Head and neck synovial cell sarcoma FINN R. AMBLE, MD, KERRY D. OLSEN, MD. ANTONIO G. NASCIMENTO. MD. and ROBERT L. FOOTE, MD.
Rochester, Minnesota
Our experience with 14 patients treated for synovial cell sarcoma during the past 30 years Is described. Thesetumors were manifested In young people whose ages ranged from 12 to 43 years. The diagnosis of synovial cell sarcoma proved difficult because approximately one third of the patients Initially received incorrect pathologic diagnoses. A painless neck mass was the most common presenting symptom. Initial treatment Included surgical excision in five patients and surgical excision and rcdlctlon therapy In nine. Recurrent or metastatic tumor occurred from 4 months to 62 months later; thus, long-term followup is Important. Four of the nine patients who were followed more than 5 years died of their disease. Favorable prognostic findings Included early diagnosis and the performance of wide surgical removal. (OTOLARYNGOL HEAD NECK SURG 1992;107:631.)
The first report of a sarcoma apparently arising from the synovial membrane was published in 1865 by Simon. I Many case reports and review articles have since appeared in the literature. I In 1927, Smith? termed this lesion synovioma, a term implying a benign disorder. The aggressive nature of this malignancy was not recognized until many years later. 2 Synovial cell sarcoma (SS) in the head and neck region was first described by Jernstrom' in 1954 in a case report of SS of the pharynx. Ten percent of these tumors have been found to arise in the head and neck region. There usually is no association with a synovial structure." These tumors present significant challenges in pathologic identification and clinical management. This article describes our experience with the behavior and treatment of this neoplasm. METHODS AND MAllRIAL
Between 1960 and 1990, 14 patients with SS were seen at the Mayo Clinic, Rochester, Minnesota. The
From the Department of Otolaryngology (Drs. Amble and Olsen), the Section of Surgical Pathology (Dr. Nascimento), and the Division of Radiation Oncology (Dr. Foote), Mayo Clinic and Mayo Foundation. Presented at the Annual Meeting of the American Academy of Otolaryngology-Head and Neck Surgery, Kansas City, Mo., Sept. 2226, 1991. Received for publication Nov. 19, 1991; revision received May 26, 1992; accepted July 24, 1992. Reprint requests: Kerry D. Olsen, MD, Mayo Clinic, 200 First St., SW, Rochester, MN 55905. 23/1/41503
average age at presentation was 30.2 years for the six male patients and 26.1 years for the eight female patients; the overall age range was 12 to 43 years. No causal factors for this disease were identified. Symptoms and Findings
The most common presenting symptom was a painless mass (eight patients)." Other symptoms included dysphagia (four patients), a painful mass (three patients), hoarseness (one patient), and headache (one patient) (Table 1). Dyspnea occurred in three patients during the course of their disease. The average duration from onset of symptoms to diagnosis was 10 months (median, 6 months; range, less than 1 month to 60 months). The most common site of occurrence was the neck, which was affected in eight patients. The primary location was the esophagus or hypopharynx in three patients, the larynx in two patients, and the parotid gland in one patient (Table 1). Only one patient had known metastatic disease at the time of diagnosis; this patient had tumor spread to the lungs and clavicle. Pathology
Material suitable for histopathologic review was available in 13 cases. The tumors varied from 1.5 to 12 cm in size. Two lesions showed a mixed cystic and solid pattern, and focal calcification was identified in two lesions. No lesions showed extensive calcification. Grossly, there were no distinguishing clinical features; the tumors varied from necrotic or cystic to solid. All 13 tumors were hypercellular lesions. Six tumors showed the classic biphasic pattern, with abundant spin631
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Table 1. Treatment and results in 14 patients with synovial cell sarcoma in the head and neck Case no.
Initial surgery
Site
Symptom
L posterior cervical triangle
Painless mass Dysphagia Headache
Biopsy
50 Gy
Painless mass
Enucleation
66 Gy
Painful mass
Painless mass
Wide excision, RND Wide excision, L RND Biopsy
50 Gy
5
R posterior cervical triangle Mid-posterior neck L neck. submental Supraglottic
6
L posterior neck
Painless mass
Wide excision
7
L supraclavicular
Painful mass
Enucleation
8
R anterior superior neck Esophagus Hypopharynx
Painless mass
Wide excision
20 Gy brachyRx 45 Gy external beam 30 Gy preop 25 Gy postop 56 Gy
Dysphagia Dysphagia Painless mass Dysphagia
Biopsy Wide excision, laryngectomy Wide excision Wide excision
Painful mass
Wide excision
Painless mass
Wide excision, RND
2 3 4
9 10 11 12 13 14
Parotid gland Esophagus, hypopharynx Larynx. hypopharynx Neck
Painless mass
Radiation
Chemotherapy
CTX, ADR, COOP, pyrazofurin; MTX, VP16, VCR Recombinant interferon 0
Status at followup (mol
000 (73)
000 (32) A- (71) A- (72)
CTX, VCR, actinomycin 0
A- (37)
ADR,CDDP
000 (12)
000 (98)
50 Gy
648 Gy Unknown amount 64.8 Gy (primary)
A+ (7)
A+ (80) A- (136)
Unknown amount
A- (39) A- (43)
MAP
A+ (9) A- (139)
A -, Alive without evidence of disease; A +, alive with disease; 000, dead of disease; AOR, Adriamycin (doxorubicin); brachyRx, brachytherapy; COOP, cisplatin; CTX, Cytoxan (cyclophosphamide); MAP, mitomycin; Adriamycin, cisplatin: MTX, methotrexate; RNO, radical neck dissection; VCR, vincristine; VP16, etoposide.
dle cells and gland-like structures (Fig. 1). In seven cases, the tumors were monophasic, composed solely of fascicles or whorls of spindle cells with no clear-cut gland-like structures. Three tumors showed features of hemangiopericytomas with a richly vascularized background; however, other areas of typical 55 could be identified. Five tumors (cases 2,3,4, 7, and 12) had fewer than 10 mitotic figures per 10 high-power fields and no necrosis. Focal necrosis was identified in cases 5 and 8. Cases 1, 11, and 13 demonstrated massive areas of necrosis (Fig. 2). In our series, diagnosing 55 was difficult, as has been described in the literature." In four of our 14 patients, the initial referral diagnosis was "mixed cell" parotid tumor, fibrous histiocytoma, neuroendocrine tumor, and rhabdomyosarcoma.
Treatment
Given the long period during which patients were treated and the various extents of tumor of the patients in this review, the surgical, radiotherapeutic, and chemotherapeutic treatments varied greatly (Table 1). The management of each case is summarized below. Case 1. In 1972, an excisional biopsy of a left posterior neck mass was followed by removal of a local recurrent tumor 2 years later. Tumor recurrence led to left radical neck dissection and parotidectomy. The presumptive diagnosis was "mixed cell" tumor. In 1976, the patient was seen at the Mayo Clinic with local tumor recurrence. A submental, suprahyoid neck dissection was performed. By 1977, the patient had local recurrence and pulmonary metastasis. Nine palliative excisions/debulkments of local or regional disease, in addition to a tracheostomy, were done until the patient's
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Volume 107 Number 5 November 1992
Head and neck synovial sarcoma
Fig. 1. Classic biphasic pattern of synovial cell sarcoma with abundant spindle cells and gland-like structures (arrows). (Hematoxylin-eosin stain; original magniftcation x 100.)
Fig. 2. Gross specimen of large esophageal synovial cell sarcoma.
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death in 1978. Radiation and chemotherapy regimens also seemed to have no effect. Case 2. This patient underwent enucleation/debulkment of a large, fixed, 8 em right posterior cervical mass. Despite postoperative radiation therapy and recombinant interferon D therapy, he died 24 months later of pulmonary metastasis with no recurrence in the local tumor bed. Case 3. This patient underwent local excision of a mid-posterior neck mass (less than 2 em). The patient came to the Mayo Clinic for additional treatment, which included wide excision of the tumor area, with anterior and posterior neck dissection. Postoperative radiation therapy to the neck was also given. The patient was well with no evidence of disease 71 months later. Case 4. A small neck mass was removed from the left submental region of this patient. SS was diagnosed, and wide excision of the biopsy site and a left radical neck dissection were performed. The patient was alive without disease 72 months later. Case 5. An excisional biopsy of tumor in the supraglottic region led to the diagnosis of rhabdomyosarcoma. Chemotherapy [cyclophosphamide (Cytoxan), vincristine, actinomycin D] was used without effect. An extended supraglottic laryngectomy was performed 2 months later because of persistent tumor. Laser treatment and tumor debulkment were necessary because of progressive airway obstruction. Pulmonary metastasis then occurred. The patient was alive with disease 7 months after the initial treatment. Case 6. This patient underwent removal of a large (more than 4 em) left posterior neck mass. After a diagnosis of SS, wide re-excision was performed with postoperative radiation therapy, brachytherapy, and external beam. The patient was alive without evidence of disease 37 months later. Case 7. A patient with !mown pulmonary metastasis and a large tumor (more than 4 em) in the left supraclavicular area was treated with debulkment, radiation therapy, and three cycles of chemotherapy [doxorubicin (Adriamycin), CDDP (cisplatin)]. There was no significant effect on tumor progression and the patient died within 12 months. Case 8. This patient had a right anterior superior neck mass treated with wide excision. Five years later, local recurrence was managed by radical neck dissection, parotidectomy, and postoperative radiation therapy. Pulmonary metastasis occurred 5 months later, and the patient died 98 months after his onset of symptoms. Case 9. A biopsy of an esophagus lesion causing dysphagia was reported as being "malignant." The primary lesion and the neck were treated with radiation.
Extensive local and regional recurrence was diagnosed as SS at the Mayo Clinic 6.5 years after the onset of the patient's symptoms. A tracheostomy for airway protection was done. The patient was alive with disease 1 year later. Case 10. A hypopharyngeal tumor was treated by removal of the medial wall of the piriform fossa. A diagnosis of fibrous histiocytoma was later changed to SS. The patient underwent a laryngectomy. There was no recurrence during the next 10 years. Case 11. A mass in the right parotid region initially was treated by local excision. After a diagnosis of SS, the patient underwent a right total parotidectomy and removal of a portion of the infratemporal fossa. Postoperative radiation therapy was also given. Four years later, there was no evidence of disease. Case 12. A teenage girl with SS of the esophagus and hypopharynx underwent an esophagectomy, laryngectomy, pharyngectomy, total thyroidectomy, mediastinal dissection, and gastric pull-up. She was to receive postoperative radiation and chemotherapy in another country. The patient was alive with no evidence of disease 39 months later. Case 13. A young male patient had massive tumor involvement of the larynx and pharynx that required a tracheotomy. A biopsy showed a neuroendocrine tumor. Subsequent treatment at our institution included a total laryngectomy, partial pharyngectomy, partial esophagectomy, and paratracheal node dissection. Postoperatively, the patient received radiation therapy to the primary lesion, neck, and mediastinum. Pulmonary metastasis occurred 8 months later. At followup, he was receiving chemotherapy. Case 14. An SS lesion in the right anterior superior neck was widely removed with a right neck dissection. Eleven years later, the patient was alive without disease. RESULTS
At the time of this study, four patients had died of their tumor (1 year to 8 years) after their initial treatment; three patients were alive with disease. An overall determinate survival curve (Kaplan-Meier method) is shown in Fig. 3. Four of nine patients with 5-year followup were alive without recurrent tumor. One patient was alive with disease 80 months after initial treatment. Long-term followup is important because death from tumor can occur up to 5 years after treatment. Two patients had survived more than 10 years without tumor recurrence. Both patients were treated with wide surgical excision alone. Nine patients underwent wide surgical excision of their tumor. Seven of these patients were alive with no recurrence at an average of 76.7 months (37 to 139
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Head and neck synovial sarcoma
635
100
80 ;:R. 0
60
.~
20
::J
C/)
0
0
2
3
4
5
6
7
8
9
10
11
12
Years after surgery Fig. 4. Survival in 14 patients with synovial cell sarcoma by treatment: wide excision vs. other surgical therapy (debulkment or biopsy),
months) after initial treatment. One of the nine patients was alive with disease 9 months later, and one patient died of disease at 98 months. Five patients were surgically treated with either biopsy or debulkment; all either died of disease or were alive with disease. The improved survival rate with wide excision vs. other surgical treatment was statistically significant, as determined by the log-rank test (p = 0.01) (Fig. 4). Six patients received postoperative radiation therapy. Four of these patients were alive without disease (average, 47.5 months) after initial treatment, and the other two were alive with disease (average, 44.5 months). Five of the six never had recurrence within the tumor bed. Four patients received radiation therapy for residual tumor or recurrence. All four died of disease (average, 53.8 months). In three, persistent or recurrent disease developed within the tumor bed (radiation dose, 50 to 55 Gy); in one, tumor did not recur within the
tumor bed (dose, 66 Gy). No patient received radiation as the sole treatment. In nine of the 10 patients who received radiation therapy, complete treatment records were available for review. No recurrences developed within the tumor bed in the four patients who received 64.8 or more Gy. However, recurrences developed within the tumor bed in four of five patients who received 0.5 to 50 Gy. Chemotherapy was known to be given to five patients because of recurrence or metastases or for palliation. Three were dead of disease (average, 39 months) and two were alive with disease (average, 8 months). Local recurrences developed in three of the 14 patients up to 56 months after initial treatment. Four patients had persistent disease after their initial operation. All seven of these patients were either dead of disease or alive with disease. No patient in this series had documented cervical nodal metastasis.
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Only one patient had known metastatic disease at the time of presentation. Distant metastasis developed later in five patients. In all cases, cancer spread only to the lungs at an average of 30.8 months (range, 8 to 62 months) after initial surgical therapy. In addition to the performance of wide surgical excision, early diagnosis seemed to affect survival favorably. In the seven patients alive without disease, the average time from onset of symptoms to diagnosis was 5.6 months. In contrast, in those patients alive with disease or dead of disease, the average time from onset of symptoms to diagnosis was 15.5 months. No correlation was noted between tumor grade or histologic findings and survival. Also, tumor size and location did not seem to influence tumor recurrence or metastasis. DISCUSSION
Head and neck 5S does not seem to arise from formed adult synovial membranes. Primary involvement of a joint cavity is rare. Batsakis" and otbers':" believe these sarcomas probably arise from synovioblastic differentiation of mesenchymal tissues. The histologic identification of a tumor as 55 is often difficult. Dystrophic calcification and calcospherites are findings in 55 from any site." 5S consisting of only one cell type has been described, but not all pathologists agree that monophasic variants exist.' According to Mackenzie," the biphasic pattern is the only histologic criterion by which the diagnosis of 55 can be made. Hajdu et al. 1 described monophasic variants (spindle cell and epithelioid) as subtypes of 55. The histopathologic findings are the same as those for tumors arising in the head and neck and in the extremities. "Classic" 55 has two cellular components: a spindle cell (fibrosarcoma-like) component and a pseudoepithelial component. 5 There are no unique gross pathologic features. The tumor can be necrotic, cystic, or solid with a pseudocapsule." The neoplasm is usually circumscribed and gray or pink. Foci of hemorrhage, calcification, or mucoid areas may be present. 5S of the head and neck tends to occur in young persons. This characteristic has been reported in the literature':':" and was found in our series. Most reports describe a male predominance, but our series had a slight female predominance (57%). There were no specific findings or symptoms that were suspicious for 55. These tumors generally present as an asymptomatic neck mass. The treatment of head and neck 55 is not well-delineated because of the rare and varied occurrence of
this cancer. Soft tissue sarcomas tend to infiltrate along fascial and muscle planes beyond the palpable tumor limit. Therefore, operation should remove all gross tumor and encompass a wide cuff of normal tissue. Operation alone has been advocated as the main treatment. 1 Several authors recommend wide surgical excision.":":" Limited resection, according to our experience and that of others, has no place in the management of 5S. 9 Roth et al. 7 found that only one of eight patients who had removal of regional lymph nodes had evidence of nodal metastasis. No patient in our series had cervical metastasis. In the absence of palpable disease, a neck dissection done prophylactically for metastasis is unnecessary. The role of radiation therapy for SS has been examined by several authors. 12·15 A study by Mamelle et al. 12 suggested that postoperative radiotherapy decreased local recurrence but did not improve long-term survival. A total dose of at least 65 Gy has been recommended for local control of this tumor" and is supported by our experience. The low doses of radiation therapy given to many patients in this series reflect the changes in radiation therapy over the 30 years of this study. The role of chemotherapy is indeterminate, but its use in adults for most sarcomas has not been helpful. 2 CONCLUSION
Head and neck SS is a rare tumor. It typically presents as an asymptomatic neck mass in young people. The diagnosis of this neoplasm is challenging in that patients often initially receive an incorrect pathologic diagnosis. Favorable prognostic factors appear to be early diagnosis and the performance of wide surgical excision. A prophylactic neck dissection is unnecessary, but it may be done to achieve wide removal of a primary neck tumor. Long-term followup is essential because recurrent or metastatic tumor can occur up to 4 years after initial therapy. The cause of death in most of our patients was a result of local recurrence and pulmonary metastasis. Five of the nine patients with followup of more than 5 years were alive, although one had known recurrent tumor. Postoperative radiation therapy with doses of 65 Gy or more may improve local control, but current chemotherapy is probably of no benefit.
REFERENCES
I. Hajdu SI, Shiu MH, Fortner JG. Tendosynovial sarcoma: a clinicopathological study of 136 cases, Cancer 1977;39:1201-17. 2. Moore DM, Berke as. Synovial sarcoma of the head and neck. Arch Otolaryngol Head Neck Surg 1987;113:311-3. 3. Jemstrom P, Synovial sarcoma of the pharynx: report of a case. Am J Clin Pathol 1954;24:957-61.
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Head and neck synovial sarcoma
4. Burton OM, Patow CA. Clinical and pathologic diagnosis. Arch Otolaryngol Head Neck Surg 1990:116:1342-5. 5. Batsakis JG. Tumors of the head and neck: clinical and pathological considerations. 2nd ed. Baltimore: Williams & Wilkins, 1979. 6. Harrison EG Jr, Black BM, Devine KD. Synovial sarcoma primary in the neck. Arch Pathol 1961:71:137-41. 7. Roth JA, Enzinger FM, Tannenbaum M. Synovial sarcoma of the neck: a followup study of 24 cases. Cancer 1975;35:124353. 8. Mackenzie DH. Synovial sarcoma: a review of 58 cases. Cancer 1966;19:169-80. 9. Lockey MW. Rare tumors of the ear, nose and throat: synovial sarcoma of the head and neck. South Med J 1976;69:316-20. 10. Oppedal BR, Reyne T, Titterud I. Synovial sarcomas of the neck: a report of two cases. J Laryngol Otol 1985;99:101-4. 11. Ariel 1M, Pack GT. Synovial sarcoma: review of 25 cases. N Engl J Med 1963;268:1272-5.
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12. Mamelle G, Richard J, Luboinski B, Schwaab G, Eschwege F, Micheau C. Synovial sarcoma of the head and neck: an account of four cases and review of the literature. Eur J Surg Oneal 1986;12:347-9. 13. Chang AE, Rosenberg SA, Glatstein EJ, Antman KH. Sarcomas of soft tissue. In: DeVita VT Jr, Hellman S, Rosenberg SA, eds. Cancer: principles and practice of oncology. 3rd ed. Philadelphia: JB Lippincott, 1989:1345-98. 14. Goepfert H, Lindberg RD, Sinkovics JG, Ayala AG. Soft-tissue sarcoma of the head and neck after puberty: treatment by surgery and postoperative radiation therapy. Arch Otolaryngol 1977;103:365-8. 15. Lindberg RD, Martin RG, Romsdahl MM, Barkley HT Jr. Conservative surgery and postoperative radiotherapy in 300 adults with soft-tissue sarcomas. Cancer 1981;47:2391-7.
AV AILABLE NOWI The FIVE-YEAR (1986-1990) CUMULATIVE INDEX TO OTOLARYNGOLOGY -HEAD AND NECK SURGERY can be purchased from the Publisher for $44.00. This comprehensive 104-page reference guide is a current presentation of all topics included in the Journal from January 1986 through December 1990 (volumes 94103)-the past 10 volumes. It incorporates complete references to more than 640 original articles, abstracts, case reports, letters, and editorials. It features 1668 Subject Headings, under which there are 3247 references. Each subject entry lists the complete article title, authorts), volume, page(s), and year of publication. In addition, it includes 3190 Author Entries, which list contributors, along with their respective titles, author-to-author referral, volume, page, and publication. To purchase, call or write: Mosby-Year Book, Inc., 11830 Westline Industrial Dr., St. Louis, Missouri 63146-3318, or telephone FREE 1-800-325-4177, extension 4351, Journal Fulfillment (in Missouri, call collect at 314-872-8370, extension 4351, Journal Fulfillment). PREPAYMENT REQUIRED. Make checks payable to Mosby-Year Book, Inc.; (all payments must be in U.S. funds drawn on a U.S. bank). Price: $44.00 in the U.S.; $50.50 in Canada; and $48.50 in other countries (price includes mailing charges).
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Otolaryngology Head and Neck Surgery March 1993
News and Announcements
held July 26-30, 1993, at the Tamarron Resort in Durango, Colorado. This 28 hour review and update will encompass all the clinically important areas of MR imaging. Important new concepts and pathological/imaging correlations in the body, musculoskeletal system, ENT, head and neck, brain, and spine will be explored. Daily case presentations will supplement these lectures and will serve to test the registrants' diagnostic abilities in MR imaging. This complete review of MR imaging will be presented by nationally recognized leaders in magnetic resonance imaging. As a result of this comprehensive review, registrants will become familiar with current applications of MR imaging and will be able to integrate many of these applications directly into their practice. Program chairmen for this presentation will be Robert Quencer, MD (University of Miami), Victor Haughton, MD (Medical College of Wisconsin). Twenty-eight credits of Category I will be available. For further information, please contact Marti Carter, CME, Inc., 11011 West Nort Ave., Milwaukee, Wisconsin 53226, or call (414) 771-9520. Ear, Nose, and Throat Diseases: 1993 Update
Children's Hospital of Pittsburgh will hold its 18th Annual Symposium, "Ear, Nose, and Throat Diseases in Children: A 1993 Update." This symposium will be held July 30-31, 1993. CME credits will be awarded.
For further information, please contact the Department of Pediatric Otolaryngology, Children's Hospital of Pittsburgh, 3705 Fifth Avenue at DeSoto St., Pittsburgh, Pennsylvania 15213, or call (412) 692-8577. Twenty-fifth Annual Meeting - Head and Neck Oncologists
The Association of Head and Neck Oncologists of Great Britain will sponsor the Twenty-fifth Annual Meeting of Head and Neck Oncology, to be held in Edinburgh, Scotland, United Kingdom, on August 23-26, 1993. International and local faculty will present extensive social and family programs. For further information, please contact Mr. P. J. Bradley, Honorary Secretary, Department of Otorhinolaryngology-Head and Neck Surgery, University Hospital, Queens Medical Centre, Nottingham, NG7 2UH, England, or phone 0602421421. Sixth International Congress on Interventlonal Ultrasound
The Sixth International Congress on Interventional Ultrasound will be held in Copenhagen, Denmark, on September 7-10, 1993. For further information, please contact Christian Nolsoe, Congress Secretary, Department of Ultrasound, Herlev Hospital, University of Copenhagen, DK-2730 HerlevDenmark, or call + 45/ 44 53 53 00 ext. 3240.
CORRECTION
The Supplement to the December 1992 issue of the JOURNAL (Volume 107, Number 6, Part 2), incorrectly listed Dr. Bruce R. Gordon as Chief of Otolaryngology at the Massachusetts Eye and Ear Institute. Dr. Joseph Nadol is Chief of Otolaryngology at the Massachusetts Eye and Ear Infirmary. Dr. Gordon is Chief of Otolaryngology at Cape Cod Hospital.
Rochester, Minnesota
Our experience with 14 patients treated for synovial cell sarcoma during the past 30 years Is described. Thesetumors were manifested In young people whose ages ranged from 12 to 43 years. The diagnosis of synovial cell sarcoma proved difficult because approximately one third of the patients Initially received incorrect pathologic diagnoses. A painless neck mass was the most common presenting symptom. Initial treatment Included surgical excision in five patients and surgical excision and rcdlctlon therapy In nine. Recurrent or metastatic tumor occurred from 4 months to 62 months later; thus, long-term followup is Important. Four of the nine patients who were followed more than 5 years died of their disease. Favorable prognostic findings Included early diagnosis and the performance of wide surgical removal. (OTOLARYNGOL HEAD NECK SURG 1992;107:631.)
The first report of a sarcoma apparently arising from the synovial membrane was published in 1865 by Simon. I Many case reports and review articles have since appeared in the literature. I In 1927, Smith? termed this lesion synovioma, a term implying a benign disorder. The aggressive nature of this malignancy was not recognized until many years later. 2 Synovial cell sarcoma (SS) in the head and neck region was first described by Jernstrom' in 1954 in a case report of SS of the pharynx. Ten percent of these tumors have been found to arise in the head and neck region. There usually is no association with a synovial structure." These tumors present significant challenges in pathologic identification and clinical management. This article describes our experience with the behavior and treatment of this neoplasm. METHODS AND MAllRIAL
Between 1960 and 1990, 14 patients with SS were seen at the Mayo Clinic, Rochester, Minnesota. The
From the Department of Otolaryngology (Drs. Amble and Olsen), the Section of Surgical Pathology (Dr. Nascimento), and the Division of Radiation Oncology (Dr. Foote), Mayo Clinic and Mayo Foundation. Presented at the Annual Meeting of the American Academy of Otolaryngology-Head and Neck Surgery, Kansas City, Mo., Sept. 2226, 1991. Received for publication Nov. 19, 1991; revision received May 26, 1992; accepted July 24, 1992. Reprint requests: Kerry D. Olsen, MD, Mayo Clinic, 200 First St., SW, Rochester, MN 55905. 23/1/41503
average age at presentation was 30.2 years for the six male patients and 26.1 years for the eight female patients; the overall age range was 12 to 43 years. No causal factors for this disease were identified. Symptoms and Findings
The most common presenting symptom was a painless mass (eight patients)." Other symptoms included dysphagia (four patients), a painful mass (three patients), hoarseness (one patient), and headache (one patient) (Table 1). Dyspnea occurred in three patients during the course of their disease. The average duration from onset of symptoms to diagnosis was 10 months (median, 6 months; range, less than 1 month to 60 months). The most common site of occurrence was the neck, which was affected in eight patients. The primary location was the esophagus or hypopharynx in three patients, the larynx in two patients, and the parotid gland in one patient (Table 1). Only one patient had known metastatic disease at the time of diagnosis; this patient had tumor spread to the lungs and clavicle. Pathology
Material suitable for histopathologic review was available in 13 cases. The tumors varied from 1.5 to 12 cm in size. Two lesions showed a mixed cystic and solid pattern, and focal calcification was identified in two lesions. No lesions showed extensive calcification. Grossly, there were no distinguishing clinical features; the tumors varied from necrotic or cystic to solid. All 13 tumors were hypercellular lesions. Six tumors showed the classic biphasic pattern, with abundant spin631
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Table 1. Treatment and results in 14 patients with synovial cell sarcoma in the head and neck Case no.
Initial surgery
Site
Symptom
L posterior cervical triangle
Painless mass Dysphagia Headache
Biopsy
50 Gy
Painless mass
Enucleation
66 Gy
Painful mass
Painless mass
Wide excision, RND Wide excision, L RND Biopsy
50 Gy
5
R posterior cervical triangle Mid-posterior neck L neck. submental Supraglottic
6
L posterior neck
Painless mass
Wide excision
7
L supraclavicular
Painful mass
Enucleation
8
R anterior superior neck Esophagus Hypopharynx
Painless mass
Wide excision
20 Gy brachyRx 45 Gy external beam 30 Gy preop 25 Gy postop 56 Gy
Dysphagia Dysphagia Painless mass Dysphagia
Biopsy Wide excision, laryngectomy Wide excision Wide excision
Painful mass
Wide excision
Painless mass
Wide excision, RND
2 3 4
9 10 11 12 13 14
Parotid gland Esophagus, hypopharynx Larynx. hypopharynx Neck
Painless mass
Radiation
Chemotherapy
CTX, ADR, COOP, pyrazofurin; MTX, VP16, VCR Recombinant interferon 0
Status at followup (mol
000 (73)
000 (32) A- (71) A- (72)
CTX, VCR, actinomycin 0
A- (37)
ADR,CDDP
000 (12)
000 (98)
50 Gy
648 Gy Unknown amount 64.8 Gy (primary)
A+ (7)
A+ (80) A- (136)
Unknown amount
A- (39) A- (43)
MAP
A+ (9) A- (139)
A -, Alive without evidence of disease; A +, alive with disease; 000, dead of disease; AOR, Adriamycin (doxorubicin); brachyRx, brachytherapy; COOP, cisplatin; CTX, Cytoxan (cyclophosphamide); MAP, mitomycin; Adriamycin, cisplatin: MTX, methotrexate; RNO, radical neck dissection; VCR, vincristine; VP16, etoposide.
dle cells and gland-like structures (Fig. 1). In seven cases, the tumors were monophasic, composed solely of fascicles or whorls of spindle cells with no clear-cut gland-like structures. Three tumors showed features of hemangiopericytomas with a richly vascularized background; however, other areas of typical 55 could be identified. Five tumors (cases 2,3,4, 7, and 12) had fewer than 10 mitotic figures per 10 high-power fields and no necrosis. Focal necrosis was identified in cases 5 and 8. Cases 1, 11, and 13 demonstrated massive areas of necrosis (Fig. 2). In our series, diagnosing 55 was difficult, as has been described in the literature." In four of our 14 patients, the initial referral diagnosis was "mixed cell" parotid tumor, fibrous histiocytoma, neuroendocrine tumor, and rhabdomyosarcoma.
Treatment
Given the long period during which patients were treated and the various extents of tumor of the patients in this review, the surgical, radiotherapeutic, and chemotherapeutic treatments varied greatly (Table 1). The management of each case is summarized below. Case 1. In 1972, an excisional biopsy of a left posterior neck mass was followed by removal of a local recurrent tumor 2 years later. Tumor recurrence led to left radical neck dissection and parotidectomy. The presumptive diagnosis was "mixed cell" tumor. In 1976, the patient was seen at the Mayo Clinic with local tumor recurrence. A submental, suprahyoid neck dissection was performed. By 1977, the patient had local recurrence and pulmonary metastasis. Nine palliative excisions/debulkments of local or regional disease, in addition to a tracheostomy, were done until the patient's
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Volume 107 Number 5 November 1992
Head and neck synovial sarcoma
Fig. 1. Classic biphasic pattern of synovial cell sarcoma with abundant spindle cells and gland-like structures (arrows). (Hematoxylin-eosin stain; original magniftcation x 100.)
Fig. 2. Gross specimen of large esophageal synovial cell sarcoma.
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death in 1978. Radiation and chemotherapy regimens also seemed to have no effect. Case 2. This patient underwent enucleation/debulkment of a large, fixed, 8 em right posterior cervical mass. Despite postoperative radiation therapy and recombinant interferon D therapy, he died 24 months later of pulmonary metastasis with no recurrence in the local tumor bed. Case 3. This patient underwent local excision of a mid-posterior neck mass (less than 2 em). The patient came to the Mayo Clinic for additional treatment, which included wide excision of the tumor area, with anterior and posterior neck dissection. Postoperative radiation therapy to the neck was also given. The patient was well with no evidence of disease 71 months later. Case 4. A small neck mass was removed from the left submental region of this patient. SS was diagnosed, and wide excision of the biopsy site and a left radical neck dissection were performed. The patient was alive without disease 72 months later. Case 5. An excisional biopsy of tumor in the supraglottic region led to the diagnosis of rhabdomyosarcoma. Chemotherapy [cyclophosphamide (Cytoxan), vincristine, actinomycin D] was used without effect. An extended supraglottic laryngectomy was performed 2 months later because of persistent tumor. Laser treatment and tumor debulkment were necessary because of progressive airway obstruction. Pulmonary metastasis then occurred. The patient was alive with disease 7 months after the initial treatment. Case 6. This patient underwent removal of a large (more than 4 em) left posterior neck mass. After a diagnosis of SS, wide re-excision was performed with postoperative radiation therapy, brachytherapy, and external beam. The patient was alive without evidence of disease 37 months later. Case 7. A patient with !mown pulmonary metastasis and a large tumor (more than 4 em) in the left supraclavicular area was treated with debulkment, radiation therapy, and three cycles of chemotherapy [doxorubicin (Adriamycin), CDDP (cisplatin)]. There was no significant effect on tumor progression and the patient died within 12 months. Case 8. This patient had a right anterior superior neck mass treated with wide excision. Five years later, local recurrence was managed by radical neck dissection, parotidectomy, and postoperative radiation therapy. Pulmonary metastasis occurred 5 months later, and the patient died 98 months after his onset of symptoms. Case 9. A biopsy of an esophagus lesion causing dysphagia was reported as being "malignant." The primary lesion and the neck were treated with radiation.
Extensive local and regional recurrence was diagnosed as SS at the Mayo Clinic 6.5 years after the onset of the patient's symptoms. A tracheostomy for airway protection was done. The patient was alive with disease 1 year later. Case 10. A hypopharyngeal tumor was treated by removal of the medial wall of the piriform fossa. A diagnosis of fibrous histiocytoma was later changed to SS. The patient underwent a laryngectomy. There was no recurrence during the next 10 years. Case 11. A mass in the right parotid region initially was treated by local excision. After a diagnosis of SS, the patient underwent a right total parotidectomy and removal of a portion of the infratemporal fossa. Postoperative radiation therapy was also given. Four years later, there was no evidence of disease. Case 12. A teenage girl with SS of the esophagus and hypopharynx underwent an esophagectomy, laryngectomy, pharyngectomy, total thyroidectomy, mediastinal dissection, and gastric pull-up. She was to receive postoperative radiation and chemotherapy in another country. The patient was alive with no evidence of disease 39 months later. Case 13. A young male patient had massive tumor involvement of the larynx and pharynx that required a tracheotomy. A biopsy showed a neuroendocrine tumor. Subsequent treatment at our institution included a total laryngectomy, partial pharyngectomy, partial esophagectomy, and paratracheal node dissection. Postoperatively, the patient received radiation therapy to the primary lesion, neck, and mediastinum. Pulmonary metastasis occurred 8 months later. At followup, he was receiving chemotherapy. Case 14. An SS lesion in the right anterior superior neck was widely removed with a right neck dissection. Eleven years later, the patient was alive without disease. RESULTS
At the time of this study, four patients had died of their tumor (1 year to 8 years) after their initial treatment; three patients were alive with disease. An overall determinate survival curve (Kaplan-Meier method) is shown in Fig. 3. Four of nine patients with 5-year followup were alive without recurrent tumor. One patient was alive with disease 80 months after initial treatment. Long-term followup is important because death from tumor can occur up to 5 years after treatment. Two patients had survived more than 10 years without tumor recurrence. Both patients were treated with wide surgical excision alone. Nine patients underwent wide surgical excision of their tumor. Seven of these patients were alive with no recurrence at an average of 76.7 months (37 to 139
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Volume 107 Number 5 November 1992
Head and neck synovial sarcoma
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Years after surgery Fig. 4. Survival in 14 patients with synovial cell sarcoma by treatment: wide excision vs. other surgical therapy (debulkment or biopsy),
months) after initial treatment. One of the nine patients was alive with disease 9 months later, and one patient died of disease at 98 months. Five patients were surgically treated with either biopsy or debulkment; all either died of disease or were alive with disease. The improved survival rate with wide excision vs. other surgical treatment was statistically significant, as determined by the log-rank test (p = 0.01) (Fig. 4). Six patients received postoperative radiation therapy. Four of these patients were alive without disease (average, 47.5 months) after initial treatment, and the other two were alive with disease (average, 44.5 months). Five of the six never had recurrence within the tumor bed. Four patients received radiation therapy for residual tumor or recurrence. All four died of disease (average, 53.8 months). In three, persistent or recurrent disease developed within the tumor bed (radiation dose, 50 to 55 Gy); in one, tumor did not recur within the
tumor bed (dose, 66 Gy). No patient received radiation as the sole treatment. In nine of the 10 patients who received radiation therapy, complete treatment records were available for review. No recurrences developed within the tumor bed in the four patients who received 64.8 or more Gy. However, recurrences developed within the tumor bed in four of five patients who received 0.5 to 50 Gy. Chemotherapy was known to be given to five patients because of recurrence or metastases or for palliation. Three were dead of disease (average, 39 months) and two were alive with disease (average, 8 months). Local recurrences developed in three of the 14 patients up to 56 months after initial treatment. Four patients had persistent disease after their initial operation. All seven of these patients were either dead of disease or alive with disease. No patient in this series had documented cervical nodal metastasis.
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Only one patient had known metastatic disease at the time of presentation. Distant metastasis developed later in five patients. In all cases, cancer spread only to the lungs at an average of 30.8 months (range, 8 to 62 months) after initial surgical therapy. In addition to the performance of wide surgical excision, early diagnosis seemed to affect survival favorably. In the seven patients alive without disease, the average time from onset of symptoms to diagnosis was 5.6 months. In contrast, in those patients alive with disease or dead of disease, the average time from onset of symptoms to diagnosis was 15.5 months. No correlation was noted between tumor grade or histologic findings and survival. Also, tumor size and location did not seem to influence tumor recurrence or metastasis. DISCUSSION
Head and neck 5S does not seem to arise from formed adult synovial membranes. Primary involvement of a joint cavity is rare. Batsakis" and otbers':" believe these sarcomas probably arise from synovioblastic differentiation of mesenchymal tissues. The histologic identification of a tumor as 55 is often difficult. Dystrophic calcification and calcospherites are findings in 55 from any site." 5S consisting of only one cell type has been described, but not all pathologists agree that monophasic variants exist.' According to Mackenzie," the biphasic pattern is the only histologic criterion by which the diagnosis of 55 can be made. Hajdu et al. 1 described monophasic variants (spindle cell and epithelioid) as subtypes of 55. The histopathologic findings are the same as those for tumors arising in the head and neck and in the extremities. "Classic" 55 has two cellular components: a spindle cell (fibrosarcoma-like) component and a pseudoepithelial component. 5 There are no unique gross pathologic features. The tumor can be necrotic, cystic, or solid with a pseudocapsule." The neoplasm is usually circumscribed and gray or pink. Foci of hemorrhage, calcification, or mucoid areas may be present. 5S of the head and neck tends to occur in young persons. This characteristic has been reported in the literature':':" and was found in our series. Most reports describe a male predominance, but our series had a slight female predominance (57%). There were no specific findings or symptoms that were suspicious for 55. These tumors generally present as an asymptomatic neck mass. The treatment of head and neck 55 is not well-delineated because of the rare and varied occurrence of
this cancer. Soft tissue sarcomas tend to infiltrate along fascial and muscle planes beyond the palpable tumor limit. Therefore, operation should remove all gross tumor and encompass a wide cuff of normal tissue. Operation alone has been advocated as the main treatment. 1 Several authors recommend wide surgical excision.":":" Limited resection, according to our experience and that of others, has no place in the management of 5S. 9 Roth et al. 7 found that only one of eight patients who had removal of regional lymph nodes had evidence of nodal metastasis. No patient in our series had cervical metastasis. In the absence of palpable disease, a neck dissection done prophylactically for metastasis is unnecessary. The role of radiation therapy for SS has been examined by several authors. 12·15 A study by Mamelle et al. 12 suggested that postoperative radiotherapy decreased local recurrence but did not improve long-term survival. A total dose of at least 65 Gy has been recommended for local control of this tumor" and is supported by our experience. The low doses of radiation therapy given to many patients in this series reflect the changes in radiation therapy over the 30 years of this study. The role of chemotherapy is indeterminate, but its use in adults for most sarcomas has not been helpful. 2 CONCLUSION
Head and neck SS is a rare tumor. It typically presents as an asymptomatic neck mass in young people. The diagnosis of this neoplasm is challenging in that patients often initially receive an incorrect pathologic diagnosis. Favorable prognostic factors appear to be early diagnosis and the performance of wide surgical excision. A prophylactic neck dissection is unnecessary, but it may be done to achieve wide removal of a primary neck tumor. Long-term followup is essential because recurrent or metastatic tumor can occur up to 4 years after initial therapy. The cause of death in most of our patients was a result of local recurrence and pulmonary metastasis. Five of the nine patients with followup of more than 5 years were alive, although one had known recurrent tumor. Postoperative radiation therapy with doses of 65 Gy or more may improve local control, but current chemotherapy is probably of no benefit.
REFERENCES
I. Hajdu SI, Shiu MH, Fortner JG. Tendosynovial sarcoma: a clinicopathological study of 136 cases, Cancer 1977;39:1201-17. 2. Moore DM, Berke as. Synovial sarcoma of the head and neck. Arch Otolaryngol Head Neck Surg 1987;113:311-3. 3. Jemstrom P, Synovial sarcoma of the pharynx: report of a case. Am J Clin Pathol 1954;24:957-61.
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Volume 107 Number 5 November 1992
Head and neck synovial sarcoma
4. Burton OM, Patow CA. Clinical and pathologic diagnosis. Arch Otolaryngol Head Neck Surg 1990:116:1342-5. 5. Batsakis JG. Tumors of the head and neck: clinical and pathological considerations. 2nd ed. Baltimore: Williams & Wilkins, 1979. 6. Harrison EG Jr, Black BM, Devine KD. Synovial sarcoma primary in the neck. Arch Pathol 1961:71:137-41. 7. Roth JA, Enzinger FM, Tannenbaum M. Synovial sarcoma of the neck: a followup study of 24 cases. Cancer 1975;35:124353. 8. Mackenzie DH. Synovial sarcoma: a review of 58 cases. Cancer 1966;19:169-80. 9. Lockey MW. Rare tumors of the ear, nose and throat: synovial sarcoma of the head and neck. South Med J 1976;69:316-20. 10. Oppedal BR, Reyne T, Titterud I. Synovial sarcomas of the neck: a report of two cases. J Laryngol Otol 1985;99:101-4. 11. Ariel 1M, Pack GT. Synovial sarcoma: review of 25 cases. N Engl J Med 1963;268:1272-5.
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12. Mamelle G, Richard J, Luboinski B, Schwaab G, Eschwege F, Micheau C. Synovial sarcoma of the head and neck: an account of four cases and review of the literature. Eur J Surg Oneal 1986;12:347-9. 13. Chang AE, Rosenberg SA, Glatstein EJ, Antman KH. Sarcomas of soft tissue. In: DeVita VT Jr, Hellman S, Rosenberg SA, eds. Cancer: principles and practice of oncology. 3rd ed. Philadelphia: JB Lippincott, 1989:1345-98. 14. Goepfert H, Lindberg RD, Sinkovics JG, Ayala AG. Soft-tissue sarcoma of the head and neck after puberty: treatment by surgery and postoperative radiation therapy. Arch Otolaryngol 1977;103:365-8. 15. Lindberg RD, Martin RG, Romsdahl MM, Barkley HT Jr. Conservative surgery and postoperative radiotherapy in 300 adults with soft-tissue sarcomas. Cancer 1981;47:2391-7.
AV AILABLE NOWI The FIVE-YEAR (1986-1990) CUMULATIVE INDEX TO OTOLARYNGOLOGY -HEAD AND NECK SURGERY can be purchased from the Publisher for $44.00. This comprehensive 104-page reference guide is a current presentation of all topics included in the Journal from January 1986 through December 1990 (volumes 94103)-the past 10 volumes. It incorporates complete references to more than 640 original articles, abstracts, case reports, letters, and editorials. It features 1668 Subject Headings, under which there are 3247 references. Each subject entry lists the complete article title, authorts), volume, page(s), and year of publication. In addition, it includes 3190 Author Entries, which list contributors, along with their respective titles, author-to-author referral, volume, page, and publication. To purchase, call or write: Mosby-Year Book, Inc., 11830 Westline Industrial Dr., St. Louis, Missouri 63146-3318, or telephone FREE 1-800-325-4177, extension 4351, Journal Fulfillment (in Missouri, call collect at 314-872-8370, extension 4351, Journal Fulfillment). PREPAYMENT REQUIRED. Make checks payable to Mosby-Year Book, Inc.; (all payments must be in U.S. funds drawn on a U.S. bank). Price: $44.00 in the U.S.; $50.50 in Canada; and $48.50 in other countries (price includes mailing charges).
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News and Announcements
held July 26-30, 1993, at the Tamarron Resort in Durango, Colorado. This 28 hour review and update will encompass all the clinically important areas of MR imaging. Important new concepts and pathological/imaging correlations in the body, musculoskeletal system, ENT, head and neck, brain, and spine will be explored. Daily case presentations will supplement these lectures and will serve to test the registrants' diagnostic abilities in MR imaging. This complete review of MR imaging will be presented by nationally recognized leaders in magnetic resonance imaging. As a result of this comprehensive review, registrants will become familiar with current applications of MR imaging and will be able to integrate many of these applications directly into their practice. Program chairmen for this presentation will be Robert Quencer, MD (University of Miami), Victor Haughton, MD (Medical College of Wisconsin). Twenty-eight credits of Category I will be available. For further information, please contact Marti Carter, CME, Inc., 11011 West Nort Ave., Milwaukee, Wisconsin 53226, or call (414) 771-9520. Ear, Nose, and Throat Diseases: 1993 Update
Children's Hospital of Pittsburgh will hold its 18th Annual Symposium, "Ear, Nose, and Throat Diseases in Children: A 1993 Update." This symposium will be held July 30-31, 1993. CME credits will be awarded.
For further information, please contact the Department of Pediatric Otolaryngology, Children's Hospital of Pittsburgh, 3705 Fifth Avenue at DeSoto St., Pittsburgh, Pennsylvania 15213, or call (412) 692-8577. Twenty-fifth Annual Meeting - Head and Neck Oncologists
The Association of Head and Neck Oncologists of Great Britain will sponsor the Twenty-fifth Annual Meeting of Head and Neck Oncology, to be held in Edinburgh, Scotland, United Kingdom, on August 23-26, 1993. International and local faculty will present extensive social and family programs. For further information, please contact Mr. P. J. Bradley, Honorary Secretary, Department of Otorhinolaryngology-Head and Neck Surgery, University Hospital, Queens Medical Centre, Nottingham, NG7 2UH, England, or phone 0602421421. Sixth International Congress on Interventlonal Ultrasound
The Sixth International Congress on Interventional Ultrasound will be held in Copenhagen, Denmark, on September 7-10, 1993. For further information, please contact Christian Nolsoe, Congress Secretary, Department of Ultrasound, Herlev Hospital, University of Copenhagen, DK-2730 HerlevDenmark, or call + 45/ 44 53 53 00 ext. 3240.
CORRECTION
The Supplement to the December 1992 issue of the JOURNAL (Volume 107, Number 6, Part 2), incorrectly listed Dr. Bruce R. Gordon as Chief of Otolaryngology at the Massachusetts Eye and Ear Institute. Dr. Joseph Nadol is Chief of Otolaryngology at the Massachusetts Eye and Ear Infirmary. Dr. Gordon is Chief of Otolaryngology at Cape Cod Hospital.
