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Synovial sarcoma mimicking thyroid carcinoma A 28-year-old woman who is a resident of an endemic goitre region presented with anterior neck swelling of 2-year duration with rapid progression over preceding 6 months. The patient had associated voice change and dysphagia; however, there were no respiratory symptoms. She also had significant weight loss for the last 6 months. There was no history of radiation exposure or thyroid cancer in the family. On examination, a large, firm, smooth surfaced swelling was palpable in the region of thyroid (Fig. 1). Trachea was pushed anteriorly and was felt throughout its course suggesting retrotracheal nature. Fine needle aspiration cytology (FNAC) from the swelling was reported as poorly differentiated/medullary carcinoma thyroid. However, serum calcitonin was within normal limits. Neck X-ray showed retrotracheal widening (Fig. 2a) and neck contrast-enhanced computed tomography revealed a heterogeneously enhancing solid cystic lesion 12.5 × 9.5 cm with peripheral calcification in retropharyngeal space presumably arising from the left lobe of thyroid. With the diagnosis of thyroid carcinoma with retrotracheal extension, patient underwent total thyroidectomy with excision of retrotracheal tumour. Intra-operatively both lobes of thyroids appeared normal and the left lobe was connected with few fibrous bands to a large well-capsulated mass in retrotracheal location that had minimal adhesion to oesophagus and pharyngeal wall without any evidence of tumour metastasis into lateral neck nodes (Fig. 2b). Patient made an uneventful recovery. Final histopathology was suggestive of synovial sarcoma showing immunostaining for cytokeratin, bcl-2 and vimentin (Fig. 3). In view of histopathology, patient received adjuvant radiotherapy and is free of disease.
Head and neck synovial sarcoma is an extremely rare malignancy representing approximately 0.1% of all head and neck cancers.1 These tumours arise from pluripotent mesenchymal cells with no definitive relationship to articular surfaces, and are named as such for their appearance and not of their origin.2 The histopathological diagnosis of synovial cell sarcoma is challenging as a result of the variable cellular and architectural morphol-
Fig. 1. Neck swelling with tattooing.
Fig. 2. (a) Lateral X-ray neck showing retrotracheal widening. (b) Intraoperative photograph showing relation of mass with adjacent structures.
© 2015 Royal Australasian College of Surgeons
ANZ J Surg •• (2015) ••–••
2
Images for surgeons
Radiotherapy, in most cases, except those with small, low-grade lesions, should be added to maximize local control.8 Chemotherapy can be considered in neoadjuvant or adjuvant setting: tumours of >5 cm in size, clinical or imaging evidence of local extension, or a high-risk site of presentation (e.g. the paraspinal neck or skull base).6 To summarize, in endemic goitre region with large goitre burden, this entity can be mistaken for thyroid disorder. In cases FNAC revealing atypical cytological features immunocytochemical examination/trucut biopsy could have helped in clinching the diagnosis. Although overall prognosis remains poor, early detection and appropriate treatment are key to achieve better outcome.
References
Fig. 3. (a) Spindle cells arranged in nodules around small tubules in a myxoid stroma (H&E, original magnification ×10). (b) Cytokeratin staining tubular structures (immunostain, original magnification ×10).
ogy and resemblance to other neoplastic processes common to the region, and is usually misdiagnosed or misclassified by the general pathologist.3 Because this has obvious implications on treatment, an expert pathological review is vital before treatment. In our case, too, on cytological examination it was reported as thyroid carcinoma. Head and neck synovial cell sarcoma has a greater metastatic risk than synovial cell sarcoma found in extremities.4 Regional metastases occur in 12.5% of patients with head and neck synovial cell sarcoma, and up to 25% of patients present with distant metastases.2,5 Our patient did not have any regional and distant metastasis in contrast to the literature. Synovial sarcomas of neck are more aggressive with high recurrence rates and poorer prognosis as compared with tumours of the upper aero-digestive tract.6 Positive tumour margin, large tumour size and advanced tumour stage are associated with poorer overall survival.7 The mainstay of treatment of synovial cell sarcoma is complete excision with wide surgical margins. Hence, every effort should be made to resect these tumours completely during initial surgery.
1. Sturgis EM, Potter BO. Sarcomas of the head and neck region. Curr. Opin. Oncol. 2003; 15: 239–52. 2. O’Sullivan PJ, Harris AC, Munk PL. Radiological features of synovial cell sarcoma. Br. J. Radiol. 2008; 81: 346–56. 3. De Bree R, van der Valk P, Kuik DJ et al. Prognostic factors in adult soft tissue sarcomas of the head and neck: a single centre experience. Oral Oncol. 2006; 42: 703–9. 4. Mullen JR, Zagars GK. Synovial sarcoma outcome following conservation surgery and radiotherapy. Radiother. Oncol. 1994; 33: 23–30. 5. Simunjak B, Petric V, Bedekovic V, Cupic´ H, Hat J. Dimensions and outcome of synovial sarcoma of the head and neck: case presentation and review of the literature. J. Otolaryngol. 2005; 34: 420–3. 6. Harb WJ, Luna MA, Patel SR, Ballo MT, Roberts DB, Sturgis EM. Survival in patients with synovial sarcoma of the head and neck: association with tumor location, size, and extension. Head Neck 2007; 29: 731– 40. 7. Kraus DH, Dubner S, Harrison LB et al. Prognostic factors for recurrence and survival in head and neck soft tissue sarcomas. Cancer 1994; 74: 697–702. 8. Tejani MA, Galloway TJ, Lango M, Ridge JA, von Mehren M. Head and neck sarcomas: a comprehensive cancer center experience. Cancers (Basel) 2013; 5: 890–900.
Naval Bansal,* MS, MCh Ranjana S. Ranade,† MD Anjali Mishra,* MS, PDC Departments of *Endocrine Surgery and †Pathology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India doi: 10.1111/ans.13113
© 2015 Royal Australasian College of Surgeons
Synovial sarcoma mimicking thyroid carcinoma A 28-year-old woman who is a resident of an endemic goitre region presented with anterior neck swelling of 2-year duration with rapid progression over preceding 6 months. The patient had associated voice change and dysphagia; however, there were no respiratory symptoms. She also had significant weight loss for the last 6 months. There was no history of radiation exposure or thyroid cancer in the family. On examination, a large, firm, smooth surfaced swelling was palpable in the region of thyroid (Fig. 1). Trachea was pushed anteriorly and was felt throughout its course suggesting retrotracheal nature. Fine needle aspiration cytology (FNAC) from the swelling was reported as poorly differentiated/medullary carcinoma thyroid. However, serum calcitonin was within normal limits. Neck X-ray showed retrotracheal widening (Fig. 2a) and neck contrast-enhanced computed tomography revealed a heterogeneously enhancing solid cystic lesion 12.5 × 9.5 cm with peripheral calcification in retropharyngeal space presumably arising from the left lobe of thyroid. With the diagnosis of thyroid carcinoma with retrotracheal extension, patient underwent total thyroidectomy with excision of retrotracheal tumour. Intra-operatively both lobes of thyroids appeared normal and the left lobe was connected with few fibrous bands to a large well-capsulated mass in retrotracheal location that had minimal adhesion to oesophagus and pharyngeal wall without any evidence of tumour metastasis into lateral neck nodes (Fig. 2b). Patient made an uneventful recovery. Final histopathology was suggestive of synovial sarcoma showing immunostaining for cytokeratin, bcl-2 and vimentin (Fig. 3). In view of histopathology, patient received adjuvant radiotherapy and is free of disease.
Head and neck synovial sarcoma is an extremely rare malignancy representing approximately 0.1% of all head and neck cancers.1 These tumours arise from pluripotent mesenchymal cells with no definitive relationship to articular surfaces, and are named as such for their appearance and not of their origin.2 The histopathological diagnosis of synovial cell sarcoma is challenging as a result of the variable cellular and architectural morphol-
Fig. 1. Neck swelling with tattooing.
Fig. 2. (a) Lateral X-ray neck showing retrotracheal widening. (b) Intraoperative photograph showing relation of mass with adjacent structures.
© 2015 Royal Australasian College of Surgeons
ANZ J Surg •• (2015) ••–••
2
Images for surgeons
Radiotherapy, in most cases, except those with small, low-grade lesions, should be added to maximize local control.8 Chemotherapy can be considered in neoadjuvant or adjuvant setting: tumours of >5 cm in size, clinical or imaging evidence of local extension, or a high-risk site of presentation (e.g. the paraspinal neck or skull base).6 To summarize, in endemic goitre region with large goitre burden, this entity can be mistaken for thyroid disorder. In cases FNAC revealing atypical cytological features immunocytochemical examination/trucut biopsy could have helped in clinching the diagnosis. Although overall prognosis remains poor, early detection and appropriate treatment are key to achieve better outcome.
References
Fig. 3. (a) Spindle cells arranged in nodules around small tubules in a myxoid stroma (H&E, original magnification ×10). (b) Cytokeratin staining tubular structures (immunostain, original magnification ×10).
ogy and resemblance to other neoplastic processes common to the region, and is usually misdiagnosed or misclassified by the general pathologist.3 Because this has obvious implications on treatment, an expert pathological review is vital before treatment. In our case, too, on cytological examination it was reported as thyroid carcinoma. Head and neck synovial cell sarcoma has a greater metastatic risk than synovial cell sarcoma found in extremities.4 Regional metastases occur in 12.5% of patients with head and neck synovial cell sarcoma, and up to 25% of patients present with distant metastases.2,5 Our patient did not have any regional and distant metastasis in contrast to the literature. Synovial sarcomas of neck are more aggressive with high recurrence rates and poorer prognosis as compared with tumours of the upper aero-digestive tract.6 Positive tumour margin, large tumour size and advanced tumour stage are associated with poorer overall survival.7 The mainstay of treatment of synovial cell sarcoma is complete excision with wide surgical margins. Hence, every effort should be made to resect these tumours completely during initial surgery.
1. Sturgis EM, Potter BO. Sarcomas of the head and neck region. Curr. Opin. Oncol. 2003; 15: 239–52. 2. O’Sullivan PJ, Harris AC, Munk PL. Radiological features of synovial cell sarcoma. Br. J. Radiol. 2008; 81: 346–56. 3. De Bree R, van der Valk P, Kuik DJ et al. Prognostic factors in adult soft tissue sarcomas of the head and neck: a single centre experience. Oral Oncol. 2006; 42: 703–9. 4. Mullen JR, Zagars GK. Synovial sarcoma outcome following conservation surgery and radiotherapy. Radiother. Oncol. 1994; 33: 23–30. 5. Simunjak B, Petric V, Bedekovic V, Cupic´ H, Hat J. Dimensions and outcome of synovial sarcoma of the head and neck: case presentation and review of the literature. J. Otolaryngol. 2005; 34: 420–3. 6. Harb WJ, Luna MA, Patel SR, Ballo MT, Roberts DB, Sturgis EM. Survival in patients with synovial sarcoma of the head and neck: association with tumor location, size, and extension. Head Neck 2007; 29: 731– 40. 7. Kraus DH, Dubner S, Harrison LB et al. Prognostic factors for recurrence and survival in head and neck soft tissue sarcomas. Cancer 1994; 74: 697–702. 8. Tejani MA, Galloway TJ, Lango M, Ridge JA, von Mehren M. Head and neck sarcomas: a comprehensive cancer center experience. Cancers (Basel) 2013; 5: 890–900.
Naval Bansal,* MS, MCh Ranjana S. Ranade,† MD Anjali Mishra,* MS, PDC Departments of *Endocrine Surgery and †Pathology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India doi: 10.1111/ans.13113
© 2015 Royal Australasian College of Surgeons
