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Objectives: To investigate the relationship between pathological diagnosis and clinical outcome of synovial sarcoma (SS) of head and neck. Methods:39 cases of SS in head and neck region from 1966 to 2011 was retrospectively studied by reviewing the pathological slices of the operative specimen and followed-up from 1 to 16 years with the mean time of four years postoperatively. Results: All patients are males aged from 8 to 66 years old. Pathologically, 18 cases (46%) are biphasic, 17 cases (44%) are monophasic and 3 cases (7%) are low-differentiated SS. No lymphatic metastasis was observed during the 1 to 16 years follow-up. 16 patients got adjuvant radiothe-rapy or chemotherapy. 9 patients died butno death was directly associated with SS. Conclusion: The loco-regional control of SS seemed related closely to the tumor location rather than pathological patterns. SS of head and neck is a special entity that has good prognosis even after several times of recur-rence, cytogenetic methods are recommend-ed to be employed to ascertain the diagnosis.

1Wen Li, MD,DDS; 1Changlin Li, MD; 2Hongying Zhang, PhD, MDDepartment of 1Otolaryngology Head &neck Surgery2 Pathology, West China Hospital, Sichuan University

In addition to very rare locations such as larynx, hypopharynx, orbit, esophagus, nasopharynx and parotid gland, the disease demonstrated a predilection for lateral upper neck. Pathologically, one case ( 3% ) was dia-gnosed as non-diferentiated SS.

22 cases (56.4%) recurred among which 6 cases(15.4%) experien-ced repeated recurrence with the most times up to 4 after sole surgical app-roach. The recurrence rate was 55.6%(10/18), 64.7%(11/17) for the biphasic and monophasic SS respectively (p>0.05). No recurrence took place in low-diferentiated group after postoperative radiotherapy. No lymphatic metastasiswas observed during the 1 to 16 years follow-up (case1, 16ys follow-up). One patient got scalp, arms, thoracic, abdominal wall and vertebral metastases during the postoperative 6-years follow-up (case2), one patient got suspicious pulmonary metastatic foci but kept still in growth and not being pathologically confirmed during the postoperative 2-years follow-up. 16 patients got adjuvant radiotherapy (16/16) or chemotherapy (3/16). The distant metastases were accompanied by increased recurrence and related operations in 4 cases (10%). 9 patients died but no death was directly associated with SS.

SS is classified histologically into two forms: monophasic and biphasic. Low-diferentiated SS was also advocated, even non-diferentiated type was also adopted. The most distinctive histologic feature of SS is its biphasic nature (epithelial and spindle), but more differentiations by immunocytoche-mistry or cytogenetics are also important especially in those places where SS rarely happened, such as larynx and esophagus, to exclude diagnostic dilemma and guide treatment plan.

There is no standard therapeutic approach based on large quantity of cases research for this disease. Given the general properties of malignant tumor, a radical resection with safe margin was recommended [2]. Postope-rative adjuvant radiotherapy may play an important role in recurrent cases or low-diferentiated category, or when the skull base, prevertebral struc-tures are involved because the compli-cated anatomy always means difficu-lties to resect with safe margin.

39 cases of SS in head and neck region from 1966 to 2011 was retrospectively studied by reviewing the histopathological slices of the speci-men, immunohistochemistry and a few cytogenetic analyses including RT-PCR ,fluorescence in situ hybridization (FISH) and DNA sequence analysis performed. The followe-up period ranged from 1 to 16 years with the mean time of 4 years postoperatively and the result was analysed with SPSS.

The loco-regional control of SS seemed related closely to the location of the tumor rather than pathological patterns. SS of head and neck is a special entity that has good prognosis even after several times of recurren-ce. Cytogenetic methods are recommended to be employed to ascertain the diagnosis in order to choose reasonable treatment protocols, to under-stand the cinical procedure and predict prognostic outcome.

Synovial sarcoma (SS) is a spindle cell tumor unrelated to mature synovial tissue, and displays variable epithelial differentiation, including glandular formation and even cartilaginous or osseous tissue formation. It has a specific chromosomal translocation t(X;18)(p11;q11). Over 80% arise in the extremities, especially around the knee [1]. SS of head and neck are extremely rare representing 5~10% of head and neck sarcoma. A limited number of SS have been reported in the parapharyngeal space, larynx, hypopharynx, and maxillofacial region. The treatment modality is controversial and the prognosis is variable with respect of tumor location, pathological categories, safe margin of resection and adjuvant postoperative radiotherapy.

INTRODUCTION

METHODS AND MATERIALS

1.Iyad S, Carlos RG, Raya S, et al. Comparing children and adults with synovial sarcoma in the surveillance, epidemiology, and end results program, 1983 to 200-5. Cancer. 2009;115: 3537-47

2.Kartha SS, Bumpous JM. Synovial sarcoma: diagno-sis, treatment, and outcomes. Laryngoscope 2002; 112: 1979–1982

CONCLUSIONS

DISCUSSION

RESULTS

REFERENCES

ABSTRACT

CONTACT

Synovial Sarcoma of Head and Neck: from Pathological Diagnosis to Clinical Outcome

Wen Li, Department of Otolaryngology Head &neck Surgery, West China Hospital, Sichuan University,610041Email: church.ent.wc@163.com

Fig1. case1 CT demonstrating a mass in the oropharynx without clear origin ( A, green arrow), with right cervical lymph nodes enlargement (B, black arrow ) but no meta-stasis as a result

Fig2. case1 the neoplasm at the aryepiglot-tic fold with 3 parts like a string of beads locating at the piryform sinus, laryngeal entrance and entrance of esophagus (C) cross section of tumor (D)

Fig3. case1 FISH demonstrating a balanced rearrangement of SS18 locus in the majority of neoplastic cells (normal locus, yellow; rearran-ged locus, separation of green and red signals).

E F

Fig4. case 2 tumor locating in the parapharyngeal space with a clear margin anterior to the carotid artery (E) and cross section of the tumor (F) demonstrating the heterogenei-ty correlating the CT quite well

Fig5. case 2 DNA sequence with SS18-SSX1 fusion, the patient is still alive one year after the third operation with larger mass in the abdominal wall and new vertebral metastasis

Fig6.case 2 tumor resection and defect repair (G), lateral view (H) 1 year after the 3rd operation, vertebral (I) and abdominal wall (J) metastases

G H I J