Laryngeal synovial sarcoma: A rare location

Nesrine Chraiet, Yosr Zenzri, Azza Gabsi, Amel Mezlini
Department of Medical Oncology, Faculty of Medicine of Tunis, Salah Azaiez Institute Tunis, El Manar University, Tunis, Tunisia

Date of Web Publication

24-Jun-2019

Correspondence Address:
Prof. Nesrine Chraiet
Department of Medical Oncology, Faculty of Medicine of Tunis, Salah Azaiez Institute Tunis, Université El Manar, Tunis
Tunisia

  Abstract 

Synovial sarcoma is an aggressive malignant mesenchymal tumor. Its incidence in oto-rhino-laryngeal location is very rare. A multidisciplinary management is recommended. We report the case of a 37-year-old male with synovial sarcoma of the larynx. We analyze the clinical, morphological, and therapeutic characteristics of this neoplasia through this observation.

Keywords: Chemotherapy, laryngeal synovial sarcoma, radiotherapy, surgery

How to cite this article: Chraiet N, Zenzri Y, Gabsi A, Mezlini A. Laryngeal synovial sarcoma: A rare location. Libyan J Med Sci 2019;3:66-7

How to cite this URL: Chraiet N, Zenzri Y, Gabsi A, Mezlini A. Laryngeal synovial sarcoma: A rare location. Libyan J Med Sci [serial online] 2019 [cited 2019 Jun 24];3:66-7. Available from: http://www.ljmsonline.com/text.asp?2019/3/2/66/261065

Introduction

Synovial sarcoma is a rare mesenchymal tumor. It represents 5% of soft-tissue sarcomas.^[1] It is common in young adult males. Synovial sarcoma is usually observed in the lower limbs.^[2] Its incidence in oto-rhino-laryngeal location is very rare, with a predilection for the hypopharynx. Complete large excision and local radiotherapy are effective in local control. Chemotherapy is indicated in the presence of metastasis.

Case Report

A 37-year-old young male, with a medical history of heavy smoking (30 pack-year smoking history) stopped for 3 years and chronic alcoholism, presented with an anterior cervical mass progressively growing for 9 months. The patient was also recently suffering from dyspnea and dysphonia. Direct laryngoscopy revealed a subglottic mass obstructing 50% of the tract. The anatomopathological examination of a biopsy concluded a biphasic synovial sarcoma, Grade III according to the French Federation of Cancer Centers Sarcoma Group (FNCLCC). A midface computed tomography (CT) scan showed an anteromedial necrotic mass of 46.2 mm × 38.6 mm budding from the glottic–subglottic area and reaching the infraglottic one [Figure 1]. This lesion caused an erosion of the thyroid cartilage. Due to worsening dyspnea, the patient underwent tracheotomy in emergency. A total laryngectomy with total thyroidectomy and cervical adenectomy was conducted. Anatomopathological features showed a spindle cell tumor with glandular differentiated areas matching with the diagnosis of biphasic synovial sarcoma. The margins were negative. Lymph nodes and thyroid were not invaded. The patient underwent adjuvant radiotherapy on the tumor bed at the dose of 51.5 Gy. Twelve months later, the CT scan revealed a 12-mm unique lesion on the superior right lung lobe [Figure 2]. A biopsy was conducted which confirmed the diagnosis of relapsed sarcoma by showing a hemangiopericytic vascularization within spindle cells positive for cytokeratin, epithelial membrane antigen (EMA), vimentin, and CD99. He underwent six courses of doxorubicin monotherapy with lung and bone progression. He is still on second-line chemotherapy by ifosfamide.

Figure 1: Midface computed tomography scan of laryngeal synovial sarcoma Click here to view

Figure 2: Lung metastasis Click here to view

Discussion

Cervical location of soft-tissue sarcoma is very rare. It represents only 1% of malignant head-and-neck tumors.^[3] Synovial sarcomas emerge from pluripotent mesenchymal cells, near or distant from joints. It represents the 4^th common sarcoma subtype, after malignant histiocytofibroma, liposarcoma, and rhabdomyosarcoma.^[4] Commonly, synovial sarcoma is seen in young adults, like our patient, with a 2.1 sex ratio. It predominates in periarticular soft tissues of the inferior limbs.^[1] The most frequent head-and-neck locations are the pharynx and the parapharyngeal space. Laryngeal synovial sarcoma is reported in only twenty cases in literature.

Clinically, it usually consists of a cervical mass of soft tissues progressively increasing with painful sensitivity to palpation. No specific imaging signs were reported except scattered calcifications that might be seen in 30% of the tumors. Magnetic resonance imaging (MRI) is the best radiological examination in soft-tissue sarcomas. Our patient did not present any calcifications in the CT scan but did not have an MRI. Histologically, two types of synovial sarcoma are known: monophasic and biphasic sarcoma. Spindle cells are found in the biphasic subtype. Some other subtypes are rare namely undifferentiated and monophasic fibrous types.^[5] In our case, the synovial sarcoma was biphasic. Immunohistochemical stains were positive for bcl2, cytokeratin, and EMA.^[6] The majority (95%) of these tumors express specific translocation t (X; 18) (p11.2; q 11.2).^[3] This translocation is correlated with the tumor production of a polymerase reverse transcriptase: SYT-SSX1; SYT-SSX2.^[7]

The cornerstone of multidisciplinary treatment is surgery. The resection should be large and complete. Recurrence-free survival is significantly increased when margins are negative (176 months vs. 33 months).^[8] The recommended margin of 5 cm for limb synovial sarcoma is not feasible in this location because of anatomy and the hemorrhagic nature of the tumor. There is no standard for the prophylactic cervical lymph node dissection. Adjuvant radiotherapy does not impact overall survival but improves locoregional control.^[9] Our patient underwent total laryngectomy with lymph node dissection and adjuvant radiotherapy at the dose of 51.5 Gy. There is no clear evidence for a chemotherapy benefit, but some experts believe that head-and-neck synovial sarcoma could delay distant metastasis in adjuvant setting and induce tumor response in metastases. Active drugs in metastatic sarcoma are doxorubicin, ifosfamide, dacarbazine, and trabectedin. Doxorubicin is the standard in the first line at the doses of 60–75 mg/m² (day 1 = day 21). No adjuvant chemotherapy was proposed to our patient. Recurrences occur in 50% of patients, especially in the first 2 years. Distant metastases are reported in 30%–42% of cases, and lungs are the most common location.^[10] Our patient had lung metastasis. He did not respond to doxorubicin and is on second-line chemotherapy with ifosfamide for lung and bone progression. The overall survival of synovial sarcoma at 5 years is 60%.^[10]

In conclusion, synovial sarcoma is a rare head-and-neck tumor. Multidisciplinary approach is mandatory which consists of a large surgery combined with adjuvant radiotherapy. It frequently relapses and has distant spread. Chemotherapy is the standard treatment for metastases. The prognosis remains poor.

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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