|Year : 1993 | Volume
| Issue : 4 | Page : 189-191
Nasal septal chondrosarcoma with visual loss
K Sharma, A Kaur, HC Taneja, I Tyagi, R Pandey
Department of Neurosurgery and Pathology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, India
Dept. of Neurosurgery, Sanjay Gandhi Post Graduate Institute of MedicalSciences,Post Box No. 375, Rai Bareli Road, Lucknow
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Sharma K, Kaur A, Taneja H C, Tyagi I, Pandey R. Nasal septal chondrosarcoma with visual loss. Indian J Ophthalmol 1993;41:189-91
|How to cite this URL:|
Sharma K, Kaur A, Taneja H C, Tyagi I, Pandey R. Nasal septal chondrosarcoma with visual loss. Indian J Ophthalmol [serial online] 1993 [cited 2020 May 30];41:189-91. Available from: http://www.ijo.in/text.asp?1993/41/4/189/25593
Chondrosarcoma of faciomaxillary area is an uncommon entity. Primary chondrosarcomas of nasal and paranasal sinus region including nasal septum rarely extend to cranial or intracranial areas unless there is recurrence. , In 1974, Fu and Perzin  reported 25 cases of primary chondrosarcoma of nasal and para nasal sinus region, to which Randall and Gray  added 8 more cases a decade later. In primary tumours, visual impairment is a rare presenting symptom.' We report a case of primary chondrosarcoma of nasal septum extending to the base of anterior cranial fossa with loss of vision in the right eye.
| Case report|| |
A 45-year-old man presented with a two-year history of recurrent bilateral nasal blockade, a six-month history of frequent frontal headache, and a one-month history of loss of vision in the right eye. About six months earlier, he was operated elsewhere for nasal blockade with no significant relief. No detailed information was available. His general and systemic examination was normal. Neurological examination revealed complete bilateral anosmia and blind right eye with primary optic atrophy. Right eye had mild proptosis with exotropia and restricted adduction. The left eye was normal. No other neurological deficit was noticed. Anterior rhinoscopy revealed presence of mucopurulent discharge and dirty fleshy mass blocking both nasal cavities. Posterior rhinoscopy revealed a mass obstructing the nasopharynx. No feature of raised intracranial pressure was found. His haematological and biochemical investigations were within normal limits. Plain X-ray of the paranasal sinuses revealed radioopaque shadow suggestive of a mass in both nasal cavities, right maxillary ethmoid and sinuses. Computed tomographic scan showed nonenhancing isodense mass filling the nasal cavity, ethmoid and maxillary sinus. Superiorly, the mass was extending extradurally upto the floor of anterior cranial fossa, reaching to the sella posteriorly. Destruction of the medial wall of both orbits was present with displacement of right orbital contents laterally [Figure - 1].
Right lateral rhinotomy was performed under general anaesthesia. A firm waxy white tumour was present in both the nasal cavities. It was attached to nasal septum filling the nasopharynx, compressing the medial wall of the right maxilla more than the left and extending into the right orbit. The right eyeball was displaced laterally. The medial wall of right optic canal was eroded and the tumour was pressing the right optic nerve. A part of the tumour was adherent to the base of anterior cranial fossa. Complete removal of the tumour was done. Histopathology revealed lobules of chondromatous tissue at places lined by nasal mucosa. Few lobules were relatively acellular and resembled mature cartilage, while others showed marked increase in cellularity with large chondrocytes having enlarged multinucleated and binucleated cells. Stroma consisted of collagen strands having congested blood vessels. Thus, a diagnosis of well-differentiated chondrosarcoma was made [Figure - 2].
Postoperative period was uneventful. His ocular movements improved completely. Vision and olfaction were same as preoperative status. Radiotherapy was given in the immediate postoperative period. A CT scan after 3 months of radiotherapy showed minimal residual tumour at lateral and posterosuperior periphery [Figure - 3]. Clinicoradiological follow-up of two years did not reveal any evidence of tumour growth [Figure - 4].
| Discussion|| |
Chondrosarcomas constitute an uncommon group of cartilaginous tumours.  In the face they commonly involve nasal cavity, para nasal sinuses, nasopharynx, larynx, and mandible.  The origin of chondrosarcomas from nasal septum is a relatively rare occurrence.  Chondrosarcoma arises from primitive mesenchymal stem cell or may arise from any tissue of cartilaginous nature having chondroblasts.
The usual age of presentation varies between 40 and 50 years with a higher incidence in men. , Faciomaxillary chondrosarcomas appear earlier than the tumours at other sites. 
Chondrosarcomas of face usually do not involve the intracranial structures primarily. Intracranial infiltration is a feature of recurrence of lesion.  Paddison and Hanks  reported a case of maxillary tumour which extended into nasal cavity, mouth, and floor of anterior cranial fossa but there was no visual loss. A review of the literature for proven cases of chondrosarcomas of nasal and paranasal sinus region, reveals that the symptomatology varies according to the site and size of tumour. Usually, nasal obstruction, nasal discharge, facial asymmetry, headache, restricted ocular movements, diplopia, proptosis, facial pain, nasal and ear fullness are presenting signs and symptoms in primary cases ,, Recurrence of lesion with intracranial involvement is characterized by visual disturbances, loss of vision, proptosis and multiple cranial nerves involvement. ,, But in our case, visual involvement was an early feature in a primary variety of tumour.
CT scan in coronal and sagittal plain is the most informative investigation in evaluating the extent and exact location of the tumour. Magnetic Resonance Imaging is equally helpful, if available. While bony destruction, calcification with isodense or hypodense nonenhancing mass, are common findings on CT scan,  calcification was not seen in our patient. Biopsy of the lesion is the only way to confirm the diagnosis. Histopathologically, atypical cells are diagnostic.
So far, no definite treatment protocol has been adopted for these tumours. Radical surgical resection is the treatment of choice. However, incomplete removal heralds early recurrence. Though chondrosarcomas are radioresistant, regression has been reported after radiotherapy. , Ryall et al have used chemotherapy along with radiotherapy in the treatment of chondrosarcomas with reportedly better results.
Overall, result of a single treatment modality is unsatisfactory. Coats et alb reported that 7 of 13 patients of facial chondrosarcoma showed better results after radical surgical resection and radiotherapy was used for palliation after recurrence. In our patient, surgery with radiotherapy has shown a significant recurrencefree interval of two years. In our opinion, radical resection is the treatment of choice for these tumours and that, patients should undergo radiotherapy soon after radical surgery to achieve better outcome.
| References|| |
Wagas S, Tochio H, Shiro Yamagiwa M,and Nishioka,H. Chondrosarcoma of ethmoid sinus extending to anterior fossa. Surg Neurol. 16:324-328, 1981.
Berkmen YM and Blatt ES. Cranial and intracranial carti laginous tumours. Clin Radiol. 19:327-333, 1968.
Randall CJ and Gray R. Nasal septal chondrosarcomas. A long term follow-up report. J Laryngolol Otolol. 82:635638, 1984.
Fu YS and Perzin KH. Non epithelial tumours of nasal cavity, paranasal sinuses, and nasopharynx. Clinico pathological study No. 3: Cartilaginous, tumor, Chondroma: Chondrosarcoma. Cancer. 34:453-463, 1974.
Cianfriglia F, Pompilli A, and Occhipinti E. Intracranial malignant cartilaginous tumours. Report of two cases and review of literature. Acta Neurochirurgica. 45:163175, 1978.
Gallagher IN and Strome M. Chondrosarcoma of facial region. Laryngoscope. 82:978-984, 1972.
Paddison GM and Hanks GE. Chondrosarcoma of maxilla. Report of a case responding to supervoltage irradiation and review of literature. Cancer. 28:616-619, 1971.
Coates HL, Pearson BW, Devine KD, and Unni KK. Chondro sarcoma of nasal cavity, paranasal sinuses and nasopharynx. Trans Am Acad Ophthalmol-Otolaryngol. 84:919-926, 1977.
Lichtenstein L and Juffe HL. Chondrosarcomas of bone. Am J Pathol. 19:553-89, 1943.
Mankin HJ, Cantley KP, Lippellio L, Schiller AL, and Campbell CJ. The biology of human chondrosarcomas. 1.Description of cases grading, and biochemical analyses. J Bone Joint Surg. 62:160-176, 1980.
Ryall RDH, Bates T, Newton KA, and Hellmann K. Combination of radiotherapy and Razoxane (ICRF) for chondrosarcoma Cancer. 44:891-895, 1979.
[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4]