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CHONDROSARCOMA OF THE NASAL SEPTUM Arif AlP, Milind Gosavi 2, Rajiv MichaeP, John Mathew 4, Mary Kurien s, Anila Korula 6
Keywords : Chondrosarcoma, nasal septnm, wide surgical excision
INTRODUCTION Chondrosarcoma are slowly growing malignant tumours of cartilaginous tissue. These turnouts usually involve pelvis, ribs, and long bones of extremities, scapula and sternum with only 5% to 10% in the head and neck region. ~ Chondrosarcoma of the nasal septurg reported in literature had extensive involvement of paranasal sinuses and skull
base at the time of diagnosis) ,2,3 Limited involvement of the nasal cavity was reported in only one patient? We present a case of nasal septal chondrosarcoma involving the nasal cavity alone with no evidence of extension or destruction of adjacent structures.
~,2Registrar, 3Lecturer, 4professor, 5Professor & Head, Department of ENT, Speech and Hearing, 6professor, Department of Pathology, Christian Medical College and Hospital, Vellore, Tamilnadu, India.
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Chondrosarcoma of the Nasal Septum i el
CASE REPORT A 26-year-old female patient presented to our hospital with complaints of persistent bilateral nasal obstruction and bifrontal headache of 2 years duration, with alteration of shape of nose of 6 months duration. There was no history of epistaxis or visual disturbance. Nasal endoscopy revealed firm smooth surfaced reddish mass arising from the septum filling both nasal cavities. Caudal part of septum was intact. P o s t e r i o r r h i n o s c o p y s h o w e d no extension into the nasopharynx. There was no palpable neck node and the rest of the examinations were within normal limits. CT scan revealed mass lesion with flakes of calcification centered on nasal septum extending to both nasal cavity with expansion and thinning of the medial wall of both maxillary antra with no involvement. Cribriform plate and orbital margins were intact. Multiple biopsies under local anaesthesia consisted of mature chondrocytes in a cartilaginous matrix with no evidence of atypia. Hence she underwent excision of the mass by mid-facial degloving approach. A large friable mass was found arising from the septum occupying the entire nasal cavity. Tumor was removed in toto. The thinned out medial wall of the both maxillary antra were removed. The mucosa of the m a x i l l a r y and e t h m o i d sinuses were normal. Postoperative period was uneventful. Final H i s t o p a t h o l o g i c a l e x a m i n a t i o n of the excised specimen showed sections of tumor composed of lobules of neoplastic chondrocytes set in a cartilaginous matrix with foci of marked nuclear atypia, nuclear enlargement with
Fig 1: Microphotograph showing chondrosarcoma comprising chondrocytes with hyperchromatic nuclei displaying mitoses and binucleation. (H&E X 200)
nucleoli, h y p e r c h r o m a t i s m , o c c a s i o n a l mitosis and multinucleation, consistent with well differentiated chondrosarcoma (Fig. I). Three years follow up after surgery shows no evidence of tumor recurrence. DISCUSSION Chondrosarcoma are uncommon tumours. Approximately 5-10% are located in the head and neck region. L,2 The site of predilection for chondrogenic tumours in head and neck region include Ethmoid sinus (50%), Maxilla (18%), Nasal septum (17%), Hard palate and Nasopharynx (6%), and Alar cartilage (3%). 4 These malignant tumours arise from primitive mesenchymal stem cells or may also arise from tissue not ordinarily containing cartilage (dura, falx, maxilla) because of multidirectional differentiation of mesenchymal cell.l The histological criteria for the proper diagnosis of this neoplasm is the presence of many cells with hyperchromatic nuclei, two or more such chondrocytes within a lacuna and giant cartilage cells with large single or multiple nuclei with clumps of chromatin and mitotic activity.These grades are distinguished on the basis of mitotic rate, cellularity and nuclear size of chondrocytes in skeletal chondrosarcoma. 5 Gallagher and Strome in 1972 stressed the need of several intranasal biopsies to confirm the diagnosis of sarcoma. 6 Chondrosarcomas tend to run a slow progressive course and displace local structures before invading them. Those in the facial region metastasise infrequently. 7,8 The treatment of choice is radical surgical excision. R a d i o t h e r a p y or c h e m o t h e r a p y is usually r e c o m m e n d e d for adjuvant treatment, residual or recurrent disease and palliation. 3 The prognosis depends on the location and extent of the tumor, adequacy of treatment and the degree of differentiation. The posterior nasal cavity, nasopharynx and sphenoid sinus are the sites with poor prognosis as tumours are already large when detected and invade the base of skull. Five-year survival rate for all stages combined is between 54% and 81% in the recent series, but disease free rate is lower. Distant metastases occur in 18% patients and usually involve lungs. 3 Life long follow up is mandatory for chondrosarcoma patients of head and neck region. 3,s CONCLUSION Though clinically and radiologically limited with multiple biopsies negative for m a l i g n a n c y , the possibility of
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sarcomatous changes in lesions of the nasal septum must be considered. Primary wide excision with adequate surgical margins must be the treatment of choice.
5.
Gallagher TM, Strome M (1972): Chondrosarcoma of the facial region. Laryngoscope 82:978-984.
6.
Myers EM, Thawley SE (1979): Maxillary chondrosarcoma. Arch Otolaryngol 105:116-118.
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Dr. Mary Kurien, Professor & Head, Department of ENT, Speech and Hearing Christian Medical College and Hospital, Vellore, Tamilnadu, India Telephone : 91 416 222102/262603 (Ext: 2798) Fax : 9l 416 232035/232103 E-mail : kurien
[email protected] ent2 @cmcvellore.ac.in
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Indian Journal of Otolaryngology and Head and Neck Surgery, Vol. 56, No. 4, October - December, 2004
