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E-JCRT CORRESPONDENCE
Year : 2015  |  Volume : 11  |  Issue : 4  |  Page : 1038

Osteosarcoma of larynx: A rare case report with review of literature


1 Department of Radiotherapy and Oncology, Post Graduate Institute of Medical Education and Research, Chandigarh, India
2 Department of Otorhinolaryngology, Post Graduate Institute of Medical Education and Research, Chandigarh, India
3 Department of Histopathology, Post Graduate Institute of Medical Education and Research, Chandigarh, India

Date of Web Publication15-Feb-2016

Correspondence Address:
Tapesh Bhattacharyya
Department of Radiation Oncology, Post Graduate Institute of Medical Education and Research, Chandigarh - 160 012
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0973-1482.139274

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 > Abstract 


Laryngeal osteosarcoma is an extremely rare and aggressive malignancy. Only very few cases of primary laryngeal osteosarcomas are reported in literature, and all have a dismal prognosis. A 50-year-old male presented with recurrent hoarseness of voice and was evaluated and diagnosed as sarcoma larynx. Detailed histopathology report of laryngectomy specimen revealed presence of osteoid, which confirmed the diagnosis of osteosarcoma larynx. He was further treated with adjuvant chemotherapy and radiation and is disease free after 9 months. Surgery remains the mainstay of treatment in this disease and role of adjuvant chemotherapy and radiotherapy is still evolving.

Keywords: Larynx, osteosarcoma, radiation


How to cite this article:
Bahl A, George P, Bhattacharyya T, Ghoshal S, Bakshi J, Das A. Osteosarcoma of larynx: A rare case report with review of literature. J Can Res Ther 2015;11:1038

How to cite this URL:
Bahl A, George P, Bhattacharyya T, Ghoshal S, Bakshi J, Das A. Osteosarcoma of larynx: A rare case report with review of literature. J Can Res Ther [serial online] 2015 [cited 2020 Oct 26];11:1038. Available from: https://www.cancerjournal.net/text.asp?2015/11/4/1038/139274




 > Introduction Top


Malignant mesenchymal tumors of the larynx constitute less than 1% of all laryngeal cancers, osteosarcoma being rarest among them. It is characterized by rapid hematogenous spread and aggressive clinical course. It is a difficult clinical diagnosis and optimum management of this rare malignancy is not well defined. Surgery remains the mainstay of treatment. To date only 17 cases have been reported in literature. We report a similar case of a patient who underwent surgery followed by adjuvant chemotherapy and radiation, and is now leading a good quality of life after 9 months of follow up.


 > Case Report Top


A 50-year-old male presented to a local hospital with complaints of hoarseness of voice of 2 months duration. He was evaluated and found to have a right vocal cord polyp for which he underwent polypectomy. His symptoms recurred in 3 months when he reported to our centre. He had no history of radiation exposure previously. Direct larygoscopy showed a slough covered ulceroproliferative growth involving the laryngeal surface of epiglottis, bilateral false cord, and the endolarynx was filled with the growth. Magnetic resonance imaging [Figure 1]a and [Figure 1]b showed a heterogeneously enhancing well defined polypoidal altered signal intensity filling the laryngeal lumen in supraglottic and glottic region and extending inferiorly in subglottic region causing marked laryngeal and hypopharyngeal luminal compromise. The initial biopsy indicated a sarcoma of larynx and hence the patient was taken up for radical surgery including total laryngectomy, total thyroidectomy and partial pharyngectomy. The gross specimen [Figure 2] showed a large proliferative growth filling in all the laryngeal cavity involving infraglottis, glottis, and supraglottis. Cut surface of the tumor was fleshy, reddish white in color with areas of necrosis. The tumor was destroying the anterior cartilage, involving thyroid gland and reaching up to 2 m from the anterior resection limit. All the resection limits were free. Microscopically [Figure 3], the tumor cells were spindly having prominent nuclei and moderate amount of cytoplasm. Brisk mitotic activity was noted along with large areas of necrosis, apoptosis, hemorrhage, and dystrophic calcification. On immunohistochemistry, the tumor cells were negative for CD99, EMA, cytokeratin, and showed positivity for vimentin. Lymphovascular emboli were present but none of the 10 dissected lymph nodes showed a tumor. Based on these findings, a diagnosis of osteogenic sarcoma was made. Patient underwent positron emission tomography postoperatively, which showed a fluoro deoxy glucose (FDG) avid lesion in the neck (SUVmax 19.2) suggesting the postoperative inflammation but no evidence of increased uptake elsewhere in the body. The patient further received chemotherapy with Doxorubicin 30 mg/m 2 on days 1-3 and Cisplatin 100 mg/m 2 on day 1 for six cycles at a 3-weekly interval. After completion of chemotherapy cycles, he was treated with external beam radiotherapy of 66 Gy in 33 fractions over 6.5 weeks by bilateral parallel opposed field using 6 MV photon in view of close resection margins. After 9 months of follow up, he is leading a good quality of life with no evidence of disease.
Figure 1: (a) MRI [axial image] showing a heterogeneously enhancing well defined polypoidal altered signal intensity filling the laryngeal lumen in supraglottic and glottic region. (b) MRI [coronal image] showing a heterogeneously enhancing well defined polypoidal altered signal intensity filling the laryngeal lumen in supraglottic and glottic region and extending inferiorly in subglottic region

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Figure 2: Gross specimen showing large proliferative growth filling the laryngeal cavity

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Figure 3: Photomicrograph showing extensive areas of osteoid formation lined by tumor cells

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 > Discussion Top


Osteosarcoma of the larynx is an extremely rare and aggressive malignancy because of its rapid hematogenous spread and its difficult clinical diagnosis.[1] Malignant sarcomas constitute less than 1% of all laryngeal neoplasms and osteosarcoma represents the rarest of these soft tissue neoplasms. Chondrosarcoma and fibrosarcoma are the most common tumors in this category. Chondrosarcoma most commonly originates from cricoid and thyroid cartilage. These are well differentiated neoplasms and carry a favorable prognosis. Fibrosarcomas of larynx usually arise from anterior vocal fold and anterior commissure and their prognosis depend on the tumor size.[2] Laryngeal osteosarcoma is an extremely rare entity, which was first described in 1942. Since then only 17 cases were reported in literature and among them only 1 was female.[1],[2],[3],[4],[5],[6],[7],[8],[9],[10],[11],[12],[13] Osteosarcoma is thought to arise either from immature bone forming cells or through a differentiation of chondroblasts and fibroblasts into osteoblasts. Exposure to radiation, previous history of retinoblastoma, Paget's disease of bone, and fibrous dysplasia are thought to be etiologic factors. The etiologic factor for osteosarcoma of larynx is not well defined. There is no direct correlation between cigarette smoking or alcohol and laryngeal osteosarcoma. The index case also had no such predisposing risk factors.

The most common presenting complaints in osteosarcoma of the larynx are hoarseness, dyspnea, and acute airway obstruction. The mean duration of symptoms was approximately 6 months before diagnosis as described in different reports.[5] Diagnosis is often made after multiple biopsies, because of the difficulty in obtaining a confirmatory specimen. To be classified as osteosarcoma, osteoid must be produced by the tumor. Osteoid in these tumors may be noncalcified, lace-like, and trabecular in appearance. Production of osteoid is important in differentiating osteosarcoma from anaplastic chondrosarcoma. The other differential diagnosis for osteosarcoma includes other mesenchymal tumors like fibrosarcoma, pseudosarcoma, and malignant fibrous histiohistiocytoma. Fibrosarcoma is a common mesenchymal neoplasm of the larynx, characterized by fibroblasts and production of collagen fibrils and lacks osteoid. Pseudosarcoma can also mimic osteosarcoma, however, it is usually a benign response to an epithelial-derived primary malignancy with bizarre, sarcomatous, stromal tissue. Malignant fibrous histiocytoma can exhibit chondroid and osteoid elements. Usually, these elements are seen in the periphery of the lesion and exhibit a high degree of differentiation.

There is no consensus regarding the management of laryngeal osteosarcomas, probably due to the rarity of the reported cases and limited experience. Osteosarcomas are treated with multimodality regimens which include surgery, chemotherapy and radiation therapy. Because of the rarity of this malignancy, no series has been able to show a dramatic improvement in survival attributable to the use of one particular therapy regimen. Most patients died soon after their initial diagnosis, mostly of pulmonary metastasis or local and regional recurrence. Of the 17 cases reported in literature, 14 were treated with surgery, 2 received a primary radiation therapy.[1],[4] Most of the patients underwent total laryngectomy, however, Van Laer et al.[14] reported performing more radical surgery in the form of total laryngectomy, total thyroidectomy, and total pharyngectomy similar to that in our patient. Two of the patients who were treated with external beam radiotherapy received doses ranging from 45 to 67 Gy.[15],[16] Another three patients received postoperative radiotherapy. The role of various chemotherapeutic agents on overall survival and disease-free survival of patients with skeletal osteosarcoma has been studied by Cooperative Osteosarcoma Study Group. Because of rarity of laryngeal osteosarcoma, data regarding role of chemotherapy is scarce. Athre et al.[4] reported treating a patient with ifosfamide- and adriamycin-based chemotherapy but the patient had progressive disease with pulmonary metastasis and died within 3 months. Adriamycin-based chemotherapy was also tried in other studies but their efficacy is not well defined.[15],[16]

In different reports, it has been seen that local recurrences are usually seen after a mean duration of 9.5 months and average time of distant metastasis was around 11 months. About 50% of the cases die from the disease within 20 months after surgery.[1],[4] The optimum treatment of osteosarcoma of larynx is yet to be defined and requires more prospective studies. Our report may throw some light in the management of this rare tumor.[17]


 > Conclusion Top


Osteosarcoma of larynx is an extremely rare malignancy. Due to paucity of data, the optimum management of this malignancy is not well defined. Upfront surgery remains the mainstay of treatment. There is definite role of chemotherapy in skeletal osteosarcoma because of propensity of hematogenous spread, however, its role in laryngeal osteosarcoma is not yet defined due to its rarity. Although osteosarcomas are relatively radioresistant, radiotherapy should be tried after judicious case selection. This case report highlights the evolving role of chemotherapy and radiotherapy in laryngeal osteosarcoma.

 
 > References Top

1.
Berge JK, Kapadia SB, Myers EN. Osteosarcoma of the larynx. Arch Otolaryngol Head Neck Surg 1998;124:207-10.  Back to cited text no. 1
    
2.
Sanaat Z, Mohammady G, Esmaili H, Emrani M, Dolatkhah R. Osteosarcoma of the larynx. Arch Iran Med 2009;12:499-502.  Back to cited text no. 2
    
3.
Arellano B, Gonzalez FM, Martinez G, Salas C, Ramirez Camacho R, Vergara J, et al. Inflammatory pseudotumor of the larynx. Acta Otorrinolaringol Esp 1999;50:236-8.  Back to cited text no. 3
    
4.
Athre RS, Vories A, Mudrovich S, Ducic Y. Osteosarcomas of the larynx. Laryngoscope 2005;115:74-7.  Back to cited text no. 4
    
5.
Dahm LJ, Schaefer SD, Carder HM, Vellios F. Osteosarcoma of the soft tissue of the larynx: Report of a case with light and electron microscopic studies. Cancer 1978;42:2343-51.  Back to cited text no. 5
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6.
Kassir RR, Rassekh CH, Kinsella JB, Segas J, Carrau RL, Hokanson JA. Osteosarcoma of the Head and neck: Meta-analysis of nonrandomized studies. Laryngoscope 1997;107:56-61.  Back to cited text no. 6
    
7.
Madrigal FM, Godoy LM, Daboin KP, Casiraghi O, Garcia AM, Luna MA. Laryngeal osteosarcoma: A clinicopathologic analysis of four cases and comparison with a carcinosarcoma. Ann Diagn Pathol 2002;6:1-9.  Back to cited text no. 7
    
8.
Rossi RM, Landas SK, Kelly DR, Marsh WL. Osteosarcoma of the larynx. Otolaryngol Head Neck Surg 1998;118:385-8.  Back to cited text no. 8
    
9.
Topaloglu I, Isiksacan V, Ulusoy S, Sisman S. Osteosarcoma of the larynx. Otolaryngol Head Neck Surg 2004;131:789-90.  Back to cited text no. 9
    
10.
Haar JG, Chaudhry AP, Karankia MD, Milley PS. Chondroblastic osteosarcoma of the larynx. Arch Otolaryngol 1978;104:477-81.  Back to cited text no. 10
    
11.
Morley AR, Cameron DS, Watson AJ. Osteosarcoma of the larynx. J Laryngol Otol 1973;87:997-1005.  Back to cited text no. 11
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12.
Shimizu KT, Selch MT, Fu YS, Anzai Y, Lufkin RB. Osteosarcoma metastatic to the larynx. Ann Otol Rhinol Laryngol 1994;103:160-3.  Back to cited text no. 12
    
13.
Sprinkle PM, Allen MS, Brookshire PF. Osteosarcoma of the larynx. A true primary sarcoma of the larynx. Laryngoscope 1966;76:325-33.  Back to cited text no. 13
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14.
van Laer CG, Helliwell TR, Atkinson MW, Stell PM. Osteosarcoma of the larynx. Ann Otol Rhinol Laryngol 1989;98:971-4.  Back to cited text no. 14
    
15.
Suchatlampong V, Sriumpai S, Khawcharoenporn V. Osteosarcoma of the larynx: The first case report in Thailand with ultrastructural study. J Med Assoc Thai 1981;64:301-7.  Back to cited text no. 15
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Remagen W, Löhr J, von Westernhagen B. Osteosarcoma of the larynx. HNO 1983;31:366-8.  Back to cited text no. 16
    
17.
Pinsolle J, LeCluse I, Demeaux H, Laur P, Rivel J, Siberchicot F. Osteosarcoma of the soft tissue of the larynx: Report of a case with electron microscopic studies. Otolaryngol Head Neck Surg 1990;102:276-80.  Back to cited text no. 17
    


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  [Figure 1], [Figure 2], [Figure 3]



 

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