La tunisie Medicale - 2010 ; Vol 88 ( n°02 ) : 122-124
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Résumé

Prérequis : Les chondrosarcomes sont des tumeurs malignes d’origine cartilagineuse à évolution lente. Ces tumeurs peuvent siéger au niveau de la tête et du cou particulièrement au niveau du massif facial avec atteinte préférentielle de la mandibule et du maxillaire. La localisation naso-sinusienne est très rare.
Observation : Nous rapportons le cas d’un homme âgé de 43 ans chez qui on découvert accidentellement un chondrosarcome nasoethmoïdo- maxillaire. Le patient a été opéré par voie endonasale sous guidage endoscopique. Une radiothérapie complémentaire a été réalisée avec une bonne évolution et une absence de récidive à 5 ans de recul.
Conclusion : La chirurgie constitue le traitement de choix des chondrosarcomes. La chirurgie endonasale peut garantir une résection tumorale complète dans des cas bien sélectionnés.

Mots Clés
Article

Chondrosarcoma is a rare, slow-growing tumor that usually arises from cartilaginous structures.
Chondrosarcomas make up only 10–20% of malignant primary bone tumors and are usually found in the pelvis, long bones, and ribs, with only 5 - 10% located in the head and neck, most commonly in the larynx, maxilla, mandible, and skull base. Chondrosarcoma of the sinonasal tract is very rare.

CASE REPORT


A 43-year-old man with no significant medical history presented a 12-month history of right-sided sinus congestion associated with clear rhinorrhea without epistaxis. Nasal endoscopic exam showed polypoid mass of the right nasal cavity. The patient was referred to radiology and a CT of the sinuses was obtained.
Unenhanced computed tomography (CT) examination of the sinuses (fig 1 and 2) showed an expansive, polypoid soft tissue mass arising in the right nasal cavity, ethmoid cells and right maxillary sinus. There was no bone erosion of the skull base and no extension into the intracranial space.

Figure 1 & 2 :
coronal and axial CT scan showing tissue mass arising in the right nasal cavity, ethmoid cells and right maxillary sinus



ethmoidectomy, resection of the lateral wall of nasal cavity and middle turbinectomy. The final pathological diagnosis was grade 3 chondrosarcoma. Treatment has followed by a radiation therapy (64 Gy). MRI was performed at the end of treatment and 3 years post operatively showing no recurrence (fig 3 and 4). The patient was considered free of disease at her 5 years follow-up.

Figure 3 & 4 :
MRI T2 and T1 with gadolium showing the vacuity of sinonsal tract with no reccurence of the tumor.




DISCUSSION

Chondrosarcoma comprises approximately 8% of primary bone tumors overall. Less than 10% of chondrosarcomas are found in the head and neck region (1, 2).
Originally identified in the literature by Lichtenstein and Bernstein in 1959 as a biphasic tumor comprised of spindle cell mesenchyme interspersed with areas of chondroid differentiation (1).
The tumor is more commonly seen in a younger subset of the population than the conventional chondrosarcoma, with fewer than 20% of the 111 cases reviewed by Nakashima, arising in patients over 40 years of age (2). A unique feature of this tumor is its ability to arise in either bone or soft tissue, with a predilection for skeletal origin of approximately two-to-one. Common skeletal sites in order of occurrence include the craniofacial bones (particularly the jaws) and spine while the meninges and lower extremity each comprise over a quarter of the extraskeletal cases (3, 4). Chondrosarcoma of the sinonasal tract is very rare (5, 8, 9). Patients may present with symptoms of nasal obstruction or discharge, chronic sinusitis, epistaxis, facial asymmetry, headache, restricted ocular movement, diplopia, proptosis, facial pain, dentition changes, or nasal/aural fullness (2-5).
Chondrosarcomas are graded on a 1 to 3 scale corresponding to a histological classification from a well-differentiated to undifferentiated tumor, based on the rate of mitoses, cellularity, and nuclear size (6). Grade 1 chondrosarcomas have abundant chondroid matrix, clusters of chondrocytes with normal or
slightly enlarged nuclei, rare nucleoli, absent mitoses, and occasional binucleation. Grade 2 chondrosarcomas appear to have less chondroid matrix and more chondrocytes than grade 1 tumors, presence of rare mitoses, slightly enlarged vesicular hyperchromatic nuclei, and multinucleation. Grade 3 chondrosarcomas display a myxoid matrix with irregularly shaped chondrocytes and increased cellularity, nuclear pleomorphism, and mitoses compared with the lowgrade chondrosarcomas (6).
The clinical and radiologic differential diagnosis includes chondroma, meningioma, osteomas, osteosarcomas, fibroosseous lesions, and chordoma (3, 7).
Diagnostic modalities by both CT and MRI are essential in preoperative planning. On CT, chondrosarcomas typically appear as a hypodense matrix with scattered small rings forming calcifications and bone erosion. MRI typically demonstrates a low-intensity T1 imaging, high-intensity T2 imaging, and intermediate enhancement with T1 with gadolium (5, 8, 9, 10).Surgical excision with wide surgical margins is the mainstay treatment. Surgical extirpation can be completed by using a variety of approaches such as lateral rhinotomy with or without medial maxillectomy, the transpalatal approach, Le Fort I osteotomy, and transnasal endoscopic approaches (11, 12, 13).The endoscopic approach avoids facial and oral incisions and produces the least morbidity.
Its use is limited by the access provided by the nasal cavity and paranasal sinuses, the vascularity, size, and extension of the tumor, and the availability of the special instrumentation (14). Similar to the treatment of other sarcomas, postoperative radiation therapy has been standard practice for mesenchymal chondrosarcoma.
Regional and distant metastasis are rare. The clinical course can be extremely protracted with relapses having occurred greater than 20 years after treatment of the primary tumor, making long-term follow-up a must in these patients (15).
In the most recent review of the literature on chondrosarcoma of the head and neck, 5-year survival rates for all histologic grades ranged from 54% to 81% (7, 9, 14).

CONCLUSION


This case report shows the feasibility of using endoscopic techniques to aid in the resection of selected malignant tumors of the nose. Although we do not advocate the use of endoscopic approaches for all malignant tumors, tumors that are limited in size and fully visualized by endoscopic techniques are amenable to endoscopic resection.

Références
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  2. Nakashima Y, Unni KK, Shives TC, Swee RG, Dahlin DC. Mesenchymal chondrosarcoma of bone and soft tissue. A review of 111 cases. Cancer 1986; 57: 2444-53.
  3. Ly J. Mesenchymal chondrosarcoma of the maxilla. AJR 2002; 179: 1077-8.
  4. Knott PD, Gannon FH, Thompson LDR. Mesenchymal chondrosarcoma of the sinonasal tract:a clinicopathological study of 13 cases with a review of the literature. Laryngoscope 2003; 113: 783-90.
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  7. Dahlin DC, Henderson ED. Mesenchymal chondrosarcoma: further observations in a new entity. Cancer 1962; 15: 410-7.
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  9. Aziz SR, Miremadi AR, McCab JC. Mesenchymal chondrosarcoma of the maxilla with diffuse metastasis: case report and literature review. J Oral Maxillofac Surg 2002; 60: 931-5.
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  12. Rassekh CH, Nuss DW, Kapadia SB, Curtin HD, Weissman JL, Janecka IP. Chondrosarcoma of the nasal septum: skull base imaging and clinicopathologic correlation. Otolaryngol Head Neck Surg 1996; 115: 29- 37.
  13. Ruark DS, Schlehaider UK, Shah JP. Chondrosarcomas of the head and neck. World J Surg 1992; 16: 1010-6.
  14. Ricardo L. Carrau, MD, FACS, Barlas Aydogan, MD, and Jennifer L. Hunt, MD. Chondrosarcoma of the sphenoid sinus resected by an Endoscopic Approach. American Journal of Otolaryngology, 2004; 25 : 274-277.
  15. Phillip K. Pellitteri a, Alfio Ferlito b, Johannes J. Fagan c, Carlos Suarez de, Kenneth O. Devaney f, Alessandra Rinaldo. Mesenchymal chondrosarcoma of the head and neck. Oral Oncology 2007; 43: 970- 975.
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