Mesenchymal Chondrosarcoma of the Sinonasal Tract: A Clinicopathological Study of 13 Cases With a Review of the Literature

Authors

  • P. Daniel Knott MD,

    1. Department of Otolaryngology and Communicative Diseases, Cleveland Clinic Foundation, Cleveland, Ohio
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  • Francis H. Gannon MD,

    1. Department of Orthopedic Pathology, Armed Forces Institute of Pathology, Washington, DC, U.S.A.
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  • Lester D. R. Thompson MD

    Corresponding author
    1. Department of Endocrine and Otorhinolaryngologic–Head and Neck Pathology, Armed Forces Institute of Pathology, Washington, DC, U.S.A.
    • Lester D. R. Thompson, MD, Department of Endocrine and Otorhinolaryngologic—Head and Neck Pathology, Building 54, Room G066-11, Armed Forces Institute of Pathology, 6825 16th Street Northwest, Washington, DC 20306-6000, U.S.A.
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  • The opinion and assertions expressed herein are the private views of the authors and are not to be construed as official or as representing the views of the Department of Defense.

Abstract

Objectives/Hypothesis 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.

Study Design 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.

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