Low-grade sinonasal sarcoma with neural and myogenic features: a clinicopathologic analysis of 28 cases.
Sarcomas of the sinonasal region are rare. We describe a distinct spindle cell sarcoma of the sinonasal region characterized by concomitant neural and myogenic differentiation. Consultation files and surgical cases from Mayo Clinic were reviewed. Twenty-eight cases were identified that met the inclusion criteria. Clinical data were collected from medical records, consultation letters, and referring pathologists. Reverse transcriptase-polymerase chain reaction for synovial sarcoma fusion transcripts was performed on 18 cases. Cytogenetic studies were performed on 2 cases. The 21 female and 7 male patients ranged in age from 24 to 85 years (mean, 52 y). All cases showed a characteristic histology, which included a cellular spindle cell neoplasm with uniform, elongate nuclei and an infiltrative growth pattern. All tumors demonstrated expression of S-100 with actin positivity in 96% of cases tested. Reverse transcriptase-polymerase chain reaction for synovial sarcoma fusion transcripts was negative in all cases tested. Cytogenetic studies conducted on 2 cases demonstrated the chromosomal translocation t(2;4). The nasal cavity (54%) and ethmoid sinus (57%) were the most commonly involved areas, singly or in combination. Follow-up information was available for 57% (16/28) of cases, with a mean of 8.3 years. Of these, 44% (7/16) experienced at least 1 recurrence. No patient has developed metastases or died of disease. We describe a unique tumor with a characteristic morphologic, immunophenotypic, and cytogenetic profile. On the basis of the locally aggressive nature of this lesion we believe it is best considered a low-grade sarcoma and suggest the term low-grade sinonasal sarcoma with neural and myogenic features.
Department of Laboratory Medicine, Mayo Clinic, Rochester, MN 55905, USA. lewis.jason@mayo.edu
Jason T
JT
Lewis
Andre M
AM
Oliveira
Antonio G
AG
Nascimento
David
D
Schembri-Wismayer
Eric A
EA
Moore
Kerry D
KD
Olsen
Joaquin G
JG
Garcia
Melissa L
ML
Lonzo
Jean E
JE
Lewis
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D014408:Biomarkers, Tumor / Q000378:metabolism; D002467:Cell Nucleus / Q000648:ultrastructure; D002889:Chromosomes, Human, Pair 2; D002894:Chromosomes, Human, Pair 4; D020732:Cytogenetic Analysis; D003593:Cytoplasm / Q000648:ultrastructure; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009669:Nose Neoplasms / Q000235:genetics / Q000378:metabolism / Q000473:pathology* / Q000601:surgery; D010255:Paranasal Sinus Neoplasms / Q000235:genetics / Q000378:metabolism / Q000473:pathology* / Q000601:surgery; D020133:Reverse Transcriptase Polymerase Chain Reaction; D012509:Sarcoma / Q000235:genetics / Q000378:metabolism / Q000473:pathology* / Q000601:surgery; D014178:Translocation, Genetic; D016896:Treatment Outcome; D055815:Young Adult
D014408:Biomarkers, Tumor