Oral SURGERY Oral MEDICINE AND&d
VOLUME
22
NUMBER
1
JULY,
PATHOLOGY
1966
Operative oral surgery Solitary myeloma of the mandible ffmvy E. Webb, M.D., Kentzeth D. Deline, M.D., (rj~d Edgar G. Harrison, Jr., X.D., Rochester, .lfin )I. SECTIONS t\\ND
3IAYO
OF PLASTIC
SURGERY
>\ND
SUR(:I~‘AL
P.\THOI,O(IY,
MAYO
CI,ISi(’
FOUNDATIOS
S
olitary my4oma of the mandible is a rat-c lesion. h scxarch of tlic rmdical literature disclosed nine cases accorded this diagnosis, and in thcsc reports fatlow-up information was rarely given. Two of the cascsl~ ? werci rcportrtl from the Mayo Clinic and will be reviewed in detail, particularly with rcgartl to thl> rvcntual development of multiple myeloma. Since so few cases of solitary m;veloma of the mandible have been reportc>tl. the chara.ct,erist,ics of solitary osseous myelonia:‘-” in general will be prexcntclct and compared, in so far as possible, with those of thcl mandibular lesion. SOLITARY OSSEOUS Age and sex
MYELOMA
This disease is discovered most freyuentl,v in patients in the fifth througlt the seventh decades of lift, with t,he peak incidcncc during the sixth deea&,. However, a few cases of solitary osseous mycloma have been reported in patients less than 30 years of age. Men are affected more commonl,v than womctl, in a ratio of three to one. Site
The bones most often affected, in order of decreasing ilium, femur, humerus, thorn& vertebrae, and skull.
frequenc~y, ilr(’ the> 1
2
O.S., O.M. & O.P. July, 1966
Webb, Defjine, and Harrison
Symptoms
The most common symptom is a palpable tumor with a history of progressively increasing pain. Fracture of the involved bone may draw the physician’s attention to the lesion. Roentgenologic
characteristics
Two different roentgenologic characteristics have been described.” One consists of a sharply demarcated osteolytic multicystic lesion located in the medulla of the bone. The cortex may or may not appear expanded. The multicystic appearance is due to thick trabeculae which cross the lesion irregularly. The other characteristic is chiefly a destructive process without sharply demarcated margins or trabeculations. Although unusual, expansion of the cortex of the involved bone is noted occasionally. Biochemical
characteristics
A patient with solitary osseous myeloma may have an abnormal electrophoretic pattern of proteinuria or proteinemia. Nassim and Crawford’ described a patient with a solitary myeloma of the sacrum (verified at necropsy) and proteinuria. Electrophoretic analysis of the proteinaceous material yielded three separate components, two of which were within the gamma-globulin range. Lane8 reported a case of solitary myeloma of the mandible in which preoperative and postoperative electrophoretic analyses of the blood serum revealed a significant decrease in the protein within the gamma-globulin range 2 months after excision of the affected hemimandible. On histochemical examination of the tumor tissue obtained from solitary osseous myelomas, Dahlin and Dockertyg found amyloid material associated with the tumor cells. This material was noted both intracellularly and extracellularly. Sonnet and associates’” cultured myeloma cells (obtained from a patient with multiple myeloma) in a medium containing glucose-Ii31. The P component subsequently was found within paraproteins located in the supernatant fluid of the culture medium. Bence Jones proteinuria has rarely been reported in solitary osseous myeloma.ll DEVELOPMENT
OF MULTIPLE
MYELOMA
IN SOLITARY
OSSEOUS MYELOMA
In 1950 Christopherson and MillerI’ stated that if a solitary myeloma of bone remained localized for a period of 3 years, multiple myeloma rarely ensued. Naylor and Chester-Williams,‘” however, reported the case of a patient who had solitary myeloma for 7 years and then manifested multiple myeloma. Carson and associates3 stated that solitary myeloma of bone may exist, although it must be extremely rare if patients so diagnosed are intermittently re-examined for multiple myeloma. Moreover, these authors reported cases in which multiple myeloma developed more than 6 years after solitary osseous myeloma had been diagnosed. Gootnick5 reported the occurrence of disseminated myeloma more than 9 years after the diagnosis of solitary myeloma. Tennent14 described a case
~olumr 22 Number 1
of solitary myeloma of the ilium in which the patient refused treatmclnt. Although it is noteworthy that this lesion remained localized for ‘llbl years, the patient died of multiple myeloma 5 years after the solitary myrloma was diagnosed. Yentis” described two cases in which disseminated myeloma developed X and 12 years after the diagnosis of so1ita.q myeloma was made. Recently, Kaye and associates’” reported two cases in which the patients had solitary myeloma for 10 and 11 years before multiple myeloma became manifest. (lohen ilnd associatesll recently reported a case in which there was dissemination ot’ thca tumor %I years after treatment of a solitary osseous mpclorna. The p;ltient was alive at the time of their report. CRITERIA FOR THE DIAGNOSIS
OF SOLITARY
MYELOMA
Willis’” recommended that pat.ients with solitary osseous myeloma be cxamined carefully and completely in order t,hat the existence of any cryptic mult,iple myeloma might be discovered. He suggested a complete roentgenologic survey of the skeletal system as well as examina.tion of the bone marrow for myeloma cells, the blood serum protein for hpperproteinemia, and the urine for Bence Jones proteinuria. The patient also should be rr-examined at. snbsequent intervals. The salient characteristics of seven cases of reported solitary myeloma of the mandible reported in the literature are listed in Table 1. The two cases reported previously from the Mayo Clinic are presented here in more detail. CASE REPORTS CASE
1
A 59.year-old woman came to this Clinic on Jan. 8, 1952. She stated that she had not,icrd a sensat,ion of pressure in the right lower jaw during the spring of 1951. Subsequently, a tooth in the right lower jaw had loosened and was removed. She related that she had felt well during the summer, until numbness developed in the jaw after she had bitten down on a hard substance while eating. Xoentgenographic examination suggested the presencr of a tumor in the angle and ascending ramus of the right lower jaw. A biopsy specimen of this lesion was removed by a dental surgeon in the patient’s home community and sent to the Mayo Clinic, where the tissue was considered to be representative of plasma,-c>ell my&ma. The patient was then treated by external irradiation. Examination of the oral cavity disclosed no swelling or inflammation in the region of the tumor, but there was slight tenderness of the tissue overlying t,he lesion. R,oentgenologic: examination of the skull, spinal column, ribs, and pelvis showed no evidence of multiple nlyeloma. Blood serum protein values were within the normal range, and no Rencr Jones protein was present. The results of a sternal bone marrow examination were negativr for my&ma W?llS. On Jan. 22, 1952, biopsy of the lesion of the right mandible revealed plasma-cell myeloma. A right partial mandibuleetomy was performed. On examination of the gross specimen, a fracture was found at the angle of the mandible. Subsequently, wit,hin 18 months following surgical removal of the mandible, the patient died of mult,iplr myeloma. This was som~~whai more than 2 years after the onset of symptoms. CASE 2
right
A 56.year-old woman came to this Clinic on Aug. 6, 1953, because of a swelling of the side of the lower jaw which, she stated, had been present, since November, 1952. She
Table
I. Cases of solitary
Reference / Sez 1 Age M 39 Hpitzer and Pricel’
myeloma of the mandible reported in the literature
Therapy 1 %zo”,f 1 Symptoms 1 I Left body k’ersistent swelling Curettage of leof mandible and great pain sion, followed by in left lower jaw external irradiation Persistent swelling and pain after extraction of left molar tooth
Excision of left half of mandible
Course
At last report patient was alive with no evidence of recurrence of lesion 35 years after removal
Christopherson and Millerl”
F
29
Posterior body and ramus of left jaw
Lanes
M
30
Left body of mandible
MancusiMaterils
M
45
Right angle of mandible
Hinds and associataslg
F
18
Right DOS- Aching pain and swell&i of gint&ior‘body of mandible giva about lower right first molar, with progressive increase of tumor size during ensuing months
Whitlock and Hughes20
M
67
From right ramus to left molar
Swelling of right Intraoral curetNo evidence of jaw gradually in- tage 12 and 8 residual discreasing over years before this ease 5 months period of 16 report; on exafter last years until flueternal approach, surgical protuant swelling of outer cortex of cedure tennis ball size mandible was reat point of chin moved and all cavities were carefully curetted
McCall and Bailey21
M
58
Right lower alveolar ridge
Lump in lower jaw of 1 month’s duration
Segmental resection of involved mandible Slow constant increase of pain with appearance of enlarging tumor
Excision of tumor, with resection of contiguous bone
Curettage of tumor five times, followed by right hemimandibulectomy ; external roentgen irradiation 2 months before hemimandibulectomy
Excision of adjacent margin of mandible with surrounding soft tissue; 6 months later, removal of residual tumor, right ramus of mandible! with suprahyold _.
No evidence of residual tumor 18 months after hemimandibulect,omy
No evidence of residual tumor more than 2 years after mandibulectomy and suprahyoid resection
claimed that she had not noticed any appreciable change in the size of the mandibular mass during the previous 10 months. Examination revealed a mass bulging the posterior portion of the horizontal ramus anti in tllc, post(xriol angle of the right mandible. A firm node, 1.5 cm. in tfiametcr, was prrwnt region of the right submaxillary triangle. Koentgenograms of the mandible were interpreted as showing a large multilocular h&on t,hat involved the horizontal and ascending ramus and the czoronoid process. The roots of the right, lower first molar xvere destroyed. Roentgenograms of the ribs, spinal column, pelvis, extremities, and skull mere judged negative for myt,loma. There was no TSenw JOWS pro tcxinuria. On Aug. 8, 1953, the lesion of the right mandible was thoroughly wrrtted and tlw rt’moved tissue was reported to represent plasma-cell myeloma. Two days later, three radium element tubes (each containing 50 mg. of radium) wwe placwl in thtl surgically formc~l eavit,y for 7 hours. Cth no clinical widenw of c~it,ht~l This patient is still alive 9 years after treatment, rwurwnw of the primary lesion or multiple myeloma.
COMMENT AND CONCLUSIONS Since only nine cases of solitary myeloma of t,hc mandible have been rqortetl in the medical literature, any conclusions would he unwarrant,cd. The avcrag+* age of these paCents was between 40 and 50 years, and the group consisted o! five men a,nd four women. The majority of the tumors w&e located in the. posterior portion of the mandible, often at the angle. The usual presenting symptoms of these patients wcrc pain and a mass. ‘\+‘k> believe that the possibility of solitary myeloma must be considered in patients with these symptoms in order that proper therapy may be given. Involvement of regional lymph nodes was not, found in patients wit,11 solitaq, tllmor of the mandible. In only one case, (Case 2) was ment,ion made of ;I. clinically involved lymph node, and sections for histologic study WCPVnot obtained. However, this lymph node was within the region irradiated by th(b radium capsules. There has been no recurrence of the lesion. The most interesting facet of this disease has been the decrease in the gammaglobulin range of electrophoretic pattern after removal of the t,umor and the manifestation of abnormal proteins in patients with solitary tumors. These findings, together with histologic evidence, 9 indicate that the abnormal pwt,cJins are produced by the tumor cells. This thesis was given further support, by tl:c dernonstrabion of intracellular and extracellular amytoid deposits which. togcthc>r wit,11 the utilization of tagged P glucose by mycloma cells in culture t,o protluc~c ta.gged I’“’ protein deposits,l” affords collaborative cvidcnce of functioning tumor cells. In the future, careful biochemical studies of patients with solitary osseo(1s myeloma may form the basis for t,he more knowledgeable preoperative diagnosis of this disease. Follow-up data on these patients are scanty. Howeve~~~on the basis of ~Vhat,is known about osseous myelomas in general, one must consider solitary mp4onr:r of the mandible as a malignant neoplasm ca,pable of widespread disseminati~~n many years following treatment of the solitary lesion. WC also susptbc’t that careful follow-up examination will reveal more casts of disseminated myclol~~a among patients with solit,ary om;seous myeloma of the rnandi ble. Sol it,nry myclonia may or may not b(l considrrc4 among the difft~rcnl r;rl
6
Webb, Devine,
and Ha.rrison
O.S., O.M. & O.P. July, 1966
diagnostic possibilities after roentgenograms of the affected mandible have been examined. It is in cases in which a roentgenogram arouses suspicion that the frozen-section technique of histologic examination is of most help to the surgeon. Moreover, when the correct diagnosis is made during the primary surgical procedure, the patient has the benefit of immediate and adequate surgical resection. The treatment of these tumors may involve either surgical resection of the afleeted portion of the mandible or irradiation. We believe that if surgical treatment is limited to curettage, then some type of irradiation should also be administered. No evidence of the superiority of either surgical therapy or irradiation has evolved. We believe that these treatment modalities should be complementary rather than mutually exclusive. REFERENCES 1. Davis,
2. 3. 4. 5. 6. 7. 8. 9. 10. 11. 12. 13. 14. 15. 16. 17. 18. 19. 20. 21.
G. D., and Havens, F. 2.: Plasma-Cell Myeloma of the Mandible, Proc. Staff Meet. Mayo Clin. 29: 569-571, 1954. Bruce, K. W., and Royer, R. Q.: Multiple Myeloma Occurring in the Jaws: B Study of 17 Cases, ORAL @JR&, ORAL MED. & ORAL PATH. 6: 729-743, 1953. Carson, C. P., Ackerman, L. V., and Maltby, J. D.: Plasma Cell Myeloma, Am. J. Clin. Path. 25: 849884, 1955. The So-Called Solitary Plasmacytoma of Bone, J. Fat. R.adiologists 8: Yentis, Irvan: 132-143, 1956. Review of Sixty-one Cases, Radiology 45,: 385390, Gootnick, L. T.: Solitary Myeloma: 1945. Paul, L. W., and Pohle, E. A.: Solitary Myeloma of Bone: A Review of the Roentgenologic Features, With Report of Four Additional Cases, Radiology 35: 651-665, 1940. Myeloma With Generalized Metabolic DisNsssim, J. R., and Crawford, T.: Solitary turbance, Brit. J. Surg. 37: 287-291, 1950. Lane, 5. L.: Plasmacytoma of the Mandible, ORAL SURG., ORAL MED. & ORAL PATH. 5: 434442, 1952. and Myeloma, Am. J. Path. 26: 581586, Dahlin, D. C., and Dockerty, M. B.: Amyloid 1950. Sonnet, J., Gillant, C., and Sokal, G.: Demonstration de la synthese de paraproteines seriques par des plasmacytes, grace au glucose uniformement marque par le 04, Rev. belge path. et med. exper. 26: 313-323, 1957-1958. Cohen, D. M., Svien, H. J., and Dahlin, D. C.: Long-Term Survival of Patients With Myeloma of the Vertebral Column, J. A. M. A. 187: 914-917, 1964. Christopherson, W. M., and Miller, A. J. : A Re-evaluation of Solitary Plasma-Cell Myeloma of Bone, Cancer 3: 240-250, 1950. Naylor, A., and Chester-Williams, F. E.: Myelomata of Bone: A Review of 25 Cases, Brit. M. J. 1: 120-124, 1954. Tennent, William: Plasmacytoma of Bone, Brit. J. Surg. 32: 471-476, 1945. Kaye, R. L., Martin, W. J., Campbell, D. C., and Lipscomb, P. R.: Long Survival in Disseminated Myeloma With Onset as Solitary Lesion: Two Cases, Ann. Int. Med. 54: 535544, 1961. Willis, R. A.: Solitary Plasmacytoma of Bone, J. Path. & Bact. 53: 77-84, 1941. Spitzer, Richard, and Price, L. W.: Solitary Myeloma of Mandible, Brit. ,M. J. 1: 1027. 1028, 1948. Mancusi-Mater-i, Giuseppe: Considerazoni su un case di plasmacitoma solitario atipico della mandibola, Gior. ital. chir. 10: 274-282, 1954. Plasma-Cell Myeloma of the Hinds-.‘. E. C., Pleasants, J. E.? and Bell, W. E.: Solitary ._ Mandible. ORAL SURG.. ORAL MED. & ORAT, PATH. 9: 193-202. 1956. Whitlock; R. I. H., and Hughes, N. C.: Solitary Myeloma of Mandible, ORAL SURG., ORAL MED. & ORAL PATH. 13: 23-33, 1960. McCall, J. W., and Bailey, C. H., Jr.: Extramedullary Plasmacytoma of the Upper Air Passage, Ann. Otol. Rhin. & Laryng. 69: 906-915, 1960.