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Neuroblastoma 01

Topic: Pediatric

Created on Thursday, February 7 2008 by jdmiles

Last modified on Thursday, February 7 2008.

A 3 year-old boy presents with worsening fever, irritability, and weight loss. There is a palpable abdominal mass on exam. Imaging shows a large mass arising from the adrenal gland. Which of the following paraneoplastic disorders is most classically associated with this type of tumor?

A) Lambert-Eaton myasthenic syndrome

B) Myoclonic encephalopathy of infants

C) Myasthenia gravis

D) Stiff-person syndrome

E) Dermatomyositis

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This question was created on February 07, 2008 by jdmiles.
This question was last modified on February 07, 2008.

ANSWERS AND EXPLANATIONS

A) Lambert-Eaton myasthenic syndrome

This answer is incorrect.

Myoclonic encephalopathy of infants, or "dancing eyes" syndrome, is associated with neuroblastoma. It is characterized by acute or subacute onset of myoclonus, cerebellar ataxia and opsoclonus ("dancing eyes"). (See References)


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B) myoclonic encephalopathy of infants

This answer is correct.

Neuroblastoma is the third most common tumor of childhood, after leukemia and primary brain tumors. Myoclonic encephalopathy of infants, or "dancing eyes" syndrome, is associated with neuroblastoma. It is characterized by acute or subacute onset of myoclonus, cerebellar ataxia and opsoclonus ("dancing eyes"). (See References)


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C) myasthenia gravis

This answer is incorrect.


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D) stiff-person syndrome

This answer is incorrect.


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E) dermatomyositis

This answer is incorrect.


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References:

1. Park, J.R., Eggert, A., and Caron, H. (2008). "Neuroblastoma: biology, prognosis, and treatment." Pediatr Clin North Am, 55(1) 97-120. (PMID:18242317)
2. DÃaz-Carballo, D., Malak, S., Bardenheuer, W., Freistuehler, M., and Reusch, H. (2008). "CYTOTOXIC ACTIVITY OF NEMOROSONE IN NEUROBLASTOMA CELLS." J Cell Mol Med (PMID:18194446)
3. Boltshauser, E., Deonna, T., and Hirt, H.R. (1979). "Myoclonic encephalopathy of infants or "dancing eyes syndrome". Report of 7 cases with long-term follow-up and review of the literature (cases with and without neuroblastoma)." Helv Paediatr Acta, 34(2) 119-33. (PMID:222713)
4. Ootsuka, S., Asami, S., Sasaki, T., Yoshida, Y., Nemoto, N., Shichino, H., Chin, M., Mugishima, H., and Suzuki, T. (2007). "Analyses of novel prognostic factors in neuroblastoma patients." Biol Pharm Bull, 30(12) 2294-9. (PMID:18057715)
5. WickstrÃ¶m, M., Johnsen, J.I., Ponthan, F., SegerstrÃ¶m, L., SveinbjÃ¶rnsson, B., Lindskog, M., LÃ¶vborg, H., Viktorsson, K., Lewensohn, R., Kogner, P., Larsson, R., and Gullbo, J. (2007). "The novel melphalan prodrug J1 inhibits neuroblastoma growth in vitro and in vivo." Mol Cancer Ther, 6(9) 2409-17. (PMID:17876040)


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pediatric
Neuroblastoma 01
Question ID: 020708137
Question written by J. Douglas Miles, (C) 2006-2009, all rights reserved.
Created: 02/07/2008
Modified: 02/07/2008
Estimated Permutations: 600