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 Table of Contents  
LETTER TO THE EDITOR
Year : 2013  |  Volume : 9  |  Issue : 1  |  Page : 163-164

Paediatric chondrosarcoma of the sinonasal region


1 Department of Surgical Oncology, Cancer Institute (WIA), Adyar, Chennai, India
2 Department of Pathology, Cancer Institute (WIA), Adyar, Chennai, India

Date of Web Publication10-Apr-2013

Correspondence Address:
Arvind Krishnamurthy
Department of Surgical Oncology, Cancer Institute (WIA), Sardar Patel Rd, Adyar, Chennai
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0973-1482.110356

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How to cite this article:
Krishnamurthy A, Gowthaman S, Majhi U. Paediatric chondrosarcoma of the sinonasal region. J Can Res Ther 2013;9:163-4

How to cite this URL:
Krishnamurthy A, Gowthaman S, Majhi U. Paediatric chondrosarcoma of the sinonasal region. J Can Res Ther [serial online] 2013 [cited 2019 Sep 17];9:163-4. Available from: http://www.cancerjournal.net/text.asp?2013/9/1/163/110356

Sir,

Chondrosarcomas are a heterogeneous group of malignant mesenchymal tumors derived from a cartilaginous origin. These tumors usually involve the pelvis and long bones. Less than 10% of the chondrosarcomas involve the craniofacial region, accounting for less than 2% of all head and neck tumors. [1] A majority of the chondrosarcomas present between the 5 th and 7 th decades although they can occur at any age; only about 14 cases of pediatric sinonasal chondrosarcoma shave been reported. [1],[2],[3] We present an additional case of a pediatric sinonasal chondrosarcoma; this case is elegantly illustrative of its clinical, radiological, and histological presentation.

A 6-year-old female child presented with prominence of her left eye and nasal obstruction of one month duration. Examination revealed proptosis of the left eye, caused due to a painless fleshy mass protruding in the left nasal cavity, a biopsy of which was suggestive of a chondrosarcoma. CT scan of paranasal sinuses showed a well-circumscribed hypodense mass in left naso-ethmoidal region with multiple specks of curvilinear calcifications, bone destruction of the medial wall of the left orbit with extension into maxillary sinuses and abutting the anterior skull base. [Figure 1]a, b CT scan of the chest was normal. She underwent an uneventful wide excision of the mass by an anterior craniofacial approach [Figure 2]. The resected specimen showed a 3 × 3 × 2cm tumor with microscopic features, suggestive of a chondrosarcoma grade 2 [Figure 3]. She was considered for adjuvant radiation in view of the intermediate histological grading (Grade 2) and close margins; she received external beam radiotherapy 50 Grey to the tumor bed. The patient is on regular follow up and remains disease-free for over 6 months.
Figure 1: (a). Axial section, (b)- Coronal section: CT scan of paranasal sinuses showed a well-circumscribed 2.9 × 2.4 × 2.0cmhypodense mass in left naso-ethmoidal region with multiple specks of curvilinear calcification and bone destruction, features highly suggestive of a chondrosarcoma

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Figure 2: Intra-operative photograph following wide excision of the tumor by an anterior craniofacial approach

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Figure 3: (H and E, 20×) Section shows a cartilaginous tumor with disorganized hypercellular hyaline cartilage with increasednumber ofbinucleated cells on one half and hyper cellular area on the other half with myxoid change. The cells are large, round, with central vesicular nuclei and prominent eosinophilic nucleoli. Mitotic activity is not well made out, no spindling is seen. The features are suggestive of chondrosarcoma Grade II

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The epidemiologic risk factors for cranio-facial chondrosarcomas remain poorly defined because of their rarity. These tumors commonly present as a painless mass that progress to symptoms such as nasal obstruction, vision, and dental abnormalities. Radical surgery with clear margins is the most effective treatment for chondrosarcomas and should be considered in all potentially operable cases. [4] The roles of chemotherapy and radiation therapy in cases of chondrosarcoma are unclear; the disease is generally considered insensitive to these treatments. Radiotherapy has, however, been used in either alone or in combination following surgery, especially in incompletely excised tumors or when located in surgically inaccessible regions, irradiation with protons or other charged particles seems beneficial in these situations. [5] The consensus therapy for base skull chondrosarcomas includes surgical resection and adjuvant radiotherapy. [5] The prognosis of these tumors is generally good; the tumor grade and complete surgical respectability are the most relevant clinical prognostic factors in patients with chondrosarcomas. This case reaffirms the importance of considering sarcomas or other neoplastic lesions in the differential diagnosis of progressive nasal obstruction and proptosis in children.

 
 > References Top

1.Gadwal SR, Famburg-Smith JC, Gannon FH.Thompson LD. Primary chondrosarcoma of the head and neck in pediatric patients. Cancer 2000;88:2180-8.  Back to cited text no. 1
    
2.Jörg S, August C, Stoll W, Alberty J. Myxoidchondrosarcoma of the maxilla in a pediatric patient. Eur Arch Otorhinolaryngol 2006;263:195-8.  Back to cited text no. 2
    
3.Kim YJ, Im SA, Lim GY, Chun HJ, Park HJ, Kim MS, et al. Myxoidchondrosarcomaof the sinonasal cavity in a child: Acase report. Korean J Radiol 2007;8:452-5.  Back to cited text no. 3
    
4.Riedel RF, Larrier N, Dodd L, Kirsch D, Martinez S, Brigman BE. The clinical management of chondrosarcoma.Curr Treat Options Oncol 2009;10:94-106.  Back to cited text no. 4
    
5.Hauptman JS, Barkhoudarian G, Safaee M, Gorgulho A, Tenn S, Agazaryan N, et al. Challenges in Linear Accelerator Radiotherapy for Chordomas and Chondrosarcomas of the Skull Base: Focus on Complications. Int J Radiat Oncol Biol Phys 2012;83:542-51.  Back to cited text no. 5
    


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  [Figure 1], [Figure 2], [Figure 3]



 

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