Synovial Sarcoma of the Head and NeckDennis M. Moore, MD, Gerald S. Berke, MD

\s=b\Synovial sarcoma, a malignant soft-tissue tumor that occurs primarily in theextremities, is seen rarely in the head andneck. Although known to behave aggres-sively in the extremities, recent reportssuggest that the course of synovial sarco-ma may be more indolent when it occursin the head and neck. Five new cases ofsynovial sarcoma of the head and neckare described herein. Of four patientswith adequate follow-up, three died ofpulmonary metastases at 1, 2\m=1/2\,and 8years following initial treatment. Thefourth patient is alive with disease at theprimary site six years after diagnosis. Thepoor prognosis for patients with this dis-ease suggests that aggressive treatmentis necessary.

(Arch Otolaryngol Head Neck Surg1987;113:311-313)

Synovial sarcoma, a malignant soft-tissue tumor that occurs primari¬ly in the extremities, is rarely seen inthe head and neck. In 1927, LawrenceWeld Smith termed this lesion synovi-oma, imparting a benign connotation.1The truly aggressive nature of thismalignancy was not recognized untilmany years later. Synovial sarcoma

accounts for 8% to 10% of all sarco¬mas. It was first described in the headand neck in 1954, when Jernstrom2reported a case of synovial sarcoma ofthe pharynx. Fewer than 50 head andneck synovial sarcomas have beenreported since. The limited clinicalexperience with this lesion in the headand neck is in sharp contrast to a

much greater experience in theextremities.Several large series of synovial sar¬

coma of the extremities have beendescribed.u5 Cadman et al4 reported134 cases of synovial sarcoma at allsites with a 25% five-year survival.An additional 136 cases, with a five-year survival of 40%, were contrib¬uted by Hajdu et al.3 The two largest

series of synovial sarcoma of the headand neck are collections of case

reports from a number of differentcenters. Roth et al6 cited a 47% five-year survival among 22 patients withsynovial sarcoma of the neck. Elevencases of orofacial synovial sarcoma

were described by Shmookler et al,7but a 66% survival in nine patientsreflected a median follow-up of only2.9 years. Reports of synovial sarcomain the head and neck literature havebeen limited to isolated casestudies.813 When discussing synovialsarcoma of the extremities, mostauthors recommend treating thislesion with aggressive surgical thera¬py followed by radiation therapy.However, recent reports suggest thatsynovial sarcoma may be indolent,and can be treated by simple excisionwithout radiotherapy.1415 Despite thecontroversy regarding treatment ofsynovial sarcoma of the head andneck, the clinical features of synovialsarcoma are similar regardless ofsite.Patients present with synovial sar¬

coma usually between age 25 to 36years, with men outnumbering women

3:2. Earlier discovery of synovial sar¬coma in the head and neck resultsfrom symptoms of pain, dysphagia,hoarseness, and cosmetic deformity.Metastasis to regional lymph nodesoccurs in 12.5% of cases in the headand neck and 23% of cases in theextremities.1'4-6 Pulmonary métastasescaused death in ten of 22 cases inRoth's series of head and neck synovi¬al sarcomas and occurs in 33% to 81%of cases in the extremities.1'3·4'6"' Thedismal prognosis for patients withsynovial sarcoma of the extremitieshas led to several treatment options.Surgical procedures for primary

sarcoma of the extremity include localexcision, en bloc resection of theentire anatomic compartment, andamputation. Sarcomas of soft tissuestend to infiltrate along fasciai andmuscle planes far beyond the palpablelimits of the tumor.17 Therefore, mostauthors conclude that surgery should

aim for removal of all gross tumorand encompass a wide cuff of normaltissue.1·4·518 Although most sarcomasare radioresistant, adjunctive radia¬tion therapy is advocated.1619 The roleof chemotherapy is not well defined.Recent attention has focused on theuse of adjuvant chemotherapy beforemetastatic disease is detectable.17 Theuse of chemotherapy in children withsarcomas may improve the control ofdistant métastases and recurrences atthe primary site.16-20 However, studiesassessing the use of chemotherapy inadults with sarcomas have been dis¬appointing in the demonstration ofsignificantly improved survival.21One major factor that may lead to a

misdiagnosis of synovial sarcoma ofthe head and neck is a divergence ofopinion about its histopathologic fea¬tures. Hajdu et al3 stated that a prim¬itive mesenchymal precursor cell dif¬ferentiates into fascia, aponeurosis,tendon, bursae, and synovial mem¬

branes (Fig l).22 The unicellular origintheory of Hajdu et al3 postulates thatmalignant degeneration of this primi¬tive mesenchymal cell causes synovialsarcoma. Contrary to earlier beliefs,synovial sarcoma seldom arises from

Fig 1.—Unicellular origin theory of tendosyno-vial sarcoma (modified from Batsakis22 withpermission).

Primitive Mesenchyme

Fascia

Fasciai AponeurosisTendonBursae

Synovial MembraneTendosynovial Sarcoma

Monophasic-Biphasic-Monophasic(Spindle) (Epithelioid)

Accepted for publication June 23, 1986.From the Division of Head and Neck Surgery,

UCLA Center for Health Sciences.Presented at the American College of Surgeons

Meeting, Western Section, Palm Springs, Calif,Feb 18, 1986.Reprint requests to Division of Head and Neck

Surgery, UCLA Center for Health Sciences, LosAngeles, CA 90024 (Dr Berke).

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formed synovial membrane, althoughmature synovium and synovial sarco¬ma exhibit a similar two-cell histolo¬gy.22-23 Classically, biphasic synovialsarcoma manifests both spindle cellsand epithelioid cells (Figs 2 and 3).3

Synovial sarcomas consisting of onlyone cell type have been described, butnot all pathologists agree thatmonophasic variants exist. Macken¬zie24 classifies monophasic synovialsarcomas as either spindle-cell sarco-

Fig 2.—Biphasic synovial sarcoma (patient 5). Note spindle cells arranged in bundles andepithelioid element centrally forming pseudogland (hematoxylin-eosin, X40).

Fig 3.—Biphasic synovial sarcoma (patient 5) (hematoxylin-eosin, X160).

mas or epithelioid sarcomas. Recog¬nizing that most synovial sarcomasare poorly differentiated mesenchy¬mal tumors, Russell25 recommendsthat staging of patients with synovialsarcoma be limited to stages 3 and 4.Despite differing opinions regardingclassification of synovial sarcoma, thehistopathology is the same for thosearising in the head and neck and thosearising in the extremities.A child recently presenting with

synovial sarcoma of the postauricularregion and the limited experiencewith this tumor in the head and neckprompted a review of the casestreated at UCLA.

PATIENTS AND METHODS

The UCLA Central Tumor Registry wassearched for records of cases of synovialsarcoma in the head and neck from 1974 to1986. Five previously unreported cases aredescribed (Table).Case 1.—A 35-year-old woman presented

for evaluation of a painless 2-cm left gingi-vobuccal lesion. There was no cervical ade¬nopathy. A biopsy specimen of the lesionwas consistent with synovial sarcoma.Results of a preoperative evaluation,including a chest roentgenogram were nor¬mal. She underwent a left composite man¬

dibulectomy and suprahyoid neck dissec¬tion with Vitallium tray and iliac crestbone graft reconstruction. She received5000 rad (50 Gy) of radiotherapy to theprimary site and left neck. At one year, a1-cm recurrence was treated with widelocal excision along with removal of theinfected Vitallium tray. Bilateral lungmétastases were diagnosed seven yearsafter initial therapy, in spite of control atthe primary site. The patient underwentsurgical removal of the lung nodules anddied of progressive pulmonary failureeight years after initial diagnosis.

Synovial Sarcoma of the Head and Neck, UCLA 1974-1985

Patient, No./Age, y/Sex Size, cm Location Treatment* Outcome1/35/F Left gingivobuccal sulcus 1975, composite resection hemimandible,

suprahyoid neck dissection, 5000 rad (50Gy); 1976, WLE recurrence; 1983, lungmétastases

Dead of disease at 8 y

Nasopharynx 1980, 6600 rad (66 Gy), chemotherapy;1982, WLE recurrence; 1983, WLErecurrence; 1985, WLE recurrence

3/21/M Hypopharynx 1983, WLE, 6490 rad (64.9 Gy),chemotherapy

1985, massive recurrence requiringtracheostomy; dead of disease at 2 Vé y

4/48/F Anterior neck 1982, WLE, thyroidectomy, 6000 rad (60Gy); 1983, neck dissection, lungmétastases; chemotherapy

Dead of disease at 12 mo

5/8/M Postauricular 1985, WLE, 5000 rad (50 Gy), chemotherapy No evidence of disease at 6 mo

*WLE indicates wide local excision.

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Case 2.—A 27-year-old woman presentedwith intermittent right otalgia. Examina¬tion disclosed a 2-cm submucosal lesion inthe right posterior nasopharynx. A biopsyspecimen showed a synovial sarcoma. Achest roentgenogram was normal. Sheunderwent 6600 rad (66 Gy) of radiothera¬py to the primary tumor, and receivedeight courses of systemic combined chemo¬therapy consisting of dacarbazine, doxoru-bicin hydrochloride, and cyclophospha¬mide. A recurrence was noted in 1982, andshe was referred to UCLA. A transpalatal,wide local excision was performed afterthe patient refused radical skull base sur¬

gery. Nasopharyngeal recurrences in 1983,1985, and 1986 were excised locally, and thepatient is currently alive with disease atthe primary site, invading the clivus.Case 3.—A 21-year-old man presented

with progressive dysphagia, odynophagia,and weight loss. Examination revealed a

4-cm submucosal mass in the pharynxextending from the fossa of Rosenmuller tothe hypopharynx. A biopsy specimenshowed synovial sarcoma. The patientunderwent wide local excision through alateral pharyngotomy approach. He re¬

ceived 6490 rad (64.9 Gy) of radiotherapyto the pharynx. The tumor recurred withinthree months and the patient was treatedwith cyclophosphamide, vincristine sul¬fate, dactinomycin, and doxorubicin withpartial response. The patient eventuallydeveloped massive recurrent tumor requir¬ing tracheostomy for airway maintenance.He died of widespread pulmonary métasta¬ses 2V2 years after initial diagnosis.Case 4.—A 48-year-old woman discov¬

ered a painless, 1-cm mass in the lowerright cervical region. Thyroid suppressivetherapy was instituted for a suspectedthyroid nodule. A thyroid scan and chestroentgenogram were normal. In December1982, a total thyroidectomy was performedwith excision of an adjacent paratrachealmass. Histopathology was consistent withsynovial sarcoma. She received 6000 rad(60 Gy) of radiotherapy to the neckthrough opposed lateral fields. She was

referred to UCLA six months later for a

4-cm recurrence. She underwent total lar¬yngectomy with radical neck dissectionand wide resection of the previous scar andoverlying soft tissue. Despite treatmentwith systemic chemotherapy, she devel¬oped bilateral lung métastases and diedone year following initial diagnosis.

Case 5.—An 8-year-old boy presentedwith a 4-cm slowly enlarging mass in theleft postauricular region in July 1985.There was no history of antecedent traumaor ear disease. An excisional biopsy speci¬men demonstrated a biphasic synovial sar¬coma. Chest roentgenogram, bone scan,and bone marrow aspirate were normal.

He then underwent wide local excision ofthe tumor bed with a 3- to 4-cm cuff of skinand normal soft tissue; reconstruction was

performed with a cervical rotationadvancement flap. He received 5000 rad (50Gy) of radiotherapy to the postauricularand occipital regions. The patient recentlycompleted a planned 12-month course ofsystemic chemotherapy with dactinomycinand vincristine and is without recurrentdisease at 15 months.

COMMENTIn summary, five cases of synovial

sarcoma of the head and neck aredescribed. Patient 5 is newly diag¬nosed. Of the remaining four patients,three are dead of lung métastases at 1,2V2 and 8 years following initial diag¬nosis. The fourth patient is alive withdisease at the primary site six yearsafter initial diagnosis. All fourpatients developed at least one recur¬rence. Three patients were treatedwith wide local excision and postoper¬ative radiotherapy, while the fourthreceived radiotherapy as a pri¬mary modality. Adjunctive systemicchemotherapy was used in threepatients.This experience, though small, sug¬

gests that synovial sarcoma of thehead and neck is equally as aggressiveas synovial sarcoma of the extremi¬ties. A marked tendency for localrecurrence and pulmonary métastaseslowers survival to approximately 40%at five years, and 25% at tenyears.137 We believe that until moreexperience with this lesion isobtained, treatment guidelines ofsynovial sarcoma of the extremitiesshould be extrapolated to cases in thehead and neck. Wide resection is indi¬cated in all cases and should encom¬

pass a large cuff of normal tissue.3·14Radiotherapy should be used postop¬eratively in all cases for the treatmentof residual microscopic disease.16·1'The role of adjuvant chemotherapy inthis disease is under investigation,but its greatest potential may lie inthe prevention or delay of distantmétastases.26The authors wish to thank John G. Batsakis,

MD, for the use of his diagram (Fig 1), andRobert Mickel, MD, and David Hanson, MD, fortheir helpful editorial comments.

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