Osteogenic Sarcoma of the Virginia A. LiVolsi, \s=b\ Six cases of osteosarcoma of the maxilla are reported, including one case that was radiation-associated and one case that arose in Paget's disease. The literature on this entity is reviewed and the clinicopathologic features of this lesion in this unusual location are discussed.
(Arch Otolaryngol 103:485-488, 1977)
Osteogeni c mas) rarely
sarcomas
prognosis.
In the nonfacial skeleton, osteosarfound most frequently in the long bones: femur, tibia, and humérus.1" These neoplasms occur in second and third decades. When osteosarcomas are seen in older patients, preexisting conditions are commonly identified, such as bone infarcts," bone radiation,12" and Paget's dis¬ ease.1"15 Osteosarcoma arising in preexisting Paget's disease of the facial bones has been reported rare¬ ly.17 The present report reviews a series of cases of primary osteosarcomas of the maxilla, including one case that arose in an individual with polyostotic Paget's disease. comas are
Accepted for publication Feb 22, 1977. From the Department of Pathology, Yale University School of Medicine, New Haven, Conn.
Reprint requests to Department of Pathology, Yale University School of Medicine, 310 Cedar St, New Haven, CT 06510.
MD
SUBJECTS AND METHODS Six patients with osteosarcoma of the maxilla, whose conditions were diagnosed and treated at the Yale-New Haven Hospital from 1955 to 1975, form the basis of this study. The clinical charts and all pathologic materials were reviewed. Fol¬ low-up data were obtained in all but one instance.
(osteosarco-
arise in the maxilla. Small series11 and individual case reports' " have described the clinical and pathological features of these lesions; most studies indicate a poor
Clinical
Maxilla
Findings
Age and Sex.—All six patients
were male whose ages at diagnosis ranged from 10 to 75 years (Table). Symptoms.—A mass in the cheek or maxillary area brought three of the six patients to medical attention. Loose teeth or dentures were noted by two of these three persons; loose dentures was the only symptom for another patient. The two remaining persons complained of epistaxis or bloody nasal discharge; one of these also experienced diplopia. Duration of symp¬ toms ranged from two weeks to six months. History.—Three of the six patients had notable preexisting conditions. One man recalled trauma to the cheek region 40 years before he developed the osteosar¬ coma; the trauma of an explosion had led to visual disturbances for most of his life. A second patient had received radiation therapy for retinoblastoma seven and five years before he developed osteosarcoma. Another person had a 30-year history of Paget's disease that involved the humérus, vertebrae, and skull bones.
subjects,
Radiologie Studies In four cases, sinus and facial radio¬ graphs demonstrated a mass that was destroying the boundaries of the maxilla. In three of these cases, sclerosis or osteo-
led to a differential diagnosis that included osteosarcoma. A benign osteogen¬ ic lesion was considered in a fifth case. The patient with Paget's disease showed a progressive change in the facial bones, but a definitive diagnosis of a superimposed neoplastic process was not made.
genesis
Pathology Gross.—The lesions occupied the entire maxilla and were associated with a scle¬ rotic process that extended into surround¬ ing soft tissues. Four tumors occurred primarily on the left side; two tumors occurred on the right side. In each patient, extension of the lesion was noted: to the ethmoid sinus in two cases, to nasopharynx in one case, and to the opposite antrum in five cases.
Microscopic—Osteogenic
sarcoma
was
identified in each case. The degree of differentiation varied (Fig 1 and 2). Osteoid formation and calcification ranged from minute to massive. Zones of neoplas¬ tic cartilage were recognized in one tumor. The proliferating cells contained markedly hyperchromatic nuclei; mitoses were easily found. In three instances, invasion into the antral mucosa was seen. Destruction of normal, preexisting bone was noted in those cases in which adequate material was resected. In one patient (case 5), histologie Paget's disease was identified at autopsy in the humérus. Destruction of the facial and cranial bones by tumor precluded histologie documentation of Paget's disease in this area.
Sites of Metastasis In four patients who died, the lungs and several bones (clavicles, humérus, ribs, and pelvic bones) were the sites involved by
Downloaded From: http://archotol.jamanetwork.com/ by a University of Arizona Health Sciences Library User on 05/25/2015
Clinical
Findings
Case
Age, yr/ Sex 1/63/M
Symptoms/ Duration
Swelling
on
Predisposing Factor
right
Extensive destruc¬ tion of right an-
None
side of face with
proptosis/6 2/48/M
Maxillary
Partial excision
None
3/56/M
Loosened den¬ tures/several wk
Injury 40
4/10/M
Bloody nasal discharge/4
Radiation therapy for retinoblastoma
yr before osteosarcoma
diagnosis
Wide excision
Sclerosing
Maxillectomy (margins of excision involved) Left maxillectomy
probably benign Left maxillary mass with invasion of ethmoid sinus
Follow-up Slight response; prompt recurrence;
Mass occupying maxilla with inva¬ sive edges process left maxilla—
6,000 rads
radiotherapy
by bone-pro¬ ducing mass
loose teeth/2 wk
mo
Postoperative Therapy
Therapy
trum
mo
mass,
Radiographie Findings
only
died of disease after 1 yr Died of disease af¬
7,500 rads
radiotherapy;
ter 15
mo
methotrexate
and ethmoldectomy
Lost to follow up
Chemotherapy— doxorubicin
hydrochloride,
Died of disease after 3 yr
dacarbazine,
methotrexate
5/75/M
Epistasis, diplopia/ Polyostotic Paget's 3 wk
disease 30 yr before osteosar¬ coma
6/32/M
Swelling on right side of face; loosened den¬ tures/3 wk
None
diagnosis
Sclerotic bone and soft tissue density in left ethmoid and
spheroid areas Ossifying lesion of maxilla, irregular edges; probably malignant growth
Biopsy performed
Radiotherapy;
me¬
thotrexate treat¬
Died of disease after 5 mo
ment
Right maxillectomy; partial left lectomy
maxil¬
7,000 rads radiother¬ Died of disease apy
Fig 1 —Moderately differentiated osteosarcoma with obvious bone formation. Note hyperchromatic malignant cells in stroma (case 4) (hematoxylin-eosin, 250).
Downloaded From: http://archotol.jamanetwork.com/ by a University of Arizona Health Sciences Library User on 05/25/2015
after 15
mo
Fig 2.—Left, Case 5. Osteosarcoma encroaching on antral mucosa (shown in on right) (hematoxylin-eosin; left, 250; right, 450). without identifiable preexisting le¬ metastatic deposits. Each of these patients patients had received radiotherapy on also showed local recurrence. Autopsy in sion.1" Trauma has been noted to be the facial area seven to 42 years the fifth patient demonstrated massive associated with bone before developing maxillary osteogen¬ frequently long invasion of the cerebral hemisphere ipsilat¬ ic sarcoma: two of nine patients who tumors, but this may be a reflection of eral to the tumor; however, no distant the fact that trauma draws attention were described by Fu and Perzin,2 métastases were identified. three described by Kragh et al,3 and to the lesion, rather than the indica¬ tion of a causal association. Several one patient each who was described by Therapy conditions of bone have been asso¬ Caron et al,1 Yannopoulos et al,9 Arlen Biopsies were performed on all lesions. ciated with the et al,5 and Sim et al." One subject in subsequent develop¬ Following the diagnosis of osteosarcoma, the present series also gave a history ment of osteosarcoma. These include further surgical excision was attempted in radiation,51--1' Paget's disease,1"15 of radiotherapy (case 4), bringing the four instances: radical maxillectomy (three bone infarcts,11 and fibrous dyspla¬ total to 14 patients who had prior cases), and wide excision (one case). Radio¬ radiation therapy. sia.1"1"17 therapy was administered to four persons Reasons for radiation therapy for In the maxilla, osteosarcomas have (as primary therapy in one case and post¬ operatively in three cases). Adjuvant been reported as having arisen in these 14 patients included treatment chemotherapy was given to three patients; association with prior radia¬ of retinoblastoma (five cases),2·1 fi¬ this included methotrexate alone in two brous dysplasia (four cases),2"1214 "be¬ fibrous dysplasia,1-2-912-14 tion,--5"15" cases, and combination therapy of doxoruand Paget's disease.1·7 A review of osseous growth" (one case),5 nign bicin hydrochloride (Adriamycin), dacarbathese factors in preexisting maxillary squamous cell carcinoma (one case),4 zine (DTIC-Dome), and methotrexate in follows. sarcomas spindle cell tumor (one case),11 and two osteogenic one case. detail
Of the five subjects who were followed up, all died of recurrent and metastatic disease.
COMMENT
Most osteosarcomas arise
anew
Radiation
Therapy
Prior radiation therapy constitutes the most commonly encountered fac¬ tor. Thus, of the recorded cases of osteosarcoma of the maxilla, 13
cases
for which the
reasons
for treat¬
ment is unknown.3
Dysplasia patients gave a history of Fibrous
Five fibrous
dysplasia,1·2·*·18·"
Downloaded From: http://archotol.jamanetwork.com/ by a University of Arizona Health Sciences Library User on 05/25/2015
and
one
pa-
tient had had a "benign osseous growth".5 Of these, five patients had received radiation therapy prior to developing osteosarcoma; the other patient1 had not.
Paget's
Rarely has
Disease
osteosarcoma of the
maxilla been associated with Paget's disease. Caron et al1 cited two cases, and Karpawich7 described one in¬ stance of such an association. Patient 5 in the present report also had a definite history of Paget's disease. Trauma A definite history of trauma pre¬ ceded the discovery of the osseous lesion by three years in one reported case," and by 40 years in case 3 of this
series.
Osteogenic sarcomas of the maxilla swelling that is often followed by pain, dental, and neuro¬ logic symptoms. The majority of patients have no notable prior history appear with
of bone disease.1-2-4 Whereas osteosarcoma of the long bones tends to occur in patients in the second decade,1" the age of most patients with osteosarcoma of the maxillae is third to fourth decade.12-4 Men are affected more frequently in
all types of osteosarcoma,10 although Dehner18 recorded the case of a 15year-old girl with maxillary osteosar¬
tion appears to be less a
important from prognostic standpoint than does the
extent of the tumor at initial
diagno¬
coma.
sis.
Radiological findings need not be diagnostic; most patients show bone destruction and an expansile growth that is often accompanied by soft
Therapy for these tumors remains primarily radical surgery. However,
tissue extension. Periosteal new-bone formation is rarely found. A malig¬ nant lesion is suspected often. How¬ ever, various appearances have been described that depend on the relative osteolytic and osteoblastic nature of the tumor.'-1'-18 Pathologically, the tumors grossly have been described as firm, fibrous, gritty, and occasionally, calcified or ossified.1-2-4 When maxillectomy spec¬ imens have been available, the lesions usually erode and extend through the preexisting bone.2 Results of histological examination also disclose a variety of patterns that depend on the degree of differentia¬ tion of the lesion. Hence, areas that are composed of malignant mesenchymal cells with foci of cartilage and osteoid formation are described.1-2-4·8 However, the amount of osteoid mate¬ rial may vary from lesion to lesion and from zone to zone in the same neoplasm.2 The degree of differentia-
because of the location, and often the extent of the neoplasm, total surgical extirpation is rarely feasible. Radio¬ therapy and, more recently, chemo¬ therapy have been tried; experience with these modalities is limited for maxillary osteosarcomas, but they appear to offer little palliation at best. The overall survival of patients with osteosarcomas has been recorded as 17%.'" Prognosis for patients with jaw bone lesions has been described as better than for those patients who have tumors that occur in long bones; this has been attributed to better differentiation of the jaw osteosarco¬ mas. Hence, Caron et al' reported a 33% survival for such patients. How¬ ever, Fu and Perzin2 had an 11% fiveyear survival in their series, and Kragh et al5 recorded a 19% diseasefree rate. The absence of any longterm survivor in the present series correlates more closely with the latter two
reports.
References AS, Hajdu SI, Strong EW: Osteogenic of the facial and cranial bones. Am J
1. Caron sarcoma
Surg 122:719-725,
1971.
YS, Perzin KH: Nonepithelial tumors of the nasal cavity, paranasal sinuses and nasophar2. Fu
clinicopathologic study: II. Osseous and including osteoma, fibrous dysplasia, ossifying fibroma, osteoblastoma, giant cell tumor and osteosarcoma. Cancer
ynx: A
fibro-osseous lesions,
33:1289-1305, 1974. 3. Kragh LV, Dahlin DC, Erich JB: Osteogenic sarcoma
of the jaws and facial bones. Am J Surg
96:496-505, 1958. 4. Roca AN, Smith JL, Jing BS: Osteosarcoma and parosteal osteogenic sarcoma of the maxilla and mandible. Am J Clin Pathol 54:625-636, 1970. 5. Arlen M, Shah IC, Higinbotham N, et al: Osteogenic sarcoma of head and neck induced by radiation therapy. NY State J Med 172:929-934, 1972.
6. Curtis ML, Elmore JS, Sotercanos GC: Osteosarcoma of the jaws. J Oral Surg 32:125\x=req-\
130, 1974.
7. Karpawich AJ: Paget's disease with osteogenic sarcoma of maxilla. Oral Surgery
11:827-834, 1958. 8. Madhaven M, Aurora AL, Sen SB: Osteogen-
ic sarcoma of the maxilla. J Laryngol Oto 88:1125\x=req-\
1129, 1974.
9. Yannopoulos K, Born AF, Griffiths CO, et al: Osteosarcoma arising in fibrous dysplasia of the facial bones. Am J Surg 107:556-564, 1964. 10. Spjut HJ, Dorfman HD, Fechner RE, et al: Tumors of Bone and Cartilage. Atlas of Tumor Pathology, series 2. Armed Forces Institute of Pathology, 1970, pt 5,pp 141-162. 11. Mirra JM, Bullough PG, Marcove RC, et al: Malignant fibrous histiocytoma and osteosarcoma in association with bone infarcts. J Bone Joint Surg 56-A:932-940, 1974. 12. Sabaras AO, Dahlin DC, Childs DS, et al:
Postradiation sarcoma of bone. Cancer 9:528-542, 1956. 13. Arlen M, Higinbotham NL, Huvos AG, et al: Radiation-induced sarcoma of bone. Cancer 28:1087-1099, 1971. 14. Sim FH, Cupps RE, Dahlin DC, et al: Postradiation sarcoma of bone. J Bone Joint Surg 54-A:1479-1489, 1972. 15. Porretta CA, Dahlin DC, Jones JM: Sarcoma in Paget's disease of bone. J Bone Joint Surg 39-A:1314-1329, 1957. 16. Huvos AG, Higinbotham NL, Miller TR: Bone sarcomas arising in fibrous dysplasia. J Bone Joint Surg 54-A:1047-1056, 1972. 17. Townsend GL, DeSanto LW: Malignant change in sphenoid sinus fibrous dysplasia. Arch Otolaryngol 92:267-271, 1970. 18. Dehner LP: Tumors of the mandible and maxilla in children. Cancer 32:112-120, 1973.
Downloaded From: http://archotol.jamanetwork.com/ by a University of Arizona Health Sciences Library User on 05/25/2015
(Arch Otolaryngol 103:485-488, 1977)
Osteogeni c mas) rarely
sarcomas
prognosis.
In the nonfacial skeleton, osteosarfound most frequently in the long bones: femur, tibia, and humérus.1" These neoplasms occur in second and third decades. When osteosarcomas are seen in older patients, preexisting conditions are commonly identified, such as bone infarcts," bone radiation,12" and Paget's dis¬ ease.1"15 Osteosarcoma arising in preexisting Paget's disease of the facial bones has been reported rare¬ ly.17 The present report reviews a series of cases of primary osteosarcomas of the maxilla, including one case that arose in an individual with polyostotic Paget's disease. comas are
Accepted for publication Feb 22, 1977. From the Department of Pathology, Yale University School of Medicine, New Haven, Conn.
Reprint requests to Department of Pathology, Yale University School of Medicine, 310 Cedar St, New Haven, CT 06510.
MD
SUBJECTS AND METHODS Six patients with osteosarcoma of the maxilla, whose conditions were diagnosed and treated at the Yale-New Haven Hospital from 1955 to 1975, form the basis of this study. The clinical charts and all pathologic materials were reviewed. Fol¬ low-up data were obtained in all but one instance.
(osteosarco-
arise in the maxilla. Small series11 and individual case reports' " have described the clinical and pathological features of these lesions; most studies indicate a poor
Clinical
Maxilla
Findings
Age and Sex.—All six patients
were male whose ages at diagnosis ranged from 10 to 75 years (Table). Symptoms.—A mass in the cheek or maxillary area brought three of the six patients to medical attention. Loose teeth or dentures were noted by two of these three persons; loose dentures was the only symptom for another patient. The two remaining persons complained of epistaxis or bloody nasal discharge; one of these also experienced diplopia. Duration of symp¬ toms ranged from two weeks to six months. History.—Three of the six patients had notable preexisting conditions. One man recalled trauma to the cheek region 40 years before he developed the osteosar¬ coma; the trauma of an explosion had led to visual disturbances for most of his life. A second patient had received radiation therapy for retinoblastoma seven and five years before he developed osteosarcoma. Another person had a 30-year history of Paget's disease that involved the humérus, vertebrae, and skull bones.
subjects,
Radiologie Studies In four cases, sinus and facial radio¬ graphs demonstrated a mass that was destroying the boundaries of the maxilla. In three of these cases, sclerosis or osteo-
led to a differential diagnosis that included osteosarcoma. A benign osteogen¬ ic lesion was considered in a fifth case. The patient with Paget's disease showed a progressive change in the facial bones, but a definitive diagnosis of a superimposed neoplastic process was not made.
genesis
Pathology Gross.—The lesions occupied the entire maxilla and were associated with a scle¬ rotic process that extended into surround¬ ing soft tissues. Four tumors occurred primarily on the left side; two tumors occurred on the right side. In each patient, extension of the lesion was noted: to the ethmoid sinus in two cases, to nasopharynx in one case, and to the opposite antrum in five cases.
Microscopic—Osteogenic
sarcoma
was
identified in each case. The degree of differentiation varied (Fig 1 and 2). Osteoid formation and calcification ranged from minute to massive. Zones of neoplas¬ tic cartilage were recognized in one tumor. The proliferating cells contained markedly hyperchromatic nuclei; mitoses were easily found. In three instances, invasion into the antral mucosa was seen. Destruction of normal, preexisting bone was noted in those cases in which adequate material was resected. In one patient (case 5), histologie Paget's disease was identified at autopsy in the humérus. Destruction of the facial and cranial bones by tumor precluded histologie documentation of Paget's disease in this area.
Sites of Metastasis In four patients who died, the lungs and several bones (clavicles, humérus, ribs, and pelvic bones) were the sites involved by
Downloaded From: http://archotol.jamanetwork.com/ by a University of Arizona Health Sciences Library User on 05/25/2015
Clinical
Findings
Case
Age, yr/ Sex 1/63/M
Symptoms/ Duration
Swelling
on
Predisposing Factor
right
Extensive destruc¬ tion of right an-
None
side of face with
proptosis/6 2/48/M
Maxillary
Partial excision
None
3/56/M
Loosened den¬ tures/several wk
Injury 40
4/10/M
Bloody nasal discharge/4
Radiation therapy for retinoblastoma
yr before osteosarcoma
diagnosis
Wide excision
Sclerosing
Maxillectomy (margins of excision involved) Left maxillectomy
probably benign Left maxillary mass with invasion of ethmoid sinus
Follow-up Slight response; prompt recurrence;
Mass occupying maxilla with inva¬ sive edges process left maxilla—
6,000 rads
radiotherapy
by bone-pro¬ ducing mass
loose teeth/2 wk
mo
Postoperative Therapy
Therapy
trum
mo
mass,
Radiographie Findings
only
died of disease after 1 yr Died of disease af¬
7,500 rads
radiotherapy;
ter 15
mo
methotrexate
and ethmoldectomy
Lost to follow up
Chemotherapy— doxorubicin
hydrochloride,
Died of disease after 3 yr
dacarbazine,
methotrexate
5/75/M
Epistasis, diplopia/ Polyostotic Paget's 3 wk
disease 30 yr before osteosar¬ coma
6/32/M
Swelling on right side of face; loosened den¬ tures/3 wk
None
diagnosis
Sclerotic bone and soft tissue density in left ethmoid and
spheroid areas Ossifying lesion of maxilla, irregular edges; probably malignant growth
Biopsy performed
Radiotherapy;
me¬
thotrexate treat¬
Died of disease after 5 mo
ment
Right maxillectomy; partial left lectomy
maxil¬
7,000 rads radiother¬ Died of disease apy
Fig 1 —Moderately differentiated osteosarcoma with obvious bone formation. Note hyperchromatic malignant cells in stroma (case 4) (hematoxylin-eosin, 250).
Downloaded From: http://archotol.jamanetwork.com/ by a University of Arizona Health Sciences Library User on 05/25/2015
after 15
mo
Fig 2.—Left, Case 5. Osteosarcoma encroaching on antral mucosa (shown in on right) (hematoxylin-eosin; left, 250; right, 450). without identifiable preexisting le¬ metastatic deposits. Each of these patients patients had received radiotherapy on also showed local recurrence. Autopsy in sion.1" Trauma has been noted to be the facial area seven to 42 years the fifth patient demonstrated massive associated with bone before developing maxillary osteogen¬ frequently long invasion of the cerebral hemisphere ipsilat¬ ic sarcoma: two of nine patients who tumors, but this may be a reflection of eral to the tumor; however, no distant the fact that trauma draws attention were described by Fu and Perzin,2 métastases were identified. three described by Kragh et al,3 and to the lesion, rather than the indica¬ tion of a causal association. Several one patient each who was described by Therapy conditions of bone have been asso¬ Caron et al,1 Yannopoulos et al,9 Arlen Biopsies were performed on all lesions. ciated with the et al,5 and Sim et al." One subject in subsequent develop¬ Following the diagnosis of osteosarcoma, the present series also gave a history ment of osteosarcoma. These include further surgical excision was attempted in radiation,51--1' Paget's disease,1"15 of radiotherapy (case 4), bringing the four instances: radical maxillectomy (three bone infarcts,11 and fibrous dyspla¬ total to 14 patients who had prior cases), and wide excision (one case). Radio¬ radiation therapy. sia.1"1"17 therapy was administered to four persons Reasons for radiation therapy for In the maxilla, osteosarcomas have (as primary therapy in one case and post¬ operatively in three cases). Adjuvant been reported as having arisen in these 14 patients included treatment chemotherapy was given to three patients; association with prior radia¬ of retinoblastoma (five cases),2·1 fi¬ this included methotrexate alone in two brous dysplasia (four cases),2"1214 "be¬ fibrous dysplasia,1-2-912-14 tion,--5"15" cases, and combination therapy of doxoruand Paget's disease.1·7 A review of osseous growth" (one case),5 nign bicin hydrochloride (Adriamycin), dacarbathese factors in preexisting maxillary squamous cell carcinoma (one case),4 zine (DTIC-Dome), and methotrexate in follows. sarcomas spindle cell tumor (one case),11 and two osteogenic one case. detail
Of the five subjects who were followed up, all died of recurrent and metastatic disease.
COMMENT
Most osteosarcomas arise
anew
Radiation
Therapy
Prior radiation therapy constitutes the most commonly encountered fac¬ tor. Thus, of the recorded cases of osteosarcoma of the maxilla, 13
cases
for which the
reasons
for treat¬
ment is unknown.3
Dysplasia patients gave a history of Fibrous
Five fibrous
dysplasia,1·2·*·18·"
Downloaded From: http://archotol.jamanetwork.com/ by a University of Arizona Health Sciences Library User on 05/25/2015
and
one
pa-
tient had had a "benign osseous growth".5 Of these, five patients had received radiation therapy prior to developing osteosarcoma; the other patient1 had not.
Paget's
Rarely has
Disease
osteosarcoma of the
maxilla been associated with Paget's disease. Caron et al1 cited two cases, and Karpawich7 described one in¬ stance of such an association. Patient 5 in the present report also had a definite history of Paget's disease. Trauma A definite history of trauma pre¬ ceded the discovery of the osseous lesion by three years in one reported case," and by 40 years in case 3 of this
series.
Osteogenic sarcomas of the maxilla swelling that is often followed by pain, dental, and neuro¬ logic symptoms. The majority of patients have no notable prior history appear with
of bone disease.1-2-4 Whereas osteosarcoma of the long bones tends to occur in patients in the second decade,1" the age of most patients with osteosarcoma of the maxillae is third to fourth decade.12-4 Men are affected more frequently in
all types of osteosarcoma,10 although Dehner18 recorded the case of a 15year-old girl with maxillary osteosar¬
tion appears to be less a
important from prognostic standpoint than does the
extent of the tumor at initial
diagno¬
coma.
sis.
Radiological findings need not be diagnostic; most patients show bone destruction and an expansile growth that is often accompanied by soft
Therapy for these tumors remains primarily radical surgery. However,
tissue extension. Periosteal new-bone formation is rarely found. A malig¬ nant lesion is suspected often. How¬ ever, various appearances have been described that depend on the relative osteolytic and osteoblastic nature of the tumor.'-1'-18 Pathologically, the tumors grossly have been described as firm, fibrous, gritty, and occasionally, calcified or ossified.1-2-4 When maxillectomy spec¬ imens have been available, the lesions usually erode and extend through the preexisting bone.2 Results of histological examination also disclose a variety of patterns that depend on the degree of differentia¬ tion of the lesion. Hence, areas that are composed of malignant mesenchymal cells with foci of cartilage and osteoid formation are described.1-2-4·8 However, the amount of osteoid mate¬ rial may vary from lesion to lesion and from zone to zone in the same neoplasm.2 The degree of differentia-
because of the location, and often the extent of the neoplasm, total surgical extirpation is rarely feasible. Radio¬ therapy and, more recently, chemo¬ therapy have been tried; experience with these modalities is limited for maxillary osteosarcomas, but they appear to offer little palliation at best. The overall survival of patients with osteosarcomas has been recorded as 17%.'" Prognosis for patients with jaw bone lesions has been described as better than for those patients who have tumors that occur in long bones; this has been attributed to better differentiation of the jaw osteosarco¬ mas. Hence, Caron et al' reported a 33% survival for such patients. How¬ ever, Fu and Perzin2 had an 11% fiveyear survival in their series, and Kragh et al5 recorded a 19% diseasefree rate. The absence of any longterm survivor in the present series correlates more closely with the latter two
reports.
References AS, Hajdu SI, Strong EW: Osteogenic of the facial and cranial bones. Am J
1. Caron sarcoma
Surg 122:719-725,
1971.
YS, Perzin KH: Nonepithelial tumors of the nasal cavity, paranasal sinuses and nasophar2. Fu
clinicopathologic study: II. Osseous and including osteoma, fibrous dysplasia, ossifying fibroma, osteoblastoma, giant cell tumor and osteosarcoma. Cancer
ynx: A
fibro-osseous lesions,
33:1289-1305, 1974. 3. Kragh LV, Dahlin DC, Erich JB: Osteogenic sarcoma
of the jaws and facial bones. Am J Surg
96:496-505, 1958. 4. Roca AN, Smith JL, Jing BS: Osteosarcoma and parosteal osteogenic sarcoma of the maxilla and mandible. Am J Clin Pathol 54:625-636, 1970. 5. Arlen M, Shah IC, Higinbotham N, et al: Osteogenic sarcoma of head and neck induced by radiation therapy. NY State J Med 172:929-934, 1972.
6. Curtis ML, Elmore JS, Sotercanos GC: Osteosarcoma of the jaws. J Oral Surg 32:125\x=req-\
130, 1974.
7. Karpawich AJ: Paget's disease with osteogenic sarcoma of maxilla. Oral Surgery
11:827-834, 1958. 8. Madhaven M, Aurora AL, Sen SB: Osteogen-
ic sarcoma of the maxilla. J Laryngol Oto 88:1125\x=req-\
1129, 1974.
9. Yannopoulos K, Born AF, Griffiths CO, et al: Osteosarcoma arising in fibrous dysplasia of the facial bones. Am J Surg 107:556-564, 1964. 10. Spjut HJ, Dorfman HD, Fechner RE, et al: Tumors of Bone and Cartilage. Atlas of Tumor Pathology, series 2. Armed Forces Institute of Pathology, 1970, pt 5,pp 141-162. 11. Mirra JM, Bullough PG, Marcove RC, et al: Malignant fibrous histiocytoma and osteosarcoma in association with bone infarcts. J Bone Joint Surg 56-A:932-940, 1974. 12. Sabaras AO, Dahlin DC, Childs DS, et al:
Postradiation sarcoma of bone. Cancer 9:528-542, 1956. 13. Arlen M, Higinbotham NL, Huvos AG, et al: Radiation-induced sarcoma of bone. Cancer 28:1087-1099, 1971. 14. Sim FH, Cupps RE, Dahlin DC, et al: Postradiation sarcoma of bone. J Bone Joint Surg 54-A:1479-1489, 1972. 15. Porretta CA, Dahlin DC, Jones JM: Sarcoma in Paget's disease of bone. J Bone Joint Surg 39-A:1314-1329, 1957. 16. Huvos AG, Higinbotham NL, Miller TR: Bone sarcomas arising in fibrous dysplasia. J Bone Joint Surg 54-A:1047-1056, 1972. 17. Townsend GL, DeSanto LW: Malignant change in sphenoid sinus fibrous dysplasia. Arch Otolaryngol 92:267-271, 1970. 18. Dehner LP: Tumors of the mandible and maxilla in children. Cancer 32:112-120, 1973.
Downloaded From: http://archotol.jamanetwork.com/ by a University of Arizona Health Sciences Library User on 05/25/2015
