SYNOVIAL SARCOMA OF THE HEAD AND NECK Roman P. Bukachevsky, MD, Robert L. Pincus, MD, Frank G. Shechtman, MD, Edward Sarti, MD, and Paul Chodosh, MD

Primary synovial sarcoma is an unusual tumor of the head and neck. Fewer than 75 cases have been reported in the literature. We have treated 7 additional cases; 3 in the hypopharynx, 2 in the parapharyngeal space, 1 in the oral pharynx and 1 in the posterior triangle of the neck. An enlarging cervical mass, voice change, and dysphagia were among the presenting complaints. CT revealed solitary nonhomogenous tumors from 3 to 7 cm in diameter. Microscopically, all cases showed a biphasic cellular pattern verified by immunohistochemical staining. Multimodality treatment consisted of surgery and postoperative radiation therapy with 3 patients receiving chemotherapy. Although the original pathology report was incorrect in 3 cases, clinical suspicion for synovial sarcoma ensured proper diagnosis. HEAD & NECK 1992;14:44-48

been recorded. Roth and associates' reviewed the pathologic features of 24 patients with synovial sarcoma of the neck on file at the Armed Forces Institute of Pathology (AFIP). In a more recent AFIP review, Enzinger and Weiss3 documented 31 additional cases occurring in the head and neck. Fewer than 75 cases have been reported to date in the literature. We have treated 7 new cases with follow-up from 2 t o 23 years and report our findings herein. CASEREPORTS

In 1967, a 12-year-old girl was seen for complaints of sore throat and minimal respiratory distress. On examination of the oral pharynx, a 4-cm pedunculated lesion was noted arising from the left posterior tonsillar pillar extending to the supraglottis. An excisional biopsy was reported as synovial sarcoma. The patient then underwent tonsillectomy, partial pharyngectomy, and removal of a portion of the base of tongue, with clear margins. Multimodality treatment consisted of 2 courses of 0.5 mg intravenous dactinomycin for 5 days administered during the first and sixth week of cobalt 60 radiation therapy. A total dose of 5800 cGy was delivered through a 6 x 6 cm single lateral portal. Because of leukopenia, her third course of dactinomycin was given 1 month later. She is alive and free of disease 23 years later. Case 1.

Despite its name, synovial sarcoma rarely arises directly from synovial membranes. More commonly the tumor is found in the vicinity of large joints and bursae, especially in the extremities. Location in the head and neck, a region poor in synovioblastic tissue, is uncommon. The first case was reported by Jersntrom in 1954.' Since then, isolated case reports have

Presented at the American Laryngological, Rhinological and Otological Society, Eastern Section Meeting, New Haven, Connecticut, January 27, 1990. From the Department of Otolarygnology-Head and Neck Surgery, New York Eye & Ear Infirmary, New York Medical College, New York. New York. Address reprint requests to (new address) Roman P Bukachevsky, 201 South Lewis St.. #114, Orange, CA 92668. Accepted for publication May 12, 1991 CCC 0148-6403192101044-05 $04.00 0 1992 John Wiley & Sons, Inc

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Synovial Sarcoma of the Head and Neck

In 1971, a 21-year-old woman offered a 16-month history of muffled voice and was found

Case 2.

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to have a cyst in her oropharynx. Excisional biopsy was reported as adenocarcinoma arising in a minor salivary gland. The patient was treated with cobalt 60 via anterior and opposed lateral 6 X 6 cm ports to a total dose of 6000 cGy. One year later a follow-up examination showed a large submucosal hypopharyngeal mass extending into the pyriform with direct extension to the midjugular area. Clinical suspicion prompted review of the original pathology slides. A diagnosis of primary synovial sarcoma was made. A total laryngopharyngectomy with radical neck dissection was performed. The patient developed pulmonary metastases and died 2 years later. In 1972, a 21-year-old man was initially seen with a 4-centimeter slowly enlarging mass located high in the right posterior triangle of his neck just behind the mastoid tip. Wide local excision revealed a biphasic synovial sarcoma with positive tumor margins on permanent section. He received 6000 cGy using cobalt 60 in 30 treatments over 57 days using opposed anteriorposterior fields measuring 11 x 16 cm. Four years later he developed a 2-cm recurrence in the occipital area which was surgically excised with negative margins for tumor. In 1986, he developed a spinal metastasis a t T4. A laminectomy was followed by 2800 cGy in 14 treatments delivered over 60 days with a 6-MeV linear accelerator. The following year another thoracic spine recurrence required surgery with postoperative radiation therapy. A 6-MeV linear accelerator was used to deliver 3600 cGy in 12 treatments over 26 days using a portal encompassing T8-Tl2. The patient developed pulmonary metastases and died in 1987, 15 years after the initial diagnosis. Case 3.

Case 4. In 1974, a 22-year-old woman sought medical care for voice change with progressive dysphagia over the past year. Intraoral inspection revealed a left parapharyngeal mass with shifting of the soft palate and tonsil toward the midline. A fullness of the left submandibular and parotid areas was noted. Surgical excision of the tumor with partial mandibulectomy was performed. Pathology was consistent with synovial sarcoma (negative margins). Cobalt 60 radiation therapy delivered through a 8 x 11 cm lateral port to a total dose of 5000 cGy was given over 5 weeks. One week later, 3 courses of chemotherapy (200 mg cyclophosphamide, 1mg vincristine, 10 mg doxorubicin per course) were given over a

Synovial Sarcoma of the Head and Neck

3-week period. The patient developed pulmonary metastases 3 years postoperatively and died. Case 5. In 1987, a 27-year-old woman complained of a foreign body sensation in her pharynx for 3 months, with recent onset of dysphagia and dyspnea. Physicial examination revealed a right lateral pharyngeal mass starting a t the lower pole of the tonsil extending inferiorly and projecting over the larynx. A CT scan revealed a right hypopharyngeal mass questionably involving retropharyngeal tissues, with calcifications present. The mass did not involve the larynx or epiglottis. After tracheostomy, direct laryngoscopy and biopsy were performed. A diagnosis of biphasic synovial sarcoma was made. She underwent a right supraomohyoid neck dissection followed by midline mandibulotomy with resection of the tumor of the pharyngeal wall, tonsil, and tongue base. The margins of resection were negative. The patient was treated with a 6-MeV linear accelerator to a total dose of 5040 cGy in 28 treatments over 57 days using opposed lateral ports measuring 7 X 13 cm. After receiving 4500 cGy, the ports were reduced to 5.5 x 13 cm to avoid further radiation to the spinal cord. She has no evidence of disease 4 years later.

In 1987, a 17-year-old girl had a 3-month history of progressive neck swelling and recent voice changes. She had been treated for acute tonsillitis with oral antibiotics several months prior. Despite treatment, cervical adenopathy persisted necessitating endoscopy. Biopsy of a hypopharyngeal mass was reported as chronic inflammation. CT revealed a large neck mass which extended into the hypopharynx. Repeat endoscopy and biopsy revealed synovial sarcoma. Preoperatively she received both chemotherapy and radiation therapy. Each course of chemotherapy consisted of cyclophosphamide (50 mg/m2), vincristine (1.5 mg/m2), and doxorubicin (75 mg/m2>administered every 3 weeks for 12 weeks. During the tenth treatment week the patient developed toxicity and the vincristine dose was reduced by 50%to 1 mg per course. Concurrent radiation therapy was delivered via a 6 MeV linear accelerator to a total dose of 4000 cGy over 5 weeks. The patient underwent tracheostomy, wide local excision with partial pharyngectomy, neck dissection, and reconstruction with a right radial forearm free flap. Surgical margins were negative for tumor. Postoperatively, chemotherapy was continued for 60 Case 6.

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weeks. Cardiac toxicity from doxorubicin (at a total dose of 360 mg) necessitated its removal from the regimen. The patient was continued on vincristine and cyclophosphamide,with the addition of 2.3 mg dactinomycin per course. The patient remains disease-free at 3-year follow-up. In 1989, a 33-year-old Hispanic man sought medical attention for a painless progressively enlarging left neck mass. Most recently he complained of hoarseness and compressive symptoms. Physical examination yielded a 4-cm mass in the left submanidibular triangle with mild bulging of the left tonsil and lateral pharyngeal wall. CT with contrast delineated a nonenhancing mass of multiple densities in the left parapharyngeal space with a shift of the larynx to the opposite side. The patient underwent tracheostomy with wide local excision of the mass via an extended midline mandibulotomy approach. Surgical pathology was reported as undifferentiated carcinoma with clear margins of resection. A revised diagnosis of biphasic synovial sarcoma was made after further pathologic review. The patient was treated with 5040 cGy over a 6-week period using cobalt 60 via opposed lateral ports (10 x 14 cm) and 1 anterior port of 7 X 20 cm dimension. He developed a cervical recurrence just outside (anterior) to the left treatment field 1.5 years later.

Case 7.

DISCUSSION

As with synovial sarcomas of the extremities, there is a reported male predominance in the head and neck. In our series females (5) outnumbered males (2) (Table 1).Patients are more fre-

quently affected in the early years of life (second to third decades). A progressively enlarging painless neck mass of several months' duration is the usual first complaint. Five of our 7 patients were initially seen with hoarseness, dyspnea, or dysphagia because of the tumor's compressive effects upon the larynx and pharynx. Synovial sarcoma may be located high in the superior aspect of the neck, in the prevertebral area from the base of skull to the hyp~pharynx,~ in the retropharyngeal and parapharyngeal areas, and in the anterior neck along the borders of the sternocleidomastoid, as well as in the orofacia1 and laryngeal sites.' They vary in size depending on their location and relationship to adjacent structures, growing somewhat larger in the neck than in the orofacial r e g i ~ nHypopha.~ ryngeal and parapharyngeal tumors were the largest in our series. Findings at surgery yielded well-circumscribed spherical multinodular or lobulated tumors covered by a thin fibrous membrane or pseudocapsule. Cystic and hemorrhagic foci were present (Figure 1). Histologically, synovial sarcomas show a biphasic cellular pattern consisting of a stroma of fibroblast spindle-like cells in which are scattered pale epithelial-like cells arranged in glandular formations, nests, or cleft-like spaces. The epithelial-like cells may be cuboidal or columnar in shape and form papillary projections. Mast cells and hyalinized scar-like areas may be found (Figure 2). Microcalcification has been found in 30% to 60% of cases, and can be detected by radiologic evaluation.6 Biphasic synovial sarcoma does not really exhibit synovial differentiation, but rather epithelial differentia-

Table l . Clinical data of 7 cases of primary synovlal sarcoma of the head and neck. Tumor size (cm)

Case

Ageisex

Anatomical site

Treatment

FOIIOW-UP

1

121F

Oral pharynx

4

NED 23 years

6

Excision, radiotherapy, chemotherapy Excision

2

21lF

Hypopharyngeal

3

21lM

Neck, posterior triangle

4

Excision, radiotherapy

4

22lF

Paraphyarngeal

7

5 6

271F 171F

Hypopharyngeal Hypopharyngeal

3 4

7

33lM

Parapharyngeal

6

Excision, radiotherapy, chemotherapy Excision, radiotherapy Chemotherapy, radiotherapy, excision Excision, radiotherapy

Dead, pulmonary metastases 2 years postop Dead, pulmonary metastases 15 years postop Dead, pulmonary metastases 3 years postop NED 4 years NED 3 years Recurrence 1.5 years

NED, no evidence of disease

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Synovial Sarcoma of the Head and Neck

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FIGURE 1. Pathologic specimen, cut in half, showing a bbulated mass covered by a thin pseudocapsule.

tion. Some pathologists believe that a monophasic form exists consisting entirely of spindle-like or epithelial-like cells.7 The myriad of histopathologic features is responsible for the divergence in diagnosis and ini-

tial misdiagnosis. Synovial sarcomas may be confused with mixed salivary tumors, fibrosarcoma, or spindle cell carcinoma, The occasional presence of calcification may misleadingly point to a metastatic thyroid tumor. Reticulin and PAS stains help to define the lesion based on its architectural characteristics. The use of immunocytochemical markers may help confirm the nature of the tumor. Cytokeratin stains positive for keratin found in the epithelial-like areas. Vimentin stains positive in the spindle-like mesenchymal areas. Both cell types can stain positive for cytokeratin; however, the number of spindlelike cells that stain keratin positive is usually small.8 Electron microscopy suggests that these tumors do not derive directly from synovia] cells despite their synovial-like appearance by light mi~roscopy.~ Like Batsakis," Roth' suggests that synovial sarcomas originate from undifferentiated or pluripotential mesenchymal cells, thus explaining their occurrence in areas devoid of normal synovial tissue. Currently, the primary mode of therapy is

FIGURE 2. Photomicrograph demonstrating biphasic cellular pattern of synovial sarcoma. Hematoxylin & eosin, x 20.

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surgical excision. Synovial sarcomas tend to spread beyond the visible and palpable limits of tumor. Radical procedures in the head and neck to achieve adequate margins are difficult to accomplish without sacrifice of vital structures. For these reasons synovial sarcomas recur locally."In the present study all patients except for 1 (case 3) had negative margins on permanent pathologic section. Nevertheless, 2 patients (cases 2 and 7) recurred locally. Most metastases are blood borne, although up to 20% spread through lymphatic channels to regional nodes." Routine lymph node dissection is usually not performed unless adenopathy is present. None of the 4 patients in our series who underwent neck dissection had lymph node metastases. Although most sarcomas of the extremities are radioresistant, postoperative radiotherapy is advocated in the head and neck for improved local control rates.l2,l3 Over the years, both the number and size of the treatment ports utilized in our series were increased. It is noteworthy that case 7 recurred just outside (anterior) to the left 10 X 14 cm treatment port. The aggressive nature of this disease in the head and neck and its poor prognosis are reaffirmed.13 The value of adjuvant chemotherapy is not firmly established as far as enhancing diseasefree survival. Lindberg found no improvement

when comparing patients treated postoperatively with radiotherapy alone and those receiving chemotherapy in addition to postoperative radiation.14 A prospective study at the National Institutes of Health is currently underway (personal communication). Most series report 40% to 50% &year survival rates.15 Our series had a 29% mortality at 3 years with an overall 43% mortality. The appearance of pulmonary metastases is usually responsible for the patient's demise (cases 2-4). As in synovial sarcomas at other sites, the size of the primary tumor may be the most important prognostic indicator. Our data support this hypothesis. Patients whose primary tumor was larger than 4 cm in diameter had a poorer outcome. Calcifying type synovial sarcoma has been reported to have a better prognosis.16 In summary, our cases had different demographic and clinical presentations from those most commonly found in synovial sarcoma of the head and neck. In three cases, clinical suspicion prompted further pathologic review and provided the correct diagnosis. It is important for the head and neck surgeon to be familiar with this tumor whose aggressive behavior carries high morbidity and mortality rates. Early proper diagnosis and treatment may improve the prognosis.

REFERENCES

1. Jernstrom P. Synovial sarcoma of the pharynx: report of a case. Am J Clin Pathol 1954;24:957-961. 2. Roth JA, Enzinger FM, Tannenbaum M. Synovial sarcoma of the neck: a follow-up study of 24 cases. Cancer 1975;35:1243- 1253. 3. Enzinger FM, Weiss SW. Soft tissue tumors. St. Louis: CV Mosby, 1983. 4. Gapanavicius BG, Behar AI, Chisin P. Synovial sarcoma of the hypopharynx. Ann Otol 1978;87:356-359. 5. Schmookler BM, Enzinger FM, Brannon RB. Orofacial synovial sarcoma. Cancer 1982;50:269-276. 6. Cadman NL, Soyle EH, Kelly PJ. Synovial sarcoma: a n analysis of 134 tumors. Cancer 1965;18:613-627. 7. Krall RA, Kostianovsky M, Patchefsky AS. Synovial sarcoma: A clinical, pathological and ultrastructural study of 26 cases supporting the recognition of a monophasic variant. Am J Surg Pathol 1981;5:137. 8. Corson JM, Weiss LM, Banks-Schlegel SP. Keratin proteins in synovial sarcoma. Am J Surg Pathol 1983;7:107- 109. 9. Michaelson MF, Brown GA, Maynard JA. Synovial sarcoma: an electron microscopic study of monophasic and biphasic forms. Cancer 1980;45:2109-2118.

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Synovial Sarcoma of the Head and Neck

10. Batsakis JG. Tumors of the head and neck: Clinical and pathological considerations. Baltimore: Williams & Wilkins, 1973357. 11. Carabell SC, Goodman RL. Radiation therapy for soft tissue sarcoma. Semin Oncol 1981;8:201-206. 12. Lindberg RD, Martin RG, Romsdahl MM. Conservative surgery and postoperative radiotherapy in 300 adults with soft tissue sarcomas. Cancer 1981;47:2391-2397. 13. Moore, Berke GS. Synovial sarcoma of the head and neck. Otolaryngol Head Neck Surg 1987;113:311-313. 14. Lindberg RD, Murphy WK, Benjamin RS. Adjuvant chemotherapy in the treatment of primary soft tissue tumors: A preliminary report. In: Martin RC, Ayala AG, eds. Management of primary bone and soft tissue tumors. Chicago: Year Book Medical Publishers, 1977:343-352. 15. Kyriakos M. Pathology of selected soft tissue tumors of the head and neck. In: Thawley SE, Panje WR, eds. Comprehensive management of head and neck tumors, 01 2. Philadelphia: WB Saunders, 1987:1265- 1267. 16. Varela-Dyran J, Enzinger FM. Calcifying synovial sarcoma. Cancer 1982;50:345-352.

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Synovial sarcoma of the head and neck.

Primary synovial sarcoma is an unusual tumor of the head and neck. Fewer than 75 cases have been reported in the literature. We have treated 7 additio...
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