|Year : 2016 | Volume
| Issue : 2 | Page : 48-51
Monophasic synovial sarcoma of the hypopharynx excised by transoral laser microsurgery
Thomas Shi1, Grace M Scott2, Bret Wehrli1, Kevin Fung2
1 Department of Pathology, Schulich School of Medicine and Dentistry, Western University, London, Ontario, Canada
2 Department of Otolaryngology-Head and Neck Surgery, Schulich School of Medicine and Dentistry, Western University, London, Ontario, Canada
|Date of Web Publication||13-Oct-2017|
Room A3-118, 339 Windermere Road, London, Ontario
Source of Support: None, Conflict of Interest: None
| Abstract|| |
An overwhelming majority of primary tumors of the hypopharynx are squamous cell carcinomas, accounting for approximately 95%. Synovial sarcoma (SS) is exceedingly rare at this anatomical site. A 41-year-old, otherwise healthy male presented with a 2-year history of dyspnea and dysphonia. Flexible laryngoscopy revealed a very large, well-circumscribed, pedunculated mass with near-total airway obstruction. On computed tomography imaging, a 4.7 cm × 2.6 cm supraglottic mass was demonstrated. Transoral CO2 laser microsurgery was carried out for en bloc removal. Final margins were clear and the patient is currently disease-free and symptom-free. Since the first case of head and neck SS was described in 1954, there have been fewer than 200 cases reported in the world literature. To the best of our knowledge, this is the first known case of a laryngeal SS that presented with airway obstruction which was managed with a negative-margin transoral CO2 laser microsurgery resection.
Keywords: Hypopharynx, synovial sarcoma, transoral CO2 laser microsurgery
|How to cite this article:|
Shi T, Scott GM, Wehrli B, Fung K. Monophasic synovial sarcoma of the hypopharynx excised by transoral laser microsurgery. J Laryngol Voice 2016;6:48-51
| Introduction|| |
An overwhelming majority of primary tumors of the hypopharynx are squamous cell carcinomas, accounting for approximately 95%. Synovial sarcoma (SS) is exceedingly rare at this anatomical site. Herein, we present a case of SS in a young male presenting with progressive airway obstruction. Beyond this rare presentation, it is the management of this case that makes it exceptional. Despite the near-total upper airway obstruction associated with this 4.2 cm × 4.1 cm × 2.7 cm SS, the mass was removed by endoscopic resection with transoral laser microsurgery (TLM), which has not been previously described. TLM has been popularized since the 1990s based on oncologic outcomes, functional outcomes, minimal recovery time, and a trend in the literature toward a lower cost to the health-care system.,,,,,,,
| Case Report|| |
A 41-year-old, otherwise healthy male presented with a 2-year history of dyspnea and dysphonia. His breathing was worse in the supine position. On physical examination, his voice was extremely muffled. His airway was stable in the upright position, but he developed stridor on lying down, suggesting airway compromise. Flexible laryngoscopy revealed a very large, well-demarcated, pedunculated mass that was almost completely obstructing the airway [Figure 1]. The mass seemed to be arising from the left superior hypopharynx wall. It was fleshy in color and mobile with movement of the hypopharynx. Due to the size of the mass, the vocal cords could not be visualized. The epiglottis was not involved. No other abnormalities of the head and neck were found. No cervical adenopathy was palpable.
On computed tomography (CT) of the neck with intravenous contrast, the mass was characterized as a 4.7 cm × 2.6 cm supraglottic mass that was partially obstructing the airway. There was no lymphadenopathy evident on the CT scan [Figure 2]. Magnetic resonance imaging with gadolinium contrast showed a mass in the hypopharynx measuring 4.2 cm × 2.7 cm × 4.1 cm [Figure 3].
At this point, the patient's airway needed to be secured and a histological diagnosis needed to be obtained. He was taken to the operating theater for an awake tracheostomy under local anesthesia, followed by panendoscopy and an attempt at endoscopic resection with the CO2 laser for both diagnostic and therapeutic purposes. A Lindholm laryngoscope was suspended, and the mass was found to be well-circumscribed, pedunculated, and arising from a medium-sized stalk from the left posterolateral pharyngeal wall. The stalk of origin was at the level of the top of the medial piriform sinus; however, the pedunculated aspect extended to the esophageal inlet inferiorly and draped completely over his glottis superiorly. The CO2 laser was used at a setting of 2 W ultrapulse mode to excise the mass, cutting through the mucosa, through the pharyngeal constrictors, and into the parapharyngeal space. The mass was removed en bloc [Figure 4]. It measured approximately 6 cm and was well-circumscribed, encapsulated, and rubbery on palpation. Esophagoscopy did not reveal any additional lesions. The area of the posterolateral pharyngeal wall was allowed to granulate. The patient was decannulated 3 days later and discharged home in good condition. His airway, voice, and swallowing symptoms completely resolved. He was subsequently seen in clinic 3 weeks following surgery; laryngoscopy was essentially normal, aside from an area of granulation on the left lateral pharyngeal wall [Figure 5]. His case was subsequently discussed at the head and neck multidisciplinary tumor board, and the consensus decision was made to clear the positive margin followed by adjuvant radiotherapy. Subsequent endoscopic resection was easily performed without the need for a tracheostomy, and the revision pharyngeal resection did not yield any active tumor cells. Since the margins were negative, it was decided that the patient would not receive adjuvant radiotherapy and instead would be closely observed. At 1.5 years postdiagnosis, the patient remains disease-free.
Pathologic examination revealed this mass to be a monophasic SS, grade 2. Moderate mitotic activity was identified. There was no necrosis or lymphovascular invasion. The deep margin of the mass was positive. By immunohistochemistry, scattered individual and small clusters of tumor cells were positive for cytokeratin AE1/AE3, epithelial membrane antigen, and S100 protein; tumor cells were diffusely positive for vimentin; tumor cells failed to stain for desmin, myogenin, actins, CD34, and cytokeratin Cam 5.2.
Nuclear fluorescence in situ hybridization (nuc ish) analysis was performed to screen for a rearrangement involving the SS18 (previously called SYT) locus using a dual color, break apart probe (Abbott Molecular) located at 18q11.2. A pattern consistent with SS18 rearrangement was detected in 73.5% (147 of 200) of cells examined. Overall, the findings were diagnostic of that of SS.
| Discussion|| |
Since the first case of head and neck SS was described in 1954, there have been fewer than 200 cases reported in the world literature.,,, There is a male predominance, with a male-to-female ratio of 3:2, and although SS can occur at any age, they most commonly arise in patients aged between 25 and 35 years.,,,
The tumor usually appears as an asymptomatic mass until it attains sufficient volume to cause pressure effects on neighboring structures. Like traditional presentations of SS in the hypopharynx, our patient was quite asymptomatic, beyond a history of dyspnea and dysphonia. With these large, well-circumscribed masses, resection with negative margins remains the foundation of therapy.,, This can be a challenging endeavor in the head and neck region. Due to this, SS in the head and neck may be more aggressive and have a more reserved prognosis.,, Disease recurrence is a significant problem, with up to 45% of patients with head and neck SS developing a local recurrence and 33% of developing distant metastatic disease.,
Here, we present a case of SS in the hypopharynx, which is quite an unusual location. SS is a malignant tumor of pluripotent mesenchymal cells of unknown lineage differentiation and without a normal cell counterpart.,,,, The fact that it is most commonly found in the periarticular areas likely accounts for its initial and continued designation as “synovial sarcoma”. However, SS does not arise from synovial cells of the joint and may be of stem cell in origin., Despite this lack of knowledge, SS is a well-characterized pathologic identity that accounts for 7%–10% of all soft-tissue tumors. The pharynx is a rare location for an SS; in one case series, 1.3% (2/150) of all SSs were found in this location.
A well-known reciprocal translocation t(X; 18)(p11.2;q11.2) is detected in >95% of SS. The fusion of SS18 gene (also called SYT) with one of the SSX genes on the X chromosome produces a fusion protein, SS18-SSX. Studies have shown SS18-SSX to act as a scaffolding protein which recruits two other proteins, ATF2 and TLE1. TLE1 further recruits PcG/HDAC and act as a repressor to inhibit transcription of ATF2-target genes, including tumor suppressors. Due to advances in cytogenetics, testing for the presence of an SS18 translocation has become a standard procedure for suspected cases of SSs.
The advent of microsurgical endoscopic procedures using the CO2 laser represents an important advance surgery allowing for less invasive, more precise, and more functional surgery. This may result in lower morbidity and a better quality of life for the patient and have important implications for the realm of head and neck oncology. For hypopharyngeal carcinoma, TLM has the same advantages as those for laryngeal cancer: faster postoperative functional recovery and shorter hospital stay compared with those of open surgery.
In conclusion, we present a case of an SS in the hypopharynx with airway obstruction, an extremely unusual presentation for this tumor. SS is a rare, malignant tumor, which is best managed with surgical resection and radiotherapy. Endoscopic resection with transoral CO2 laser microsurgery has not been previously described for this disease entity. This approach has clear advantages over open partial or total laryngopharyngeal resection with respect to functional outcomes. This case has demonstrated that endoscopic resection is technically possible and is associated with minimal morbidity. Long-term follow-up is necessary, especially given the high rate of relapse.
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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]