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Expansile mass of the body of the mandible
J Oral Maxillofac 50:627-632.
Surg
1992
Expansile Mass of the Body of the Mandible STANLEY KERPEL, DDS,* PAUL FREEDMAN, DDS,t AND STEPHEN H. TROYER, DDS, MSDS dible and the root resorption of the involved teeth, suggest an aggressive, probably malignant, lesion. The radiograph obtained 3 years previously excludes a number of benign lesions that would take many years to reach the size of this lesion. The presence of calcified deposits suggest that mixed radiolucent/radiopaque lesions must be considered. The differential diagnosis basically includes the following: 1) benign odontogenic cysts or neoplasms; 2) benign nonodontogenic neoplasms; and 3) malignant neoplasms. Benign odontogenic cysts or neoplasms to be considered include dentigerous cyst, odontogenic keratocyst, ameloblastoma, calcifying odontogenic cyst (Gorlin’s cyst), calcifying epithelial odontogenic tumor (Pindborg tumor), odontogenic myxoma, adenomatoid odontogenic tumor, and central cementifying fibroma. Dentigerous cyst. The dentigerous cyst can be an aggressive lesion at times. It can result in expansion of bone with facial asymmetry, as well as cause root resorption of adjacent teeth. Rarely, dentigerous cysts may erode the cortical plates of bone. Paresthesia is not common. Radiographically, the lesion appears as a radiolucency with a thin sclerotic border; it invariably involves the crown of an unerupted tooth. The latter would tend to make the diagnosis of dentigenous cyst unlikely. Odontogenic keratocyst. The odontogenic keratocyst most often occurs in the third decade of life. It is seen more frequently in males than females and the mandible is involved much more frequently than the maxilla. Radiographically, the lesion appears as a unilocular or multilocular radiolucency, usually with a thin sclerotic border. The lesion may penetrate bone as well as expand it. Multiple odontogenic keratocysts can occur, and in some instances they are associated with the basal cell nevus syndrome. Ameloblastoma. An ameloblastoma can occur at any age; however, nearly half of the lesions occur between the ages of 20 and 40 years. Ameloblastomas typically begin as central lesions that are slowly destructive and tend to expand bone rather than perforate it. Paresthesia is not common. The mural ameloblastoma usually arises in the wall of a dentigerous cyst. Radiographically, ameloblastomas are classically described as multilocular radiolucent lesions. In advanced
Case Presentation In March 198 1, a 42-year-old white man was referred for evaluation of a swelling of the right mandibular vestibule (Fig I). Except for a slight tingling of the lower lip, there were no other symptoms. The swelling was hard, with no apparent crepitation noted on palpation, and was covered by intact mucosa. Extraorally there was a subtle expansion of the right submandibular region. Radiographs showed the presence of a large, predominantly radiolucent lesion extending from the right mandibular premolar region to the distal of the right third molar (Fig 2). The lesion appeared compartmentalized and contained calcified deposits within its center. There was slight root resorption of the involved teeth. The anterior component of the lesion was not well delineated, and the inferior border appeared to be irregularly eroded in one area. The patient’s medical history was essentially negative, as were all his laboratory findings. Of interest in his past dental history was transient temporomandibular joint discomfort on the right side 3 years before. A panoramic radiograph taken at that time (Fig 3) was interpreted as normal, and shortly thereafter his symptoms subsided. Differential
Diagnosis
Stephen H. Troyer, DOS, MSD
The availability of a panoramic radiograph obtained 3 years prior to this examination is certainly helpful, since it indicates that the lesion is of relatively recent origin. This radiograph does not indicate the presence of dental pathology in the area of the current radiolucent lesion and all teeth in the area appear to be in good repair. Restorations in the posterior teeth appear to be reasonably small and do not seem to involve the pulp. There is no obvious widening of the periodontal membrane involving any of these teeth. The clinical and radiographic findings, particularly the irregular erosion of the inferior border of the man-
* Attending, Department of Oral Pathology, Booth Memorial Medical Center, Flushing, NY. t Assistant Director, Department of Oral Pathology, Booth Memorial Medical Center, Flushing, NY. $ In private practice, Evansville, IN. Addressreprint requests to Dr Kerpel: Oral Pathology Laboratory, Inc, 56-45 Main St, Flushing, NY 11355. 8 1992 American
Association
of Oral and Maxillofacial
Surgeons
0278-2391/92/5006-0015$3.00/O
627
626
EXPANSILE MASS OF BODY OF THE MANDIBLE
FIGURE 1. Photograph taken at initial presentation showing a swelling of the right mucobuccal fold.
cases they appear compartmentalized, with septa of bone within the radiolucent mass. Calcifving odontogenic cyst (Gorlin’s cyst). The calcifying odontogenic cyst may occur at any age. In its early stages of development, the lesion may closely resemble a dentigerous cyst radiographically. Later in the course, varying amounts of calcified radiopaque material are seen scattered throughout the lesion. Calcifying epithelial odontogenic tumor (Pindborg’s tumor). The mean age of occurrence of the calcifying epithelial odontogenic tumor is 40 years, with no significant sex predilection. It occurs twice as frequently in the mandible as in the maxilla. Radiographically, the lesion has a variable appearance. Although it may appear as a diffuse or well-circumscribed unilocular radiolucency, it also may be multilocular. There may be scattered flecks of calcification present.
FIGURE 2. Panoramic radiograph showing the presence of a destructive, predominantly radiolucent lesion with ill-defined borders. There are calcified deposits present just inferior to the mandibular second molar.
FIGURE 3. Normal radiograph taken 3 years previously (A) compared with recent radiograph (B).
Adenomatoid odontogenic tumor. The adenomatoid odontogenic tumor is usually found in patients under 20 years of age. Females are involved twice as frequently as males and the maxilla is involved twice as frequently as the mandible. Unlike the ameloblastoma, this lesion occurs most frequently anterior to the canine both in the maxilla and mandible. Radiographically, it appears as a destructive lesion of bone that may or may not be well circumscribed, but many times resembles a dentigerous cyst. There is usually a unilocular radiolucent appearance, but the lesion may contain radiopaque foci. Root resorption is rare. Odontogenic myxoma. The odontogenic myxoma usually occurs between the second and third decades of life. Males are involved less frequently than females. The mandible is involved slightly more frequently than the maxilla. Radiographically, the odontogenic myxoma appears as a central lesion of the jaws that may expand the bone and cause cortical plate destruction. Displacement of teeth is common; however, root resorption is less frequent. Central cementijjying$broma. The central cementifying fibroma usually occurs in the third or fourth decade of life. Females are involved twice as frequently as males. Radiographically, the lesion has a variable appearance depending on its stage of development. In its early stages, it appears as a radiolucent lesion with no evidence of internal radiopacities. As the tumor matures, multiple opacities appear within the radiolucent area until eventually it appears as a radiopaque mass. The lesion has a centrifugal growth pattern, resulting in the production of a round tumor mass as it expands the inferior border of the mandible. Some believe that the central cementifying fibroma and the
KERPEL, FREEDMAN.
AND TROYER
central ossifying fibroma are closely related, in that their age of occurrence, sex predilection, radiographic appearance, location, and clinical behavior are similar. Nonodontogenic neoplasms that should be considered include neurolemmoma and chondroma. Neurokmmoma. The neurolemmoma may arise at any age and there is no sex predilection. Intraorally, the neurolemmoma is most commonly reported as a soft-tissue lesion. A number of cases have been reported involving the mandible, with the tumor apparently arising from the inferior alveolar nerve. Radiographitally, the central lesions in bone may produce considerable destruction, with expansion of the cortical plates, resembling what might be expected of a much more aggressive lesion. The chondroma is a benign central Chondroma. tumor of cartilage that is common in the bony skeleton, but relatively uncommon in the maxilla or mandible. The lesion may appear at any age and shows no sex predilection. It has been reported to occur in the body of the mandible. Radiographically, the lesion presents as an irregular radiolucent or mixed radiolucent/radiopaque area. It is destructive and has been reported to cause root resorption of adjacent teeth. Malignant neoplasms to be considered include the following: osteosarcoma (osteogenic sarcoma), malignant schwannoma (neurogenic sarcoma), chondrosarcoma, fibrosarcoma, Ewing’s sarcoma, primary lymphoma of bone, and metastatic lesions to the mandible from other primary sites. Osteosarcoma of the jaws occurs Osteosarcoma. at a later age than osteosarcoma involving the long bones, with the usual age at the time of occurrence reported to be between 27 to 33 years. Males are involved more frequently than females and tumors of the mandible are more common than those of the maxilla. Osteosarcoma has been reported to be preceded by trauma. It also has been reported with increased frequency in bone affected by osteitis deformans (Paget’s disease). In nearly all osteosarcomas of the jaw that have been reported, however, there was no preceding history of trauma or of osteitis deformans. Bone subjected to therapeutic radiation has been reported to undergo malignant transformation. A number of cases of osteosarcoma developing after radiation therapy for benign jaw lesions (ie, fibrous dysplasia and giant cell granuloma) have been reported. Radiographically, osteosarcoma may present as an osteolytic process with minimal calcified bone formation or as a sclerosing bone-forming lesion. The presence of irregular trabeculae of bone radiating outward on the periphery of the lesion has been described as the “sun-ray” appearance of osteosarcoma. The tumor destroys cortical plates as it progresses, expanding and eventually perforating them. The osteolytic form
629 is essentially a destructive process, resulting in an irregular radiolucency with both expansion and destruction of the cortical plates. An early reported manifestation of osteosarcoma is symmetrical widening of the periodontal ligament space surrounding one or more teeth on the dental radiograph. This is reported to occur prior to any other radiographic evidence of the presence of a neoplasm, and therefore is of great diagnostic significance. Malignant schwannoma. The malignant schwannoma appears most frequently between the ages of 30 and 60 years. Male and female incidence are similar, with the mandible involved more frequently than the maxilla. The presenting symptoms may include pain and/or paresthesia. Radiographically, the lesion may appear as a diffuse radiolucency that is characteristic of a malignant neoplasm. It may also appear as a smooth radiolucency associated with the mandibular canal when the tumor originates from the inferior alveolar nerve. Chondrosarcoma. The chondrosarcoma occurs most frequently between the ages of 30 and 60 years. Males are involved twice as frequently as females. A distinctive type of chondrosarcoma, the mesenchymal chondrosarcoma, occurs most frequently between the ages of 10 and 30 years and has no sex predilection. Radiographically, the lesion usually appears as an irregular radiolucent area in bone that may produce considerable destruction. Occasionally there is calcification of the neoplastic cartilage. Fibrosarcoma. Intraorally, the fibrosarcoma may arise in any location, but is commonly involved with the periosteum of the maxilla or mandible. The lesion is most common before 50 years of age. Radiographically, it appears as a destructive malignant neoplasm. Ewing’s sarcoma is an uncomEwing’s sarcoma. mon malignant lesion that occurs primarily in people aged 5 to 25 years. The mandible is involved more frequently than the maxilla. Lip paresthesia has been reported. Radiographically, the lesion is destructive and may produce an irregular, diffuse radiolucency. Lesions may also be radiopaque, resembling sclerosing osteomylitis. Formation of layers of subperiosteal bone is characteristic, producing the “onion skin” appearance. Osteophytes within the lesion also may produce the “sun-ray” appearance associated with osteosarcoma. Primary lymphoma of bone. Primary lymphoma of bone is an uncommon disease of the jaws, occurring somewhat more frequently in the mandible than the maxilla. The disease usually occurs prior to 40 years of age and males are involved more frequently than females. Paresthesia has been reported. Radiographitally, the lesion appears as an invasive, destructive, malignant neoplasm. The diffuse radiolucency many
630 times involves the alveolar process, with destruction of bone and adjacent teeth. Metastatic lesions to mandible from other primary sites. Although the jaws are not thought to be a common site for metastasis, metastatic tumors to the jaws are of significance as their appearance may be the first indication of malignancy at a distance site. The lesions may be asymptomatic, but paresthesia has been reported due to involvement of the inferior alveolar nerve. Expansion of the jaw occurs frequently. The mandible is involved much more frequently than the maxilla. Radiographically, there is no characteristic appearance. The metastatic lesions may cause bone destruction and appear as radiolucencies, but certain lesions produce bone, giving a mixed radiolucent/radiopaque appearance. Primary tumors that may metastasize to the jaws include those involving the breast, lung, and kidney. Tumors involving the thyroid, prostate, colon, and stomach have been reported to metastisize less frequently. Summary. A differential diagnosis has been presented that includes a number of benign nonodontogenie cysts and neoplasms, benign nonodontogenic neoplasms, and malignant neoplasms. Obviously, a biopsy of the lesion is indicated to establish the final diagnosis. Although a number of the benign lesions discussed should be considered in the differential diagnosis, the clinical and radiographic evidence suggest a malignant neoplasm. Factors that favor a malignant neoplasm include the following: 1) the presence of a large destructive radiolucent lesion with no evidence of dental pathology (ie, impacted teeth, periapical pathology); 2) obvious invasion and destruction of the inferior border of the mandible; 3) resorption of roots of teeth adjacent to the lesion. Of the malignant neo-
FIGURE 4. Medium-power photomicrogmph showing tumor bone on the left and cartilage-like material bordered by difise sheets of malignant osteoblasts on the right (hematoxyln-eosin stain, original magnification X 100).
EXPANSILE MASS OF BODY OF THE MANDIBLE
FIGURE 5. High-power photomicrograph showing clusters of cells with large hyperchromatic nuclei, some containing prominent nucleoli. Moderate pleopmorphism of the tumor cells, as well as malignant bone, also is noted.
plasms discussed, the osteosarcoma is the most common neoplasm exhibiting the clinical and radiographic appearance of the case presented. These characteristics include: 1) age of the patient at the time the lesion is diagnosed; 2) more frequent involvement in males; 3) more common occurrence in the mandible; 4) radiographic appearance as a destructive radiolucency with expansion and destruction of cortical plates; 5) varying amounts of calcification present within the radiolucency. SUBSEQUENT
COURSE
An incisional biopsy was performed under local anesthesia and consisted of fragments of soft tissue that were gritty in areas. Microscopically, the tumor was composed of focally cellular mesenchymal tissue exhibiting both osteoid formation and cartilage, which showed endochondral ossification (Fig 4). Cellular foci were found alternating with areas that were sparsely populated by the tumor cells. The tumor cells were large, spindled to polyhedral in shape, and contained hyperchromatic nuclei with prominent nucleoli (Fig 5). Obvious pleomorphism, as well as the appearance of occasional atypical mitoses, was also present. The microscopic appearance was consistent with osteogenic sarcoma, chondroblastic type. The patient was referred for treatment. He was treated first with five courses of high-dose methotrexate and then underwent a right hemimandibulectomy with removal of the submandibular gland and regional lymph nodes. Several courses of postoperative chemotherapy were administered after discharge. The case was closely followed and approximately 1 year after completion of chemotherapy the patient was admitted to the hospital for reconstruction of the mandible. During the procedure, abnormal tissue was noted; on biopsy it was found to be recurrent tumor. This was surgically removed and the reconstruction was not performed. The patient did well for several years, but then recurrent disease as well as metastasis to the lung developed. The patient died of his disease approximately 6 years from the time of diagnosis.
631
KERPEL, FREEDMAN, AND TROYER
Discussion Osteogenic sarcoma is a malignant osteoid-producing tumor usually found in the metaphysis of a long bone. Approximately one half of the reported cases are located around the knee joint.’ Six percent to 7% of all cases occur within the jaw bones.2 The great majority of lesions of the long bones occur within the second and third decade of life, whereas those found in the jaws are seen at least a decade later.2$3Although most studies indicate a male predilection for this disease, this finding is not totally consistent.4.5 Regardless of the location, pain and swelling are the most common presenting symptoms.’ A clinical classification of osteogenic sarcoma based on its location in and around bone has evolved over the years. s.6Classic or conventional osteogenic sarcoma occurs as an endosseous lesion and represents about three quarters of all cases.5 Those associated with the surface of a bone include parosteal Cjuxtacortical) osteogenic sarcoma and, less commonly, periosteal osteogenic sarcoma. The former has a better prognosis than classic osteogenic sarcoma because it tends to wrap around the underlying bone. Periosteal osteogenic sarcoma also may spare the medullary space by involving the periosteal surface of the cortex.6 The classification of osteogenic sarcoma also includes several other types6 Among these are telangiectatic osteogenic sarcoma, which is quite vascular and resembles an aneurysmal bone cyst; multicentric osteogenic sarcoma, and those osteogenic sarcomas arising in a preexisting condition. The latter usually refers to an osteogenic sarcoma developing in Paget’s disease, in a tumor that has been radiated, or at a site in the field of radiation6,’ There also exists high-grade surface and soft-tissue osteogenic sarcomas. Different radiographic patterns may be seen in patients with osteogenic sarcoma.5 The extent of destruction is dependent on the duration and aggressiveness of the disease. The amount of calcified material being produced dictates how much lysis or sclerosis is seen radiologically. The three radiographic patterns reported in osteogenic sarcomas are osteolytic, osteosclerotic (osteoblastic), and mixed. Some studies report the more pure forms to be predominant, whereas others suggest the mixed type to be the most common.3,8,9 Huvos has reported that lytic lesions are much more frequently seen in osteogenic sarcomas occurring in older people.’ In jaw tumors, Garrington” reported a fairly equal distribution of these three patterns, with about one quarter of cases showing a “sunburst” appearance of tumor bone. Clark,3 in his study of jaw tumors, found that the osteoblastic type was the most frequently encountered pattern in the maxilla (50%), whereas in the mandible the osteolytic form predominated (43%).
The histologic subtypes of osteogenic sarcomas are based on the predominant stromal pattern. The three most common histologic patterns are osteoblastic, chondroblastic, and fibroblastic.6 In a study of 650 cases, Dahlin found that slightly more than one half of all cases were osteoblastic and the rest were almost evenly split between the other two patterns. However, both he and Clark noted that the chondroblastic type accounted for nearly one half of the jaw tumors.‘*3 In the past, other histologic subtypes such as anaplastic, mixed, telangiectatic,3~6~8and small cell variants also have been described.” A grading system modeled after the Broder’s method for epidermoid carcinomas has been used by many pathologists. In Dahlin’s series, more than 85% were high-grade (grade III to IV) osteosarcomas.3 tnterestingly, in Clark’s study of osteosarcomas of the jaws, the cases overall were better differentiated, with over 90% being grade II or grade III.6 Before the middle 1970s it was widely accepted that patients with osteogenic sarcoma did not experience much more than a 20% 5-year survival rate despite ablative surgery, which was the most widely used form of treatment.” Although local control of disease would often be achieved, distant metastasis usually developed within a short time. Since then, clinical trials using chemotherapeutic agents such as vincristine and methotrexate with leucovorin rescue, bleomycin, cyclophosphamide, and actinomycin D, and doxorubicin have reported 5-year survival rates of 45% to 80%.“,‘2 Induction chemotherapy prior to definitive surgery has also been used, its use rationalized by speculation that most patients with osteogenic sarcoma have undetected metastatic disease at the time of treatment.12 The populations studied in these trials were usually young age groups with high-grade osteogenic sarcomas of the extremities. As previously stated, osteogenic sarcomas of the jaws tend to be better-differentiated tumors than those of the extremities and also appear not to metastasize as readily.3 Although Gamington and Clark3 reported 35% and 40% 5-year survival rates, respectively, Clark3 and RussI emphasized that in those patients treated with radical surgery the survival rates were considerably higher. Thus, the role of chemotherapy in treatment of osteogenic sarcomas of the jawbones remains unresolved. References I. Dahlin DC, Unni KK: Bone tumors: General aspects and data on 8,542 cases (ed 4). Springfield, IL, Thomas: 1986, p 269 2. Ganington GE. Scofield HH. Comvn J. et al: Osteosarcoma of the J%W: An&is of 56 c&es. Cance; 20:311, 1967 3. Clark JL, Unni KK, Dahlin DC, et al: Osteosarcoma of the jaw. Cancer 51:231 I. 1983
632 4. Stark A, Kreicbergs A, Nilsonne U, et al: The age of osteosarcoma patients is increasing: An epidemiological study of osteosarcoma in Sweden 1971 to 1984. J Bone Joint Surg [B] 72:89, 1990 5. Edeiken-Monroe B, Edeiken J, Jacobson HG: Osteosarcoma. Semin Roentgen01 24: 153, 1989 6. Unni KK, Dahlin DC: Osteosarcoma: Pathology and classification. Semin Roentgen01 24: 143, 1989 7. Dickens P, Wei WI, Sham JS: Osteosarcoma of the maxilla in Hong Kong Chinese postirradiation for nasopharyngeal carcinoma: A report of four cases. Cancer 66: 1924, 1990 8. Huvos, AG: Bone Tumors: Diagnosis, treatment and prognosis. Philadelphia, PA, Saunders, 1979, p 47
EXPANSILE MASS OF BODY OF THE MANDIBLE
9. Huvos AG: Osteogenic sarcoma of bones and soft tissues in older persons: A clinicopathologic analysis of 117 patients older than 60 years. Cancer 57: 1442, 1986 10. Simi FH, Unni KK, Beaubout JW, et al: Osteosarcoma with small cells simulating Ewing’s tumor. J Bone Joint Surg [A] 61:207, 1979 11. Link MP, Goorin AM, Miser AW. et al: The effect of adjuvant chemotherapy on relapse-free survival in patients with osteosarcoma of the extremity. N Engl J Med 3 14:1600, 1986 12. Goorin AM, Abelson HT, Frei III EF: Osteosarcoma: Fifteen years later. N Engl J Med 313:1637, 1985 13. Russ JE, Jesse RH: Management of osteosarcoma of the maxilla and mandible. Am J Surg 140:572, 1980
Surg
1992
Expansile Mass of the Body of the Mandible STANLEY KERPEL, DDS,* PAUL FREEDMAN, DDS,t AND STEPHEN H. TROYER, DDS, MSDS dible and the root resorption of the involved teeth, suggest an aggressive, probably malignant, lesion. The radiograph obtained 3 years previously excludes a number of benign lesions that would take many years to reach the size of this lesion. The presence of calcified deposits suggest that mixed radiolucent/radiopaque lesions must be considered. The differential diagnosis basically includes the following: 1) benign odontogenic cysts or neoplasms; 2) benign nonodontogenic neoplasms; and 3) malignant neoplasms. Benign odontogenic cysts or neoplasms to be considered include dentigerous cyst, odontogenic keratocyst, ameloblastoma, calcifying odontogenic cyst (Gorlin’s cyst), calcifying epithelial odontogenic tumor (Pindborg tumor), odontogenic myxoma, adenomatoid odontogenic tumor, and central cementifying fibroma. Dentigerous cyst. The dentigerous cyst can be an aggressive lesion at times. It can result in expansion of bone with facial asymmetry, as well as cause root resorption of adjacent teeth. Rarely, dentigerous cysts may erode the cortical plates of bone. Paresthesia is not common. Radiographically, the lesion appears as a radiolucency with a thin sclerotic border; it invariably involves the crown of an unerupted tooth. The latter would tend to make the diagnosis of dentigenous cyst unlikely. Odontogenic keratocyst. The odontogenic keratocyst most often occurs in the third decade of life. It is seen more frequently in males than females and the mandible is involved much more frequently than the maxilla. Radiographically, the lesion appears as a unilocular or multilocular radiolucency, usually with a thin sclerotic border. The lesion may penetrate bone as well as expand it. Multiple odontogenic keratocysts can occur, and in some instances they are associated with the basal cell nevus syndrome. Ameloblastoma. An ameloblastoma can occur at any age; however, nearly half of the lesions occur between the ages of 20 and 40 years. Ameloblastomas typically begin as central lesions that are slowly destructive and tend to expand bone rather than perforate it. Paresthesia is not common. The mural ameloblastoma usually arises in the wall of a dentigerous cyst. Radiographically, ameloblastomas are classically described as multilocular radiolucent lesions. In advanced
Case Presentation In March 198 1, a 42-year-old white man was referred for evaluation of a swelling of the right mandibular vestibule (Fig I). Except for a slight tingling of the lower lip, there were no other symptoms. The swelling was hard, with no apparent crepitation noted on palpation, and was covered by intact mucosa. Extraorally there was a subtle expansion of the right submandibular region. Radiographs showed the presence of a large, predominantly radiolucent lesion extending from the right mandibular premolar region to the distal of the right third molar (Fig 2). The lesion appeared compartmentalized and contained calcified deposits within its center. There was slight root resorption of the involved teeth. The anterior component of the lesion was not well delineated, and the inferior border appeared to be irregularly eroded in one area. The patient’s medical history was essentially negative, as were all his laboratory findings. Of interest in his past dental history was transient temporomandibular joint discomfort on the right side 3 years before. A panoramic radiograph taken at that time (Fig 3) was interpreted as normal, and shortly thereafter his symptoms subsided. Differential
Diagnosis
Stephen H. Troyer, DOS, MSD
The availability of a panoramic radiograph obtained 3 years prior to this examination is certainly helpful, since it indicates that the lesion is of relatively recent origin. This radiograph does not indicate the presence of dental pathology in the area of the current radiolucent lesion and all teeth in the area appear to be in good repair. Restorations in the posterior teeth appear to be reasonably small and do not seem to involve the pulp. There is no obvious widening of the periodontal membrane involving any of these teeth. The clinical and radiographic findings, particularly the irregular erosion of the inferior border of the man-
* Attending, Department of Oral Pathology, Booth Memorial Medical Center, Flushing, NY. t Assistant Director, Department of Oral Pathology, Booth Memorial Medical Center, Flushing, NY. $ In private practice, Evansville, IN. Addressreprint requests to Dr Kerpel: Oral Pathology Laboratory, Inc, 56-45 Main St, Flushing, NY 11355. 8 1992 American
Association
of Oral and Maxillofacial
Surgeons
0278-2391/92/5006-0015$3.00/O
627
626
EXPANSILE MASS OF BODY OF THE MANDIBLE
FIGURE 1. Photograph taken at initial presentation showing a swelling of the right mucobuccal fold.
cases they appear compartmentalized, with septa of bone within the radiolucent mass. Calcifving odontogenic cyst (Gorlin’s cyst). The calcifying odontogenic cyst may occur at any age. In its early stages of development, the lesion may closely resemble a dentigerous cyst radiographically. Later in the course, varying amounts of calcified radiopaque material are seen scattered throughout the lesion. Calcifying epithelial odontogenic tumor (Pindborg’s tumor). The mean age of occurrence of the calcifying epithelial odontogenic tumor is 40 years, with no significant sex predilection. It occurs twice as frequently in the mandible as in the maxilla. Radiographically, the lesion has a variable appearance. Although it may appear as a diffuse or well-circumscribed unilocular radiolucency, it also may be multilocular. There may be scattered flecks of calcification present.
FIGURE 2. Panoramic radiograph showing the presence of a destructive, predominantly radiolucent lesion with ill-defined borders. There are calcified deposits present just inferior to the mandibular second molar.
FIGURE 3. Normal radiograph taken 3 years previously (A) compared with recent radiograph (B).
Adenomatoid odontogenic tumor. The adenomatoid odontogenic tumor is usually found in patients under 20 years of age. Females are involved twice as frequently as males and the maxilla is involved twice as frequently as the mandible. Unlike the ameloblastoma, this lesion occurs most frequently anterior to the canine both in the maxilla and mandible. Radiographically, it appears as a destructive lesion of bone that may or may not be well circumscribed, but many times resembles a dentigerous cyst. There is usually a unilocular radiolucent appearance, but the lesion may contain radiopaque foci. Root resorption is rare. Odontogenic myxoma. The odontogenic myxoma usually occurs between the second and third decades of life. Males are involved less frequently than females. The mandible is involved slightly more frequently than the maxilla. Radiographically, the odontogenic myxoma appears as a central lesion of the jaws that may expand the bone and cause cortical plate destruction. Displacement of teeth is common; however, root resorption is less frequent. Central cementijjying$broma. The central cementifying fibroma usually occurs in the third or fourth decade of life. Females are involved twice as frequently as males. Radiographically, the lesion has a variable appearance depending on its stage of development. In its early stages, it appears as a radiolucent lesion with no evidence of internal radiopacities. As the tumor matures, multiple opacities appear within the radiolucent area until eventually it appears as a radiopaque mass. The lesion has a centrifugal growth pattern, resulting in the production of a round tumor mass as it expands the inferior border of the mandible. Some believe that the central cementifying fibroma and the
KERPEL, FREEDMAN.
AND TROYER
central ossifying fibroma are closely related, in that their age of occurrence, sex predilection, radiographic appearance, location, and clinical behavior are similar. Nonodontogenic neoplasms that should be considered include neurolemmoma and chondroma. Neurokmmoma. The neurolemmoma may arise at any age and there is no sex predilection. Intraorally, the neurolemmoma is most commonly reported as a soft-tissue lesion. A number of cases have been reported involving the mandible, with the tumor apparently arising from the inferior alveolar nerve. Radiographitally, the central lesions in bone may produce considerable destruction, with expansion of the cortical plates, resembling what might be expected of a much more aggressive lesion. The chondroma is a benign central Chondroma. tumor of cartilage that is common in the bony skeleton, but relatively uncommon in the maxilla or mandible. The lesion may appear at any age and shows no sex predilection. It has been reported to occur in the body of the mandible. Radiographically, the lesion presents as an irregular radiolucent or mixed radiolucent/radiopaque area. It is destructive and has been reported to cause root resorption of adjacent teeth. Malignant neoplasms to be considered include the following: osteosarcoma (osteogenic sarcoma), malignant schwannoma (neurogenic sarcoma), chondrosarcoma, fibrosarcoma, Ewing’s sarcoma, primary lymphoma of bone, and metastatic lesions to the mandible from other primary sites. Osteosarcoma of the jaws occurs Osteosarcoma. at a later age than osteosarcoma involving the long bones, with the usual age at the time of occurrence reported to be between 27 to 33 years. Males are involved more frequently than females and tumors of the mandible are more common than those of the maxilla. Osteosarcoma has been reported to be preceded by trauma. It also has been reported with increased frequency in bone affected by osteitis deformans (Paget’s disease). In nearly all osteosarcomas of the jaw that have been reported, however, there was no preceding history of trauma or of osteitis deformans. Bone subjected to therapeutic radiation has been reported to undergo malignant transformation. A number of cases of osteosarcoma developing after radiation therapy for benign jaw lesions (ie, fibrous dysplasia and giant cell granuloma) have been reported. Radiographically, osteosarcoma may present as an osteolytic process with minimal calcified bone formation or as a sclerosing bone-forming lesion. The presence of irregular trabeculae of bone radiating outward on the periphery of the lesion has been described as the “sun-ray” appearance of osteosarcoma. The tumor destroys cortical plates as it progresses, expanding and eventually perforating them. The osteolytic form
629 is essentially a destructive process, resulting in an irregular radiolucency with both expansion and destruction of the cortical plates. An early reported manifestation of osteosarcoma is symmetrical widening of the periodontal ligament space surrounding one or more teeth on the dental radiograph. This is reported to occur prior to any other radiographic evidence of the presence of a neoplasm, and therefore is of great diagnostic significance. Malignant schwannoma. The malignant schwannoma appears most frequently between the ages of 30 and 60 years. Male and female incidence are similar, with the mandible involved more frequently than the maxilla. The presenting symptoms may include pain and/or paresthesia. Radiographically, the lesion may appear as a diffuse radiolucency that is characteristic of a malignant neoplasm. It may also appear as a smooth radiolucency associated with the mandibular canal when the tumor originates from the inferior alveolar nerve. Chondrosarcoma. The chondrosarcoma occurs most frequently between the ages of 30 and 60 years. Males are involved twice as frequently as females. A distinctive type of chondrosarcoma, the mesenchymal chondrosarcoma, occurs most frequently between the ages of 10 and 30 years and has no sex predilection. Radiographically, the lesion usually appears as an irregular radiolucent area in bone that may produce considerable destruction. Occasionally there is calcification of the neoplastic cartilage. Fibrosarcoma. Intraorally, the fibrosarcoma may arise in any location, but is commonly involved with the periosteum of the maxilla or mandible. The lesion is most common before 50 years of age. Radiographically, it appears as a destructive malignant neoplasm. Ewing’s sarcoma is an uncomEwing’s sarcoma. mon malignant lesion that occurs primarily in people aged 5 to 25 years. The mandible is involved more frequently than the maxilla. Lip paresthesia has been reported. Radiographically, the lesion is destructive and may produce an irregular, diffuse radiolucency. Lesions may also be radiopaque, resembling sclerosing osteomylitis. Formation of layers of subperiosteal bone is characteristic, producing the “onion skin” appearance. Osteophytes within the lesion also may produce the “sun-ray” appearance associated with osteosarcoma. Primary lymphoma of bone. Primary lymphoma of bone is an uncommon disease of the jaws, occurring somewhat more frequently in the mandible than the maxilla. The disease usually occurs prior to 40 years of age and males are involved more frequently than females. Paresthesia has been reported. Radiographitally, the lesion appears as an invasive, destructive, malignant neoplasm. The diffuse radiolucency many
630 times involves the alveolar process, with destruction of bone and adjacent teeth. Metastatic lesions to mandible from other primary sites. Although the jaws are not thought to be a common site for metastasis, metastatic tumors to the jaws are of significance as their appearance may be the first indication of malignancy at a distance site. The lesions may be asymptomatic, but paresthesia has been reported due to involvement of the inferior alveolar nerve. Expansion of the jaw occurs frequently. The mandible is involved much more frequently than the maxilla. Radiographically, there is no characteristic appearance. The metastatic lesions may cause bone destruction and appear as radiolucencies, but certain lesions produce bone, giving a mixed radiolucent/radiopaque appearance. Primary tumors that may metastasize to the jaws include those involving the breast, lung, and kidney. Tumors involving the thyroid, prostate, colon, and stomach have been reported to metastisize less frequently. Summary. A differential diagnosis has been presented that includes a number of benign nonodontogenie cysts and neoplasms, benign nonodontogenic neoplasms, and malignant neoplasms. Obviously, a biopsy of the lesion is indicated to establish the final diagnosis. Although a number of the benign lesions discussed should be considered in the differential diagnosis, the clinical and radiographic evidence suggest a malignant neoplasm. Factors that favor a malignant neoplasm include the following: 1) the presence of a large destructive radiolucent lesion with no evidence of dental pathology (ie, impacted teeth, periapical pathology); 2) obvious invasion and destruction of the inferior border of the mandible; 3) resorption of roots of teeth adjacent to the lesion. Of the malignant neo-
FIGURE 4. Medium-power photomicrogmph showing tumor bone on the left and cartilage-like material bordered by difise sheets of malignant osteoblasts on the right (hematoxyln-eosin stain, original magnification X 100).
EXPANSILE MASS OF BODY OF THE MANDIBLE
FIGURE 5. High-power photomicrograph showing clusters of cells with large hyperchromatic nuclei, some containing prominent nucleoli. Moderate pleopmorphism of the tumor cells, as well as malignant bone, also is noted.
plasms discussed, the osteosarcoma is the most common neoplasm exhibiting the clinical and radiographic appearance of the case presented. These characteristics include: 1) age of the patient at the time the lesion is diagnosed; 2) more frequent involvement in males; 3) more common occurrence in the mandible; 4) radiographic appearance as a destructive radiolucency with expansion and destruction of cortical plates; 5) varying amounts of calcification present within the radiolucency. SUBSEQUENT
COURSE
An incisional biopsy was performed under local anesthesia and consisted of fragments of soft tissue that were gritty in areas. Microscopically, the tumor was composed of focally cellular mesenchymal tissue exhibiting both osteoid formation and cartilage, which showed endochondral ossification (Fig 4). Cellular foci were found alternating with areas that were sparsely populated by the tumor cells. The tumor cells were large, spindled to polyhedral in shape, and contained hyperchromatic nuclei with prominent nucleoli (Fig 5). Obvious pleomorphism, as well as the appearance of occasional atypical mitoses, was also present. The microscopic appearance was consistent with osteogenic sarcoma, chondroblastic type. The patient was referred for treatment. He was treated first with five courses of high-dose methotrexate and then underwent a right hemimandibulectomy with removal of the submandibular gland and regional lymph nodes. Several courses of postoperative chemotherapy were administered after discharge. The case was closely followed and approximately 1 year after completion of chemotherapy the patient was admitted to the hospital for reconstruction of the mandible. During the procedure, abnormal tissue was noted; on biopsy it was found to be recurrent tumor. This was surgically removed and the reconstruction was not performed. The patient did well for several years, but then recurrent disease as well as metastasis to the lung developed. The patient died of his disease approximately 6 years from the time of diagnosis.
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Discussion Osteogenic sarcoma is a malignant osteoid-producing tumor usually found in the metaphysis of a long bone. Approximately one half of the reported cases are located around the knee joint.’ Six percent to 7% of all cases occur within the jaw bones.2 The great majority of lesions of the long bones occur within the second and third decade of life, whereas those found in the jaws are seen at least a decade later.2$3Although most studies indicate a male predilection for this disease, this finding is not totally consistent.4.5 Regardless of the location, pain and swelling are the most common presenting symptoms.’ A clinical classification of osteogenic sarcoma based on its location in and around bone has evolved over the years. s.6Classic or conventional osteogenic sarcoma occurs as an endosseous lesion and represents about three quarters of all cases.5 Those associated with the surface of a bone include parosteal Cjuxtacortical) osteogenic sarcoma and, less commonly, periosteal osteogenic sarcoma. The former has a better prognosis than classic osteogenic sarcoma because it tends to wrap around the underlying bone. Periosteal osteogenic sarcoma also may spare the medullary space by involving the periosteal surface of the cortex.6 The classification of osteogenic sarcoma also includes several other types6 Among these are telangiectatic osteogenic sarcoma, which is quite vascular and resembles an aneurysmal bone cyst; multicentric osteogenic sarcoma, and those osteogenic sarcomas arising in a preexisting condition. The latter usually refers to an osteogenic sarcoma developing in Paget’s disease, in a tumor that has been radiated, or at a site in the field of radiation6,’ There also exists high-grade surface and soft-tissue osteogenic sarcomas. Different radiographic patterns may be seen in patients with osteogenic sarcoma.5 The extent of destruction is dependent on the duration and aggressiveness of the disease. The amount of calcified material being produced dictates how much lysis or sclerosis is seen radiologically. The three radiographic patterns reported in osteogenic sarcomas are osteolytic, osteosclerotic (osteoblastic), and mixed. Some studies report the more pure forms to be predominant, whereas others suggest the mixed type to be the most common.3,8,9 Huvos has reported that lytic lesions are much more frequently seen in osteogenic sarcomas occurring in older people.’ In jaw tumors, Garrington” reported a fairly equal distribution of these three patterns, with about one quarter of cases showing a “sunburst” appearance of tumor bone. Clark,3 in his study of jaw tumors, found that the osteoblastic type was the most frequently encountered pattern in the maxilla (50%), whereas in the mandible the osteolytic form predominated (43%).
The histologic subtypes of osteogenic sarcomas are based on the predominant stromal pattern. The three most common histologic patterns are osteoblastic, chondroblastic, and fibroblastic.6 In a study of 650 cases, Dahlin found that slightly more than one half of all cases were osteoblastic and the rest were almost evenly split between the other two patterns. However, both he and Clark noted that the chondroblastic type accounted for nearly one half of the jaw tumors.‘*3 In the past, other histologic subtypes such as anaplastic, mixed, telangiectatic,3~6~8and small cell variants also have been described.” A grading system modeled after the Broder’s method for epidermoid carcinomas has been used by many pathologists. In Dahlin’s series, more than 85% were high-grade (grade III to IV) osteosarcomas.3 tnterestingly, in Clark’s study of osteosarcomas of the jaws, the cases overall were better differentiated, with over 90% being grade II or grade III.6 Before the middle 1970s it was widely accepted that patients with osteogenic sarcoma did not experience much more than a 20% 5-year survival rate despite ablative surgery, which was the most widely used form of treatment.” Although local control of disease would often be achieved, distant metastasis usually developed within a short time. Since then, clinical trials using chemotherapeutic agents such as vincristine and methotrexate with leucovorin rescue, bleomycin, cyclophosphamide, and actinomycin D, and doxorubicin have reported 5-year survival rates of 45% to 80%.“,‘2 Induction chemotherapy prior to definitive surgery has also been used, its use rationalized by speculation that most patients with osteogenic sarcoma have undetected metastatic disease at the time of treatment.12 The populations studied in these trials were usually young age groups with high-grade osteogenic sarcomas of the extremities. As previously stated, osteogenic sarcomas of the jaws tend to be better-differentiated tumors than those of the extremities and also appear not to metastasize as readily.3 Although Gamington and Clark3 reported 35% and 40% 5-year survival rates, respectively, Clark3 and RussI emphasized that in those patients treated with radical surgery the survival rates were considerably higher. Thus, the role of chemotherapy in treatment of osteogenic sarcomas of the jawbones remains unresolved. References I. Dahlin DC, Unni KK: Bone tumors: General aspects and data on 8,542 cases (ed 4). Springfield, IL, Thomas: 1986, p 269 2. Ganington GE. Scofield HH. Comvn J. et al: Osteosarcoma of the J%W: An&is of 56 c&es. Cance; 20:311, 1967 3. Clark JL, Unni KK, Dahlin DC, et al: Osteosarcoma of the jaw. Cancer 51:231 I. 1983
632 4. Stark A, Kreicbergs A, Nilsonne U, et al: The age of osteosarcoma patients is increasing: An epidemiological study of osteosarcoma in Sweden 1971 to 1984. J Bone Joint Surg [B] 72:89, 1990 5. Edeiken-Monroe B, Edeiken J, Jacobson HG: Osteosarcoma. Semin Roentgen01 24: 153, 1989 6. Unni KK, Dahlin DC: Osteosarcoma: Pathology and classification. Semin Roentgen01 24: 143, 1989 7. Dickens P, Wei WI, Sham JS: Osteosarcoma of the maxilla in Hong Kong Chinese postirradiation for nasopharyngeal carcinoma: A report of four cases. Cancer 66: 1924, 1990 8. Huvos, AG: Bone Tumors: Diagnosis, treatment and prognosis. Philadelphia, PA, Saunders, 1979, p 47
EXPANSILE MASS OF BODY OF THE MANDIBLE
9. Huvos AG: Osteogenic sarcoma of bones and soft tissues in older persons: A clinicopathologic analysis of 117 patients older than 60 years. Cancer 57: 1442, 1986 10. Simi FH, Unni KK, Beaubout JW, et al: Osteosarcoma with small cells simulating Ewing’s tumor. J Bone Joint Surg [A] 61:207, 1979 11. Link MP, Goorin AM, Miser AW. et al: The effect of adjuvant chemotherapy on relapse-free survival in patients with osteosarcoma of the extremity. N Engl J Med 3 14:1600, 1986 12. Goorin AM, Abelson HT, Frei III EF: Osteosarcoma: Fifteen years later. N Engl J Med 313:1637, 1985 13. Russ JE, Jesse RH: Management of osteosarcoma of the maxilla and mandible. Am J Surg 140:572, 1980