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Presented by Hind Nassar, M.D.. and prepared by ChanJuan Shi, M.D., Ph.D.
Case 5: 33 year old male with a partially cystic mass in the retropharyngeal area.
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1. Question
Week 387: Case 5
33 year old male with a partially cystic mass in the retropharyngeal areaimages/nassar033009-5a.jpg
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images/nassar033009-5e.jpgCorrect
Answer: MPNST
Histology: see below
Discussion: The tumor is an MPNST arising in a plexiform neurofibroma in a patient with a clinical history of neurofibromatosis type I. Patients with NF1 harbor a 10% lifetime risk of developing an MPNST that typically arise from a preexisting plexiform neurofibroma. In rare cases they can arise do novo in patients with NF1. These tumors have a poor overall prognosis, mainly when they are high grade.
Incorrect
Answer: MPNST
Histology: see below
Discussion: The tumor is an MPNST arising in a plexiform neurofibroma in a patient with a clinical history of neurofibromatosis type I. Patients with NF1 harbor a 10% lifetime risk of developing an MPNST that typically arise from a preexisting plexiform neurofibroma. In rare cases they can arise do novo in patients with NF1. These tumors have a poor overall prognosis, mainly when they are high grade.
