Quiz-summary
0 of 1 questions completed
Questions:
- 1
Information
Presented by William Westra, M.D. and prepared by Ali Ansari-Lari, M.D.,Ph.D.
Case 1: A 79 year-old woman with a large and destructive mass filling the maxillary sinus
You have already completed the quiz before. Hence you can not start it again.
Quiz is loading...
You must sign in or sign up to start the quiz.
You have to finish following quiz, to start this quiz:
Results
Time has elapsed
Categories
- Not categorized 0%
- 1
- Answered
- Review
-
Question 1 of 1
1. Question
Week 128: Case 1
A 79 year-old woman with a large and destructive mass filling the maxillary sinus/images/Bill1a.JPG
/images/Bill1b.JPG
/images/Bill1c.JPG
/images/Bill1d.JPGCorrect
Answer: Sinonasal undifferentiated carcinoma (i.e. SNUC)
Histology: The tumor demonstrates a nested and trabecular pattern of growth. The tumor cells are medium-sized and polygonal with oval nuclei and inconspicuous nucleoli. High cell turnover is evidenced by geographic zones of necrosis, brisk mitotic activity, and numerous apoptotic bodies. Notably absent is any evidence of squamous differentiation, gland formation, neurofibrillary stroma or Homer Wright rosettes. Immunohistochemical studies (not shown) show that the tumor cells are strongly immunoreactive for AE1:AE3; but they are not immunoreactive for S100, HMB45, chromogranin or synaptophysin. A special stain for mucin was also negative.
Discussion: Sinonasal undifferentiated carcinoma (SNUC) is an aggressive tumor presumably derived from the Schneiderian membrane (i.e. the surface epithelium lining the sinonasal tract). Its clinical features are dominated by its aggressive nature. At the time of diagnosis, most patients have involvement not only of the nasal cavity, but multiple paranasal sinuses as well. Secondary involvement of the nasopharynx, orbit and cranial cavity has often occurred by the time these patients come to clinical attention. Patients commonly present with visual deficits, cranial nerve palsies and proptosis. The median patient survival is less than 6 months.
SNUCs are undifferentiated neoplasms that are cytokeratin positive and often NSE positive but show no further specific differentiation at the microscopic or immunohistochemical levels. Indeed, the diagnosis of SNUC is one of exclusion and should not be rendered if there is any morphologic (e.g. squamous differentiation, glandular formations) or immunohistochemical (e.g. melanocyctic differentiation) evidence of specific tumor differentiation. Not surprisingly, the differential diagnosis of SNUC includes a large group of poorly differentiated malignant neoplasms. The entity that is perhaps most easily confused with SNUC is a high grade olfactory neuroblastoma. Some might even argue that many SNUCs actually represent esthesioneuroblastomas at one extreme end of its histologic spectrum (i.e. grade 4 esthesioneuroblastomas). Again, a diagnosis of SNUC should not be rendered if the tumor demonstrates neurofibrillary stroma, Homer Wright rossetts, and/or immunoreactivity for synaptophysin.
Incorrect
Answer: Sinonasal undifferentiated carcinoma (i.e. SNUC)
Histology: The tumor demonstrates a nested and trabecular pattern of growth. The tumor cells are medium-sized and polygonal with oval nuclei and inconspicuous nucleoli. High cell turnover is evidenced by geographic zones of necrosis, brisk mitotic activity, and numerous apoptotic bodies. Notably absent is any evidence of squamous differentiation, gland formation, neurofibrillary stroma or Homer Wright rosettes. Immunohistochemical studies (not shown) show that the tumor cells are strongly immunoreactive for AE1:AE3; but they are not immunoreactive for S100, HMB45, chromogranin or synaptophysin. A special stain for mucin was also negative.
Discussion: Sinonasal undifferentiated carcinoma (SNUC) is an aggressive tumor presumably derived from the Schneiderian membrane (i.e. the surface epithelium lining the sinonasal tract). Its clinical features are dominated by its aggressive nature. At the time of diagnosis, most patients have involvement not only of the nasal cavity, but multiple paranasal sinuses as well. Secondary involvement of the nasopharynx, orbit and cranial cavity has often occurred by the time these patients come to clinical attention. Patients commonly present with visual deficits, cranial nerve palsies and proptosis. The median patient survival is less than 6 months.
SNUCs are undifferentiated neoplasms that are cytokeratin positive and often NSE positive but show no further specific differentiation at the microscopic or immunohistochemical levels. Indeed, the diagnosis of SNUC is one of exclusion and should not be rendered if there is any morphologic (e.g. squamous differentiation, glandular formations) or immunohistochemical (e.g. melanocyctic differentiation) evidence of specific tumor differentiation. Not surprisingly, the differential diagnosis of SNUC includes a large group of poorly differentiated malignant neoplasms. The entity that is perhaps most easily confused with SNUC is a high grade olfactory neuroblastoma. Some might even argue that many SNUCs actually represent esthesioneuroblastomas at one extreme end of its histologic spectrum (i.e. grade 4 esthesioneuroblastomas). Again, a diagnosis of SNUC should not be rendered if the tumor demonstrates neurofibrillary stroma, Homer Wright rossetts, and/or immunoreactivity for synaptophysin.
