Primary reticulum-cell sarcoma of the mandible Review
Robert L. Campbell,* Chapel Hill, N. C. UNIVERSITY
David E. Kelly,” CAROLINA
of a case
and E. Jefferson’ Burkes, Jr.,** OF DENTISTRY
A 52.year-old man developed intermittent swelling, pain, and paresthesia during a one-year period prior to the histologic diagnosis of reticulum-cell sarcoma. The clinical profile and radiographic examination suggested either chronic osteomyelitis or malignancy. Treatment was confined to radiation therapy with resolution of previously reported cases of the malignancy but a guarded prognosis. Twenty-two primary reticulum-cell sarcoma of the mandible are reviewed.
rimary reticulum-cell sarcoma of the mandible has been reported infrequently since classification of this malignancy in 1939.5r G An additional case and review of the American literature since that published by Gerry and Williams7 in 1955 is presented here. This case emphasizes the difficulty of establishing an early diagnosis, even after biopsy, and illustrates the extensiveness of the disease process and its duration without early metastasis. REVIEW
OF THE LITERATURE
Reticulum-cell sarcoma (RCS) of bone was first accepted by Ewing for inclusion in the Bone Sarcoma Registry of the American College of Surgeons in 1939.“9 F In that same year, Parker and JacksonI presented a study of seventeen cases of this disease entity, one of which was located in the mandible. Since that time, approximately twenty-two cases of primary RCS in the mandible have been reported in the American literature (Table I). In addition to these, Harrigan and associates9 reported a case which arose in the subcutaneous tissue of the chin and apparently invaded the mandible. * Assistant **Associate
of Oral Surgery. of Oral Pathology.
F M M M
68 9 22 19
Rteg, Dahlin, and Gores Hteg, Dahlin, and Gores Rtrg, Dahlin, and Gores Blake and Beck
Ivins Ivins Gerry Gerry
and and and and
Dahlin Dahlin Williams Williams’
Wang and Fleischli Wang and Fleischli Halperin and Williams Taylor, ,ilexander, and Kramer Shoji ant1 Miller
M M F
54 46 41
Melrose, Beveridge, and Abrams Wilson and Pugh
*Represents the same patient; survey by Gerry and Williams.
Parker and Jackson Szutus and Hsirh
Hody of mandible
Painless bump, 3 months R,ound, firm swelling, 3 K&us months painless swelling, Right and left body and Bilateral 4 months symphysis Swelling, inability to open, Body 6 weeks Painless swelling, 7 months R.amus and angle Painless swelling, 4 months Ramus and angle Painful swelling, 6 months Ramus Painless mass below inciR.ight body to left premolar sors, 6 months, mobility of teeth Painless swelling, anterior Right body to left premolar mandible, 6 months, mobility of teeth Pain, swelling, parest,hesia, Not stated duration unknown Pain, swelling, paresthesia, Sot stated duration unknown Pain, swelling, paresthesia, sot stated duration unknown Pain and swelling, 2 weeks, Right body mobility of teeth Pain and swelling, 3 years Left mandible Pain ant1 swelling, 9 da.ys Left mandible Pain, swelling, paresthesia, Symphysis, right body, 3 weeks, mobility of teeth left body Painless swelling, 3 weeks, Left angle mobility of teeth Pain and swelling, duration Left body unknown Tntermittent paresthesia, 1 Left body year Paresthesia and pain, 3 Hymphysis years Pain and swelling, 60 days, Rymphysis mobility of teeth 2 cases Not stated presented in a case report by Stoopack and in a literature
Sex and age were recorded for sixteen of the patients reported in the literature. Of these, twelve were males and four were females (a ratio of 3 :1 ) This coincides with the reported series of RCS in all locations in the body in which there was approximately a 3 :l male predominance.‘5, ID The most common site of the tumor is reported to be the long bones.l”, IQ,20 The youngest patient with RCS of the mandible was 8 years of age, and the oldest was 69. The mean age was 32.2 years, with 56 per cent of the patients being under 30 years old (Table I). This agrees with the findings of previously mentioned surveys.15, I93*O CLINICAL
Shoji and Miller’” reported that pain was the most common presenting qymptom in their review of forty-seven patients with RCS of bone. With RCS
Oral Surg. June, 1975
of the mandible, swelling was the most common presenting symptom, with mandibular pain second, followed by paresthesia of the lip on the affected side (Table I). Other presenting symptoms have been resorption4 and loosening of the teeth.8s I13I73I8 Prior to the report by Gerry and Williams’ in 1959, mobility of teeth was not mentioned as a symptom, but since then loosening of teeth has been reported as an early symptom in six of fifteen cases. No evidence of migration of teeth has been reported. RADIOGRAPHIC
There is apparently no characteristic appearance or site of RCS on roentgenographic examination. The most commonly reported site is the posterior body of the mandible near the ramus, where there is a large amount of mcdullary bone. Radiographic findings that have been described include variations from a diffuse, mottled area of rarefaction consistent with osteomyelitis to an increased radiodensity suggesting osteoblastic activity at, the subperiosteal, peripheral margin of the lesion.‘, 15,X69*O However, osteolysis is the most dominant pattern. Other findings include destruction of the lamina dura,‘l. I7 resorption of the roots of teeth,” and pathologic fracture of the affected bone.” This latter finding is usually associated with a patient who has a radiographic appearance consistent with osteomyelitis of long bone.‘” Fracture of the mandible has been documented by Harrigan and colleagues.” TREATMENT
Historically, RCS has been treated by several different methods. Radiation, surgery, and chemotherapy, alone or in combination, have all been used. The doses of radiation have usually been between 2,000 and 4,000 rads; however, doses as low as 1,500 rads and as high as 6,000 rads have also been used.5’ Is Surgically, hemimandibulectomy and radical neck dissection have been reported.“’ Chemotherapeutic agents have generally been used in locally extensive or metastatic lesions-not for a cure but, rather, to limit the disease and make Definitive treatment has been surgery or radiation the curative modality.‘” determined by staging the disease.10 In Stage 1, with the tumor limited to one lymph node area, irradiation has been very effective St,age I is the only stage in which surgery may be curative. In Stage II, with two adjacent lymph node areas on the same side of the diaphragm, radiation therapy is the generally accepted mode of treatment. In advanced Stages III and IV, chemotherapy is usually added to radiation therapy. Where there is a wide spread lymphadenopathy or visceral involvement, Stage IV, chemotherapy has been the primary mode of treatmentI” CASE REPORT A 52.year-old Negro man was seen in the Oral Surgery Clinic in July, 1974, with facial asymmetry of the right body and ramus of the mandible. Facial swelling extended from the inferior border, superiorly, to approximately one half the distance up the lateral surface of the ramus (Fig. 1).
Pig. 1. Facial Past
right angle and ramus of the mandible.
Since December, 1973, the patient had been treated by a local physician for essential hypertension. Treatment had included diazepam, chlorthalidone, and methyldopa. He admitted to urinary difficulties a,nd a previous diagnosis of benign prostatic hypertrophy which did not require specific treatment. History
In June, 1973, the patient sought dental treatment for relief of pain localized to the mandibular right first molar. The tooth was mobile and “periodontally diseased,” according to his local dentist, and subsequently was extracted without difficulty. Within 1 week after extraction, soft-tissue swelling developed on the lingual surface anterior to the extraction site. Oral penicillin was prescribed, and the patient was told to return if the swelling did not subside. In August, 1973, the mandibular right second premolar was painful and mobile, and x-ray examination again revealed “periodontal disease” (Fig. 2). The tooth was extracted, and recovery was uneventful. In December, 1973, the patient developed a swelling intraorally in the lingual vestibule adjacent to the first molar and second premolar area and extra-orally as previously described. Paresthesia of the lower lip occurred at this time but resolved, as did the swelling, while the patient was on a l-week course of oral penicillin. All swelling and the paresthesia resolved with no further symptoms until July, 1974, when the patient returned to his dentist with complaints of pain and swelling, but no paresthesia. Physical
Problem 1 involved a firm, nontender, bonelike overgrowth of tissue intraorally (Fig. 3). The gingiva lingual to the second premolar area and remaining first premolar was soft and edematous, grayish purulent exudate around the tooth. The first premolar was extremely mobile and nonvital to appropriate thermal and electrical stimuli. Ko dysesthesias were present along the inferior alveolar or lingual nerve distributions. Palpation of the right neck revealed a firm, freely movable, nontender node at the junction of the anterior border of the sternocleidomastoid muscle and the angle of the mandible. Problem 2 involved essential hypertension with grade 2 changes in eye ground. During his hospital course, blood pressures ranged from 160/90 to 210,030. The patient had not been taking his medicine regularly prior to admission but was restarted on methyldopa, 250 mg. three times a day.
Fig. d. Periapical
Fig. J. Intraoral Radiographic
Oral Surg. June, 1975
1 year prior
view of bonelike
to biopsy and diagnosis.
caused by tumor growth.
of the right A periapical radiograph taken in August, 1973, prior to the extraction second premolar (Fig. 2), revealed a radiolucent appearance in the molar and premolar areas. Complete loss of lamina dura around the second premolar and interseptal bone loss between the first and second premolars was present. However, no biopsy was performed at this time. The Panorex, lateral oblique, and posteroanterior films of the mandible taken in July, 1974, revealed large osteolytic lesions extending from the first premolar posteriorly to include the body, angle, and ramus of the mandible to within 1.5 cm. of the coronoid notch (Fig. 4). The destructive process in the ramus appeared as several large marrow spaces connected together, producing a honey comb appearance (Fig. 5). Complete loss of normal bone pattern at the angle of the mandible prohibited recognition of the inferior alveolar canal. A concave, osteolytic area at the inferior border and a radiopaquc mass in the third molar area could be seen (Fig. 6). Several punched-out, circumscribed, ra.diolucent lesions were present in the body of the mandible near the mental foramen (Fig. 5). Roentgenograms of the chest were negative for any disease process. A metastatic bone series revealed no evidence
Pig. 4. Posteroanterior
of lytic arthritic Laboratory
view showing osteolysis
sarcoma of masu&ible
of ramus and angle of mandible.
or sclerotic lesions to suggest the presence of metastatic changes in the pelvic girdle and spine were present.
disease. Some degenerative
Clinical laboratory studies of blood, serum, and urine revealed only an elevated alkaline phosphatase value of 50 I.mh (normal, 20 to 48). Peripheral blood studies to reveal any evidence of a malignant condition or chronic infectious process. Hospital
On July 9, 1974, with the patient under local anesthesia and intravenous sedation, an incisional biopsy was performed to obtain bone from two sites and tissue adjacent to’ the lingual surface of the first premolar. Upon direct visualization of the ramus and body from an incision across the retromolar pad, a pathologic fracture of the mandible was evident by its mobility. Periosteum was reflected with difficulty from the underlying lesion, which appeared as a grayish, avascular callus. A 1 cm. square section of bone was removed, revealing necrotic fibrous tissue which was friable lvhen attempts were made to remove the specimen. On the assumption that osteomyelitis was the correct diagnosis, the extraction of the mobile first premolar, placement of an irrigation catheter, and a closed reduction of the fracture using Ivy loop immobilization mere performed. Another biopsy was performed approximately 2 weeks later to confirm the diagnosis. Microscopic
Three specimens were submitted-one each of soft tissue and bone on July 10 and a second bone specimen on July 22. The tissues examined showed extensive necrosis and obliteration of recognizable histologic of the connective tissue by pattern. In some areas there was dense, diffuse infiltration pleomorphic cells with sparse cytoplasm. The nuclei were ovoid with irregular outlines and
Fig. 5. Lateral
oblique view of mandible
Fig. 6. Panorex radiograph lesion at inferior bordrr.
crest and osteolytic
coarse chromatin distribution (Fig. 7 ) . In some areas of that specimen these present in small clusters. Scatterrd ~11s rest~nll)ling lymphohlnsts were present, few areas cells recognizable as lymphocytes WWY predominant.
~11s were anrl in :I
Further evaluation was undertaken after the diagnosis of RCS to establish the presence of metastxtic disease. A liver scan showrtl a defect in the left lobe which uxs biopsied and resolved as normal. LynlI)ll:Ingiogr:lnls, boric marrow biopsy, rp~troc~hlear nodo biopsy, stereo chest films, and a metnstatic boric scan ww all negative for tumor. A final diagnosis of primary reticulum-cell sarcoma of the rn:~ndiblr~ w:ts c~stal~lishr~d. ‘l’rrntmeut consisted of 200 rads a day for 14 days to the affectrtl :lr,la ant1 follo\v-up for adequacy of treatment and possible recurrence. The appearauce of dowse, opaque boric of tllct sir type was evident 2 months after radiation therapy (Fig. 8).
Volume 39 Number 6
Fig. 7. Microscopic section showing eosin stain. Magnification, x500.)
sarcoma of mamWde
dense, scar type
The location, age, sex, and symptoms in this patient were consistent with those of the average patient with RCS of bone. A radiolucency in the molar and premolar area was seen on a periapical film taken 1 year prior to biopsy. A panoramic radiograph of the mandible may have led to earlier diagnosis, particularly in view of recurring postextraction complications. Radiographically, RCS has not been associated with specific findings.15 IIowever, a diffuse osteolytic lesion without a definite order is most common in RCS of either long
Oral Surg. Junr, 19%
or flat hones.*” In our patient there was a loss of lamina dura but no resorption of roots or displacement of teeth. Root resorption was present in an 8-yea],-old patient* but was not mentioned in other cases of RCS of the mandible. Some authors describe a moth-eaten appearancell; others mention either a subpcriosteal osteolytic or an osteogenic phenomenon.‘* I5 RCS usually arises in mrdullary bone; however, it has been described as originating in soft tissue and spreading to bone.” This could explain a subperiostcal osteolytic appearance at the inferior border of the mandible, as seen in this case. Qomc degree of osteogenic reaction seen at the peripheral margin of bone has been described in 45 per cent of the cases of RCS of long bones. 2” Cerrv and Williams’ described a patient with a “sunburst” effect., but there has been only one other report of a rcactivc proliferative process in the mandible.‘” Both the ostcolytic phenomenon at the inferior border and the osteogenic process at the superior border ha.ve been seen in our patient (Fig. 5). An alkaline phosphatasc level of 50 Imp reflects a slight elevation that has been reported in other cases.c,,‘* This elevation seems to confirm the radiographic finding that there was an attempt to produce now bone at the superior border of the lesion, either in an effort to repair the pathologic fracture or as a defense against the tumor. Boston and associates” stated that, laboratory studies of this type are nonspecific and of Iit,tlc diagnostic value. The importance of the origin of the tumor map reflect in survival rates. In RCS of long bones, there is a median survival of 7 years for primary lesions confined more or less to bone, whereas soft-tissue RCS has a %,/&year survival rate.l’ Whether or not the soft-tissue lesion represents t,hc tlirect extension oi the primary tumor is diffic~ult to determine. There are too few lesions of the mandible to develop any survival statistics for the head and neck comparable to those for RCS of long bones. One might speculate that the s-year survival rates might be better for patients with RCS of flat bones than for those with longbone involvement, because of the more superficial location and, consequentIF, earlier detection. Earlier diagnosis of RCS may have been hintlcred by the history of the lesion in this patient. Minc,v and Warnoek’” reported that swelling subsided with antibiotic treatment. Occasionally remission caould be quite long; 3 to 6 months has been reported.!‘, I2 Temporary paresthesia with swelling followed by remission of symptoms with antibiotic therapy is again reported here. This apparently led to the early, but erroneous, diagnosis of abscess or osteompclitis. Shoji anti Niller’” have intcr.jccted the possibility that the etiology of RCS ma;\ be related to iat,rogenic or accidental trauma or infection after which malignant change develops. This might explain the remission of symptoms after antibiotic therapy. Clinically, reticulum-cell sarcoma has been most often tles~ribcd as grayish pink, glistening, brainlike, or fish-meat in appcarance.“‘~ I11Ii Periostcum ma? be firmly attachctl t,o the underlying tumor to sucaha degree as to make dissection difficult. Melrosc and associatesll reported areas of necrosis within the lesion, as demonstrated in this case. Biopsy of ncocrotic areas or friable tumors that can hc easily c+rushed (luring instrumentation may lead to the diagnosis of
sorcon~n of ??la~&ble
chronic inflammation.17 Wang and FleischlilQ reported that seven of twenty-one cases in their series of RCS of all bones were erroneously diagnosed initially. Differentiation of RCS from Ewing’s sarcoma, multiple myeloma, and metastatic carcinoma is often made with difficulty.4, 5, I8 The importance of this distinction for treatment and prognosis is obvious. Steg and colleaguesl” used the classification transition’al type for those lesions of borderline histology. They noted the difficulty in categorizing lesions in the malignant lymphoma group of diseases, and we shared that frustration. Although most areas of the tumor were consistent with reticulum-cell sarcoma, transitional areas could be found. Although special stains for reticulin were used, revealing thin fibrils throughout the intact portions of the lesion, the significance of these fibrils has been quest,ioned.l Stains for glycogen were negative. Boston and co-authors” have stated that exact histologic categorization of lesions within the malignant lymphoma group is not practical, especially since the histologic appearance did not provide significant clues regarding the probability of survival. SUMMARY
A review of the literature and an additional case of primary reticulum-cell sarcoma of the mandible in a 52-year-old man have been presented. The patient reported that he experienced pain and swelling intermittently for 1 year. Radiographically, the area of bone destruction was consistent with osteomyelitis. A biopsy revealed reticulum-cell sarcoma. Additional examinations determined that the neoplasm was confined to the mandible. The patient was subsequently treated with radiation therapy to the affected area. Emphasis has been placed on the difficulty in diagnosis and the varied radiographic appearance of the tumor. There should always be a high degree of suspicion when a seemingly obvious disease tends to take an unexpected course.
REFERENCES 1. Aegerter, 2. 3. 4. 5. 6. 7. 8.
E., and Kirkpatrick, J. A.: Orthopedic Diseases, ed. 3, Philadelphia, 1968, W. B. Saunders Company, pp. 708-716. Annel. P. W.: Reticulum-Cell Sarcoma in the Jams. ORAL &JR& 26: 92-95. 1968. Barclay, J. K. : Reticulum Cell Sarcoma: Report of Case, J. Oral Surg. 29: 734-736, 1971. Blake, M. N., and Beck, L.: Reticulum Cell Sarcoma; Report of Case, J. Oral Surg. 21: 165-168, 1963. Boston, H. C., Jr., Dahlin, D. G., Ivins, J. C., and Cupps, R. F.: Malignant Lymphoma (So-called Reticulum Cell Saacoma) of Bone, Cancer 34: 1131-1137, 1974. Coley, B. L., Higinbotham, N. L., and Groesbeck, H. P.: Primary Reticulum Cell Sarcoma of Bone, Radiology 55: 641-658. 1950. Gerry, k. G., and Williams, S. F.: Primary Reticulum Cell Sarcoma of the Mandible, ORAL fkJRG. 8: 56%581,1955. Ha.lperin, S., and Williams, A. C.: Reticulum-Cell Sarcoma of the Mandible, ORAL SURG.
28: 32-35. 1969. 9. Harriganj W. F., Butler, F. S., and Rchaffer, A. B.: Reticulum Cell Sarcoma of the Chin, J. Oral Surg. 12: 210.214, 1954. 10. Larsen, R. R.. Hill, G. J., and Ratzer. E. R,.: Reticulum Cell Sarcoma in Head and
Neck Surgery,‘Am. J. Surg: 123: 338.342, 1972. 11. Melrose, R. J., Beveridge, E., and Abrams, A. M.: Malignant Lymphoma, J. South. Calif. Dent. Assoc. 37: 507-511, 1969. 12. Mincy, D. I,., and Warnock, M. L. : Primary Malignant Lymphoma of the Mandible: ReDort of Case. J. Oral Surg. 32: 221-224. 1974. 13. Pafker, F., and Jackson, I?.: Primary Reticulum Cell Sarcoma of Bone, Surg. Gynecol. Obstet. 68: 45-53, 1939.
Oral Surg. June, 1975
14. Potdar, G. G. Primary Reticulum Cell Sarcoma of Bone in Western India, Br. J. Cancer 24: 48-55, 1970. Reticulum Cell Sarcoma of Bone, Cancer 28: 15. Shoji, H., and Miller, T. H.; Primary 1234-1244. 1971. Lymphoma of the Mandible and 16. Steg, R. G., Dahlin, D. C., and Gores, R,. J.: Malignant Maxillarv Region, ORAL SURG. 12: 128-141. 1959. 17. Stoopa&, J. ‘c.: ‘Reticulum Cell Sarcoma of the Mandible: Report of Case, J. Oral Surg. 17: 73-79, 1959. Reticulum Cell Sarcoma 18. Taylor, C. G., Alexander, R. E., and Kramer, H. S.: Primary of Mandible: Report of Case, J. Oral Surg. 28: 218.221, 1970. Reticulum Cell Sarcoma of Bone, Cancer 22: 19. Wang, C. C., and Fleischli, D. J.: Primary 994-998, 1968. Reticulum Cell Sarcoma of Bone With 20. Wilson, T. W., and Pugh, D. G.: Primary Emphasis on Roentgen Aspects, Radiology 65: 343-351, 1955. Reprint
Dr Robert L. Campbell University of North Carolina School of Dentistry Chapel Hill, N. C. 27514