Nasal cavity, paranasal sinuses, nasopharynx
Other tumors
Chondrosarcoma - mesenchymal

Author: Abul Ala Syed Rifat Mannan, M.D. (see Authors page)
Editor: Songyang Yuan, M.D., Ph.D.

Revised: 23 May 2018, last major update August 2014

Copyright: (c) 2004-2018, PathologyOutlines.com, Inc.

PubMed Search: Chondrosarcoma mesenchymal nasal

See also: Bone chapter
Cite this page: Mannan, A.A.S.R. Chondrosarcoma - mesenchymal. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/nasalmesenchymalchondrosarcoma.html. Accessed October 17th, 2018.
Definition / general
  • Malignant small round cell tumor with focal cartilaginous differentiation and often with a pericytomatous vascular pattern
Epidemiology
  • Mesenchymal chondrosarcoma of the sinonasal tract (MCSNT) is extremely rare and accounts for 0.07% of all benign and malignant primary sinonasal tract tumors (Cancer 1959;12:1142)
  • Affects women twice as commonly as men
  • Usually presents in third and fourth decades of life; mean age at presentation is 34.8 years
Sites
  • Within the sinonasal tract, maxillary sinus is most commonly affected, followed by ethmoid sinus and nasal cavity (Laryngoscope 2003;113:783)
Clinical features
  • Epistaxis and nasal obstruction are the most common presenting symptoms
Prognostic factors
  • High grade tumor with unpredictable prognosis
  • High rate of local recurrence
  • Hematogenous metastasis to lungs is common
Case reports
Treatment
  • Surgical resection, with or without adjuvant chemotherapy or radiotherapy remains the treatment of choice
Gross description
  • Tumor size varies from 3.2 - 9.0 cm
  • Tumor grows as irregular mucosa covered mass, with frequent extension into adjacent structures including orbit, cribriform plate, dura, palate
  • Often has a fish flesh appearance, with chalky foci of calcification
Microscopic (histologic) description
  • Bimorphic pattern typical of mesenchymal chondrosarcoma elsewhere
  • Characterized by richly cellular, undifferentiated, small mesenchymal cells frequently arranged in a pericytomatous pattern, intermixed with islands of relatively well differentiated and comparatively benign looking cartilage
  • Relative amounts of the two elements may vary
Positive stains