Submandibular synovial sarcoma mimicking a vascular lesion: A rare case report

Naresh Kumar; Neeraj Kumar Dhiman; Shankar Singh; Ajit Kumar Vishwakarma

doi:10.4103/srmjrds.srmjrds_76_17

CASE REPORT

Year : 2018 | Volume : 9 | Issue : 2 | Page : 100-102

Submandibular synovial sarcoma mimicking a vascular lesion: A rare case report

Naresh Kumar, Neeraj Kumar Dhiman, Shankar Singh, Ajit Kumar Vishwakarma
Department of Oral and Maxillofacial Surgery, Faculty of Dental Sciences, Institute of Medical Sciences, Banaras Hindu University, Varanasi, Uttar Pradesh, India

Date of Web Publication

18-Jun-2018

Correspondence Address:
Shankar Singh
Department of Oral and Maxillofacial Surgery, Faculty of Dental Sciences, Institute of Medical Sciences, Banaras Hindu University, Varanasi, Uttar Pradesh
India

Source of Support: None, Conflict of Interest: None

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DOI: 10.4103/srmjrds.srmjrds_76_17

Abstract

Synovial sarcoma is a soft-tissue sarcoma of periarticular surfaces in close association with tendon sheaths, bursae, and joint capsules. Synovial sarcoma represents 5.6%–10% of all soft-tissue sarcomas. Knox suggested the name synovial sarcoma in the year 1936. These tumors are usually not found in soft tissues of head and neck region, and majority of tumors believed to take origin from paravertebral connective tissue spaces and manifest as parapharyngeal and solitary retropharyngeal masses near the carotid bifurcation. We present a rare case of biphasic synovial sarcoma of the submandibular region in a young male patient.

Keywords: Biphasic, malignant tumor, synovial sarcoma

How to cite this article:
Kumar N, Dhiman NK, Singh S, Vishwakarma AK. Submandibular synovial sarcoma mimicking a vascular lesion: A rare case report. SRM J Res Dent Sci 2018;9:100-2

How to cite this URL:
Kumar N, Dhiman NK, Singh S, Vishwakarma AK. Submandibular synovial sarcoma mimicking a vascular lesion: A rare case report. SRM J Res Dent Sci [serial online] 2018 [cited 2023 May 28];9:100-2. Available from: https://www.srmjrds.in/text.asp?2018/9/2/100/234595

Introduction

Synovial sarcoma is a malignant tumor of pluripotent mesenchymal cells.^{[1],[2],[3],[4]} This tumor is commonly found in periarticular tissues, with few cases occurring in the head and neck region.^{[1],[2],[3],[4],[5]} Guadagnolo reported a large series of 150 cases of synovial sarcoma and only 9 involving the head and neck region.^[6] The first reported case of synovial sarcoma of head and neck region was described by Jernstrom in 1954 involving the pharynx.^[7] Male adolescents and young adults are most frequently affected, mainly involving the deep soft tissue of the extremities, especially in the knee area.^[8],[9]

Case Report

A 24-year-old man reported to outdoor unit of our hospital with 6 months history of painless, well-demarcated, firm swelling of about 6 cm × 4 cm × 5.5 cm extending from submental region to angle of mandible [Figure 1]. Overlying skin was normal in texture without any surface temperature elevation and ulceration. The patient underwent fine-needle aspiration cytology (FNAC); findings were suggestive of nonspecific vascular pathology with hemorrhagic aspirate. Computed tomography (CT) face angiography imaging features were more in favor of hypervascular solid cystic tumor with internal hemorrhage and intense enhancement of solid component in arterial and venous phase and feeding artery from left external carotid artery [Figure 2]. Submandibular gland and carotid body were uninvolved. Differential diagnosis of lymphoma, dermatofibroma, dermoid cyst, chondroma of thyroid cartilage, and arteriovenous malformation were suspected. Surgical removal of the tumor with ligation of left external carotid artery was proposed under general anesthesia. The lesion was exposed through submandibular incision given in second crease of skin of neck from midline up to the posterior border of sternocleidomastoid muscle. External carotid artery was dissected, identified through its branches and ligated as a precautionary measure. Tumor was well defined and filled with semisolid material [Figure 3]. Tumor was excised and sent for histopathological examination. Histopathological sections [Figure 4] and [Figure 5] show a biphasic tumor with epithelial and spindle cell component. Epithelial component consists of large pale cells with abundant cytoplasm arranged in glands, cords, and nests. Glandular lumen shows eosinophilic secretions. Spindle cell component shows small spindle cells arranged in fascicles having round cytoplasm. Mitotic figure 10/hpf identified with focal areas of hemorrhage. Necrosis was also noted. Patient was advised for radiation therapy immediately.

Figure 1: Submandibular swelling extending from midline to angle of mandible

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Figure 2: Computed tomography face angiography imaging showing hypervascular solid cystic tumor with feeding artery from left external carotid artery

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Figure 3: Intraoperative image showing lesion

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Figure 4: Photomicrograph showing epithelial cells and spindle cells (×20)

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Figure 5: Photomicrograph showing epithelial cells and spindle cells with areas of necrosis (×40)

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Discussion

Synovial sarcoma is a soft-tissue tumor occurring in the biphasic or monophasic type and consisting of epithelial and/or spindle cell components. Biphasic type occurs more commonly. Synovial sarcoma is a rare but one of the most common types of malignant nonrhabdomyosarcomatous soft-tissue sarcoma.^[10] In the head and neck region, synovial sarcoma originates from primitive mesenchyma with no synovial association.^[11],[12] Patients usually present a painless slow-growing mass during their third and fourth decade of life with associated compressive or infiltrative symptom of surrounding structures.^[10] Synovial sarcoma mainly affects male adolescents and young adults. Consistent with the literature, our patient too presented with painless left submandibular mass which was gradual in onset, slow-growing mass; however, he did not complain of any compressive or infiltrative symptoms. Some of the predisposing factors to development of sarcoma include genetic factors, viral infection in immunocompromised patients, irradiation, chronic lymphedema, environmental carcinogens, and trauma.^[10] Diagnosis of synovial sarcoma is very difficult for oral pathologists because of its relatively rare occurrence in maxillofacial region. On CT scan or magnetic resonance imaging (MRI) scan, synovial sarcoma usually demonstrates features of homogeneous solid mass. However, it may present heterogeneous signals based on the degree of hemorrhage, necrosis, or calcification.^[13] Recent study by Tateishi et al.^[14] showed that CT and MRI scans allow prognostic prediction in patients with synovial sarcoma. Proximal distribution, tumor size larger than 5 cm, absence of calcification, presence of hemorrhage, and presence of triple signal pattern are significantly associated with disease-free survival rate. Despite relatively slow growth, long-term survival for synovial sarcomas from anywhere, including the head and neck, is not good.^[5] Synovial sarcoma in the head and neck region has an aggressive nature and a reserved prognosis.^[3] Several factors have been evaluated for prognosis, and the most significant were tumor size and deep extension at the time of primary treatment with survival varying inversely with these two factors.^[5],[11]

Conclusion

Treatment of synovial sarcoma is essentially surgical resection although tumor is sensitive to chemotherapy and radiotherapy. It is more likely to be misdiagnosed because of rarity of this neoplasm in the maxillofacial region. An oral and maxillofacial surgeon should be familiar with this aggressive tumor, which carries high mortality, morbidity, and relapse rate. Synovial sarcoma should be considered in the differential diagnosis of well-defined inhomogeneous tumors of submandibular region in young adults.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

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