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Occult Mediastinal Ganglioneuroblastoma Presenting with Myoclonic Encephalopathy as Paraneoplastic Syndrome

Journal of the Korean Pediatric Society 1994;37(5):695-700.
Published online May 15, 1994.
Occult Mediastinal Ganglioneuroblastoma Presenting with Myoclonic Encephalopathy as Paraneoplastic Syndrome
Hahng Lee1, Dong Ki Han1, Jae Won Oh1, In Joon Seol1, Eun Kyung Hong2, Seok Chol Jeon3
1Department of Pediatrics, College of Medicine, Han yang University, Seoul, Korea
2Department of Pathology, College of Medicine, Han yang University, Seoul, Korea
3Department of Radiology, College of Medicine, Han yang University, Seoul, Korea
간대성 근령련 뇌증의 부신생물 증후군으로 진단된 잠재성 종격동 신경절아 세포종
이항1, 한동기1, 오재원1, 설인준1, 홍은경2, 전석철3
1한양대학교 의과대학 소아과학교실
2한양대학교 의과대학 병리학교실
3한양대학교 의과대학 진단방사선과학교실
Ganglioneruroblastoma and neuroblastoma are among commonest types of childhood malignancy and a number of unique paraneoplastic syndromes have been associated with both localized and disseminated neuroblastoma. The coincidence of neuroblastoma and myoclonic encephalopathy or other paraneoplastic syndromes occurs relatively rare, and therefore, failure to recognize this association could result in delays in both diagnosis and treatment, and the result could prove to be unfortunately fatal. The mechanism which underlies the remote damaging effect of neural crest tumor, especially neuroblastoma, on the nervous system resulting in myoclonic encephalopathy is by no means clear. In addition the nature and the extent of the pathologic lesion are inconsistent. We experienced a case of myoclonic encephalopathy associated with an occult mediastinal ganglioneuroblastoma in a 22-month-old girl who was hospitalized for inability to walk without support and tilting of the head to the left side. She became increasingly ataxic, and during the hospitalization myoclonic jerks of upper extremities and head along with chaotic, rapidly flickering, multidirectional spontaneous eye movements, were noted. Laboratory data included normal complete blodd count, urinalysis, BUN and creatinine, electrolytes and bone marrow. Chest X-ray and chest CT revealed a relatively well marginated right posterior mediastinal mass. In a 24 hours urine excretion test, VMA and catecholamines were increased. Over the next 2 weeks, a surgical exploration revealed a right posterior mediastinal mass. Microscopically the mass proved to be a ganglioneuroblastoma, extending to right innominate artery and right axillary lymph nodes. Within 2 weeks after the surgery, radiotherapy (2,400 rads) and chemotherapy (CTX, DTIC, VER) were started, but corticosteroid was not used. She has been free of tumor and abnormal neurological systemic symptoms and signs for 1 1/2 year since the completion of chemotherapy. In the 3 1/2 years follow-up period, her neurologic symptoms has completely resolved by the completion of 2 years chemotherapy. We report a case of mycoclonic encephalopathy associated with hidden gang-lioneuroblastoma in 22-month-old girl.
Key Words: Myoclonic encephalopathy, Opsoclonus, Ataxia, Paraneoplastic syndrome, Neroblastoma, Ganglioneuroblastoma

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