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Osteogenic sarcoma of the mandible
Northwestern OSTEOGENIC
SARCOMA A
FREDERICK
University
W.
CASE
OF THE MANDIBTJE REPORT
MERRIFIELD,
D.D.S., M.D.
T
HE bone structure of the body is affected by injury, infection, nutrition, and new growths. The bones of the jaws are no exception, but they show a tendency to resist disease better than other bones. Osteomyelitis of the mandible is rarely the intractable type of disease that it may be elsewhere. Osteogenic sarcoma of the mandible, the subject of this report, is a rare disease compared to its incidence in other bones, notably the tibia, femur, and pelvis. Ewing, Codman, Geschickter and Copeland, Waldron, and others have devoted themselves to the problem of the classification of bone tumors and have made great progress in this regard. The difficulties were well expressed by Homan. “Since bone, which is mesoblastic in origin, is perhaps the most highly finished product of the primitive fibroblast, it is not surprising that the tissue of its tumors would vary from the most primitive to the most differentiated, and that, according to the general rule, new growths of bone should be, on the one hand, remarkbly malignant and on the other, almost completely benign. There is, moreover, a most confusing variety of appearances in any one tumor. Thus a classification of these tumors is difficult.” All authorities are in agreement that true tumors of jawbones, per se, are rare. Padgett emphasizes the relationship between normal bone development and tumor formation and gives reasons based upon this observation for the scarcity of true bone tumors in the bones of the jaw. All bone components are derived from preformed connective tissue. In nonmembranous bones, fetal cartilage cells give rise to adult cartilage cells, and this adult cartilage is ossified by a gradual process and true bone is formed. In the early stages of either variety of bone formation, new growth is unlikely to occur. It is in bone formed from cartilage, in which the transition steps are delayed over a Ionger period of development, that tumor formation in bone is likely to occur. Specifically, the reasons for the variety of tumors of the jawbones proper, based upon the foregoing observations, are that the bones of the jaws are preThe areas of cartilagenous origin in the mandible, dominately membranous. with one exception, pass through the cellular transit,ion completely and are calcified by the one of the first year. The exception is the epiphysis of the condyle which becomes calcified at about the fifteenth year. Osteogenic sarcoma is most likely to appear in the second decade of life, in otherwise strong and healthy young persons when intensive development of bone occurs. The
Department
of Oral
Surgery,
Northwestern 389
University
Dental
School,
Chicago,
Ill.
290
Ii're.derickW. Merrifietd
Trauma, as an exciting factor in the disease, has been the observation of most authors. Kolodny believes that 70 per cent of patients over 10 years of age have such a history. Ewing, however, analyzes the etiological factor of trauma and draws particular attention to the proliferative activity of fibroblasts, osteoblasts, and endothelium which can be separated from sarcoma only with difficulty, noted in the callus of healing bone injuries. In this connection, we have knowledge of tissue from a subperiosteal swelling of the mandible, following extraction of an infected tooth, reported on frozen section as osteogenic sarcoma. The jaw was resected, and a later report on the tissue removed at operation was: “Inflammatory Ewing is of the opinion that while tissue.” all varieties of sarcoma have been attributed to trauma, injury is only one of the essential factors. He believes that “many or most cases” fail to give a history of trauma, that many tumors arise in bones prot,ected from injury, and that occasionally bone sarcomas are multiple. The mandible is probably the most frequently injured of the facial bones, but osteogenic sarcoma occurs less frequently here than in the maxilla by the proportion of one to two, and Christensen’s classification of one thousand bone tumors includes eleven osteogenic tumors in the maxilla and only seven in the mandible. The clinical diagnosis of osteogenic sarcoma may be difficult. Persistent, increasing, and unexplained pain is the earliest symptom and one which may be overlooked. The aching, boring character of the pain, which is worse at night, is similar to the onset of osteomyelitis of the jaw. In osteomyelitis, however, there is associated dental disease, which is usually absent in sarcoma. The symptoms may be present some time before relief is sought. Mr. R. E., the subject of this report, had teeth extracted for relief of “toothache.” The clinical findings vary greatly with the particular type of tumor. In this case, the facial contour was changed without skin invasion, but the growth was rather slow; the general condition of the patient was excellent and there was no evidence of lung invasion. There was submaxillary lymph node enlargement, but of inflammatory type. X-ray examination, as is usual, was somewhat confusing and films of diagnostic value were obtained only after repeated effort. The report on the first film was “osteomyelitis of mandible.” CASE
REPORT
History.-Mr. R. E. Until two years ago, the patient was well and healthy, at which time he noticed a pea-sized swelling on the gum of the lingual surface of the lower left cuspid. Prior to this (three to four months), the patient’s pipe (in his mouth) was hit by a log chain The pipe stem was driven against the labial surfaces of the incisors from a winch on a truck. and cuspid on the left side, driving them loose. These teeth straightened out spontaneously No other exciting factors were recalled. and apparently returned to normal. One month after the lesion was first noticed, it ruptured spontaneously, releasing a yelThis ulceration healed quickly. lowish thin fluid. At this time, there was no increase in size. From this time until April, 1943, the lesion gradually increased in size. In April, the patient had all of his lower teeth removed (against the advice of his dentist) because he said that There were apparently no comdecayed and pieces chipped off easily. ” they were “badly plications immediately following the extractions. Three to four weeks after the extraction, the swelling began to increase, becoming The lesion progressed slowly in size until the pavisible on the exterior of the left cheek. At the clinic, the patient states that a biopsy was taken and tient went to a dental clinic. The patient continued on at the clinic he was informed the diagnosis was “sarcomic tumor. ”
Osteogenic Sarcoma
of Mandible
291
for two months, refusing operation, which was advised. The jaw did not improve and sequestra of ‘ ‘ bonylike ’ ’ substance up to about 1 cm. in size began to be expelled from the lesion. It was uncomfortable, but not painful, at the time these pieces were extruded. The A moderate amount of bleeding patient has removed several pieces himself with tweezers. was the only consequence of each removal. The only discomfort or pain reported was a feeling occasionally as though <(a pin were sticking him.” This pain (‘is not severe, may occur at any time, and almost any place in the region of the jaw on the left. ” On Jan. 31, 1944, the patient was referred to the tumor clinic of Northwestern University Medical School. Examination.-A well-developed, well-nourished white man, aged 38 years, not acutely good general health, with nothing of any signifiill. The patient was found to be in fairly cance except in the mouth. Blood and urine examination3 were normal. Kahn and Waasermann reaction3 were negative.
Fig.
1.
Examination of the mouth revealed a diffuse, hard rind slightly tender mass involving the left side of the mandible, from the cuspid to the second molar areas. The gums showed marked hypertrophy and the continuity of the mucosa was broken in several places by craterlike ulcers with gritt,y bases and containing gritty material. The upper teeth were carious and covered with tartar on the left side. The lower teeth had been removed. The left side of the face was moderately enlarged, but the skin was unaffected. In the neck, the left upper anterior cervical nodes were palpable, rather soft, and freely movable. Smaller node3 were palpable on the right side. Report obtained on the original biopsy was verified. The diagnosis of “osteogenic sarcoma” was made. X-Ray Examination.-Jan. 24, 1944. “The flms of the left jaw region show a great amount of soft tissue swelling which was observed clinically. In addition, there is an enlargement of the body of the mandible on the left. The radiating new bone formation extends superiorly into the soft tissues of the alveolar surface giving this lesion a malignant aspect and an invasive character. New bone formation suggests osteogenic sarcoma. ” (Fig. I.)
bone were
‘( Impression: Malignant bone tumor, body of left mandible” Jan. 21, 1944. “Chest films showed a healthy chest with no or soft tissue structures” (Fig. 2) (A. F. Galloway). Jan. 31, 1944. “Re-examination of the jaw shows the same The opinion remains the observed on the previous Alm study. Impression: Pavors osteomyelitis rather than malignancy (Dr. Impression : Not convinced films indicate malignancy (Dr. ( ( B
Fig.
(A. F:,Galloway). evidence of “metastasis findings same” I I C”). ’ ‘) .
to
more clearly than (A. F. Galloway).
4.
Operalice Note.-The patient was operated upon on Feb. 17, 1944, under avertin anesthesia. The left side of the face was exposed. A’ midline incision was made in the inferior labium, carried to the inferior border of the mandible and then posteriorly to the angle of the jew. A flap was made by sharp disscetion and turned back. The jaw was cut through at the midline and also through the ramus abpre the angle, with a Gigli saw. The mass was freed from the floor of the mouth and removed. There was no undue hemorrhage. The facial artery and vein were the only vessels ligated except for the usual small bleeders. The intraoral mucous membrane was approximated where possible and the skin incision was closed with black silk. A .Penrose drain was inserted and a moderate pressure bandage applied. portion surface
Pathologic Report.-Gross Description: IL The gross specimen of the mandible, 9 cm. in length, together with surrounding is edentulous and has a nodular irregular appearance. Near
(Fig. 3) consists of a tissues. The mucosal the posterior end of the
294
Frederick
Fig.
5.
IV. Merrifield
(Magnification
X50.)
Osteogenic Sarcoma of Mandible
Fig.
6.
(Magnitlcation
X150.)
295
PredetickW. Merrifield
296
bone, there is a nodular round swelling 4$$ area, the appearance is that of a firm fibrous periosteum. The bone itself does not appear
by
4 cm. in diameter. tumor having a radial to be greatly affected.
On section structure
through this arising in the
Microscopic : “ Three blocks, six sections. The first block (Fig. 4) is partially covered with the uncornified stratified squamous epithelium of the mouth, showing marked elongation of the rete pegs. Lymphocytes and plasma cells are numerous in the subcutaneous tissue. The deeper portions are composed of tumor tissue, consisting of osteoid tissue containing spicules of newly formed bone (Fig. 6). Occasional masses of cartilage are present, some of which show myxomatous change. The basal portion is covered with granulation tissue and purulent exudate.
Fig.
7.
(Magnification
X400.)
(I The second block (Fig. 5) is also composed of osteoid tissue and myxomatous cartilage. Occasional areas of calcification are present in the osteoid tissue. Some voluntary muscle and fibrous tissue are present on the outer surface. The tumor appears to be well encapsulated at this point and does not invade beyond the capsule. “The
third follicles. is no evidence of
primary
block (Fig. 7) consists of a small lymph node The sinusoids are choked with small lymphocytes metastasis in this section.”
containing numerous and plasma cells.
small There
: “ Osteogenic: node. ”
I)iaynosia
gional
lymph Comment:
“There
s:tr~om:t
is no evidence
o t“ the
left
chronic
mandible;
of metastasis”
(D.
lymphadenitis
in
re-
0. Manshardt)).
Postoperatde CozLrse.-There was an elevation of temperature for six days to a maximum of 102.4” F. on the first postoperative day, and becoming normal on the seventh day. Some of the skin sutures and the rubber drain were removed on the fifth day. The remaining There was drainage from sutures were removed on the seventh and eleventh days, respectively. the external wound for about three weeks, and snrall bone splinters were extruded. Complete healing then took place.
Fig.
The patient reported for healed perfectly. The external from the prolonged drainage. operated side. This is unusual Function was good and he was back at his usual work. There
was
at this
time
S.
observation in January, 1945. The mucosa of the mouth had wound was healed, hut with a rather irregular scar, resulting Strangely enough, there was no deviation of the jaw to the in a resected, edentulous jaw (see Fig. 8). the
patient
no evidence
had
no complaint
of recurrence
relative
to his
ability
to eat,
and
or of metastasis.
REFEREXCES Ewing, Homans, Hertzler,
James : Neoplastic Diseases, ed. 3, Philadelphia, 1928, W. B. Saunders Co. John : Textbook of Surgery, ed. 3, Springfield, Ill., 1935, Charles C Thomas. Arthur E. : Surgical Pathology of the Mouth and Jaws, Philadelphia, 1938, J. B. Lippincott Co. Codman, E. A.: Diagnosis of Osteogenic Sarcoma, Surg., Gynec. & Obst. 42: 381, 1926. Geschickter, C. F., and Copeland, M. M.: Tumors of Bone, New York, 1931, The American Institute of Cancer. Thoma, Kurt H.: Oral Pathology, ed. 2, St. Louis, 1944, The C. 1’. Mosbv Co. Padgett, E. C.: Surgical Diseases of the Mouth and Jaws, Philadelphia, 1938, W. B. Saunders Co.
SARCOMA A
FREDERICK
University
W.
CASE
OF THE MANDIBTJE REPORT
MERRIFIELD,
D.D.S., M.D.
T
HE bone structure of the body is affected by injury, infection, nutrition, and new growths. The bones of the jaws are no exception, but they show a tendency to resist disease better than other bones. Osteomyelitis of the mandible is rarely the intractable type of disease that it may be elsewhere. Osteogenic sarcoma of the mandible, the subject of this report, is a rare disease compared to its incidence in other bones, notably the tibia, femur, and pelvis. Ewing, Codman, Geschickter and Copeland, Waldron, and others have devoted themselves to the problem of the classification of bone tumors and have made great progress in this regard. The difficulties were well expressed by Homan. “Since bone, which is mesoblastic in origin, is perhaps the most highly finished product of the primitive fibroblast, it is not surprising that the tissue of its tumors would vary from the most primitive to the most differentiated, and that, according to the general rule, new growths of bone should be, on the one hand, remarkbly malignant and on the other, almost completely benign. There is, moreover, a most confusing variety of appearances in any one tumor. Thus a classification of these tumors is difficult.” All authorities are in agreement that true tumors of jawbones, per se, are rare. Padgett emphasizes the relationship between normal bone development and tumor formation and gives reasons based upon this observation for the scarcity of true bone tumors in the bones of the jaw. All bone components are derived from preformed connective tissue. In nonmembranous bones, fetal cartilage cells give rise to adult cartilage cells, and this adult cartilage is ossified by a gradual process and true bone is formed. In the early stages of either variety of bone formation, new growth is unlikely to occur. It is in bone formed from cartilage, in which the transition steps are delayed over a Ionger period of development, that tumor formation in bone is likely to occur. Specifically, the reasons for the variety of tumors of the jawbones proper, based upon the foregoing observations, are that the bones of the jaws are preThe areas of cartilagenous origin in the mandible, dominately membranous. with one exception, pass through the cellular transit,ion completely and are calcified by the one of the first year. The exception is the epiphysis of the condyle which becomes calcified at about the fifteenth year. Osteogenic sarcoma is most likely to appear in the second decade of life, in otherwise strong and healthy young persons when intensive development of bone occurs. The
Department
of Oral
Surgery,
Northwestern 389
University
Dental
School,
Chicago,
Ill.
290
Ii're.derickW. Merrifietd
Trauma, as an exciting factor in the disease, has been the observation of most authors. Kolodny believes that 70 per cent of patients over 10 years of age have such a history. Ewing, however, analyzes the etiological factor of trauma and draws particular attention to the proliferative activity of fibroblasts, osteoblasts, and endothelium which can be separated from sarcoma only with difficulty, noted in the callus of healing bone injuries. In this connection, we have knowledge of tissue from a subperiosteal swelling of the mandible, following extraction of an infected tooth, reported on frozen section as osteogenic sarcoma. The jaw was resected, and a later report on the tissue removed at operation was: “Inflammatory Ewing is of the opinion that while tissue.” all varieties of sarcoma have been attributed to trauma, injury is only one of the essential factors. He believes that “many or most cases” fail to give a history of trauma, that many tumors arise in bones prot,ected from injury, and that occasionally bone sarcomas are multiple. The mandible is probably the most frequently injured of the facial bones, but osteogenic sarcoma occurs less frequently here than in the maxilla by the proportion of one to two, and Christensen’s classification of one thousand bone tumors includes eleven osteogenic tumors in the maxilla and only seven in the mandible. The clinical diagnosis of osteogenic sarcoma may be difficult. Persistent, increasing, and unexplained pain is the earliest symptom and one which may be overlooked. The aching, boring character of the pain, which is worse at night, is similar to the onset of osteomyelitis of the jaw. In osteomyelitis, however, there is associated dental disease, which is usually absent in sarcoma. The symptoms may be present some time before relief is sought. Mr. R. E., the subject of this report, had teeth extracted for relief of “toothache.” The clinical findings vary greatly with the particular type of tumor. In this case, the facial contour was changed without skin invasion, but the growth was rather slow; the general condition of the patient was excellent and there was no evidence of lung invasion. There was submaxillary lymph node enlargement, but of inflammatory type. X-ray examination, as is usual, was somewhat confusing and films of diagnostic value were obtained only after repeated effort. The report on the first film was “osteomyelitis of mandible.” CASE
REPORT
History.-Mr. R. E. Until two years ago, the patient was well and healthy, at which time he noticed a pea-sized swelling on the gum of the lingual surface of the lower left cuspid. Prior to this (three to four months), the patient’s pipe (in his mouth) was hit by a log chain The pipe stem was driven against the labial surfaces of the incisors from a winch on a truck. and cuspid on the left side, driving them loose. These teeth straightened out spontaneously No other exciting factors were recalled. and apparently returned to normal. One month after the lesion was first noticed, it ruptured spontaneously, releasing a yelThis ulceration healed quickly. lowish thin fluid. At this time, there was no increase in size. From this time until April, 1943, the lesion gradually increased in size. In April, the patient had all of his lower teeth removed (against the advice of his dentist) because he said that There were apparently no comdecayed and pieces chipped off easily. ” they were “badly plications immediately following the extractions. Three to four weeks after the extraction, the swelling began to increase, becoming The lesion progressed slowly in size until the pavisible on the exterior of the left cheek. At the clinic, the patient states that a biopsy was taken and tient went to a dental clinic. The patient continued on at the clinic he was informed the diagnosis was “sarcomic tumor. ”
Osteogenic Sarcoma
of Mandible
291
for two months, refusing operation, which was advised. The jaw did not improve and sequestra of ‘ ‘ bonylike ’ ’ substance up to about 1 cm. in size began to be expelled from the lesion. It was uncomfortable, but not painful, at the time these pieces were extruded. The A moderate amount of bleeding patient has removed several pieces himself with tweezers. was the only consequence of each removal. The only discomfort or pain reported was a feeling occasionally as though <(a pin were sticking him.” This pain (‘is not severe, may occur at any time, and almost any place in the region of the jaw on the left. ” On Jan. 31, 1944, the patient was referred to the tumor clinic of Northwestern University Medical School. Examination.-A well-developed, well-nourished white man, aged 38 years, not acutely good general health, with nothing of any signifiill. The patient was found to be in fairly cance except in the mouth. Blood and urine examination3 were normal. Kahn and Waasermann reaction3 were negative.
Fig.
1.
Examination of the mouth revealed a diffuse, hard rind slightly tender mass involving the left side of the mandible, from the cuspid to the second molar areas. The gums showed marked hypertrophy and the continuity of the mucosa was broken in several places by craterlike ulcers with gritt,y bases and containing gritty material. The upper teeth were carious and covered with tartar on the left side. The lower teeth had been removed. The left side of the face was moderately enlarged, but the skin was unaffected. In the neck, the left upper anterior cervical nodes were palpable, rather soft, and freely movable. Smaller node3 were palpable on the right side. Report obtained on the original biopsy was verified. The diagnosis of “osteogenic sarcoma” was made. X-Ray Examination.-Jan. 24, 1944. “The flms of the left jaw region show a great amount of soft tissue swelling which was observed clinically. In addition, there is an enlargement of the body of the mandible on the left. The radiating new bone formation extends superiorly into the soft tissues of the alveolar surface giving this lesion a malignant aspect and an invasive character. New bone formation suggests osteogenic sarcoma. ” (Fig. I.)
bone were
‘( Impression: Malignant bone tumor, body of left mandible” Jan. 21, 1944. “Chest films showed a healthy chest with no or soft tissue structures” (Fig. 2) (A. F. Galloway). Jan. 31, 1944. “Re-examination of the jaw shows the same The opinion remains the observed on the previous Alm study. Impression: Pavors osteomyelitis rather than malignancy (Dr. Impression : Not convinced films indicate malignancy (Dr. ( ( B
Fig.
(A. F:,Galloway). evidence of “metastasis findings same” I I C”). ’ ‘) .
to
more clearly than (A. F. Galloway).
4.
Operalice Note.-The patient was operated upon on Feb. 17, 1944, under avertin anesthesia. The left side of the face was exposed. A’ midline incision was made in the inferior labium, carried to the inferior border of the mandible and then posteriorly to the angle of the jew. A flap was made by sharp disscetion and turned back. The jaw was cut through at the midline and also through the ramus abpre the angle, with a Gigli saw. The mass was freed from the floor of the mouth and removed. There was no undue hemorrhage. The facial artery and vein were the only vessels ligated except for the usual small bleeders. The intraoral mucous membrane was approximated where possible and the skin incision was closed with black silk. A .Penrose drain was inserted and a moderate pressure bandage applied. portion surface
Pathologic Report.-Gross Description: IL The gross specimen of the mandible, 9 cm. in length, together with surrounding is edentulous and has a nodular irregular appearance. Near
(Fig. 3) consists of a tissues. The mucosal the posterior end of the
294
Frederick
Fig.
5.
IV. Merrifield
(Magnification
X50.)
Osteogenic Sarcoma of Mandible
Fig.
6.
(Magnitlcation
X150.)
295
PredetickW. Merrifield
296
bone, there is a nodular round swelling 4$$ area, the appearance is that of a firm fibrous periosteum. The bone itself does not appear
by
4 cm. in diameter. tumor having a radial to be greatly affected.
On section structure
through this arising in the
Microscopic : “ Three blocks, six sections. The first block (Fig. 4) is partially covered with the uncornified stratified squamous epithelium of the mouth, showing marked elongation of the rete pegs. Lymphocytes and plasma cells are numerous in the subcutaneous tissue. The deeper portions are composed of tumor tissue, consisting of osteoid tissue containing spicules of newly formed bone (Fig. 6). Occasional masses of cartilage are present, some of which show myxomatous change. The basal portion is covered with granulation tissue and purulent exudate.
Fig.
7.
(Magnification
X400.)
(I The second block (Fig. 5) is also composed of osteoid tissue and myxomatous cartilage. Occasional areas of calcification are present in the osteoid tissue. Some voluntary muscle and fibrous tissue are present on the outer surface. The tumor appears to be well encapsulated at this point and does not invade beyond the capsule. “The
third follicles. is no evidence of
primary
block (Fig. 7) consists of a small lymph node The sinusoids are choked with small lymphocytes metastasis in this section.”
containing numerous and plasma cells.
small There
: “ Osteogenic: node. ”
I)iaynosia
gional
lymph Comment:
“There
s:tr~om:t
is no evidence
o t“ the
left
chronic
mandible;
of metastasis”
(D.
lymphadenitis
in
re-
0. Manshardt)).
Postoperatde CozLrse.-There was an elevation of temperature for six days to a maximum of 102.4” F. on the first postoperative day, and becoming normal on the seventh day. Some of the skin sutures and the rubber drain were removed on the fifth day. The remaining There was drainage from sutures were removed on the seventh and eleventh days, respectively. the external wound for about three weeks, and snrall bone splinters were extruded. Complete healing then took place.
Fig.
The patient reported for healed perfectly. The external from the prolonged drainage. operated side. This is unusual Function was good and he was back at his usual work. There
was
at this
time
S.
observation in January, 1945. The mucosa of the mouth had wound was healed, hut with a rather irregular scar, resulting Strangely enough, there was no deviation of the jaw to the in a resected, edentulous jaw (see Fig. 8). the
patient
no evidence
had
no complaint
of recurrence
relative
to his
ability
to eat,
and
or of metastasis.
REFEREXCES Ewing, Homans, Hertzler,
James : Neoplastic Diseases, ed. 3, Philadelphia, 1928, W. B. Saunders Co. John : Textbook of Surgery, ed. 3, Springfield, Ill., 1935, Charles C Thomas. Arthur E. : Surgical Pathology of the Mouth and Jaws, Philadelphia, 1938, J. B. Lippincott Co. Codman, E. A.: Diagnosis of Osteogenic Sarcoma, Surg., Gynec. & Obst. 42: 381, 1926. Geschickter, C. F., and Copeland, M. M.: Tumors of Bone, New York, 1931, The American Institute of Cancer. Thoma, Kurt H.: Oral Pathology, ed. 2, St. Louis, 1944, The C. 1’. Mosbv Co. Padgett, E. C.: Surgical Diseases of the Mouth and Jaws, Philadelphia, 1938, W. B. Saunders Co.