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Presented by Peter Illei, M.D. and prepared by Andrea Subhawong, M.D.
Case 1: 2 year old boy with a large abdominal/retroperitoneal mass.
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Question 1 of 1
1. Question
Week 395: Case 1
2 year old boy with a large abdominal/retroperitoneal mass. Immunostains were positive for chromogranin and synaptophysin. Cytokeratin was negative.images/6.1.09.01a.jpg
images/6.1.09.01b.jpg
images/6.1.09.01c.jpgCorrect
Answer: Neuroblastoma, undifferentiated
Histology: none provided
Discussion: Sections show a small round blue cell tumor that exhibits areas of necrosis and a solid pattern less growth. The tumor cells have a high N:C ratio and are round to oval nuclei with finely granular chromatin. Small nucleoli are also noted. The mitosis-karyorrhexis index is high and no stromal component is identified. These findings are most consistent with an undifferentiated stroma-poor neuroblastoma representing unfavorable histology.
Immunostains (not shown) were positive for synaptophysin and chromogranin and negative for desmin, myogenin, WT1, CD99, Cam5.2 and AE1/AE3 negative. This staining pattern supports the diagnosis and helps exclude other small blue cell tumors in the differential diagnosis. This tumor was n-myc amplified.
Wilms tumor or nephroblastoma can have diffuse blastemal growth patterns where the tumor cells are small, closely packed and have frequent mitosis. The blastemal cells are vimentin positive and may show focal expression of NSE, desmin and cytokeratin. In addition, the blastemal cells are typically WT-1 positive (nuclear staining). Desmoplastic small round cell tumor (DSRCT) may exhibit similar histology, however, the tumor cells may exhibit epithelial, muscular or neural differentiation. Most cases are cytokeratin, desmin (Cytoplasmic dot-like staining), EMA, vimentin and NSE positive. Myogenin and myoD1 are typically negative.
Primitive neuroectodermal tumors (PNET) are typically CD99, FLI-1 and vimentin positive and can also express BCl-2, cytokeratin and NSE and a number of other neural markers.
Incorrect
Answer: Neuroblastoma, undifferentiated
Histology: none provided
Discussion: Sections show a small round blue cell tumor that exhibits areas of necrosis and a solid pattern less growth. The tumor cells have a high N:C ratio and are round to oval nuclei with finely granular chromatin. Small nucleoli are also noted. The mitosis-karyorrhexis index is high and no stromal component is identified. These findings are most consistent with an undifferentiated stroma-poor neuroblastoma representing unfavorable histology.
Immunostains (not shown) were positive for synaptophysin and chromogranin and negative for desmin, myogenin, WT1, CD99, Cam5.2 and AE1/AE3 negative. This staining pattern supports the diagnosis and helps exclude other small blue cell tumors in the differential diagnosis. This tumor was n-myc amplified.
Wilms tumor or nephroblastoma can have diffuse blastemal growth patterns where the tumor cells are small, closely packed and have frequent mitosis. The blastemal cells are vimentin positive and may show focal expression of NSE, desmin and cytokeratin. In addition, the blastemal cells are typically WT-1 positive (nuclear staining). Desmoplastic small round cell tumor (DSRCT) may exhibit similar histology, however, the tumor cells may exhibit epithelial, muscular or neural differentiation. Most cases are cytokeratin, desmin (Cytoplasmic dot-like staining), EMA, vimentin and NSE positive. Myogenin and myoD1 are typically negative.
Primitive neuroectodermal tumors (PNET) are typically CD99, FLI-1 and vimentin positive and can also express BCl-2, cytokeratin and NSE and a number of other neural markers.
