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Ren MY, Li J, Li RM, Wu YX, Han RJ, Zhang C. Primary orbital monophasic synovial sarcoma with calcification: A case report. World J Clin Cases 2022; 10:1623-1629. [PMID: 35211602 DOI: 10.12998/wjcc.v10.i5.1623 ren my, li j, li rm, wu yx, han rj, zhang c. primary orbital monophasic synovial sarcoma with calcification: a case report. world j clin cases 2022; 10(5): 1623-1629 [pmid: 35211602 doi:10.12998/wjcc.v10.i5.1623] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/31/2021] [Revised: 10/28/2021] [Accepted: 12/28/2021] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Synovial sarcoma is a malignant mesenchymal neoplasm with variable epithelial differentiation. Most synovial sarcoma cases are reported in young adults and can arise in any body site. Notably, primary orbital synovial sarcoma is rare. CASE SUMMARY An 8-year-old east Asian girl with 1-month history of gradual painless proptosis and lacrimation of the right eye was admitted. The patient presented with painless proptosis, downward eyeball displacement, and upward movement disorders. According to clinical manifestations, imaging examinations and postoperative immunohistochemical examinations, the diagnosis was monophasic synovial sarcoma with calcification. The patient underwent anterior orbitotomy procedure for removal of the right orbital mass under general anesthesia. The diagnosis of monophasic synovial sarcoma with calcification was confirmed finally through histological and immunohistochemical exam. The follow-up period was 6 mo, and no recurrence was observed during this period. CONCLUSION Primary orbital monophasic synovial sarcoma with calcification is a rare sarcoma, and clinical manifestations and imaging results are not specific. The tumor may present similar features as a benign tumor. Comprehensive analysis of clinical, radiological, and pathological findings is critically important for making the right diagnosis. Conventional treatment approach for synovial sarcoma is surgical resection with adjuvant or neoadjuvant radiotherapy, which is highly effective for localized tumors.
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Affiliation(s)
- Ming-Yu Ren
- Department of Orbital Disease and Ocular Tumor, Hebei Eye Hospital, Xingtai 054001, Hebei Province, China.
| | - Jing Li
- Intensive Care Unit, Xingtai Third Hospital, Xingtai 054001, Hebei Province, China
| | - Rui-Miao Li
- Department of Orbital Disease and Ocular Tumor, Hebei Eye Hospital, Xingtai 054001, Hebei Province, China
| | - Yi-Xiang Wu
- Department of Orbital Disease and Ocular Tumor, Hebei Eye Hospital, Xingtai 054001, Hebei Province, China
| | - Rui-Juan Han
- Department of Orbital Disease and Ocular Tumor, Hebei Eye Hospital, Xingtai 054001, Hebei Province, China
| | - Chi Zhang
- Department of Orbital Disease and Ocular Tumor, Hebei Eye Hospital, Xingtai 054001, Hebei Province, China
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Ren MY, Li J, Li RM, Wu YX, Han RJ, Zhang C. Primary orbital monophasic synovial sarcoma with calcification: A case report. World J Clin Cases 2022; 10:1623-1629. [PMID: 35211602 PMCID: PMC8855275 DOI: 10.12998/wjcc.v10.i5.1623] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/31/2021] [Revised: 10/28/2021] [Accepted: 12/28/2021] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Synovial sarcoma is a malignant mesenchymal neoplasm with variable epithelial differentiation. Most synovial sarcoma cases are reported in young adults and can arise in any body site. Notably, primary orbital synovial sarcoma is rare. CASE SUMMARY An 8-year-old east Asian girl with 1-month history of gradual painless proptosis and lacrimation of the right eye was admitted. The patient presented with painless proptosis, downward eyeball displacement, and upward movement disorders. According to clinical manifestations, imaging examinations and postoperative immunohistochemical examinations, the diagnosis was monophasic synovial sarcoma with calcification. The patient underwent anterior orbitotomy procedure for removal of the right orbital mass under general anesthesia. The diagnosis of monophasic synovial sarcoma with calcification was confirmed finally through histological and immunohistochemical exam. The follow-up period was 6 mo, and no recurrence was observed during this period. CONCLUSION Primary orbital monophasic synovial sarcoma with calcification is a rare sarcoma, and clinical manifestations and imaging results are not specific. The tumor may present similar features as a benign tumor. Comprehensive analysis of clinical, radiological, and pathological findings is critically important for making the right diagnosis. Conventional treatment approach for synovial sarcoma is surgical resection with adjuvant or neoadjuvant radiotherapy, which is highly effective for localized tumors.
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Affiliation(s)
- Ming-Yu Ren
- Department of Orbital Disease and Ocular Tumor, Hebei Eye Hospital, Xingtai 054001, Hebei Province, China
| | - Jing Li
- Intensive Care Unit, Xingtai Third Hospital, Xingtai 054001, Hebei Province, China
| | - Rui-Miao Li
- Department of Orbital Disease and Ocular Tumor, Hebei Eye Hospital, Xingtai 054001, Hebei Province, China
| | - Yi-Xiang Wu
- Department of Orbital Disease and Ocular Tumor, Hebei Eye Hospital, Xingtai 054001, Hebei Province, China
| | - Rui-Juan Han
- Department of Orbital Disease and Ocular Tumor, Hebei Eye Hospital, Xingtai 054001, Hebei Province, China
| | - Chi Zhang
- Department of Orbital Disease and Ocular Tumor, Hebei Eye Hospital, Xingtai 054001, Hebei Province, China
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Quan H, Sreekissoon S, Wang Y. Synovial sarcoma of the head and neck: A review of reported cases on the clinical characteristics and treatment methods. Front Cell Dev Biol 2022; 10:1077756. [PMID: 36684451 PMCID: PMC9853006 DOI: 10.3389/fcell.2022.1077756] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/23/2022] [Accepted: 12/12/2022] [Indexed: 01/08/2023] Open
Abstract
Synovial sarcoma (SS) is a high-grade soft-tissue sarcoma that occurs predominantly in older children and young adults in their thirties. It is usually very challenging to diagnose and treat synovial sarcoma in the head and neck region. The purpose of this review is to investigate the clinical manifestations and different treatment methods in the management of primary synovial sarcoma of the head and neck. HNSS has an aggressive nature and poor prognosis. Surgical resection, radiotherapy, and chemotherapy are the primary treatment methods. Typically, surgical resection with negative margins remains the foundation of therapy, which is not very easily achieved in the head and neck due to its complex anatomical structure and the presence of many blood vessels and nerves. However, synovial sarcoma has a high recurrence rate, so aggressive management and close follow-up are warranted for the optimal outcome.
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Affiliation(s)
- Hongzhi Quan
- Department of Oral Maxillofacial Surgery, Xiangya Stomatological Hospital and School of Stomatology, Central South University, Changsha, Hunan, China
| | - Senjeet Sreekissoon
- Department of Oral Maxillofacial Surgery, Xiangya Stomatological Hospital and School of Stomatology, Central South University, Changsha, Hunan, China
| | - Yan Wang
- School and Hospital of Stomatology, Wenzhou Medical University, Wenzhou, Zhejiang, China
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Xu P, Chen J. Primary Synovial Sarcoma of the Orbit. OPHTHALMOLOGY AND EYE DISEASES 2017; 9:1179172117701732. [PMID: 28469480 PMCID: PMC5397295 DOI: 10.1177/1179172117701732] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 12/07/2016] [Accepted: 01/31/2017] [Indexed: 12/17/2022]
Abstract
Purpose: To describe the clinical, pathological, and immunohistochemical characteristics and therapies of a rare case of primary synovial sarcoma in the orbit. Design: Retrospective interventional case report. Participant: A 6-year-old girl with pathologically proven synovial sarcoma in the orbit. The diagnosis was confirmed by immunohistochemistry. Methods: The patient was treated with right lateral orbital and right temporal tumor resection, followed by chemotherapy. She was followed up every 3 months for 1 year. Results: The tumor was excised, and the patient received 5 courses of chemotherapy. She did well during the initial first-year follow-up with no recurrent signs. Conclusions: We reported the sixth case of primary synovial sarcoma in the orbit and the first case of a 6-year-old girl.
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Affiliation(s)
- Pei Xu
- Department of Ophthalmology, Tongji Hospital, Huazhong University of Science and Technology, Wuhan, People's Republic of China.,Department of Ophthalmology & Visual Sciences, University of Texas Medical Branch, Galveston, TX, USA.,Department of Neuroscience and Cell biology, University of Texas Medical Branch, Galveston, TX, USA
| | - Jianbin Chen
- Department of Ophthalmology, Tongji Hospital, Huazhong University of Science and Technology, Wuhan, People's Republic of China
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Primary orbital synovial sarcoma: A clinicopathologic review with a differential diagnosis and discussion of molecular genetics. Surv Ophthalmol 2017; 62:227-236. [DOI: 10.1016/j.survophthal.2016.09.001] [Citation(s) in RCA: 11] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/16/2016] [Revised: 09/03/2016] [Accepted: 09/09/2016] [Indexed: 12/13/2022]
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Abstract
PURPOSE To describe the first reported case of intraocular synovial sarcoma. METHODS A 29-year-old man was enucleated for a blind, painful eye. Pathologic examination revealed an unexpected intraocular spindle cell tumor. RESULTS Immunohistochemical characterization revealed diffuse reactivity of the tumor cells for vimentin and focal positivity for epithelial markers pankeratin and epithelial membrane antigen. Melanoma markers were negative. Fluorescent in situ hybridization studies identified a t(X;18) (p11.2;q11.2) translocation, establishing a final diagnosis of synovial sarcoma. There was no evidence of extraocular extension as the resected margin of the optic nerve was free of tumor. Further imaging studies revealed no extraocular primary site or metastasis. CONCLUSION The incidental discovery of an intraocular malignancy in this case underscores the importance of routine histopathologic analysis of all enucleated globes. To the authors' knowledge, this is the first reported case of an intraocular synovial sarcoma, either as metastasis or as primary site.
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Liu K, Duan X, Yang L, Yu Y, Liu B. Primary synovial sarcoma in the orbit. J AAPOS 2012; 16:582-4. [PMID: 23158553 DOI: 10.1016/j.jaapos.2012.09.002] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/03/2012] [Revised: 08/21/2012] [Accepted: 09/03/2012] [Indexed: 12/24/2022]
Abstract
Synovial sarcoma is a rare malignant neoplasm that accounts for approximately 6% to 9% of all soft-tissue tumors. It occurs predominately in upper and lower extremities of young adults. We report the first case of a primary orbital synovial sarcoma in an 18-month-old girl.
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Affiliation(s)
- Ke Liu
- Department of Ophthalmology, 2nd Xiangya Hospital, Central South University, China
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Oh HN, Yang JW, Kim JL. A Case of Primary Orbital Synovial Sarcoma. JOURNAL OF THE KOREAN OPHTHALMOLOGICAL SOCIETY 2012. [DOI: 10.3341/jkos.2012.53.5.716] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/23/2022]
Affiliation(s)
- Ha Na Oh
- Department of Ophthalmology, Busan Paik Hospital, Inje University College of Medicine, Busan, Korea
| | - Jae Wook Yang
- Department of Ophthalmology, Busan Paik Hospital, Inje University College of Medicine, Busan, Korea
| | - Jung Lim Kim
- Department of Ophthalmology, Busan Paik Hospital, Inje University College of Medicine, Busan, Korea
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Kusuma S, Skarupa DJ, Ely KA, Cmelak AJ, Burkey BB. Synovial Sarcoma of the Head and Neck: A Review of its Diagnosis and Management and a Report of a Rare Case of Orbital Involvement. EAR, NOSE & THROAT JOURNAL 2010. [DOI: 10.1177/014556131008900612] [Citation(s) in RCA: 14] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/16/2022] Open
Abstract
Synovial sarcoma is typically an aggressive malignant tumor of the soft tissues, usually in the extremities, that affects young adults. Tumors of the head and neck are rare. Reported head and neck sites have included the hypopharynx (the most common site), the oropharynx, the larynx, and the soft tissues of the neck; only 4 cases of orbital involvement have been previously reported. We describe a case of synovial sarcoma of the medial canthus, which we discovered during a review of a tumor registry. The patient, an 18-year-old woman, underwent conservative excision and postoperative radiation therapy. Long-term follow-up detected no evidence of recurrence. Because there is no established, consistent approach to the treatment of synovial sarcoma of the head and neck, we also present a consensus management plan based on our review of the literature.
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Affiliation(s)
- Shashidhar Kusuma
- Department of Plastic Surgery, The Cleveland Clinic, Strongsville Family Health Center, Strongsville, Ohio
| | - David J. Skarupa
- Department of Plastic Surgery, The Cleveland Clinic, Strongsville Family Health Center, Strongsville, Ohio
| | - Kim A. Ely
- From the Vanderbilt Bill Wilkerson Center for Otolaryngology and Communication Sciences, Nashville, Tenn
| | - Anthony J. Cmelak
- From the Vanderbilt Bill Wilkerson Center for Otolaryngology and Communication Sciences, Nashville, Tenn
| | - Brian B. Burkey
- From the Vanderbilt Bill Wilkerson Center for Otolaryngology and Communication Sciences, Nashville, Tenn
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Hartstein ME, Silver FL, Ludwig OJ, O'Connor DM. Primary Synovial Sarcoma. Ophthalmology 2006; 113:2093-6. [PMID: 17074567 DOI: 10.1016/j.ophtha.2006.04.037] [Citation(s) in RCA: 16] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/19/2005] [Revised: 04/08/2006] [Accepted: 04/08/2006] [Indexed: 11/22/2022] Open
Abstract
PURPOSE To describe the clinicopathologic and immunohistochemical features and treatment of a rare case of primary synovial sarcoma of the orbit. DESIGN Retrospective interventional case report. PARTICIPANT A 14-year-old young man with histologically proven synovial sarcoma of the orbit. The diagnosis was confirmed by demonstration of a specific chromosomal translocation by polymerase chain reaction studies. METHODS The patient was treated with orbital exenteration and followed up for 18 months at regular intervals. RESULTS The tumor was excised completely, and the patient has done well during the initial 1-year follow-up with no sign of recurrence. CONCLUSIONS We report the fourth case of synovial sarcoma of the orbit and, at 14 years of age, the youngest patient reported.
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Affiliation(s)
- Morris E Hartstein
- Department of Ophthalmology, Saint Louis University Eye Institute, Saint Louis University School of Medicine, St. Louis, Missouri 63104, USA.
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Vera J, García MD, Marigil M, Abascal M, Lopez JI, Ligorred L. Biphasic synovial sarcoma of the abdominal wall. Virchows Arch 2006; 449:367-72. [PMID: 16855839 DOI: 10.1007/s00428-005-0076-2] [Citation(s) in RCA: 12] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/01/2005] [Accepted: 08/19/2005] [Indexed: 12/18/2022]
Abstract
Synovial sarcoma arising in the abdominal wall is a rare tumor. We report a case of a 38-year-old man who complained of abdominal pain. Physical examination revealed a firm mobile mass, 25 cm in diameter, in the left lower abdominal wall. The tumor was first thought to be a sarcoma arising from the omentum or mesentery. During surgery, a large tumor was found attached to the inner surface of the abdominal wall and compressing the gastrointestinal tract. On microscopic examination the tumor corresponded to a biphasic synovial sarcoma immunoreactive for cytokeratins (AE1/AE3, 7 and 19), epithelial membrane antigen and carcinoembryonic antigen in the epithelial tumor cells, for E-cadherin especially in their glandular structure, vimentin, CD99, and CD56 in the spindle cell component and for bcl-2 protein. The tumor recurred at the same site, and clinical course progressed to death 3 months after the initial diagnosis.
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Affiliation(s)
- Jesús Vera
- Department of Pathology, Hospital General San Jorge, Avenida Martinez de Velasco, 36, 22071, Huesca, Spain.
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