a typical opsoclonus-myoclonus syndrome within a few hours, presenting vertigo, cerebellous ataxia, multidirectional involuntary ocular movements and non-rhythmic axial and segmentary myoclonia. Brain computed tomography and magnetic resonance imaging demonstrated discrete diffuse anomalies of the white substance predominating in the pons. The cerebrospinal fluid showed discrete lymphocytosis. Antineuron antibodies were negative. No cause could be identified until the development 11 months later of pleomorphic T-cell mediastino-cervical lymphoma. The patient responded moderately to a CHOP regimen which had no effect on the opsoclonus-myoclonus syndrome. Death occurred after a 16-month course due to pulmonary complications. DISCUSSION: Neuroblastoma and infectious causes predominate in opsoclonus-myoclonus syndromes observed in children; in adults, the predominant cause is cancer. Antineuron, anti-Ri and anti-Hu antibodies can be evidenced in some cases, arguing in favor of a paraneoplastic mechanism. Recent reports have evidenced MRI anomalies in the pons and the cerebellum, anatomically well correlated with the opsoclonus-myoclonus syndrome. Besides small-cell bronchogenic anaplastic cancer, the possibility of cancer of the breast and uterus, and both non-Hodgkin and Hodgkin lymphoma should be explored, knowing the cancer develops several month after the opsoclonus-myoclonus syndrome.
