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Presented by William Westra, M.D. and prepared by Orin Buetens, M.D.
Case 4: 46 year-old man with nosebleeds.
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1. Question
Week 19: Case 4
46 year-old man with nosebleeds./images/1818a.jpg
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Answer: Esthesioneuroblastoma
Histology: At low power, the tumor grows as lobules within the submucosa. The overlying respiratory mucosa appears normal. Vessels showing prominent glomeruloid-type of endothelial proliferation separate the tumor lobules. This type of vascular proliferation has been associated with tumors showing neuroendocrine differentiation.
At higher power, the tumor cells have round-to-oval uniform nuclei with small nucleoli. Cell borders are indistinct. Necrosis and significant mitotic activity are not present. Rosettes and pseudorosettes are not identified.
Discussion: Esthesioneuroblastoma (olfactory neuroblastoma) are tumors derived from the olfactory neuroepithelium. They typically present as polypoid masses that arise high and deep within the nasal cavity (i.e. from the olfactory epithelium). They can occur across a wide range of ages, but demonstrate a bimodal peak in the second and sixth decades of life.
Esthesioneuroblastomas are included in the differential diagnosis that includes a spectrum of other primitive appearing round cell-tumors. In the sinonasal tract, this list includes malignant mucosal melanoma, rhabdomyosarcoma, lymphoma, PNET/Ewing’s group tumors, and others. Clearly the clinical history is helpful in sorting through this differential diagnosis. In addition, immunohistochemistry plays a valuable role. The expected immunohistochemical profile of a typical esthesioneuroblastoma is as follows:
Chromogranin / Synaptophysin: positive;
Neurofilament: positive;
S100: positive in compressed cells at periphery of lobules;
Cytokeratin: variably positive (scattered positive in about 25%) of tumors;
EMA: generally negative;
HMB45: negative;
Desmin: negative;
Leukocyte common antigen: negative;
O13: negative.The expected behavior of esthesioneuroblastoma is dictated by tumor stage, but tumor grade may also be predictive. One of the more commonly employed classification schemes (the Hyams’ Histologic Grading System) using a 4 tiered grading scheme. As tumors become more poorly differentiated (i.e. Grade 3 and 4), they loose many of the more characteristic morphologic features of esthesioneuroblastoma such as its neurofibrillary matrix, Homer Wright rosettes, and uniform cytology. Thus at the far end of the morphologic spectrum, esthesioneuroblastoma may be difficult to distinguish from sinonasal undifferentiated carcinoma.
Incorrect
Answer: Esthesioneuroblastoma
Histology: At low power, the tumor grows as lobules within the submucosa. The overlying respiratory mucosa appears normal. Vessels showing prominent glomeruloid-type of endothelial proliferation separate the tumor lobules. This type of vascular proliferation has been associated with tumors showing neuroendocrine differentiation.
At higher power, the tumor cells have round-to-oval uniform nuclei with small nucleoli. Cell borders are indistinct. Necrosis and significant mitotic activity are not present. Rosettes and pseudorosettes are not identified.
Discussion: Esthesioneuroblastoma (olfactory neuroblastoma) are tumors derived from the olfactory neuroepithelium. They typically present as polypoid masses that arise high and deep within the nasal cavity (i.e. from the olfactory epithelium). They can occur across a wide range of ages, but demonstrate a bimodal peak in the second and sixth decades of life.
Esthesioneuroblastomas are included in the differential diagnosis that includes a spectrum of other primitive appearing round cell-tumors. In the sinonasal tract, this list includes malignant mucosal melanoma, rhabdomyosarcoma, lymphoma, PNET/Ewing’s group tumors, and others. Clearly the clinical history is helpful in sorting through this differential diagnosis. In addition, immunohistochemistry plays a valuable role. The expected immunohistochemical profile of a typical esthesioneuroblastoma is as follows:
Chromogranin / Synaptophysin: positive;
Neurofilament: positive;
S100: positive in compressed cells at periphery of lobules;
Cytokeratin: variably positive (scattered positive in about 25%) of tumors;
EMA: generally negative;
HMB45: negative;
Desmin: negative;
Leukocyte common antigen: negative;
O13: negative.The expected behavior of esthesioneuroblastoma is dictated by tumor stage, but tumor grade may also be predictive. One of the more commonly employed classification schemes (the Hyams’ Histologic Grading System) using a 4 tiered grading scheme. As tumors become more poorly differentiated (i.e. Grade 3 and 4), they loose many of the more characteristic morphologic features of esthesioneuroblastoma such as its neurofibrillary matrix, Homer Wright rosettes, and uniform cytology. Thus at the far end of the morphologic spectrum, esthesioneuroblastoma may be difficult to distinguish from sinonasal undifferentiated carcinoma.
