In patients who require a gastric pull-up, despite bilateral injury to the vocal cords and regurgitation, preservation of the larynx can improve their quality of life. Â© 2021 The Authors. Clinical Case Reports published by John Wiley & Sons Ltd

Ahmadi, A.

Firouzabadi, M.D.

Ghanbari, H.

Roomiani, M.

Sanaei, A.

eprints Iran University of Medical Sciences

Clin Case Rep. 2021;9:1699–1703.    | 1699wileyonlinelibrary.com/journal/ccr31 |  INTRODUCTIONWe present the case of a 49- year- old man with odynopha-gia, dysphagia, and regurgitation. Endoscopy showed a par-tially obstructed mass in upper esophagus with ulceration of overlying mucosa. The imaging findings indicated a hetero-geneous intraluminal mass in the esophagus. The patient un-derwent successful total esophagectomy and gastric pull- up with laryngeal preservation.A synovial sarcoma is a soft tissue sarcoma subtype, which is assumed to arise from mesenchymal cells.1 The name is derived from its microscopic resemblance to the sy-novium under light microscopy. Synovial sarcoma accounts for approximately 5%- 10% of all soft tissue sarcomas.2 Synovial sarcoma of digestive system is an extremely rare phenomenon.3 Among sarcomas, primary synovial sarcoma of the esophagus is  an uncommon tumor that may present with dysphagia, odynophagia, and loss of weight.4 It occurs at a male- to- female ratio of 3:2, especially in young adults and adolescents.5 Here, we describe a case of synovial sar-coma of the esophagus, based on immunohistochemical (IHC) staining.2 |  CASE PRESENTATIONThe patient was a 49- year- old man with odynophagia, dys-phagia to liquids, and regurgitation for two years, which had worsened over the last month. He refused any loss of appe-tite, loss of weight, or aspiration, with no clinically signifi-cant lymphadenopathy in the head or neck. The laboratory data were within the normal ranges. The gastrointestinal (GI) endoscopy showed a partially obstructed mass in the upper esophagus with ulcerating overlying mucosa. The postcricoid region appeared free of tumor, based on direct laryngoscopy, and biopsy of the submucosal mass of the upper esophagus suggested sarcoma/sarcomatoid carcinoma. However, an-other pathologist reported a poorly differentiated synovial sarcoma with extensive ulceration and necrosis. The com-puted tomography (CT) scan of the thorax revealed a seg-ment of circumferential esophageal wall thickening and a large heterogeneous intraluminal mass in the cervical es-ophagus with compression effects on the posterior wall of trachea, without extension (Figure 1).The results of magnetic resonance imaging (MRI) showed a large heterogeneous intraluminal solid mass, measuring Received: 28 June 2020 | Revised: 28 December 2020 | Accepted: 6 January 2021DOI: 10.1002/ccr3.3881  C A S E  R E P O R TGastric pull- up with laryngeal preservation of the primary synovial sarcoma of the esophagus: A case reportAslan Ahmadi |   Mohammad Dehghani Firouzabadi |   Ayda Sanaei  |   Hadi Ghanbari  |   Maryam RoomianiThis is an open access article under the terms of the Creative Commons Attribution- NonCommercial- NoDerivs License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non- commercial and no modifications or adaptations are made.© 2021 The Authors. Clinical Case Reports published by John Wiley & Sons Ltd.ENT and Head & Neck Research Center, The Five Senses Health Institute, Iran University of Medical Sciences, Tehran, IranCorrespondenceHadi Ghanbari and Maryam Roomiani, MD, ENT and Head & Neck Research Center, The Five Senses Institute, Iran University of Medical Sciences, Tehran, Iran.Emails: ghanbari_md@iums.ac.ir; maryamroomiani3@gmail.comAbstractIn patients who require a gastric pull- up, despite bilateral injury to the vocal cords and regurgitation, preservation of the larynx can improve their quality of life.K E Y W O R D Sear, nose throat1700 |   AHMADI et Al.57 × 47 × 43 mm in the upper thoracic esophagus, which led to the anterior displacement of trachea, with low T1 and high T2 signals and marked enhancement in the post- contrast images. No fat plane was observed between the lesion and the prevertebral fascia. Therefore, esophageal sarcoma was considered, as squamous cell carcinoma (SCC) and adeno-carcinoma are less probable (Figure 2). Due to the absence of a postcricoid area superior to the tumor, the patient was a candidate for esophagectomy and gastric pull- up with laryn-geal preservation (Figure 3).Written informed consent was obtained from the patient before surgery. Using the lateral pharyngotomy approach, un-likely imaging findings included prevertebral fascia involve-ment and simple detachment and resection of tumor from the prevertebral fascia and the retropharyngeal space with a safe margin. The superior margin of the tumor was dissected from the postcricoid region, with no evidence of erosion (negative for tumor deposits on the frozen section). Following esoph-agus resection, the anterior aspect of the proximal part of the stomach was stitched to the normal mucosal strip in the F I G U R E  1  A, B, Axial view of chest CT shows a large heterogenous intraluminal mass in cervical esophagus with compression effect on posterior wall of tracheaF I G U R E  2  Pre- operative neck MRI. A- F, Sagittal and axial plane of T1, B, T1 Fat suppression, D&F T1 Post GAD, E: T2   | 1701AHMADI et Al.superior part of the resected site, and the posterolateral part of the stomach was stitched to the remnant retropharyngeal mucosa with Vicryl 3- 0. The left recurrent laryngeal nerve was sacrificed due to tumor involvement.On the first postoperative day, the patient was evaluated with a flexible fiberoptic bronchoscope through the nose. The mucosal color was pink, and the laryngeal tissue was viable. Although the right recurrent laryngeal nerve was preserved, double true vocal cord (TVC) paralysis occurred. The patient successfully adhered to an oral diet after no leaking confir-mation, based on the gastrografin study. He was discharged from the hospital with a jejunostomy tube and a tracheostomy tube, which remained for two and six months, respectively (Figure 4). At 12 months after surgery, he could talk without tracheostomy and perform activities. The specimen size was 7 × 4.5 × 3 cm, consisting of a firm creamy mass, measuring 5 × 4.5 × 3 cm with hemorrhagic spots, which were reported as synovial sarcoma of the esophagus (Figures 5 & 6). Also, proximal and distal margins of the specimen were free from the tumor, and vascular invasion was not identified.3 |  DISCUSSIONSynovial sarcoma is a rare type of soft tissue sarcoma, which is typically found in the extremities and adjacent to tendon F I G U R E  3  A; Lateral Pharyngectomy approach, tumor identified (asterisk) and B; tumor completely released from trachea (white arrow) and C; mass totally resected with esophagus (plus sign)(A) (B) (C)F I G U R E  4  Indirect laryngoscopy, 6 mo after surgery, left TVC paralysis was noted (asterisk)F I G U R E  5  Low power microscopic examination with hematoxylin and eosin (H&E) staining showed area of necrosis and also esophageal wall infiltration by hypercellular sheets of tumor cells arranged in fascicles1702 |   AHMADI et Al.sheaths and joint capsules.6 A synovial sarcoma of the head and neck is extremely rare and accounts for only 3% to 9% of all sarcomas.7,8 In this regard, a review study by F. Sista et al showed that the stomach was the most common site of monophasic synovial sarcoma, followed by jejunoileal, duo-denal, and colorectal regions. Although monophasic synovial sarcoma has not been reported in the esophagus, approxi-mately 75% of biphasic synovial sarcomas of the digestive system occur in the esophagus.9The CT scan findings typically indicate a well- defined het-erogeneous soft tissue mass. Also, CT scan is useful for detect-ing tumor calcifications. However, MRI is the imaging modality of choice to stage the tumor locally. The tumor is commonly hypointense to muscles and heterogeneous on T1- and T2- weighted images, respectively.1 The T2- weighted MRI images may demonstrate the "triple sign" due to the presence of areas with low, medium, and high signal intensities.6 Histologically, they are classified into three types: monophasic, biphasic, and poorly differentiated. The monophasic type consists of entirely spindle- shaped cells. The biphasic type is composed of both spindle- shaped and epithelial components, while the poorly differentiated type is characterized by a predominantly round or short spindle- cell morphology.10 In the present case, a poorly differentiated synovial sarcoma was reported. Overall, the char-acteristic IHC profile for the diagnosis of synovial sarcoma includes the expression of transducer- like enhancer of split 1 (TLE- 1), CD99, partial expression of cytokeratin, and epithe-lial membrane antigen (EMA).11 In our case, CK5, CK6, CK7, TLE1, CD99, BCL- 2, and CKAE1/AE3 were positive.Cytogenetically, translocation between X and 18 chro-mosomes is seen in more than 90% of synovial sarcomas.12 Generally, a combination of surgery and adjuvant therapy is used to treat synovial sarcoma. Once an adequate clear mar-gin is not achievable, radiotherapy is performed. Our patient underwent total esophagectomy and gastric pull- up with laryngeal preservation; however, radiotherapy was not per-formed because of no evidence of residual tumor. Also, the larynx was preserved in our case.The factors  indicating a  poor  prognosis  include a large tumor size, cystic or hemorrhage components, tumor location in the head, neck, or trunk, older age, a poorly differentiated type on histology, heterogeneity, necrosis, a high nuclear grade, a high mitotic rate, and p53 mutations. The overall five- year survival is estimated at 36% to 76%.13,14 Although advanced imaging investigations, such as MRI and/or CT scan, are particularly useful in diagnosing neoplasms of the neck,15 synovial sarcoma of the GI tract needs to be differ-entiated from malignant gastrointestinal stromal tumor, leio-myosarcoma, and sarcomatoid carcinoma.34 |  CONCLUSIONAlthough rare, synovial sarcoma should be considered in the differential diagnosis of esophageal lesions. In our patient, the type of surgery, that is, esophagectomy and gastric pull- up with laryngeal preservation, was selected, based on the involved tumor areas with major impacts on the patient's quality of life.ACKNOWLEDGMENTSPublished with written consent of the patient.CONFLICT OF INTERESTNone declared.AUTHOR CONTRIBUTIONSAslan Ahmadi, Mohammad Dehghani Firouzabadi, Ayda Sanaei, Hadi Ghanbari, and Maryam Roomiani: involved in conception and design of work. Aslan Ahmadi, Mohammad Dehghani Firouzabadi, Ayda Sanaei, Hadi Ghanbari, and Maryam Roomiani: critically revised the article. All the au-thors approved the final version and have the agreement to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.ETHICAL APPROVALThis study protocol was approved by the local ethics com-mittee of the Iran University of Medical Sciences. Informed consent was obtained from the patient before the study.F I G U R E  6  A- C, High power microscopic examination with H&E staining showed areas of necrosis and highly cellular fascicles of round to oval cells with high N/C ratio, hyperchromatic nuclei, and inconspicuous nucleoli(A) (B) (C)   | 1703AHMADI et Al.DATA AVAILABILITY STATEMENTAll data generated or analyzed during this study are included in this submitted article.ORCIDAyda Sanaei   https://orcid.org/0000-0003-2967-9734 Hadi Ghanbari   https://orcid.org/0000-0002-3551-1906 Maryam Roomiani   https://orcid.org/0000-0003-0282-2318 REFERENCES 1. Tsang KH, Chan WSW, Chan MK, Lai KC, Chan A. Synovial Sarcoma: Epidemiology, Prognosis, and Imaging in a Tertiary Referral Centre, Hong Kong. J Radiol. 2016;19(1):19- 27. 2. Hoang NT, Acevedo LA, Mann MJ, Tolani B. A review of soft- tissue sarcomas: translation of biological advances into treatment measures. Cancer Manag Res. 2018;10:1089- 1114. 3. Billings SD, Meisner LF, Cummings OW, Tejada E. Synovial sarcoma of the upper digestive tract: a report of two cases with demonstration of the X;18 translocation by fluorescence in situ hy-bridization. Mod Pathol. 2000;13(1):68- 76. 4. Doroudinia A. Synovial Sarcoma of the Esophagus: a case report and review of literature. Middle East J Digest Dis. 2017;9(2):111. 5. Nath K, Gupta R, Agarwal SP, Kumar S. Synovial Sarcoma of the Head and Neck: A Report of 2 Cases. Journal of Case Reports. 2016;6:124- 128. 6. Terra S, Aesif SW, Maleszewski JJ, Folpe AL, Boland JM. Mediastinal synovial sarcoma: clinicopathologic analysis of 21 cases with molecular confirmation. Am J Surg Pathol. 2018;42(6):761- 766. 7. Alsharief AN, Fageeh M, Alabdulkarim Y. Monophasic synovial sarcoma presenting as a primary ileal mass: a case report and re-view of the literature. J Med Case Rep. 2012;6:83. 8. Bukachevsky RP, Pincus RL, Shechtman FG, et al. Synovial sar-coma of the head and neck. Head Neck. 1992;14(1):44- 48. 9. Sista F, Penna AD, Abruzzese V, Leardi S, Amicucci G. Intestinal intussusception by monophasic synovial sarcoma: case report and literature review. Chirurgia (Bucur). 2015;110(4):391- 395. 10. So IT, Cho KB, Lee JY, et al. A primary gastric synovial sar-coma: A case report and literature review. Medicine (Baltimore). 2017;96(49):e8904. 11. Zaccarini DJ, Deng X, Tull J, Maciak C, Valente AL, Zhang S. Expression of TLE- 1 and CD99 in carcinoma: pitfalls in diagno-sis of synovial sarcoma. Appl Immunohistochem Mol Morphol. 2018;26(6):368- 373. 12. Desar IME, Fleuren EDG, van der Graaf WTA. Systemic treat-ment for adults with synovial sarcoma. Curr Treat Options Oncol. 2018;19(2):13. 13. Krieg AH, Hefti F, Speth BM, et al. Synovial sarcomas usually metastasize after %3e5 years: a multicenter retrospective analysis with minimum follow- up of 10 years for survivors. Ann Oncol. 2011;22(2):458- 467. 14. Spurrell EL, Fisher C, Thomas JM, Judson IR. Prognostic factors in advanced synovial sarcoma: an analysis of 104 patients treated at the Royal Marsden Hospital. Ann Oncol. 2005;16(3):437- 444. 15. Ahmadi A, Hirbod H, Cheraghipoor M, Izadi F. Schwannoma of the brachial plexus presented as a neck mass: a case report and review of the literature. Int J Otolaryngol Head Neck Surg. 2015;4(02):104- 107.How to cite this article: Ahmadi A, Firouzabadi MD, Sanaei A, Ghanbari H, Roomiani M. Gastric pull- up with laryngeal preservation of the primary synovial sarcoma of the esophagus: A case report. Clin Case Rep. 2021;9:1699– 1703. https://doi.org/10.1002/ccr3.3881

Gastric pull-up with laryngeal preservation of the primary synovial sarcoma of the esophagus: A case report

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Gastric pull-up with laryngeal preservation of the primary synovial sarcoma of the esophagus: A case report

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