Quiz-summary
0 of 1 questions completed
Questions:
- 1
Information
Presented by George Netto, MD and prepared by Shien Micchelli, M.D.
Case 2: A 37 year old white man presented with persistent nasal obstruction and recurrent epistaxis.
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 258: Case 2
A 37 year old white man presented with persistent nasal obstruction and recurrent epistaxis. On imaging studies, he was found to have a 5 cm left nasopharyngeal mass.images/2_27_06_2a.jpg
images/2_27_06_2b.jpg
images/2_27_06_2c.jpg
images/2_27_06_2d.jpg
images/2_27_06_2e.jpgCorrect
Answer: Juvenile Nasopharyngeal angiofibroma
Histology: The tumor is characterized by the presence of a densly collagenized, paucicellular stroma separating variable sized thin walled vascular structures. Toward the periphery of the lesion a slight increase in stromal cellularity is imparted by a population of bland stellate shaped cells. The vessels range from slit like structures devoid of muscular wall to larger dialated vessels with thin muscular cuff. The stromal cells lack cytologic atypia and mitotic activity.
Discussion: For all practical considerations, juvenile nasopharyngeal angiofibroma (JNA) is a tumor of the male gender primarily occurring in adolescent boys. As is the case in our patient, JNA can occasionally be encountered in middle aged adults. JNA is thought to be a testosteron driven neoplasm given the presence of testosterone receptors in stromal and endothelial cell as shown by immunohistochemistry. The stromal cells are fibroblastic/myofibroblastic in nature and can occasionally demonstrate multinucleation and “ganglion-like” appearance.
JNA’s have an overall favorable prognosis. The exception being cases where extension into surrounding structures (eg. orbital fossa and cranium) prohibits a complete resection. It remains to be seen whether recent suggestions relating such locally aggressive behavior to alterations in C-MYC protoncogene can be validated.
The increased frequency of JNA in male patients with Familial Adenomatous Polyposis (FAP) syndrome has pointed to a potential pathogenic role of APC/Beta-Catenin. Indeed, activating Beta-Catenin mutations have been shown in several sporadic cases of JNA. Finally, the recent demonstration of a simultaneous germline and somatic frameshift mutation of APC gene in a JNC patient from an FAP family further establish JNA as an integral FAP tumor.
Reference(s):
– Schick B, Wemmert S, Jung V, Steudel WI, Montenarh M, Urbschat S. Genetic heterogeneity of the MYC oncogene in advanced juvenile angiofibromas. Cancer Genet Cytogenet. 2006;164(1):25-31.
– Valanzano R, Curia MC, Aceto G, Veschi S, De Lellis L, Catalano T, La Rocca G, Battista P, Cama A, Tonelli F, Mariani-Costantini R. Genetic evidence that juvenile nasopharyngeal angiofibroma is an integral FAP tumour. Gut. 2005;54(7):1046-7.
– Abraham SC, Montgomery EA, Giardiello FM, Wu TT. Frequent beta-catenin mutations in juvenile nasopharyngeal angiofibromas. Am J Pathol. 2001;158(3):1073-8.Incorrect
Answer: Juvenile Nasopharyngeal angiofibroma
Histology: The tumor is characterized by the presence of a densly collagenized, paucicellular stroma separating variable sized thin walled vascular structures. Toward the periphery of the lesion a slight increase in stromal cellularity is imparted by a population of bland stellate shaped cells. The vessels range from slit like structures devoid of muscular wall to larger dialated vessels with thin muscular cuff. The stromal cells lack cytologic atypia and mitotic activity.
Discussion: For all practical considerations, juvenile nasopharyngeal angiofibroma (JNA) is a tumor of the male gender primarily occurring in adolescent boys. As is the case in our patient, JNA can occasionally be encountered in middle aged adults. JNA is thought to be a testosteron driven neoplasm given the presence of testosterone receptors in stromal and endothelial cell as shown by immunohistochemistry. The stromal cells are fibroblastic/myofibroblastic in nature and can occasionally demonstrate multinucleation and “ganglion-like” appearance.
JNA’s have an overall favorable prognosis. The exception being cases where extension into surrounding structures (eg. orbital fossa and cranium) prohibits a complete resection. It remains to be seen whether recent suggestions relating such locally aggressive behavior to alterations in C-MYC protoncogene can be validated.
The increased frequency of JNA in male patients with Familial Adenomatous Polyposis (FAP) syndrome has pointed to a potential pathogenic role of APC/Beta-Catenin. Indeed, activating Beta-Catenin mutations have been shown in several sporadic cases of JNA. Finally, the recent demonstration of a simultaneous germline and somatic frameshift mutation of APC gene in a JNC patient from an FAP family further establish JNA as an integral FAP tumor.
Reference(s):
– Schick B, Wemmert S, Jung V, Steudel WI, Montenarh M, Urbschat S. Genetic heterogeneity of the MYC oncogene in advanced juvenile angiofibromas. Cancer Genet Cytogenet. 2006;164(1):25-31.
– Valanzano R, Curia MC, Aceto G, Veschi S, De Lellis L, Catalano T, La Rocca G, Battista P, Cama A, Tonelli F, Mariani-Costantini R. Genetic evidence that juvenile nasopharyngeal angiofibroma is an integral FAP tumour. Gut. 2005;54(7):1046-7.
– Abraham SC, Montgomery EA, Giardiello FM, Wu TT. Frequent beta-catenin mutations in juvenile nasopharyngeal angiofibromas. Am J Pathol. 2001;158(3):1073-8.
