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Periosteal osteogenic sarcoma of the mandible
Short communications & case reports Periosteal osteogenic sarcoma of the mandible Richard J. Zarbo, M.D.. D.iU.D.,* Joseph A. Regezi. D.D.S., MS.,** Shan R. Baker, M.D..*** Hartford, Corm., and Ann Arbor, h4ich. HARTFORD
HOSPITAL
AND
UNIVERSITY
and
OF MICHIGAN
Periosteal osteosarcoma, a variant of juxtacortical osteosarcoma, is seldom seen in the jaws. The clinical, radiographic, and histopathologic features of a rare mandibular periosteal osteosarcoma presenting as a deep mass in the area of the parotid are reported. The clinicopathologic features that distinguish this lesion from the more common parosteal osteosarcoma are also discussed. (ORAL SURG. 57643-647, 1984)
0
steosarcomas can be divided into intramedullary (central) and juxtacortical (peripheral) types on the basis of clinical, histopathologic, and radiographic characteristics. These two types reflect distinct differences in biologic behavior. Recent evidence has been presented showing that juxtacortical osteosarcomas can be further subdivided into two clinicopathologic varieties-parosteal osteosarcoma and per&teal osteosarcoma.’The purposesof this article are (1) to report an example of a rare mandibular periosteal osteosarcoma which presented as a softtissue mass mimicking a parotid tumor and (2) to discuss the differentiating features of parosteal and periosteal osteosarcomas. CASE REPORT
A 57-year-old man had a mass in the left parotid gland area which had slowly increased over the last month. The masscaused no symptoms and did not change in size with mastication. No facial muscle weaknesswas noted. There was full jaw mobility but the patient did report past temporomandibular joint discomfort. Physical examina*Department of Pathology, Hartford Hospital. **Department of Oral Pathology, School of Dentistry, The University of Michigan. ***Department of Otolaryngology, Head and Neck Surgery, The University of Michigan.
tion disclosed a 3 x 3 cm mass anterior to the left ear, overlying the ascending ramus of the mandible. The lesion was not palpable intraorally. A recent sialogram of the left parotid gland showed normal ductal pattern. The initial clinical impression was that of a parotid gland neoplasm, probably arising in the deep lobe. Two days prior to surgical intervention the patient noted hypesthesia over the distribution of the left inferior alveolar nerve. Concern about a malignant neoplasm of the deep lobe of the parotid gland indicated the need for a preoperative radiograph. There were no abnormal findings on the panographicfilm. At the time of parotidectomy, the parotid gland appeared normal. There was, however, a tumescence along the ascending ramus of the mandible. This area was biopsied. Frozen-section diagnosis was sarcoma, and permanentsection diagnosis was periosteal osteosarcoma.Treatment consisted of left hemimandibulectomy. The resected posterior left mandible contained a wellcircumscribed, firm 3.5 X 3.5 X 3 cm massthat was white, glassy, and gritty but easily sectioned with a blade. The tumor circumferentially covered the condyle and a portion of the posterior ramus. The articular surface of the condyle and the coronoid processwere spared (Fig. 1). Horizontal serial sections showedthat the tumor was closely apposed to the cortical surface of the ramus without gross involvement of the medullary cavity. Right-angle specimen radiographs showed a relatively lucent massoverlying an intact cortex. Fine focal calcified spicules radiated a short distance from the cortex. The medulla was not involved (Fig. 2). 643
644
Zarbo, Regezi, and Baker
Oral Surg. June , 1984
tained lacelike osteoid (Fig. 5). Well-circumscribed foci of neoplasm extended into medial soft tissue. Regional lymph nodesand parotid gland were not involved. One year after surgical treatment, there was no evidence of recurrence. DISCUSSION
Fig. 2. Radiograph of surgical specimen demonstrating the lucent tumor massoverlying intact cortex and medulla of mandible.
Histologically, the tumor was limited to the periosteum with only focal erosion of the cortical mandibular bone (Fig. 3). The neoplasm was pseudoencapsulatedand was composed predominantly of lobules of malignant myxochondroid connective tissue with small irregular areas of ossification (Fig. 4). Fine osteoid and bony trabeculae radiated from the cortex a short distance, corresponding to the radiographic spiculee. A poorly differentiated spindle cell stroma surrounded central chondroid zones and con-
The radiographic and histopathologic features of the case presented here fulfill the criteria established for periosteal osteosarcoma, namely an illdefined radiolucency on an intact bone surface with no involvement of the marrow cavity, often with fine spicules of bone perpendicular to the cortex; grossly soft and lobulated; and histologically composed of poorly differentiated malignant cartilage, bone, osteoid, and fibrous components containing fine, lacelike osteoid. Subclassification of juxtaccrtical lesions into parosteal and periosteal* (regarded by some as lowgrade and high-grade juxtacortical osteosarcomas, respectively)3x4appears warranted because of the distinctive clinical, microscopic, and behavioral differences that follow. Periosteal osteosarcoma is much less common than parosteal osteosarcoma. It has a 2 to 1 male predominance and a peak occurrence at about 20 years5 The lesion is usually smaller in size than the parosteal osteosarcoma and commonly involves the upper tibia1 metaphysis. Only one of twenty-three periosteal osteosarcomas in Dahlin’s series involved the mandible.6 Parosteal osteosarcoma occurs over a
Volume57 Number6
Periosteal osteogenic sarcoma of mandible
645
3. The predominantly cartilaginous neoplasm overlies and focally erodes cortical mandibular hone. (Hematoxylin and eosin stain. Magnification, X 10.)
Fig.
Fig. 4. Spindle cell stroma and focally calcified malignant cartilage. (Hematoxylin and eosin stain. Magnification, X 160.)
wide age range and peaks at about 39 years.5 It is more common in females than in males (3:2) when long bones are affected but more common in males when the jaws are affected.7 The tumor most commonl y involves the distal femoral metaphysis and tends to be large and slow growing.
In periosteal osteosarcoma the cortex is radiographically intact and sometimes thickened, \uith no involvement of the underlying marrow cavity. Because it is predominantly cartilaginous, th e mass is more radiolucent and has a more poorly Idefined periphery. On occasion, the limits of the lesicIn may
646
Zarbo, Regezi, and Baker
Oral Surg. June, 1984
Fig. 5. Eosinophilic lacelike osteoid in spindle cell stroma. (Hematoxylin and eosin stain. Magnification, x160.)
be defined by a periosteal response in the form of a Codman’s triangle.s It may have variably sized perpendicular calcified spicules radiating from the cortex. However, the tumor matrix is not as radiographically dense or homogeneous as the parosteal osteosarcoma.SParosteal osteosarcomais characteristically radiodense, more at the base than at the periphery. Although attached to the underlying bone by a broad pedicle, there is no radiographic continuity with the underlying marrow cavity. There is often a radiolucent clear space between the tumor and the normal bone cortex on which the tumor lies, since it oftens encircles but does not attach to the cortex. New periosteal bone formation is absent. Grossly, the periosteal osteosarcoma is lobulated and well delimited without infiltration into surrounding soft tissue. Unlike parosteal osteosarcoma, this tumor does not overgrow the base of origin. There may be minimal tumor infiltration into the cortex, without medullary involvement.’ The latter criterion is essential if one is to differentiate between an intramedullary osteosarcomathat has permeated the cortex and formed a soft-tissue massand a periosteal osteosarcoma. Histologically, the periosteal neoplasm is composedof lobules of poorly differentiated malignant cartilage which may show central ossification. Malignant cartilage and osteoid radiate from the cortex. The diagnostic pattern of fine, lacelike osteoid is found in the chondroid islands and among intervening malignant spindle cells. This pattern
helps distinguish this entity from juxtacortical chondrosarcoma. Again, radiographic features are essential criteria since fields of intramedullary osteosarcoma and periosteal osteosarcoma may be histologically identical. In parosteal osteosarcoma tumor margins are lobulated and smooth, but there may be small satellite foci in soft tissue. In contrast, the neoplasm is histologically well differentiated and is characterized by a spindle cell stroma with minimal atypia and rare mitotic figures separating irregular trabeculae of bone. The periphery is less ossified than the base; it may have a lobulated cartilaginous cap or may be irregular because of linear extensions into soft tissue.4’* Medullary involvement, unusual at initial presentation, can be seen in recurrent tumors but does seem to adversely effect prognosis.8Because of its bland microscopic appearance, the ditferential diagnosis may include osteoma, osteochondroma, heterotopic ossification, and myositis ossificans. In long-bone sites the prognosis for periosteal osteosarcomais regarded as being considerably better than that for conventional intramedullary osteosarcoma but poorer than that for parosteal osteosarcoma.’ Resection is the favored treatment for small lesions. Periosteal osteosarcoma is capable of local recurrence and distant metastasis. The parosteal osteosarcoma is generally regarded as a low-grade neoplasm that is locally aggressive and may recur if incompletely excised. It is considered to have mini-
Volume 57 Number 6
ma1 metastatic potential6 Recommended treatment for long-bone parosteal osteosarcomais resection for small lesions and amputation for very large lesions.8 Juxtacortical osteosarcomas (inclusive of both parosteal and periosteal types) are uncommon neoplasms that comprise about 5% of all osteosarcomas of the skeleton.6They are rarely seenin the jaws.7*9It is not known if juxtacortical osteosarcomasof the jaws are substantially different in behavior from those occurring in long bones. An overall better survival rate has been reported for intramedullary osteosarcomas of the jaw as compared to other sites.‘OMore casesmust be reported before meaningful conclusions can be drawn comparing the treatment and prognosis of parosteal and periosteal osteosarcomas in the jaws. REFERENCES
1. Unni KK, Dahlin DC, Beabout JW: Periosteal osteogenic sarcoma. Cancer 37: 2476-2485, 1976. 2. Dahlin DC, Unni KK: Osteosarcoma of bone and its important recognizable varieties. Am J Surg Pathol 1: 61-72, 1977.
Periosteal osteogenic sarcoma of mandible
647
3. Huvos AG: Bone tumors: Diagnosis, treatment, and prognosis, Philadelphia, 1970, W.B. Saunders Company. 4. Ahuja SC, Villacin AB, Smith J, Bullough PG, Huvos AG, Marcove RC: Juxtacortical (parosteal) osteogenic sarcoma. J Bone Joint Surg 59-A: 632-647, 1977. 5. deSantos LA, Murray JA, Finklestein JB, Spjut HJ, Ayala AG: The radiographic spectrum of periosteal-osteosarcdma. Radiology 127: 123-129, 1978. 6. Dahlin DC: Bone tumors; General aspects and data on 6,221 cases, ed. 3, Springfield, Ill., 1978, Charles C Thomas Publisher. 7. Bras JM, Dormer R, van der Kwast WAM, Snow GB, van der Waal L: Juxtacortical osteogenic sarcoma of the jaws. ORAL SURG 50: 535-544, 1980. 8. Unni KK, Dahlin DC, Beabout JW, Ivins JC: Parosteal
osteogenic sarcoma. Cancer 37: 2455-2475, 1976. 9. Banerjee SC: Juxtacortical osteosarcomaof mandible: review
of literature and report of case. J Oral Surg 39: 535-538, 1981. 10. Garrington G, Scofield HH, Cornyn J, Hooker SP: Osteosarcoma of the jaws: analysis of 56 cases. Cancer 20: 277-391, 1967. Reprint requests to:
Dr. Richard J. Zarbo Department of Pathology Hartford Hospital 80 Seymour St. Hartford, CT 06115
HOSPITAL
AND
UNIVERSITY
and
OF MICHIGAN
Periosteal osteosarcoma, a variant of juxtacortical osteosarcoma, is seldom seen in the jaws. The clinical, radiographic, and histopathologic features of a rare mandibular periosteal osteosarcoma presenting as a deep mass in the area of the parotid are reported. The clinicopathologic features that distinguish this lesion from the more common parosteal osteosarcoma are also discussed. (ORAL SURG. 57643-647, 1984)
0
steosarcomas can be divided into intramedullary (central) and juxtacortical (peripheral) types on the basis of clinical, histopathologic, and radiographic characteristics. These two types reflect distinct differences in biologic behavior. Recent evidence has been presented showing that juxtacortical osteosarcomas can be further subdivided into two clinicopathologic varieties-parosteal osteosarcoma and per&teal osteosarcoma.’The purposesof this article are (1) to report an example of a rare mandibular periosteal osteosarcoma which presented as a softtissue mass mimicking a parotid tumor and (2) to discuss the differentiating features of parosteal and periosteal osteosarcomas. CASE REPORT
A 57-year-old man had a mass in the left parotid gland area which had slowly increased over the last month. The masscaused no symptoms and did not change in size with mastication. No facial muscle weaknesswas noted. There was full jaw mobility but the patient did report past temporomandibular joint discomfort. Physical examina*Department of Pathology, Hartford Hospital. **Department of Oral Pathology, School of Dentistry, The University of Michigan. ***Department of Otolaryngology, Head and Neck Surgery, The University of Michigan.
tion disclosed a 3 x 3 cm mass anterior to the left ear, overlying the ascending ramus of the mandible. The lesion was not palpable intraorally. A recent sialogram of the left parotid gland showed normal ductal pattern. The initial clinical impression was that of a parotid gland neoplasm, probably arising in the deep lobe. Two days prior to surgical intervention the patient noted hypesthesia over the distribution of the left inferior alveolar nerve. Concern about a malignant neoplasm of the deep lobe of the parotid gland indicated the need for a preoperative radiograph. There were no abnormal findings on the panographicfilm. At the time of parotidectomy, the parotid gland appeared normal. There was, however, a tumescence along the ascending ramus of the mandible. This area was biopsied. Frozen-section diagnosis was sarcoma, and permanentsection diagnosis was periosteal osteosarcoma.Treatment consisted of left hemimandibulectomy. The resected posterior left mandible contained a wellcircumscribed, firm 3.5 X 3.5 X 3 cm massthat was white, glassy, and gritty but easily sectioned with a blade. The tumor circumferentially covered the condyle and a portion of the posterior ramus. The articular surface of the condyle and the coronoid processwere spared (Fig. 1). Horizontal serial sections showedthat the tumor was closely apposed to the cortical surface of the ramus without gross involvement of the medullary cavity. Right-angle specimen radiographs showed a relatively lucent massoverlying an intact cortex. Fine focal calcified spicules radiated a short distance from the cortex. The medulla was not involved (Fig. 2). 643
644
Zarbo, Regezi, and Baker
Oral Surg. June , 1984
tained lacelike osteoid (Fig. 5). Well-circumscribed foci of neoplasm extended into medial soft tissue. Regional lymph nodesand parotid gland were not involved. One year after surgical treatment, there was no evidence of recurrence. DISCUSSION
Fig. 2. Radiograph of surgical specimen demonstrating the lucent tumor massoverlying intact cortex and medulla of mandible.
Histologically, the tumor was limited to the periosteum with only focal erosion of the cortical mandibular bone (Fig. 3). The neoplasm was pseudoencapsulatedand was composed predominantly of lobules of malignant myxochondroid connective tissue with small irregular areas of ossification (Fig. 4). Fine osteoid and bony trabeculae radiated from the cortex a short distance, corresponding to the radiographic spiculee. A poorly differentiated spindle cell stroma surrounded central chondroid zones and con-
The radiographic and histopathologic features of the case presented here fulfill the criteria established for periosteal osteosarcoma, namely an illdefined radiolucency on an intact bone surface with no involvement of the marrow cavity, often with fine spicules of bone perpendicular to the cortex; grossly soft and lobulated; and histologically composed of poorly differentiated malignant cartilage, bone, osteoid, and fibrous components containing fine, lacelike osteoid. Subclassification of juxtaccrtical lesions into parosteal and periosteal* (regarded by some as lowgrade and high-grade juxtacortical osteosarcomas, respectively)3x4appears warranted because of the distinctive clinical, microscopic, and behavioral differences that follow. Periosteal osteosarcoma is much less common than parosteal osteosarcoma. It has a 2 to 1 male predominance and a peak occurrence at about 20 years5 The lesion is usually smaller in size than the parosteal osteosarcoma and commonly involves the upper tibia1 metaphysis. Only one of twenty-three periosteal osteosarcomas in Dahlin’s series involved the mandible.6 Parosteal osteosarcoma occurs over a
Volume57 Number6
Periosteal osteogenic sarcoma of mandible
645
3. The predominantly cartilaginous neoplasm overlies and focally erodes cortical mandibular hone. (Hematoxylin and eosin stain. Magnification, X 10.)
Fig.
Fig. 4. Spindle cell stroma and focally calcified malignant cartilage. (Hematoxylin and eosin stain. Magnification, X 160.)
wide age range and peaks at about 39 years.5 It is more common in females than in males (3:2) when long bones are affected but more common in males when the jaws are affected.7 The tumor most commonl y involves the distal femoral metaphysis and tends to be large and slow growing.
In periosteal osteosarcoma the cortex is radiographically intact and sometimes thickened, \uith no involvement of the underlying marrow cavity. Because it is predominantly cartilaginous, th e mass is more radiolucent and has a more poorly Idefined periphery. On occasion, the limits of the lesicIn may
646
Zarbo, Regezi, and Baker
Oral Surg. June, 1984
Fig. 5. Eosinophilic lacelike osteoid in spindle cell stroma. (Hematoxylin and eosin stain. Magnification, x160.)
be defined by a periosteal response in the form of a Codman’s triangle.s It may have variably sized perpendicular calcified spicules radiating from the cortex. However, the tumor matrix is not as radiographically dense or homogeneous as the parosteal osteosarcoma.SParosteal osteosarcomais characteristically radiodense, more at the base than at the periphery. Although attached to the underlying bone by a broad pedicle, there is no radiographic continuity with the underlying marrow cavity. There is often a radiolucent clear space between the tumor and the normal bone cortex on which the tumor lies, since it oftens encircles but does not attach to the cortex. New periosteal bone formation is absent. Grossly, the periosteal osteosarcoma is lobulated and well delimited without infiltration into surrounding soft tissue. Unlike parosteal osteosarcoma, this tumor does not overgrow the base of origin. There may be minimal tumor infiltration into the cortex, without medullary involvement.’ The latter criterion is essential if one is to differentiate between an intramedullary osteosarcomathat has permeated the cortex and formed a soft-tissue massand a periosteal osteosarcoma. Histologically, the periosteal neoplasm is composedof lobules of poorly differentiated malignant cartilage which may show central ossification. Malignant cartilage and osteoid radiate from the cortex. The diagnostic pattern of fine, lacelike osteoid is found in the chondroid islands and among intervening malignant spindle cells. This pattern
helps distinguish this entity from juxtacortical chondrosarcoma. Again, radiographic features are essential criteria since fields of intramedullary osteosarcoma and periosteal osteosarcoma may be histologically identical. In parosteal osteosarcoma tumor margins are lobulated and smooth, but there may be small satellite foci in soft tissue. In contrast, the neoplasm is histologically well differentiated and is characterized by a spindle cell stroma with minimal atypia and rare mitotic figures separating irregular trabeculae of bone. The periphery is less ossified than the base; it may have a lobulated cartilaginous cap or may be irregular because of linear extensions into soft tissue.4’* Medullary involvement, unusual at initial presentation, can be seen in recurrent tumors but does seem to adversely effect prognosis.8Because of its bland microscopic appearance, the ditferential diagnosis may include osteoma, osteochondroma, heterotopic ossification, and myositis ossificans. In long-bone sites the prognosis for periosteal osteosarcomais regarded as being considerably better than that for conventional intramedullary osteosarcoma but poorer than that for parosteal osteosarcoma.’ Resection is the favored treatment for small lesions. Periosteal osteosarcoma is capable of local recurrence and distant metastasis. The parosteal osteosarcoma is generally regarded as a low-grade neoplasm that is locally aggressive and may recur if incompletely excised. It is considered to have mini-
Volume 57 Number 6
ma1 metastatic potential6 Recommended treatment for long-bone parosteal osteosarcomais resection for small lesions and amputation for very large lesions.8 Juxtacortical osteosarcomas (inclusive of both parosteal and periosteal types) are uncommon neoplasms that comprise about 5% of all osteosarcomas of the skeleton.6They are rarely seenin the jaws.7*9It is not known if juxtacortical osteosarcomasof the jaws are substantially different in behavior from those occurring in long bones. An overall better survival rate has been reported for intramedullary osteosarcomas of the jaw as compared to other sites.‘OMore casesmust be reported before meaningful conclusions can be drawn comparing the treatment and prognosis of parosteal and periosteal osteosarcomas in the jaws. REFERENCES
1. Unni KK, Dahlin DC, Beabout JW: Periosteal osteogenic sarcoma. Cancer 37: 2476-2485, 1976. 2. Dahlin DC, Unni KK: Osteosarcoma of bone and its important recognizable varieties. Am J Surg Pathol 1: 61-72, 1977.
Periosteal osteogenic sarcoma of mandible
647
3. Huvos AG: Bone tumors: Diagnosis, treatment, and prognosis, Philadelphia, 1970, W.B. Saunders Company. 4. Ahuja SC, Villacin AB, Smith J, Bullough PG, Huvos AG, Marcove RC: Juxtacortical (parosteal) osteogenic sarcoma. J Bone Joint Surg 59-A: 632-647, 1977. 5. deSantos LA, Murray JA, Finklestein JB, Spjut HJ, Ayala AG: The radiographic spectrum of periosteal-osteosarcdma. Radiology 127: 123-129, 1978. 6. Dahlin DC: Bone tumors; General aspects and data on 6,221 cases, ed. 3, Springfield, Ill., 1978, Charles C Thomas Publisher. 7. Bras JM, Dormer R, van der Kwast WAM, Snow GB, van der Waal L: Juxtacortical osteogenic sarcoma of the jaws. ORAL SURG 50: 535-544, 1980. 8. Unni KK, Dahlin DC, Beabout JW, Ivins JC: Parosteal
osteogenic sarcoma. Cancer 37: 2455-2475, 1976. 9. Banerjee SC: Juxtacortical osteosarcomaof mandible: review
of literature and report of case. J Oral Surg 39: 535-538, 1981. 10. Garrington G, Scofield HH, Cornyn J, Hooker SP: Osteosarcoma of the jaws: analysis of 56 cases. Cancer 20: 277-391, 1967. Reprint requests to:
Dr. Richard J. Zarbo Department of Pathology Hartford Hospital 80 Seymour St. Hartford, CT 06115