Mesenchymal chondrosarcoma of the sinonasal tract: a clinicopathological study of 13 cases with a review of the literature.
OBJECTIVE
Mesenchymal chondrosarcoma of the sinonasal tract is a rare, malignant tumor of extraskeletal origin. Isolated cases have been reported in the English literature, with no large series evaluating the clinicopathological aspects of these tumors.


METHODS
Retrospective review.


METHODS
Thirteen patients with sinonasal mesenchymal chondrosarcoma were retrieved from the Otorhinolaryngologic-Head and Neck Registry of the Armed Forces Institute of Pathology.


RESULTS
Nine women and 4 men (age range, 11 to 83 y; mean age, 38.8 y) presented with nasal obstruction (n = 8), epistaxis (n = 7), or mass effect (n = 4), or a combination of these. No patients reported prior head and neck irradiation. The maxillary sinus was the most common site of involvement (n = 9), followed by the ethmoid sinuses (n = 7) and the nasal cavity (n = 5). Tumors had an overall mean size of 5.1 cm. Microscopically, the tumors displayed a small, blue, round cell morphology appearance arranged in a hemangiopericytoma-like pattern with foci of cartilaginous matrix. All cases were managed by surgery with adjuvant radiation therapy (n = 4) and/or chemotherapy (n = 3). The overall mean survival was 12.1 years, although five of six patients who developed local recurrences died of disease (mean survival, 6.5 y). Six patients were alive and disease free (mean survival, 17.3 y), and two patients were lost to follow-up.


CONCLUSIONS
Mesenchymal chondrosarcoma of the sinonasal tract is an aggressive tumor with a predilection for young women. The pattern of growth and scarcity of cartilaginous matrix result in frequent misdiagnosis. Recurrence develops in approximately one-third of patients and seems to predict a poor prognosis. Aggressive, exenterative surgery combined with adjuvant therapy appears to yield the best clinical outcome.
Knott
P Daniel
PD
Department of Otolaryngology and Communicative Diseases, Cleveland Clinic Foundation, Ohio, USA.
Gannon
Francis H
FH
Thompson
Lester D R
LD
D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D018211:Chondrosarcoma, Mesenchymal / Q000000981:diagnostic imaging* / Q000473:pathology* / Q000628:therapy; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D008875:Middle Aged; D009367:Neoplasm Staging; D010255:Paranasal Sinus Neoplasms / Q000000981:diagnostic imaging* / Q000473:pathology* / Q000628:therapy; D012189:Retrospective Studies; D014057:Tomography, X-Ray Computed