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Presented by Fred Askin, M.D. and prepared by Natasha Rekhtman, M.D., Ph.D.
Case 5: The patient is a 58 year-old male with decreased hearing and a mass in the middle ear.
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1. Question
Week 231: Case 5
The patient is a 58 year-old male with decreased hearing and a mass in the middle ear.images/7 15 05 case 5 1.jpg
images/7 15 05 case 5 2.jpg
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images/7 15 05 case 5 4.jpgCorrect
Answer: Paraganglioma
Histology: Classic Paraganglioma is a well differentiated tumor resembling normal paraganglia and composed of Chief (Type 1) cells, arrayed in nests or lobules (“Zellballen”) and surrounded by an inconspicuous single layer of sustentacular or Type 2 cells. The polygonal Chief cells have central, round to oval nuclei with a very finely stippled chromatin and inconspicuous nucleoli. Chromogranin and synaptophysin positivity in the Chief cells and S-100 related protein positivity in the sustentacular cells is helpful in the diagnosis.
Discussion: Intravascular material in this case represents the fact that the patient received therapeutic embolization prior to excision of the lesion. Paragangliomas in the skull may show extensive distortion and the characteristic organoid or cell ball pattern may be difficult to find.
Reference(s):
– Kleihues P, Cavenee WK, Editors, Pathology and Genetics of Tumors of the Nervous System, IARC Press, Lyon, France, 2000.Incorrect
Answer: Paraganglioma
Histology: Classic Paraganglioma is a well differentiated tumor resembling normal paraganglia and composed of Chief (Type 1) cells, arrayed in nests or lobules (“Zellballen”) and surrounded by an inconspicuous single layer of sustentacular or Type 2 cells. The polygonal Chief cells have central, round to oval nuclei with a very finely stippled chromatin and inconspicuous nucleoli. Chromogranin and synaptophysin positivity in the Chief cells and S-100 related protein positivity in the sustentacular cells is helpful in the diagnosis.
Discussion: Intravascular material in this case represents the fact that the patient received therapeutic embolization prior to excision of the lesion. Paragangliomas in the skull may show extensive distortion and the characteristic organoid or cell ball pattern may be difficult to find.
Reference(s):
– Kleihues P, Cavenee WK, Editors, Pathology and Genetics of Tumors of the Nervous System, IARC Press, Lyon, France, 2000.
