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Oral synovial sarcoma: Report of a case
DE
1001
ARAUJO AND MONTEIRO
J Oral Maxlllofac
Surg
47 1001-1003.1989
Oral Synovial Sarcoma: Report of a Case VERA C. DE ARAljJO*
AND DARCY CM. MONTElROt
The intraoral occurrence of synovial sarcoma is extremely rare; the world literature reveals only 12 published cases. Most of the lesions occurred on the base of the tongue, and only six occurred elsewhere (Table 1). ‘-10 Although synovial sarcomas in the mouth are rare. knowledge of the existence of these tumors is of practical importance for clinicians and pathologists. This report presents an additional case of synovial sarcoma involving the oral mucosa, reviews its clinicopathologic features, and describes an immunoperoxidase study which constitutes a valuable aid to diagnosis. Report of a Case A 36-year-old man had a 3-month history of a 2-cm painless, firm, nodular mass on his right buccal mucosa. No neck masses or lymphadenopathy were noted at the time of examination. The patient was otherwise in good health.The clinical impression was of a benign neoplasm, either a fibroma or a salivary gland tumor. The lesion was excised, and the specimen was sent for histologic examination. The postoperative period was uncomplicated. and examination up to 7 months later showed the patient to be clinically free of disease. PATHOLOGIC FINDINGS Grossly. the lesion consisted of an oval, rubbery. soft mass measuring 2 x 2 x 1.5 cm. The cut surfaces of the bisected specimen had a homogenous whitish-tan color. Histologically, the tumor was composed of two distinct cell types. One type appeared to be a spindle-shaped cell with a scant cytoplasm and oval nucleus distributed in bands or sheets. Interspersed within this cellular mass were duct-like or slit-like structures lined by cuboidal epithelial-like ceils. The tumor was not encapsulated, and infiltrated the surrounding tissues. Mitoses were rarely seen (Figs 1 and 2). Received from the University of SPo Paulo, Brazil. * Professor and Chairman, Department of Oral Pathology. School of Dentistry. t Associate Professor, Department of Pathology, School of Medicine. Address correspondence and reprint requests to Dr Arauju: Disciplina de Pathologia Bucal, Faculdade de Odontologia. Universidade de Sao Paulo, CP 8216, Sao Paulo. SP, Brasil. ,C 1989 American Association of Oral and Maxillofacial geons 0278-2391/89/4709-0020$3.00/0
Sur-
Immunohistochemical study revealed that the lining cells of the duct-like spaces were positive for keratin and epithelial membrane antigen (EMA). The spindle-shaped cells were positive for vimentin (Figs 3, 4. and 5). The tumor cells were negative for S-100 protein, glial librillary acidic protein (GFAP), and neuron-specific enolase CNSE). Discussion
Synovial sarcoma is a well-defined entity that accounts for up to 10% of all soft-tissue sarcomas.” The tumor occurs primarily in young adults in the deep soft tissues of the extremities, most commonly the lower thigh-knee region.’ Histologically, the synovial sarcoma is characterized by a biphasic cellular pattern formed by the proliferation of a spindle-cell component and the presence of duct-like and slit-like spaces lined by cuboidal epithelial-like cells. A monophasic pattern lacking either the epithelial-like cells or the spindle cells is also said to exist, but most investigators are very reluctant to make the diagnosis of synovial sarcoma in such cases.” According to Mackenzie.13 this biphasic pattern is the only valid histologic criterion by which synovial sarcomas may be diagnosed with certainty. Table 1. Summary of Intraoral Cases Previously Reported Age (yr)
Sex
Location
23 21 40 21 16 25
F M M M M M M
Moore and Berke’
35 34 16 35
M F
Base of tongue Base of tongue Base of tongue Base of tongue Base of tongue Base of tongue, and tonsil Tonsil Tonsil Dorsal tongue. L Gingivobuccal
Massarelli et al’ Nunez-Alonso et al”’
19 I1
M M
Palate Retromolar, L
Reference
Mir-Abedy ’ Novotny and Fort’ Moussavi and Ghodsi’ Guzman et al” Holtz and Magielski’ Engelhardt and Laefstedth Shmookler et al’
M
sulcus
-
ORAL SYNOVIAL SARCOMA
FIGURE 3. Positive staining of duct-like cells for kerdtin: peroxidase-antiperoxidase (PAP) method. 1:400 dilution. counterstained with Mayer’s hematoxylin. (Original magnification. X400.)
The histogenesis of synovial sarcoma remains controversial. An origin from synovia, epithelium, and neural tissue has been postulated. According to Batsakis et aLI most synovial sarcomas do not arise from formed adult synovial membranes, and primary involvement of a joint cavity is infrequent. The synovial sarcomas occurring in the neck and
oral cavity are examples of this condition. The synovial tissue in the tendinous part of the cervical and facial muscles, and the totipotentiality of mesenchymal cells, probably account for the origin of the tumor in this location. Particularly in our case, the fascia of the masseter or buccinator can be considered as potential sites of origin of the tumor. Except for the extremely unusual location. the case reported presents clinically and histologically the usual characteristics of a synovial sarcoma. Our patient was a 36-year-old man with a 3-month history of a painless nodular mass in the buccal mucosa. The conventional type of synovial sarcoma occurs primarily in adults in the third and fourth decades. being more frequent in men than in women, with a reported ratio of 3:2.” The classic histologic biphasic pattern was seen in this patient. The immunohistochemical study revealed that the duct-like cells were positive for keratin and EMA. EMA has been demonstrated in exocrine glands where it is confined to the luminal surface of cuboidal and nonmucinous columnar epithelium of
FIGURE 2. Biphasic appearance of synovial sarcoma. Cuboidal cells lining large clefts interspersed by fusiform cells can be seen. (Hematoxylin and eosin. Original magnification, X 160.)
FiGURE 4. Positive staining of fusiform cells for vimentin. (PAP, 1:75. Original magnification. X 160.)
FIGURE 1. Tumor mass in the deep portion of the buccal mucosa. (Hematoxylin and eosin. Original magnification. x63.)
1003
DF ARAUJO AND MONTEIRO
literature is reviewed. Immunohistochemical findings concerning the histogenesis of the tumor are discussed. References I. Mir-Abedy M: Considerations 2. 3. 4.
FIGURE 5. Presence of EMA in duct-like cells (PAP. I:lO. Original magnification, X400.1
5. 6. 7.
acini and ducts.“-‘s This finding is in accordance with the electron-microscopic findings of Gabbiani et al,” who showed microvilli at the luminal surface of synovioma duct-like cells. Vimentin was detected in the fusiform cells. Leader et al,” based on the positivity of the tumor cells to keratin and vimentin filaments, have suggested that synovial sarcoma might be regarded as a carcinosarcoma. Nevertheless, it is very important to remember that in other tumors, such as the salivary gland tumors of intercalated duct origin, luminal cells are keratin positive, whereas nonluminal cells (myoepithelial cells) are vimentin positive, even though both have the same epithelial origin (Araujo et al, manuscript in preparation). The small group of tumors reported with oral location do not permit conclusions regarding optimal treatment. It is noteworthy, however, that in the survivors’ group surgery was the main treatment used, whereas in the fatal cases the basic treatment consisted of irradiation and chemotherapy.
8. 9. IO.
1I. 12. 13. 14. IS.
16.
17.
18.
Summary
A case of synovial sarcoma arising in the buccal mucosa of a %-year-old man is reported, and the
19.
of the base of the tongue and its tumors. Ann d’Otolaryngol Chirurg Cervico-Fat 791547. 1962 Novotny GM. Fort TC: Synovial sarcoma of the tongue. Arch Otolaryngol 94:77. 1971 Moussavi H, Ghodsi S: Synovial sarcoma of the tongue: Report of a case. J Laryngol Otol 88:795, 1974 Guzman P, Tort-es HO. Gauza RC: Synovial sarcomas of the oral cavity (foramen caecum): Report of a case and review of the literature. Prensa Med Mex 40:215. 1975 Holtz F. Magielski JE: Synovial sarcomas of the tongue base. Arch Otolaryngol 111:271, 1985 Engelhardt SJ. Laefstedt SW: Synovial sarcoma of tonsil and tongue base. South Med J 76:243. 1983 Shmookler BM. Enzinger FM, Brannon RB: Orofaciai synovial sarcoma. A clinicopathologic study of I1 new cases and review of the literature. Cancer 50:269, 1982 Moore DM. Berke GS: Synovial sarcoma of the head and neck. Arch Otolaryngol Head Neck Surg Il3:31 I, 1987 Massarelli G, Tanda F. Salis B: Synovial sarcoma of the soft palate: Report of a case. Hum Pathol 9:341, 1978 Nunez-Alonso C. Gashti EM, Christ ML: Maxillofacial synovial sarcoma: Light- and electron-microscopic study of two classes. Am J Surg Pathol 3:23. 1979 Leader M. Pate1 J. Collins M. et al: Synovial sarcomas. True carcinosarcomas? Cancer 59:2096, 1987 Mitcherling JJ. Collins EM. Tomich CE, et al: Synovial sarcoma of the neck-Report of case. J Oral Surg 34:64, 1976 Mackenzie DH: Synovial sarcoma. A review of 58 cases. Cancer 19:169. 1966 Batsakis JG. Nishiyama RH. Sullinger GD: Synovial sarcomas of the neck. Arch Otolaryngol 85:113. 1967 Krugman ME, Rosin HD, Toker C: Synovial sarcoma of the head and neck. Arch Otolaryngol Head Neck Surg 98:53, I973 Cordell J. et al: Production of monoclonal antibodies against human epithelial membrane antigen for use in diagnostic immunocytochemistry. Br J Cancer 52:347. 1985 Heyderman E, Steele K, Ormerod MG: A new antigen on the epithelial membrane: Its immunoperoxidase localization in normal and neoplastic tissue. J Clin Pdthol 37:35. 1979 Ellis IO, Robins RA, Elston CW, et al: A monoclonal antibody. NCRC-I I. raised to human breast carcinoma I. Production and immunohistological characterization. Histopathology 8:501. 1984 Gabbiani G. Kaye GI. Lattes R. et al: Synovial sarcoma. Electron-microscopic study of a typical case. Cancer 28:1031, 1971
1001
ARAUJO AND MONTEIRO
J Oral Maxlllofac
Surg
47 1001-1003.1989
Oral Synovial Sarcoma: Report of a Case VERA C. DE ARAljJO*
AND DARCY CM. MONTElROt
The intraoral occurrence of synovial sarcoma is extremely rare; the world literature reveals only 12 published cases. Most of the lesions occurred on the base of the tongue, and only six occurred elsewhere (Table 1). ‘-10 Although synovial sarcomas in the mouth are rare. knowledge of the existence of these tumors is of practical importance for clinicians and pathologists. This report presents an additional case of synovial sarcoma involving the oral mucosa, reviews its clinicopathologic features, and describes an immunoperoxidase study which constitutes a valuable aid to diagnosis. Report of a Case A 36-year-old man had a 3-month history of a 2-cm painless, firm, nodular mass on his right buccal mucosa. No neck masses or lymphadenopathy were noted at the time of examination. The patient was otherwise in good health.The clinical impression was of a benign neoplasm, either a fibroma or a salivary gland tumor. The lesion was excised, and the specimen was sent for histologic examination. The postoperative period was uncomplicated. and examination up to 7 months later showed the patient to be clinically free of disease. PATHOLOGIC FINDINGS Grossly. the lesion consisted of an oval, rubbery. soft mass measuring 2 x 2 x 1.5 cm. The cut surfaces of the bisected specimen had a homogenous whitish-tan color. Histologically, the tumor was composed of two distinct cell types. One type appeared to be a spindle-shaped cell with a scant cytoplasm and oval nucleus distributed in bands or sheets. Interspersed within this cellular mass were duct-like or slit-like structures lined by cuboidal epithelial-like ceils. The tumor was not encapsulated, and infiltrated the surrounding tissues. Mitoses were rarely seen (Figs 1 and 2). Received from the University of SPo Paulo, Brazil. * Professor and Chairman, Department of Oral Pathology. School of Dentistry. t Associate Professor, Department of Pathology, School of Medicine. Address correspondence and reprint requests to Dr Arauju: Disciplina de Pathologia Bucal, Faculdade de Odontologia. Universidade de Sao Paulo, CP 8216, Sao Paulo. SP, Brasil. ,C 1989 American Association of Oral and Maxillofacial geons 0278-2391/89/4709-0020$3.00/0
Sur-
Immunohistochemical study revealed that the lining cells of the duct-like spaces were positive for keratin and epithelial membrane antigen (EMA). The spindle-shaped cells were positive for vimentin (Figs 3, 4. and 5). The tumor cells were negative for S-100 protein, glial librillary acidic protein (GFAP), and neuron-specific enolase CNSE). Discussion
Synovial sarcoma is a well-defined entity that accounts for up to 10% of all soft-tissue sarcomas.” The tumor occurs primarily in young adults in the deep soft tissues of the extremities, most commonly the lower thigh-knee region.’ Histologically, the synovial sarcoma is characterized by a biphasic cellular pattern formed by the proliferation of a spindle-cell component and the presence of duct-like and slit-like spaces lined by cuboidal epithelial-like cells. A monophasic pattern lacking either the epithelial-like cells or the spindle cells is also said to exist, but most investigators are very reluctant to make the diagnosis of synovial sarcoma in such cases.” According to Mackenzie.13 this biphasic pattern is the only valid histologic criterion by which synovial sarcomas may be diagnosed with certainty. Table 1. Summary of Intraoral Cases Previously Reported Age (yr)
Sex
Location
23 21 40 21 16 25
F M M M M M M
Moore and Berke’
35 34 16 35
M F
Base of tongue Base of tongue Base of tongue Base of tongue Base of tongue Base of tongue, and tonsil Tonsil Tonsil Dorsal tongue. L Gingivobuccal
Massarelli et al’ Nunez-Alonso et al”’
19 I1
M M
Palate Retromolar, L
Reference
Mir-Abedy ’ Novotny and Fort’ Moussavi and Ghodsi’ Guzman et al” Holtz and Magielski’ Engelhardt and Laefstedth Shmookler et al’
M
sulcus
-
ORAL SYNOVIAL SARCOMA
FIGURE 3. Positive staining of duct-like cells for kerdtin: peroxidase-antiperoxidase (PAP) method. 1:400 dilution. counterstained with Mayer’s hematoxylin. (Original magnification. X400.)
The histogenesis of synovial sarcoma remains controversial. An origin from synovia, epithelium, and neural tissue has been postulated. According to Batsakis et aLI most synovial sarcomas do not arise from formed adult synovial membranes, and primary involvement of a joint cavity is infrequent. The synovial sarcomas occurring in the neck and
oral cavity are examples of this condition. The synovial tissue in the tendinous part of the cervical and facial muscles, and the totipotentiality of mesenchymal cells, probably account for the origin of the tumor in this location. Particularly in our case, the fascia of the masseter or buccinator can be considered as potential sites of origin of the tumor. Except for the extremely unusual location. the case reported presents clinically and histologically the usual characteristics of a synovial sarcoma. Our patient was a 36-year-old man with a 3-month history of a painless nodular mass in the buccal mucosa. The conventional type of synovial sarcoma occurs primarily in adults in the third and fourth decades. being more frequent in men than in women, with a reported ratio of 3:2.” The classic histologic biphasic pattern was seen in this patient. The immunohistochemical study revealed that the duct-like cells were positive for keratin and EMA. EMA has been demonstrated in exocrine glands where it is confined to the luminal surface of cuboidal and nonmucinous columnar epithelium of
FIGURE 2. Biphasic appearance of synovial sarcoma. Cuboidal cells lining large clefts interspersed by fusiform cells can be seen. (Hematoxylin and eosin. Original magnification, X 160.)
FiGURE 4. Positive staining of fusiform cells for vimentin. (PAP, 1:75. Original magnification. X 160.)
FIGURE 1. Tumor mass in the deep portion of the buccal mucosa. (Hematoxylin and eosin. Original magnification. x63.)
1003
DF ARAUJO AND MONTEIRO
literature is reviewed. Immunohistochemical findings concerning the histogenesis of the tumor are discussed. References I. Mir-Abedy M: Considerations 2. 3. 4.
FIGURE 5. Presence of EMA in duct-like cells (PAP. I:lO. Original magnification, X400.1
5. 6. 7.
acini and ducts.“-‘s This finding is in accordance with the electron-microscopic findings of Gabbiani et al,” who showed microvilli at the luminal surface of synovioma duct-like cells. Vimentin was detected in the fusiform cells. Leader et al,” based on the positivity of the tumor cells to keratin and vimentin filaments, have suggested that synovial sarcoma might be regarded as a carcinosarcoma. Nevertheless, it is very important to remember that in other tumors, such as the salivary gland tumors of intercalated duct origin, luminal cells are keratin positive, whereas nonluminal cells (myoepithelial cells) are vimentin positive, even though both have the same epithelial origin (Araujo et al, manuscript in preparation). The small group of tumors reported with oral location do not permit conclusions regarding optimal treatment. It is noteworthy, however, that in the survivors’ group surgery was the main treatment used, whereas in the fatal cases the basic treatment consisted of irradiation and chemotherapy.
8. 9. IO.
1I. 12. 13. 14. IS.
16.
17.
18.
Summary
A case of synovial sarcoma arising in the buccal mucosa of a %-year-old man is reported, and the
19.
of the base of the tongue and its tumors. Ann d’Otolaryngol Chirurg Cervico-Fat 791547. 1962 Novotny GM. Fort TC: Synovial sarcoma of the tongue. Arch Otolaryngol 94:77. 1971 Moussavi H, Ghodsi S: Synovial sarcoma of the tongue: Report of a case. J Laryngol Otol 88:795, 1974 Guzman P, Tort-es HO. Gauza RC: Synovial sarcomas of the oral cavity (foramen caecum): Report of a case and review of the literature. Prensa Med Mex 40:215. 1975 Holtz F. Magielski JE: Synovial sarcomas of the tongue base. Arch Otolaryngol 111:271, 1985 Engelhardt SJ. Laefstedt SW: Synovial sarcoma of tonsil and tongue base. South Med J 76:243. 1983 Shmookler BM. Enzinger FM, Brannon RB: Orofaciai synovial sarcoma. A clinicopathologic study of I1 new cases and review of the literature. Cancer 50:269, 1982 Moore DM. Berke GS: Synovial sarcoma of the head and neck. Arch Otolaryngol Head Neck Surg Il3:31 I, 1987 Massarelli G, Tanda F. Salis B: Synovial sarcoma of the soft palate: Report of a case. Hum Pathol 9:341, 1978 Nunez-Alonso C. Gashti EM, Christ ML: Maxillofacial synovial sarcoma: Light- and electron-microscopic study of two classes. Am J Surg Pathol 3:23. 1979 Leader M. Pate1 J. Collins M. et al: Synovial sarcomas. True carcinosarcomas? Cancer 59:2096, 1987 Mitcherling JJ. Collins EM. Tomich CE, et al: Synovial sarcoma of the neck-Report of case. J Oral Surg 34:64, 1976 Mackenzie DH: Synovial sarcoma. A review of 58 cases. Cancer 19:169. 1966 Batsakis JG. Nishiyama RH. Sullinger GD: Synovial sarcomas of the neck. Arch Otolaryngol 85:113. 1967 Krugman ME, Rosin HD, Toker C: Synovial sarcoma of the head and neck. Arch Otolaryngol Head Neck Surg 98:53, I973 Cordell J. et al: Production of monoclonal antibodies against human epithelial membrane antigen for use in diagnostic immunocytochemistry. Br J Cancer 52:347. 1985 Heyderman E, Steele K, Ormerod MG: A new antigen on the epithelial membrane: Its immunoperoxidase localization in normal and neoplastic tissue. J Clin Pdthol 37:35. 1979 Ellis IO, Robins RA, Elston CW, et al: A monoclonal antibody. NCRC-I I. raised to human breast carcinoma I. Production and immunohistological characterization. Histopathology 8:501. 1984 Gabbiani G. Kaye GI. Lattes R. et al: Synovial sarcoma. Electron-microscopic study of a typical case. Cancer 28:1031, 1971