|Year : 2015 | Volume
| Issue : 1 | Page : 21-23
Laryngeal synovial sarcoma: Case report and literature review
D Sridhar Reddy, K Srinivas, Ch Sravan Kumar, Chandra Sekhar
Department of Otorhinolaryngology, Osmania Medical College, Hyderabad, Telangana, India
|Date of Web Publication||17-Dec-2015|
D Sridhar Reddy
Plot No: 138, Mallikarjun Nagar, North GSI Post, Chintalkunta, L. B. Nagar, Hyderabad - 500 068, Telangana
Source of Support: None, Conflict of Interest: None
| Abstract|| |
A 17-year-old female patient presented with complaints of difficulty in swallowing of 1-year duration, which was gradually progressive, associated with throat pain radiating to the left ear. There is no history of change in voice. There is no history of an injury or previous surgery. Computed tomography scanning confirmed a mass arising from the left aryepiglottic fold. On direct laryngoscopy, a mass was seen on the left aryepiglottic fold, which was excised and subjected to immunohistochemistry which revealed synovial sarcoma (SS). SS is a very rare tumor in the larynx, we report this case for its rarity.
Keywords: Aryepiglottic fold tumor, endoscopy, synovial sarcoma, tumor in larynx
|How to cite this article:|
Reddy D S, Srinivas K, Kumar CS, Sekhar C. Laryngeal synovial sarcoma: Case report and literature review. J Laryngol Voice 2015;5:21-3
| Introduction|| |
Only 10% of soft tissue sarcomas are synovial in type. Synovial sarcomas (SSs) are aggressive malignant soft tissue tumors that are thought to arise from pluripotent mesenchymal cells.  SS is a rare neoplasm. They usually involve large joints within the lower extremities. Only 3% of cases arise in the head and neck. , Its occurrence in the larynx is very rare and may present with airway obstruction, dysphagia, stridor, and necessitating tracheostomy. These cases are very rarely reported in the literature. The histological appearance resembles a synovial membrane hence the name SS. 
| Case report|| |
A 17-year-old female patient presented with complaints of difficulty in swallowing of 1-year duration, which was gradually progressive, associated with throat pain radiating to the ear. There is no history of change in voice. There is no history of an injury or previous surgery in the past. She did not have any relief from medical treatment.
On clinical examination, laryngeal crepitus was present, no palpable lymph nodes, on endoscopic examination; there was a smooth globular pinkish soft swelling over the left aryepiglottic fold, nearly 50 mm × 50 mm, almost obscuring the view of both the vocal cords and left pyriform fossa. Movements of the arytenoids were normal on both sides [Figure 1].
|Figure 1: Large cystic swelling over left ary-epiglottic fold on endoscopic examination of larynx|
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Computed tomography of the neck in axial, coronal, and saggital views showed circumscribed low attenuation mass in the left supraglottic region, relatively obliterating the left pyriform fossa, compressing and displacing the hypopharynx anteriorly and to the right [Figure 2],[Figure 3].
|Figure 2: Computed tomography of larynx - axial sections showing circumscribed low attenuation mass in the left supra glottic region|
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Under general anesthesia, and with prior consent for tracheostomy, larynx was exposed with Macintosh laryngoscope and with the help of a 4 mm 0° nasal endoscope, the mass was excised in toto and sent for histopathological analysis, followed by immunohistochemistry.
Biopsy revealed undifferentiated carcinoma, immunohistochemistry showed LCA, CK, S-100 negative, KI-67 positive, on further evaluation smooth muscle antibody is negative, vimentin is positive, CD-99, and BCL-2 was done and was intensely positive. Epithelial membrane antigen was focally positive, thus proving the tumor to be SS of the left aryepiglottic fold. We have evaluated the case regarding distant metastasis by ultrasonography abdomen, X-ray chest and detailed whole body clinical examination, and ruled out distant metastases [Figure 4],[Figure 5].
The patient was subjected to 44 Gy of postoperative external beam radiotherapy and was followed up endoscopically for 6 months which showed no recurrence.
| Discussion|| |
Squamous cell carcinoma accounts for over 90% of all laryngeal cancers.  Laryngeal SS is an extremely rare form of laryngeal malignancy. The histological appearance resembles a synovial membrane hence, the name SS.  Although the term SS implies an origin from the joint linings, <10% are intra-articular. SS account for approximately 10% of all soft tissue sarcomas and ranks as the fourth most common sarcoma.  The majority develop in the deep soft tissue and about 60-70% involve the lower extremity.
Sarcomas occur uncommonly in the head and neck region in the pediatric population. 
SS of head and neck is extremely rare with less than 20 cases in the literature arising from the larynx.  SS are morphologically biphasic or monophasic. The histologic hallmark of biphasic SS is dual lines of differentiation (i.e., epithelial-like and mesenchymal-like). Many SS are monophasic, being composed of only spindled cells or, very rarely, epithelial cells. Common sites of metastases are the lung, skeleton, and occasionally the regional lymph nodes.  BCL-2 protein expression has been described as a characteristic marker of SS and is useful for its differentiation from other sarcomas. Cytokeratin and CD-99 are also used in detecting SS. 
Prognosis of patients with SS depends on extent, grade, and size of the primary.  The 5-year survival rate has been reported to be approximately 70-80%, and the 10-year survival rate approximately 50%.  The treatment of SS is multimodal. Radical surgical excision is generally accepted as the mainstay of therapy and adjuvant chemotherapy comprising of doxorubicin and ifosfamide has been utilized for high-grade SS. Adjuvant radiotherapy has also been shown to reduce the local recurrence rates but not overall survival rates. 
| Conclusion|| |
SS are aggressive malignant soft tissue tumors that are thought to arise from pluripotent mesenchymal cells. Only 3% of cases arise in the head and neck. Prognosis of patients with SS depends on extent, grade, and size of the primary. The treatment of SS is multimodal. Radical surgical excision is generally accepted as the mainstay of therapy and adjuvant chemotherapy utilized for high-grade SS.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]