Alveolar soft-part sarcoma of the tongue Report of a case
Masa-Aki Takita, Masaharu Morishita, Mitsuhiko Iriki-ln, Masayoshi Sakuda, Yasuo Fukuda*, Takeshi Ishida* The 2nd Department of Oral and Maxillofacial Surgery and *Clinical Laboratory, Hospital attached to the Osaka University Faculty of Dentistry, Japan
M.-A. Takita, M. Morishita, M. Iriki-In, M. Sakuda, Y. Fukuda, T. Ishida: Alveolar soft-part sarcoma of the tongue. Report of a case. Int. J. Oral Maxillofae. Surg. 1990; 19." 110-112. Abstract. A case of alveolar soft-part sarcoma (ASPS) of the tongue in a 19-yearold boy is presented. He underwent a hemiglossectomy and received chemotherapy and has been free of disease for 3 years. The origin of intracytoplasmic periodic acid-Shiff(PAS)-positive crystals found in the tumor and the histogenesis of ASPS are briefly discussed.
Alveolar soft-part sarcoma(ASPS) is a very rare malignant tumor of unknown origin. The lesion presents almost always in the muscles of the extremities, and only a few cases in the oral and perioral region have been reported 4'5'14'15'19'2°.The tumor is usually fatal, as in almost all cases the clinical resemblance to a benign lesion leads to delayed or inadequate treatment.
Report of a case In 1985, a 19-year-oldboy first noted a tumor on the dorsal aspect of his tongue. The swelling increased gradually in size, but was not otherwise bothering. Since it was thought to be a fibroma, an excisional biopsy was performed at another hospital. The histopathological diagnosis of ASPS was made. The patient was referred to our clinic for additional therapy. The past medical history was non-contributory and the results of ordinary laboratory studies were within normal ranges. Examination revealed a 17 x 28 mm scar-like mass at the right dorsum of the tongue and an enlarged right-submandibular lymph node. Metastatic lesions were not detectible by X-ray and CT examination. Because of the possibility of some local remaining tumor tissue, a hemiglossectomyand suprahyoid neck dissection were performed. The specimen, however, was negative for tumor cells. Postoperative chemoterapy with cyclophosphamide (total 4.2 g), actinomycin D (total 3.6 g), and vincristine (total 9 mg) was given to prevent distant metastasis. Periodical examination of the patient for the past 3 years revealed no recurrence or metastasis.
Histological and immunohistochemical findings. The biopsy specimen from the first operation was reexamined. The specimen was formalin-fixed, paraffin-embedded, and stained with hematoxilin-eosin (HE), and a periodic acid-Schiff (PAS) reaction was carried out with and without diastase pretreatment. The tumor, which showed a uniform pseudoalveolar or organoid pattern with delicate vascular channels and fine fibrous septa, was composed of large, polygonal cells with a fine granular cytoplasm (Fig. 1). Mitotic figures were scarce, but a separated cluster of tumor cells was observed within a blood vessel (Fig. 2). The PAS reaction revealed a varying amount of intracytoplasmic diastase-resistant granular material (Fig. 3). The tumor was diagnosed as ASPS. Tumor specimens were also studied immunohistochemically, by the avidinbiotin-peroxidase complex (ABC) technique. The primary antibodies employed were monoclonal anti-musclespecific actin (HHF 35, Enzo Biochem, USA), polyclonal anti-desmin (DAKO, USA), and monoclonal anti-vimentin (V9, DAKO, USA). The tumor cells were positive for actin (Fig. 4), occasionally positive for desmin, but negative for vimentin. Discussion ASPS is a tumor of unknown histogenesis first described by CHRrSTOPHERSON
Key words: alveolar soft-part sarcoma;
tongue. Accepted for publication 7 November 1989
et al. 6 in 1952 as a distinct clinicopathological entity. Synonyms previously used include: malignant variant of granular cell myoblastoma and nonchromaffin paraganglioma. CHRISTOPHERSON et al. categorized ASPS separately from other recognized tumors because of its unique histological structure and clinical course. The following is a brief summary of ASPS, based on recent reports 2'~2-~5'2°. The tumor occurs primarily in adolescents and young adults. It is somewhat more frequent in women than in men, the ratio approximately being 2:1. The tumor is almost always found within the skeletal muscles of the extremities. In the head and neck region, the tongue and orbit are most frequently affected. Of 143 cases reviewed by ENZ~NGER & W~Iss1°, 62% (88 cases) occurred in the extremities, 27% (39 cases) in the head and neck (orbit, 16; tongue, 10; and 13 cases elsewhere) al~d 11% (16 cases) in the trunk. The tumor seems to have a predilection for the right side of the body. The lesions are usually slowgrowing, well-circumscribed masses with no distinguishing clinical features that would suggest malignant disease. The histological features of this tumor are, however, striking, and vary little from case to case. ASPS tumor cells are large cells with fine granular cytoplasm that assume a uniform pseudoalveolar or organoid pattern. Many cells contain diastase-resistant crystals that are PAS positive.
Alveolar soft-part sarcoma o f the tongue
111
Fig. 1. The tumor has a pseudoalveolar or organoid structure with cluster of polygonal cells (HE × 100).
Fig. 2. Tumor cluster within a peripheral blood vessel (HE × 25), indicates possibility of micrometastasis.
Fig. 3. Note the intracytoplasmic PAS-posifive granules (PAS with diastase pretreatment × 250).
Fig. 4. Immunostaining for muscle-specificactin (x 100). Tumor cell cytoplasm is diffusely positive (left), but its reactivity is weaker than that of smooth muscle cells in a blood vessel wall (upper right).
These PAS-positive materials, which represent crystalline structures composed of protein-carbohydrate complexes, are seen by electron microscopy as membrane-bounded crystals with a regular periodicitf ,lz. Some investigators have suggested that these structures develop from granules formed as secretory products in the Golgi apparatus 9,15. FISHER & REIDBORD I1, however, pointed out the resemblance between the crystals in ASPS and Z-band-derived rod-like structures. Recently, using computer simulation, MUKAI et a1.18 demonstrated that the structure of these crystals has many similarities to that of actin filaments. As to the origin of ASPS, paraganglionic mesoderm22, striated muscle 11, and a renin-producing tumor s have been proposed. Recent immunohistological studies on ASPS, including this study,
support the myogenic origin, based on the presence of actin and/or desmin (muscle-specific antigens) in the tumor cells 7A7.WANGet al? 1 reported a case of ASPS, which arose in a site previously irradiated as treatment for a hemangioma arising from the pia of the cervical cord. The tumor, usually, has a poor prognosis with early local recurrence and late metastasis to the lung, brain, and skeleton. Multiple metastases are not uncommon13. On the basis of data from a series reported by LmBERMANet al. 16, the 2-year survival rate is 83%; the 5year, 59%; and 10-year, 47%. They noted distant metastases in 28 of 46 cases. ASPS is regarded as a tumor with a tendency towards long-delayed metastases. ASPS is a slow-growing tumor with vascular invasion and this is more extensive than would appear to be the
case on clinical inspection. Postoperative chemotherapy after complete resection of tumor is probably required to prevent distant metastasis of the tumor 3.
References
1. ACKERMANLV. Surgical pathology, 6th ed. St Louis: Mosby, 1981: 1463-5. 2. BATSAKISJG, REGEZIJA, RICEDH. The pathology of head and neck tumors: Fibroadipose tissue and skeletal muscle, part 8. Head Neck Surg. 1980: 3: 145-68. 3. BAUMES, F1CKENSCIqERL, NACHMANJB, IDRISS F. Pulmonary resection and chemotherapy for metastatic alveolar soft-part sarcoma. Cancer 1981: 47: 1946-8. 4. BUCHANANG. Two rare tumors involving infratemporal fossa: alveolar soft part sarcoma and haemangiopericytoma. J Laryngol Otol 1975: 89: 375-89. 5. CALDWELLJB, HUGHESKW, FADELLEJ.
112
Takita et al.
Alveolar soft part sarcoma of the tongue. J Oral Surg 1956: 14: 342-5. 6. CnRISTOPI-mRSON WM, FOOTE FW, STEWART FW. Alveolar soft-part sarcomas, structurally characteristic tumors of uncertain histogenesis. Cancer 1952: 5: 100-11. 7. DENK H, KREPLER R, ARTLIEB U, et al. Proteins of intermediate filaments. An immunohistochemical and biochemical approach to the classification of soft tissue tumors. Am J Pathol 1983: ll0: 193-208. 8. DESCHRYVER-KECSKEMETIK, KRAUS FT, ENGLEMAN W. Alveolar soft-part sarcoma, a malignant angioreninoma, histochemical, immunocytochemical and electron-microscopic study of four cases. Am J Surg Pathol 1982: 6: 5-18. 9. EKFORSTO, KALIMOH, RANTAKOKKOV, LATVALAM, PARVINENM. Alveolar soft part sarcoma. A report of two cases with some histochemical and ultrastructurat observations. Cancer 1979: 43: 1672-7. 10. ENZINGERFM, WEISSSW. Soft tissue tumors, 2nd ed. St Louis: Mosby, 1988: 926-36. 11. FISHERER, REtDBORDH. Electron micro-
scopic evidence suggesting the myogenous derivation of the so-called alveolar soft part sarcoma. Cancer 1971: 27: 150-9. 12. FONT RL, JURCO S, ZIMMERMANLE. Alveolar soft-part sarcoma of the orbit: A clinicopathologic analysis of seventeen cases and a review of the literature. Hum Pathol 1982: 13: 569-79. 13. Ho K-L. Sarcoma metastatic to the central nervous system: report of three cases and review of the literature. Neurosurg 1979: 5: 44-8. 14. KING VB, FEE WE. Alveolar soft part sarcoma of the tongue. Am J Otolaryngol 1983: 4: 363-6. 15. KOMORI A, TAKEDAY, KAKnCHI T. Alveolar soft part sarcoma of the tongue. Oral Surg 1984: 57: 532-9. 16. LIEBERMANPH, FOOTEFW, STEWARTFW, BERG JW. Alveolar soft-part sarcoma. JAMA 1966: 198: 1047-51. 17. MUKAIM, TORIKATAC, IRI H, et al. Alveolar soft part sarcoma. A review of histogenesis and further studies based on electron microscopy, immunohistochemistry, and biochemistry. Am J Surg Pathol 1983: 7: 679-89.
18. MUKAI M, TORIKATAC, IRI H, et al. Alveolar soft part sarcoma. An elaboration of a three-dimensional configuration of the crystalloids by digital image processing. Am J Pathol 1984: 116: 398-406. 19. OLSON RAJ, PERKINSKD. Alveolar so• part sarcoma in the oral cavity: Report of case. J Oral Surg 1976: 34: 73-6. 20. SPECTOR RA, TRAVIS LW, SMITH J. Alveolar soft part sarcoma of the head and neck. Laryngoscope 1979. 89: 1301-6. 21. WANG S, MIRRA J, BHUTA S. Alveolar soft part sarcoma following radiotherapy for a spinal hemangioma. Cancer 1984: 53: 2655-60. 22. WELSH RA, BRAY DM, SHIPKEY FH, MEYER AT. Histogenesis of alveolar soft part sarcoma. Cancer 1972: 29. 191-204. Address:
Masa-Aki Takita 2nd Dept. of Oral and Maxillofacial Surgery Osaka University Faculty of Dentistry 1-8 Yamadaoka, Suita Osaka 565 Japan
Masa-Aki Takita, Masaharu Morishita, Mitsuhiko Iriki-ln, Masayoshi Sakuda, Yasuo Fukuda*, Takeshi Ishida* The 2nd Department of Oral and Maxillofacial Surgery and *Clinical Laboratory, Hospital attached to the Osaka University Faculty of Dentistry, Japan
M.-A. Takita, M. Morishita, M. Iriki-In, M. Sakuda, Y. Fukuda, T. Ishida: Alveolar soft-part sarcoma of the tongue. Report of a case. Int. J. Oral Maxillofae. Surg. 1990; 19." 110-112. Abstract. A case of alveolar soft-part sarcoma (ASPS) of the tongue in a 19-yearold boy is presented. He underwent a hemiglossectomy and received chemotherapy and has been free of disease for 3 years. The origin of intracytoplasmic periodic acid-Shiff(PAS)-positive crystals found in the tumor and the histogenesis of ASPS are briefly discussed.
Alveolar soft-part sarcoma(ASPS) is a very rare malignant tumor of unknown origin. The lesion presents almost always in the muscles of the extremities, and only a few cases in the oral and perioral region have been reported 4'5'14'15'19'2°.The tumor is usually fatal, as in almost all cases the clinical resemblance to a benign lesion leads to delayed or inadequate treatment.
Report of a case In 1985, a 19-year-oldboy first noted a tumor on the dorsal aspect of his tongue. The swelling increased gradually in size, but was not otherwise bothering. Since it was thought to be a fibroma, an excisional biopsy was performed at another hospital. The histopathological diagnosis of ASPS was made. The patient was referred to our clinic for additional therapy. The past medical history was non-contributory and the results of ordinary laboratory studies were within normal ranges. Examination revealed a 17 x 28 mm scar-like mass at the right dorsum of the tongue and an enlarged right-submandibular lymph node. Metastatic lesions were not detectible by X-ray and CT examination. Because of the possibility of some local remaining tumor tissue, a hemiglossectomyand suprahyoid neck dissection were performed. The specimen, however, was negative for tumor cells. Postoperative chemoterapy with cyclophosphamide (total 4.2 g), actinomycin D (total 3.6 g), and vincristine (total 9 mg) was given to prevent distant metastasis. Periodical examination of the patient for the past 3 years revealed no recurrence or metastasis.
Histological and immunohistochemical findings. The biopsy specimen from the first operation was reexamined. The specimen was formalin-fixed, paraffin-embedded, and stained with hematoxilin-eosin (HE), and a periodic acid-Schiff (PAS) reaction was carried out with and without diastase pretreatment. The tumor, which showed a uniform pseudoalveolar or organoid pattern with delicate vascular channels and fine fibrous septa, was composed of large, polygonal cells with a fine granular cytoplasm (Fig. 1). Mitotic figures were scarce, but a separated cluster of tumor cells was observed within a blood vessel (Fig. 2). The PAS reaction revealed a varying amount of intracytoplasmic diastase-resistant granular material (Fig. 3). The tumor was diagnosed as ASPS. Tumor specimens were also studied immunohistochemically, by the avidinbiotin-peroxidase complex (ABC) technique. The primary antibodies employed were monoclonal anti-musclespecific actin (HHF 35, Enzo Biochem, USA), polyclonal anti-desmin (DAKO, USA), and monoclonal anti-vimentin (V9, DAKO, USA). The tumor cells were positive for actin (Fig. 4), occasionally positive for desmin, but negative for vimentin. Discussion ASPS is a tumor of unknown histogenesis first described by CHRrSTOPHERSON
Key words: alveolar soft-part sarcoma;
tongue. Accepted for publication 7 November 1989
et al. 6 in 1952 as a distinct clinicopathological entity. Synonyms previously used include: malignant variant of granular cell myoblastoma and nonchromaffin paraganglioma. CHRISTOPHERSON et al. categorized ASPS separately from other recognized tumors because of its unique histological structure and clinical course. The following is a brief summary of ASPS, based on recent reports 2'~2-~5'2°. The tumor occurs primarily in adolescents and young adults. It is somewhat more frequent in women than in men, the ratio approximately being 2:1. The tumor is almost always found within the skeletal muscles of the extremities. In the head and neck region, the tongue and orbit are most frequently affected. Of 143 cases reviewed by ENZ~NGER & W~Iss1°, 62% (88 cases) occurred in the extremities, 27% (39 cases) in the head and neck (orbit, 16; tongue, 10; and 13 cases elsewhere) al~d 11% (16 cases) in the trunk. The tumor seems to have a predilection for the right side of the body. The lesions are usually slowgrowing, well-circumscribed masses with no distinguishing clinical features that would suggest malignant disease. The histological features of this tumor are, however, striking, and vary little from case to case. ASPS tumor cells are large cells with fine granular cytoplasm that assume a uniform pseudoalveolar or organoid pattern. Many cells contain diastase-resistant crystals that are PAS positive.
Alveolar soft-part sarcoma o f the tongue
111
Fig. 1. The tumor has a pseudoalveolar or organoid structure with cluster of polygonal cells (HE × 100).
Fig. 2. Tumor cluster within a peripheral blood vessel (HE × 25), indicates possibility of micrometastasis.
Fig. 3. Note the intracytoplasmic PAS-posifive granules (PAS with diastase pretreatment × 250).
Fig. 4. Immunostaining for muscle-specificactin (x 100). Tumor cell cytoplasm is diffusely positive (left), but its reactivity is weaker than that of smooth muscle cells in a blood vessel wall (upper right).
These PAS-positive materials, which represent crystalline structures composed of protein-carbohydrate complexes, are seen by electron microscopy as membrane-bounded crystals with a regular periodicitf ,lz. Some investigators have suggested that these structures develop from granules formed as secretory products in the Golgi apparatus 9,15. FISHER & REIDBORD I1, however, pointed out the resemblance between the crystals in ASPS and Z-band-derived rod-like structures. Recently, using computer simulation, MUKAI et a1.18 demonstrated that the structure of these crystals has many similarities to that of actin filaments. As to the origin of ASPS, paraganglionic mesoderm22, striated muscle 11, and a renin-producing tumor s have been proposed. Recent immunohistological studies on ASPS, including this study,
support the myogenic origin, based on the presence of actin and/or desmin (muscle-specific antigens) in the tumor cells 7A7.WANGet al? 1 reported a case of ASPS, which arose in a site previously irradiated as treatment for a hemangioma arising from the pia of the cervical cord. The tumor, usually, has a poor prognosis with early local recurrence and late metastasis to the lung, brain, and skeleton. Multiple metastases are not uncommon13. On the basis of data from a series reported by LmBERMANet al. 16, the 2-year survival rate is 83%; the 5year, 59%; and 10-year, 47%. They noted distant metastases in 28 of 46 cases. ASPS is regarded as a tumor with a tendency towards long-delayed metastases. ASPS is a slow-growing tumor with vascular invasion and this is more extensive than would appear to be the
case on clinical inspection. Postoperative chemotherapy after complete resection of tumor is probably required to prevent distant metastasis of the tumor 3.
References
1. ACKERMANLV. Surgical pathology, 6th ed. St Louis: Mosby, 1981: 1463-5. 2. BATSAKISJG, REGEZIJA, RICEDH. The pathology of head and neck tumors: Fibroadipose tissue and skeletal muscle, part 8. Head Neck Surg. 1980: 3: 145-68. 3. BAUMES, F1CKENSCIqERL, NACHMANJB, IDRISS F. Pulmonary resection and chemotherapy for metastatic alveolar soft-part sarcoma. Cancer 1981: 47: 1946-8. 4. BUCHANANG. Two rare tumors involving infratemporal fossa: alveolar soft part sarcoma and haemangiopericytoma. J Laryngol Otol 1975: 89: 375-89. 5. CALDWELLJB, HUGHESKW, FADELLEJ.
112
Takita et al.
Alveolar soft part sarcoma of the tongue. J Oral Surg 1956: 14: 342-5. 6. CnRISTOPI-mRSON WM, FOOTE FW, STEWART FW. Alveolar soft-part sarcomas, structurally characteristic tumors of uncertain histogenesis. Cancer 1952: 5: 100-11. 7. DENK H, KREPLER R, ARTLIEB U, et al. Proteins of intermediate filaments. An immunohistochemical and biochemical approach to the classification of soft tissue tumors. Am J Pathol 1983: ll0: 193-208. 8. DESCHRYVER-KECSKEMETIK, KRAUS FT, ENGLEMAN W. Alveolar soft-part sarcoma, a malignant angioreninoma, histochemical, immunocytochemical and electron-microscopic study of four cases. Am J Surg Pathol 1982: 6: 5-18. 9. EKFORSTO, KALIMOH, RANTAKOKKOV, LATVALAM, PARVINENM. Alveolar soft part sarcoma. A report of two cases with some histochemical and ultrastructurat observations. Cancer 1979: 43: 1672-7. 10. ENZINGERFM, WEISSSW. Soft tissue tumors, 2nd ed. St Louis: Mosby, 1988: 926-36. 11. FISHERER, REtDBORDH. Electron micro-
scopic evidence suggesting the myogenous derivation of the so-called alveolar soft part sarcoma. Cancer 1971: 27: 150-9. 12. FONT RL, JURCO S, ZIMMERMANLE. Alveolar soft-part sarcoma of the orbit: A clinicopathologic analysis of seventeen cases and a review of the literature. Hum Pathol 1982: 13: 569-79. 13. Ho K-L. Sarcoma metastatic to the central nervous system: report of three cases and review of the literature. Neurosurg 1979: 5: 44-8. 14. KING VB, FEE WE. Alveolar soft part sarcoma of the tongue. Am J Otolaryngol 1983: 4: 363-6. 15. KOMORI A, TAKEDAY, KAKnCHI T. Alveolar soft part sarcoma of the tongue. Oral Surg 1984: 57: 532-9. 16. LIEBERMANPH, FOOTEFW, STEWARTFW, BERG JW. Alveolar soft-part sarcoma. JAMA 1966: 198: 1047-51. 17. MUKAIM, TORIKATAC, IRI H, et al. Alveolar soft part sarcoma. A review of histogenesis and further studies based on electron microscopy, immunohistochemistry, and biochemistry. Am J Surg Pathol 1983: 7: 679-89.
18. MUKAI M, TORIKATAC, IRI H, et al. Alveolar soft part sarcoma. An elaboration of a three-dimensional configuration of the crystalloids by digital image processing. Am J Pathol 1984: 116: 398-406. 19. OLSON RAJ, PERKINSKD. Alveolar so• part sarcoma in the oral cavity: Report of case. J Oral Surg 1976: 34: 73-6. 20. SPECTOR RA, TRAVIS LW, SMITH J. Alveolar soft part sarcoma of the head and neck. Laryngoscope 1979. 89: 1301-6. 21. WANG S, MIRRA J, BHUTA S. Alveolar soft part sarcoma following radiotherapy for a spinal hemangioma. Cancer 1984: 53: 2655-60. 22. WELSH RA, BRAY DM, SHIPKEY FH, MEYER AT. Histogenesis of alveolar soft part sarcoma. Cancer 1972: 29. 191-204. Address:
Masa-Aki Takita 2nd Dept. of Oral and Maxillofacial Surgery Osaka University Faculty of Dentistry 1-8 Yamadaoka, Suita Osaka 565 Japan
