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Presented by Pedram Argani, M.D. and prepared by Hillary Ross, M.D.
Case 2: This is a 3 year old male with opsoclonus.
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Question 1 of 1
1. Question
Week 474: Case 2
This is a 3 year old male with opsoclonus.images/1alex/04052011case2image1.jpg
images/1alex/04052011case2image2.jpg
images/1alex/04052011case2image3.jpgCorrect
Answer: Neuroblastoma
Histology: This is a small round cell tumor that involves the adrenal. The lesional cells have minimal schwannian stroma separating them. These cells are associated with prominent neuropil, and occasional cells (less than 5%) demonstrate more vesicular chromatin with evident nucleoli, which indicates abortive ganglion cell differentiation. These are the typical features of a neuroblastoma, stroma poor, poorly differentiated. In this age group, this is an unfavorable prognosis tumor. The current case was associated with the opsoclonus-myoclonus syndrome, which is a paraneoplastic syndrome associated with neuroblastoma.
Discussion: Primitive neuroectodermal tumor could show focal Homer Wright rosettes with neuropil; however, this should not show the abundant neuropil outside of Homer Wright rosettes seen in the current case, and ganglion cell differentiation would not be seen in a PNET. Rhabdomyosarcoma would not have the abundant neuropil of the current lesion, and would label for muscle markers like myogenin and desmin. Malignant lymphoma would be composed of more discohesive cells lacking evidence of neural differentiation.
Incorrect
Answer: Neuroblastoma
Histology: This is a small round cell tumor that involves the adrenal. The lesional cells have minimal schwannian stroma separating them. These cells are associated with prominent neuropil, and occasional cells (less than 5%) demonstrate more vesicular chromatin with evident nucleoli, which indicates abortive ganglion cell differentiation. These are the typical features of a neuroblastoma, stroma poor, poorly differentiated. In this age group, this is an unfavorable prognosis tumor. The current case was associated with the opsoclonus-myoclonus syndrome, which is a paraneoplastic syndrome associated with neuroblastoma.
Discussion: Primitive neuroectodermal tumor could show focal Homer Wright rosettes with neuropil; however, this should not show the abundant neuropil outside of Homer Wright rosettes seen in the current case, and ganglion cell differentiation would not be seen in a PNET. Rhabdomyosarcoma would not have the abundant neuropil of the current lesion, and would label for muscle markers like myogenin and desmin. Malignant lymphoma would be composed of more discohesive cells lacking evidence of neural differentiation.
