Mesenchymal chondrosarcoma of the maxilla
Tadahiro Ito, Hiroyoshi Hiratsuka, Gen-iku Kohama Department of Oral Surgery, Sapporo Medical College, Japan
Report of a case T. Ito, H. Hiratsuka, G. Kohama: Mesenchymal chondrosarcoma of the maxilla. Int. J. Oral Maxillofac. Surg. 1991; 20." 44-45. Abstract. L i g h t m i c r o s c o p y revealed a n atypical case o f m e s e n c h y m a l c h o n d r o s a r c o m a o f the maxilla in a 62-year-old man.
M e s e n c h y m a l c h o n d r o s a r c o m a ( M C ) is relatively rare a n d characterized b y a worse p r o g n o s i s t h a n o t h e r f o r m s o f c h o n d r o s a r c o m a . It h a s a strong tendency to metastasize to d i s t a n t sites. Predilection sites o f the t u m o r are t h e rib a n d jaws 6. A survey o f 23 cases o f M C o f the jaws h a s s h o w n a predilection for occurrence in the 2nd a n d 3rd decades I. A case is presented of a n elderly p a t i e n t with M C o f the maxilla.
Case report A 62-year-old man was referred to our clinic, complaining of a swelling in the right maxilla which had grown rapidly from a small nodule on the right side of his nose. Examination revealed a firm, painless tumor of 3 x 4 cm. There were no signs of involvement of the cranial nerve. There was some diffuse buccaI expansion of the gingiva, with pus discharging through two fistula. X-ray and CT examination demonstrated an ill-defined expansile radiopaque lesion with high-density tissue proliferation of the anterior wall of the maxillary sinus and a mixed low- and high-density mass anterior to the hard tissue mass. An incisional biopsy was performed and a preliminary diagnosis of atypical chondroma was made. The patient was admitted for a partial maxillectomy. Microscopic examination of the resected specimen revealed a large amount of chondroid material in a lobulated arrangement. The cells were large but variable in size in different locations. Small variably shaped cartilaginous islands separated by sheets of poorly differentiated mesenchymal cells were observed (Fig. 1). Calcification was prominent in the central zone of the chondroid material (Fig. 2). Highly cellular masses composed of small basophilic round and ovoid cells with scanty cytoplasm admixed with spindle-shaped cells were also seen in the peripheral portions of the cartilaginous tissues (Fig. 3). These cells proliferated around numerous capillaries, but the cellular regions occupied only a small part
of the neoplasm. Mitotic figures were rarely seen. Periodic acid-Schiff stains with or without diastase were positive for glycogen. A histopathologic diagnosis of MC was made. During follow-up examination, the patient complained of pain in the right orbit, and both poor and double vision. A C T scan demonstrated bone destruction at the base of the skull. The patient underwent 7 cycles of chemotherapy with methotrexate (total 70 g), cisplatin (total 560 nag), and adriamycin (total 280 mg), but no tumor reduction was seen. Forty-seven months after the first examination the patient died with multiple distant metastases.
Discussion M C is a n aggressive n e o p l a s m first described by LICHTENSTEIN & BERNSTEIN4 as a distinct clinicopathologic entity. The h i s t o p a t h o l o g i c a p p e a r a n c e o f M C has been well described 14. These t u m o r s
Key words: mesenchymal chondrosarcoma. Accepted for publication 29 August 1990
m a y d e m o n s t r a t e a b u n d a n t sheets or clusters o f highly u n d i f f e r e n t i a t e d mese n c h y m a l cells interspersed w i t h small islands o f well-differentiated cartilaginous tissues in w h i c h calcification a n d metaplastic b o n e f o r m a t i o n is often observed. T h e present t u m o r exhibited a large a m o u n t o f well-differentiated cartilaginous m a t e r i a l at the center a n d atypical microscopic features w h i c h differ f r o m previous reports. These microscopic features a n d the presence o f dental infection m i g h t lead to a misdiagnosis o f atypical c h o n d r o m a . A similar case described b y POTOAR et al. 5 was initially diagnosed as o s t e o c h o n droma. Diagnosis o f this type o f t u m o r is often difficult as p o i n t e d o u t by other a u t h o r s 2,7. Radical surgery, if possible, has b e e n suggested as the t r e a t m e n t o f choice for
Fig. 1. Histologic section showing characteristic appearance. Chondroid island and undifferentiated elements are seen (H&E, magnification x 118)
Mesenchymal chondrosarcoma of the maxilla
45
t h a t the 5-year survival rate was 4 2 % , a n d at 10 years 2 8 % o f the M C patients were alive w i t h or w i t h o u t disease. Chemotherapy and radiotherapy have been used as a t e r m i n a l measure, whereas several a u t h o r s have r e p o r t e d the usefulness o f a c o m b i n e d a p p r o a c h u s i n g radiotherapy and/or chemotherapy and surgery 3
?
Acknowledgement. The authors thank Dr. Noriyuki Sato, Department of Pathology, Sapporo Medical College, for his critical advice. References
Fig. 2. Histologic section stained by the Kossa method. Calcification is prominent in the central zone of the chondroid material (magnification x 118)
1. CHRISTENSENRE JR. Mesenchymal chondrosarcoma of the jaws. Oral Surg 1982: 54: 197-206. 2. HOLLINS RR, LYDIATTDD, MARKIN RS, DAVIS LF. Mesenchymal chondrosarcoma: a case report. J Oral Maxillofac Surg 1987: 45: 72-5. 3. Huvos AG, ROSEN G, DABSKAM, MARCOVE RC. Mesenchymal chondrosarcoma. A clinicopathologic analysis of 35 patients with emphasis on treatment. Cancer 1983: 51: 1230-7. 4. LICHTENSTEIN L, BERNSTEIN D. Unusual benign and malignant chondroid tumors of bone. A survey of some mesenchymal cartilage tumors and malignant chondroblastic tumors, including a few mutticentric ones, as well as many atypical benign chondroblastomas and chondromyxoid fibromas. Cancer 1959: 12:1142-57. 5. POTDAR GG, SRIKHANDE as• Chondrogenic tumors of the jaws. Oral Surg 1970: 30:649 58. 6. SALVADOR AH, BEABOUT JW, DAHLIN DC. Mesenchymal ehondrosareoma: Observations on 30 new cases. Cancer 1971: 28:605 13. 7. WILLIAMS HK, EDWARDS MB, ADEKEYE EO. Mesenchymal chondrosarcoma. Int J Oral Maxillofac Surg 1987: 16:119-24.
Fig. 3. High power view of the undifferentiated mesenchymal cells. Mitotic figures are seen (H&E, magnification x 235)
MC. R e c u r r e n c e or late d i s t a n t m e t a s t a sis, however, occurs in one t h i r d to one h a l f o f the cases• SALVADOR et al. 6 have
Address: r e p o r t e d t h a t the survival o f their 30 patients w i t h M C varied f r o m 6 m o n t h s to 13.5 years. H u v o s et al. 3 h a v e stated
Hiroyoshi Hiratsuka, D.D.S. Ph.D. Department of Oral Surgery Sapporo Medical College South 1 West 16, Chuo-ku Sapporo 060, Japan
Tadahiro Ito, Hiroyoshi Hiratsuka, Gen-iku Kohama Department of Oral Surgery, Sapporo Medical College, Japan
Report of a case T. Ito, H. Hiratsuka, G. Kohama: Mesenchymal chondrosarcoma of the maxilla. Int. J. Oral Maxillofac. Surg. 1991; 20." 44-45. Abstract. L i g h t m i c r o s c o p y revealed a n atypical case o f m e s e n c h y m a l c h o n d r o s a r c o m a o f the maxilla in a 62-year-old man.
M e s e n c h y m a l c h o n d r o s a r c o m a ( M C ) is relatively rare a n d characterized b y a worse p r o g n o s i s t h a n o t h e r f o r m s o f c h o n d r o s a r c o m a . It h a s a strong tendency to metastasize to d i s t a n t sites. Predilection sites o f the t u m o r are t h e rib a n d jaws 6. A survey o f 23 cases o f M C o f the jaws h a s s h o w n a predilection for occurrence in the 2nd a n d 3rd decades I. A case is presented of a n elderly p a t i e n t with M C o f the maxilla.
Case report A 62-year-old man was referred to our clinic, complaining of a swelling in the right maxilla which had grown rapidly from a small nodule on the right side of his nose. Examination revealed a firm, painless tumor of 3 x 4 cm. There were no signs of involvement of the cranial nerve. There was some diffuse buccaI expansion of the gingiva, with pus discharging through two fistula. X-ray and CT examination demonstrated an ill-defined expansile radiopaque lesion with high-density tissue proliferation of the anterior wall of the maxillary sinus and a mixed low- and high-density mass anterior to the hard tissue mass. An incisional biopsy was performed and a preliminary diagnosis of atypical chondroma was made. The patient was admitted for a partial maxillectomy. Microscopic examination of the resected specimen revealed a large amount of chondroid material in a lobulated arrangement. The cells were large but variable in size in different locations. Small variably shaped cartilaginous islands separated by sheets of poorly differentiated mesenchymal cells were observed (Fig. 1). Calcification was prominent in the central zone of the chondroid material (Fig. 2). Highly cellular masses composed of small basophilic round and ovoid cells with scanty cytoplasm admixed with spindle-shaped cells were also seen in the peripheral portions of the cartilaginous tissues (Fig. 3). These cells proliferated around numerous capillaries, but the cellular regions occupied only a small part
of the neoplasm. Mitotic figures were rarely seen. Periodic acid-Schiff stains with or without diastase were positive for glycogen. A histopathologic diagnosis of MC was made. During follow-up examination, the patient complained of pain in the right orbit, and both poor and double vision. A C T scan demonstrated bone destruction at the base of the skull. The patient underwent 7 cycles of chemotherapy with methotrexate (total 70 g), cisplatin (total 560 nag), and adriamycin (total 280 mg), but no tumor reduction was seen. Forty-seven months after the first examination the patient died with multiple distant metastases.
Discussion M C is a n aggressive n e o p l a s m first described by LICHTENSTEIN & BERNSTEIN4 as a distinct clinicopathologic entity. The h i s t o p a t h o l o g i c a p p e a r a n c e o f M C has been well described 14. These t u m o r s
Key words: mesenchymal chondrosarcoma. Accepted for publication 29 August 1990
m a y d e m o n s t r a t e a b u n d a n t sheets or clusters o f highly u n d i f f e r e n t i a t e d mese n c h y m a l cells interspersed w i t h small islands o f well-differentiated cartilaginous tissues in w h i c h calcification a n d metaplastic b o n e f o r m a t i o n is often observed. T h e present t u m o r exhibited a large a m o u n t o f well-differentiated cartilaginous m a t e r i a l at the center a n d atypical microscopic features w h i c h differ f r o m previous reports. These microscopic features a n d the presence o f dental infection m i g h t lead to a misdiagnosis o f atypical c h o n d r o m a . A similar case described b y POTOAR et al. 5 was initially diagnosed as o s t e o c h o n droma. Diagnosis o f this type o f t u m o r is often difficult as p o i n t e d o u t by other a u t h o r s 2,7. Radical surgery, if possible, has b e e n suggested as the t r e a t m e n t o f choice for
Fig. 1. Histologic section showing characteristic appearance. Chondroid island and undifferentiated elements are seen (H&E, magnification x 118)
Mesenchymal chondrosarcoma of the maxilla
45
t h a t the 5-year survival rate was 4 2 % , a n d at 10 years 2 8 % o f the M C patients were alive w i t h or w i t h o u t disease. Chemotherapy and radiotherapy have been used as a t e r m i n a l measure, whereas several a u t h o r s have r e p o r t e d the usefulness o f a c o m b i n e d a p p r o a c h u s i n g radiotherapy and/or chemotherapy and surgery 3
?
Acknowledgement. The authors thank Dr. Noriyuki Sato, Department of Pathology, Sapporo Medical College, for his critical advice. References
Fig. 2. Histologic section stained by the Kossa method. Calcification is prominent in the central zone of the chondroid material (magnification x 118)
1. CHRISTENSENRE JR. Mesenchymal chondrosarcoma of the jaws. Oral Surg 1982: 54: 197-206. 2. HOLLINS RR, LYDIATTDD, MARKIN RS, DAVIS LF. Mesenchymal chondrosarcoma: a case report. J Oral Maxillofac Surg 1987: 45: 72-5. 3. Huvos AG, ROSEN G, DABSKAM, MARCOVE RC. Mesenchymal chondrosarcoma. A clinicopathologic analysis of 35 patients with emphasis on treatment. Cancer 1983: 51: 1230-7. 4. LICHTENSTEIN L, BERNSTEIN D. Unusual benign and malignant chondroid tumors of bone. A survey of some mesenchymal cartilage tumors and malignant chondroblastic tumors, including a few mutticentric ones, as well as many atypical benign chondroblastomas and chondromyxoid fibromas. Cancer 1959: 12:1142-57. 5. POTDAR GG, SRIKHANDE as• Chondrogenic tumors of the jaws. Oral Surg 1970: 30:649 58. 6. SALVADOR AH, BEABOUT JW, DAHLIN DC. Mesenchymal ehondrosareoma: Observations on 30 new cases. Cancer 1971: 28:605 13. 7. WILLIAMS HK, EDWARDS MB, ADEKEYE EO. Mesenchymal chondrosarcoma. Int J Oral Maxillofac Surg 1987: 16:119-24.
Fig. 3. High power view of the undifferentiated mesenchymal cells. Mitotic figures are seen (H&E, magnification x 235)
MC. R e c u r r e n c e or late d i s t a n t m e t a s t a sis, however, occurs in one t h i r d to one h a l f o f the cases• SALVADOR et al. 6 have
Address: r e p o r t e d t h a t the survival o f their 30 patients w i t h M C varied f r o m 6 m o n t h s to 13.5 years. H u v o s et al. 3 h a v e stated
Hiroyoshi Hiratsuka, D.D.S. Ph.D. Department of Oral Surgery Sapporo Medical College South 1 West 16, Chuo-ku Sapporo 060, Japan
