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Reticulum-cell sarcoma of the mandible
Reticulum-cell sarcoma of the mandible Xtephen Halperin, New York, N.Y. DEPARTMENT
D.D.S.,” mtl ;I rthw
OF ORAL
SURGERY,
HARLEM
(,‘. U’illinnrs, D.D.8.,‘” HOSPITAL
CENTER
P
rimary reticulum-cell sarcoma of bone is a malignant process that arises in a single focus in bone and is capable of both regional and distant metastasis. It is moderately sensitive to radiation. Those affected are young adults, with a slight male preponderance. The long bones, particularly the femur, are most commonly involved.i, j, G The roentgenographic appearance is one of bone rarefaction, periosteal reaction, and soft-tissue swelling.?, 3 These findings, however, are not specific for the disease.” It should be noted that the disease often extends beyond what is apparent on the roentgenograms. The diagnosis is made on the basis of clinical history, physical examination, roentgcnographic study, and histologic findngs. It is only by constant awareness of this disease that an early diagnosis can be made and prompt treatment instituted. The treatment of primary reticulum-cell sarcoma of bone varies from one institution to another. Radical surgery and/or radiotherapy is generally considered to be the accepted mode of treatment. Failure to cure by these methods is generally due to distant metastatic disease. Local recurrence is uncommon. Chemotherapy does not play a primary role in the treatment of the disease, although it becomes increasingly useful for palliation in patients with generalized lesions. CASE REPORT On Aug. 14, 1967, a 41-year-old Negro woman came to the Oral Surgery Clinic at Harlem Hospital with a chief complaint of “pain in the left jaw and numbness of the bottom lip on the left side.” The patient stated that on July 25, 1967, all her lower front t&h had been removed l)y a private dentist because of a swelling of the gingiva. According lo the patient, this swelling did not decrease after the extractions but, began to enlarge to its present size. Oral examination revealed a buecal swelling which extended from the mandibular right cuspid region to the left first premolar. This circumscribed swelling had no definite area of fluctuance, yet it was resilient. The remaining tooth (mandibular left first premolar) was quite painful to percussion and had slight mobility. Radiographic examination revealed an irregular rndiolucent osteolytic area in the right “Resident, *“Director,
32
Department Department
of Oral Surgery, 1967-1968. of Oral Surgery.
Volume Number
Reticulum-cell
28 1
sarcoma of mandible
33
mandibular cuspid region. Sequestrating bony fragments were also noted within the area of radiolucency. (2) an The patient’s past medical history included (1) measles and mumps in childhood; appendectomy in 1956 with no complications; (3) the birth of a deformed child in 1956; and (4) pyelonephritis during pregnancy in 1965, with uncomplicated delivery of a normal child. The patient denied having any allergies. Local anesthesia was induced with 2 per cent lidocaine hydrochloride with epinephrine 1 :lOO,OOO, and a biopsy was performed. A midalveolar ridge incision was made from the mandibular left first premolar to the right cuspid, and the labial mucoperiosteum x-as reflected. lobulatetl, The initial impression was that the underlying soft tissue WRS fleshy, brainlike,
Fig. 1. High-power detail of pleomorphism of cells with formation of apparent pseudopods. Tumor cells are larger than small lymphocytes, their nuclei less dense, and their cytoplasm less deeply stained. (Hcmatoxylin and cosin stain. Magnification x1,000. j
Pig. 8. Silver laid down between
impregnation stain cells. (Magnification,
has been used to outline ~1,000).
abundant
reticulum
strands
34
Halperin
and
U7illicms
and quite friable. During r~~moval of wveral wctions oi’ this l~rownish tissw, its li~~~~~orrl~agi~~ nature became apparent. The 1al)ial wrtic*al plate w*i. c Y c’rotletl. with awas of &prwsions and sharp spiny elevations. Fenc5tration of thcl rortiral platr was nottvl in the wgion of tllc osteolytic part of the lesion. Both soft-tissw fragnlt~rlts ant1 WwttPd sl”4rllcws of bonV ~vere submitted for pathologic examination. Microscopic examination wv-t~ali~rl sections of scv~~ral fragmt,nts of cellular neoplasm with features of a malignant lymphoma c*omposrd mainly of Iymphocytie or lgmphoblastic cells and numerous uniformly la.rger (*ells with scanty angular cytoplasmio processes which were interpreted as reticulum cells. TllfYw, in lurn, swmc~l to lw the predominant 41 component in several foci. Multinwlcated wlls appeared to lw histiocytic in nature but not wnspicuous. The tumor was supplied by num~~rous small l~lootl vtwels and showetl interspwscd spiculw of I)onc, awas of mild fil)rosis, and Romc nwrwis. The high rate of mitotic activity per higIll~owc’r field attwtcd to the malignant natuw of the lymphoma (Fig. 1 ). The deposition 01 filww swn with rc+cdurri stains appeared to favor a wticulumlarge amounts of wticulum wew conI~11 sarcoma (Fig. 2). The microwopic: swtions of the decalcified Irony fragments patiljle with the previous drscription of the soft-t issue neoplasm. The microscopic diagnosis \ras malignant lymphoma, mainly of the reticulunl-cell type, origininating in thcx mantlil~lc. The patient was admitted to the hospital on Aug. 19, 1967, for a more complete examination. Physical examination upon admission yielded no findings pertinent to the present condition. A clwst film showed thinning and elongation of peripheral markings in both upper lobes Ijut no recent pxrrnchymal infiltrat,ion or consolidation. The rardio-aortic silhouette was nord wmplcte l)onc survey was negative for metastasis but remal in size and configuration. series showed no invealed t,hn ostclolytic. It‘sion of the rnandibl~~. An upper gastrointestinal pyclogram revealed volvement of the esophagus, stomwh, and duodenum. An intravenous moderate hydroncphrosis on both sitles, slightly more marked in the right kidney. Both ureters and the urinary bladder, as far as ~isualizrd, pwscnted normal appearances. Laboratory findings were all within normal limits, except for a l)orderline lwkopenia. Consultations with the Dc~partmrnts of Ht~matology and Radiology were requested. The Hematology Department stated that the prril~heral blood smear and bone marrow were un‘I’ircay conc*urrwl Ivitli Ilie Radiology Department’s sugremarkable except for 111~leukopenia. gestion that, immediatr supraroltagc thc%lpy l)c instituted bcr;tuso of the radiosensitivity of the tumor. A total dose of 6,000 r owr a &wwk ptsriod wts rc~c,ortlrllerl(lctl by the Radiology Clinic, the patient was givw a local Wpartmcnt. On Aug. 24, 1967, in tlic Oral Surgwy anesthetic and the following twth \!-wC CxtracttYl : 6321 I 123
14
Complete alveolectomies were performed to afford adequate tissue coverage and to help prevent future osteoradionecrosis. for radiaThe patient was discharged on Sept. 9, 1967, to be sent to Delafield Hospital was to be given over a B-meek period, tion therapy on an outpatient basis. The radiotherapy and the total dose was to be 6,000 r. At the termination of the radiotherapy, and for one month thereafter, the neoplasm seemed to decrease in size. However, on follow-up examination in the Oral Surgery Clinic, an increase of t.lro tumor mass about the chin region was noted. The patient was immediately referrrtl back to Delafield Hospital, and on Nov. 19, 1967, a radical resection was performed. The surgical procedure included a partial mandibulectomy suprahyoid dissection. A pathologic from the right angle to the left angle, \\ith bilateral review of the specimen disclosed that all margins were free of disease. At present, the patient is under the care of the Plastic Surgery Service at Columbia-Presbyterian Hospital in New York City. She is receiving grafts 11y multiple-stage tub??, with the donor site being the
DISCUSSION
The feature of academic interest in this case wa.s the determination of the true location of the primary lesion. One can suppose from the total workup
Volume Number
28 1
Reticuhn-cell
sarcoma of mmdible
35
that the mandibular osteolytic lesion was a direct extension from a soft-tissue primary lesion in the mandible. The histologic appearance in each case was practically indistinguishable as reticulum-cell sarcoma.l, 5, 7 Another point of consideration was the order and timing of treatment. IRetrospectively, the patient might have been better treated by initial radiation, with less delay in the wide resection.“, ‘3 D SUMMARY We have presented the case of a 41-year-old Negro woman with a reticulumccl1 sarcoma of the mandible. The patient was initially treated with supravoltage radiation and then underwent a wide resection. Delayed reconstruction with multiple-stage tubes is now in progress. We would like to thank Dr. E. Moscovic,
Department
of Pathology,
for his kind assistance.
REFERENCES
Sarcoma 1. Coley, B. L., Higinbotham, N. L., and Groesbeck, H. P.: Primary Reticulum-Cell of Bone, Radiology 56: 641-658, 1950. 2. Sherman, R. S., and Snyder, R. E.: Roentgen Appearance of Primary Reticulum-Cell Sarcoma of Bone, Am. J. Roentgenol. 58: 291-306, 1947. 3. Wilson, T. W., and Pugh, D. G.: Primary Reticulum-Cell Sarcoma of Bone With Emphasis on Roentgen Aspects, Radiology 65: 343-351, 1955. 4. Wang, C. C.: Current Concepts: Treatment of Primary Reticulum-Cell Sarcoma of Bone, New England J. Med. 278: 1331-1372, 1968. 5. Szutu, C., and Hsieh, Chill-Kuang: Primary Reticulum-Cell Sarcoma of Bone; Report of Two Cases With Bone Regeneration Following Roentgenotherapy. Ann. Surg. 115: 280-291, 1942. 6. Tillman, H. H.: Malignant Lymphomas Involving the Oral Cavity and Surrounding Structures, ORAL SURG., ORAL MED.& ORAL PATH.~.~: 60,1965. D. C.: Reticulum-Cell Sarcoma of Bone, J. Boric & Joint Surg. 7. Ivins, J. C., and Dahlin, 3.5-A: 835-842. 19.53. 8. Oldham, L.: Reticulum-Cell Sarcoma of the Maxilla, D. Practitioner 16: 13, 1965. A. D., and Vickers, R. A.: Primary Reticulum-Cell Sarcoma of the Mouth, J. 9. Chaudry, Oral Surg., Anesth. & Hosp. D. Serv. 20: 159-162, 1962.
D.D.S.,” mtl ;I rthw
OF ORAL
SURGERY,
HARLEM
(,‘. U’illinnrs, D.D.8.,‘” HOSPITAL
CENTER
P
rimary reticulum-cell sarcoma of bone is a malignant process that arises in a single focus in bone and is capable of both regional and distant metastasis. It is moderately sensitive to radiation. Those affected are young adults, with a slight male preponderance. The long bones, particularly the femur, are most commonly involved.i, j, G The roentgenographic appearance is one of bone rarefaction, periosteal reaction, and soft-tissue swelling.?, 3 These findings, however, are not specific for the disease.” It should be noted that the disease often extends beyond what is apparent on the roentgenograms. The diagnosis is made on the basis of clinical history, physical examination, roentgcnographic study, and histologic findngs. It is only by constant awareness of this disease that an early diagnosis can be made and prompt treatment instituted. The treatment of primary reticulum-cell sarcoma of bone varies from one institution to another. Radical surgery and/or radiotherapy is generally considered to be the accepted mode of treatment. Failure to cure by these methods is generally due to distant metastatic disease. Local recurrence is uncommon. Chemotherapy does not play a primary role in the treatment of the disease, although it becomes increasingly useful for palliation in patients with generalized lesions. CASE REPORT On Aug. 14, 1967, a 41-year-old Negro woman came to the Oral Surgery Clinic at Harlem Hospital with a chief complaint of “pain in the left jaw and numbness of the bottom lip on the left side.” The patient stated that on July 25, 1967, all her lower front t&h had been removed l)y a private dentist because of a swelling of the gingiva. According lo the patient, this swelling did not decrease after the extractions but, began to enlarge to its present size. Oral examination revealed a buecal swelling which extended from the mandibular right cuspid region to the left first premolar. This circumscribed swelling had no definite area of fluctuance, yet it was resilient. The remaining tooth (mandibular left first premolar) was quite painful to percussion and had slight mobility. Radiographic examination revealed an irregular rndiolucent osteolytic area in the right “Resident, *“Director,
32
Department Department
of Oral Surgery, 1967-1968. of Oral Surgery.
Volume Number
Reticulum-cell
28 1
sarcoma of mandible
33
mandibular cuspid region. Sequestrating bony fragments were also noted within the area of radiolucency. (2) an The patient’s past medical history included (1) measles and mumps in childhood; appendectomy in 1956 with no complications; (3) the birth of a deformed child in 1956; and (4) pyelonephritis during pregnancy in 1965, with uncomplicated delivery of a normal child. The patient denied having any allergies. Local anesthesia was induced with 2 per cent lidocaine hydrochloride with epinephrine 1 :lOO,OOO, and a biopsy was performed. A midalveolar ridge incision was made from the mandibular left first premolar to the right cuspid, and the labial mucoperiosteum x-as reflected. lobulatetl, The initial impression was that the underlying soft tissue WRS fleshy, brainlike,
Fig. 1. High-power detail of pleomorphism of cells with formation of apparent pseudopods. Tumor cells are larger than small lymphocytes, their nuclei less dense, and their cytoplasm less deeply stained. (Hcmatoxylin and cosin stain. Magnification x1,000. j
Pig. 8. Silver laid down between
impregnation stain cells. (Magnification,
has been used to outline ~1,000).
abundant
reticulum
strands
34
Halperin
and
U7illicms
and quite friable. During r~~moval of wveral wctions oi’ this l~rownish tissw, its li~~~~~orrl~agi~~ nature became apparent. The 1al)ial wrtic*al plate w*i. c Y c’rotletl. with awas of &prwsions and sharp spiny elevations. Fenc5tration of thcl rortiral platr was nottvl in the wgion of tllc osteolytic part of the lesion. Both soft-tissw fragnlt~rlts ant1 WwttPd sl”4rllcws of bonV ~vere submitted for pathologic examination. Microscopic examination wv-t~ali~rl sections of scv~~ral fragmt,nts of cellular neoplasm with features of a malignant lymphoma c*omposrd mainly of Iymphocytie or lgmphoblastic cells and numerous uniformly la.rger (*ells with scanty angular cytoplasmio processes which were interpreted as reticulum cells. TllfYw, in lurn, swmc~l to lw the predominant 41 component in several foci. Multinwlcated wlls appeared to lw histiocytic in nature but not wnspicuous. The tumor was supplied by num~~rous small l~lootl vtwels and showetl interspwscd spiculw of I)onc, awas of mild fil)rosis, and Romc nwrwis. The high rate of mitotic activity per higIll~owc’r field attwtcd to the malignant natuw of the lymphoma (Fig. 1 ). The deposition 01 filww swn with rc+cdurri stains appeared to favor a wticulumlarge amounts of wticulum wew conI~11 sarcoma (Fig. 2). The microwopic: swtions of the decalcified Irony fragments patiljle with the previous drscription of the soft-t issue neoplasm. The microscopic diagnosis \ras malignant lymphoma, mainly of the reticulunl-cell type, origininating in thcx mantlil~lc. The patient was admitted to the hospital on Aug. 19, 1967, for a more complete examination. Physical examination upon admission yielded no findings pertinent to the present condition. A clwst film showed thinning and elongation of peripheral markings in both upper lobes Ijut no recent pxrrnchymal infiltrat,ion or consolidation. The rardio-aortic silhouette was nord wmplcte l)onc survey was negative for metastasis but remal in size and configuration. series showed no invealed t,hn ostclolytic. It‘sion of the rnandibl~~. An upper gastrointestinal pyclogram revealed volvement of the esophagus, stomwh, and duodenum. An intravenous moderate hydroncphrosis on both sitles, slightly more marked in the right kidney. Both ureters and the urinary bladder, as far as ~isualizrd, pwscnted normal appearances. Laboratory findings were all within normal limits, except for a l)orderline lwkopenia. Consultations with the Dc~partmrnts of Ht~matology and Radiology were requested. The Hematology Department stated that the prril~heral blood smear and bone marrow were un‘I’ircay conc*urrwl Ivitli Ilie Radiology Department’s sugremarkable except for 111~leukopenia. gestion that, immediatr supraroltagc thc%lpy l)c instituted bcr;tuso of the radiosensitivity of the tumor. A total dose of 6,000 r owr a &wwk ptsriod wts rc~c,ortlrllerl(lctl by the Radiology Clinic, the patient was givw a local Wpartmcnt. On Aug. 24, 1967, in tlic Oral Surgwy anesthetic and the following twth \!-wC CxtracttYl : 6321 I 123
14
Complete alveolectomies were performed to afford adequate tissue coverage and to help prevent future osteoradionecrosis. for radiaThe patient was discharged on Sept. 9, 1967, to be sent to Delafield Hospital was to be given over a B-meek period, tion therapy on an outpatient basis. The radiotherapy and the total dose was to be 6,000 r. At the termination of the radiotherapy, and for one month thereafter, the neoplasm seemed to decrease in size. However, on follow-up examination in the Oral Surgery Clinic, an increase of t.lro tumor mass about the chin region was noted. The patient was immediately referrrtl back to Delafield Hospital, and on Nov. 19, 1967, a radical resection was performed. The surgical procedure included a partial mandibulectomy suprahyoid dissection. A pathologic from the right angle to the left angle, \\ith bilateral review of the specimen disclosed that all margins were free of disease. At present, the patient is under the care of the Plastic Surgery Service at Columbia-Presbyterian Hospital in New York City. She is receiving grafts 11y multiple-stage tub??, with the donor site being the
DISCUSSION
The feature of academic interest in this case wa.s the determination of the true location of the primary lesion. One can suppose from the total workup
Volume Number
28 1
Reticuhn-cell
sarcoma of mmdible
35
that the mandibular osteolytic lesion was a direct extension from a soft-tissue primary lesion in the mandible. The histologic appearance in each case was practically indistinguishable as reticulum-cell sarcoma.l, 5, 7 Another point of consideration was the order and timing of treatment. IRetrospectively, the patient might have been better treated by initial radiation, with less delay in the wide resection.“, ‘3 D SUMMARY We have presented the case of a 41-year-old Negro woman with a reticulumccl1 sarcoma of the mandible. The patient was initially treated with supravoltage radiation and then underwent a wide resection. Delayed reconstruction with multiple-stage tubes is now in progress. We would like to thank Dr. E. Moscovic,
Department
of Pathology,
for his kind assistance.
REFERENCES
Sarcoma 1. Coley, B. L., Higinbotham, N. L., and Groesbeck, H. P.: Primary Reticulum-Cell of Bone, Radiology 56: 641-658, 1950. 2. Sherman, R. S., and Snyder, R. E.: Roentgen Appearance of Primary Reticulum-Cell Sarcoma of Bone, Am. J. Roentgenol. 58: 291-306, 1947. 3. Wilson, T. W., and Pugh, D. G.: Primary Reticulum-Cell Sarcoma of Bone With Emphasis on Roentgen Aspects, Radiology 65: 343-351, 1955. 4. Wang, C. C.: Current Concepts: Treatment of Primary Reticulum-Cell Sarcoma of Bone, New England J. Med. 278: 1331-1372, 1968. 5. Szutu, C., and Hsieh, Chill-Kuang: Primary Reticulum-Cell Sarcoma of Bone; Report of Two Cases With Bone Regeneration Following Roentgenotherapy. Ann. Surg. 115: 280-291, 1942. 6. Tillman, H. H.: Malignant Lymphomas Involving the Oral Cavity and Surrounding Structures, ORAL SURG., ORAL MED.& ORAL PATH.~.~: 60,1965. D. C.: Reticulum-Cell Sarcoma of Bone, J. Boric & Joint Surg. 7. Ivins, J. C., and Dahlin, 3.5-A: 835-842. 19.53. 8. Oldham, L.: Reticulum-Cell Sarcoma of the Maxilla, D. Practitioner 16: 13, 1965. A. D., and Vickers, R. A.: Primary Reticulum-Cell Sarcoma of the Mouth, J. 9. Chaudry, Oral Surg., Anesth. & Hosp. D. Serv. 20: 159-162, 1962.