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Resident's Page: Imaging
January 1998

Imaging Quiz Case 2

Author Affiliations
 

R. NICKBRYANMDS. JAMESZINREICHMD

Arch Otolaryngol Head Neck Surg. 1998;124(1):111. doi:10.1001/archotol.124.1.108

Malignant PNSTs make up approximately 10% of all soft tissue sarcomas.1 Similar to other soft tissue sarcomas, they tend to recur locally and spread hematogenously, with lymph node metastasis being rare.2 There is a strong association of these tumors with neurofibromatosis type 1 (NF-1, von Recklinghausen neurofibromatosis).3 In addition, 10% of cases occur in patients with occupational or therapeutic radiation exposure.4 Despite aggressive treatment of these tumors with surgery and adjuvant therapy, the overall prognosis remains poor. Survival is related to tumor size, location, and association with NF-1.5 In sporadic cases of malignant PNST, the 5-year survival rate is 50% to 75%. Patients with NF-1 have a worse prognosis, with a 15% to 30% 5-year survival rate.1

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