522
PERIOSTEAL
19. Schultz W, Whitten JB: Mucoepidermoid carcinoma in the mandible: Report of a case. J Oral Surg 27:337, 1969 20. Frederickson C, Cherrick HM: Central mucoepidermoid carcinoma of the jaws. J Oral Med 33:80, 1978 21. Unal T, Zeytinoglu B: Central mucoepidermoid carcinoma of the jaws. J Oral Med 40:202, 1985 22. Pincock JL, El-Mofty SK: Recurrence of cystic central mucoepidermoid tumor of the mandible. Int J Oral Surg 14:81, 1985 23. Stoch RB, Smith I: Mucoepidermoid carcinoma of the mandible: Report of case. J Oral Surg 3856, 1980 24. Lepp H, cited in Hertz J: Mucus secreting tumors of the jaws. Acta Chir Stand 103:276, 1952 25. Hertz J: Mucus secreting tumors of the jaws. Acta Chir Stand 103:276, 1952
J Oral Maxillofac 46:522-526,
OSTEOSARCOMA
OF THE MAXILLA
26. Sonesson A: Mucus-secreting and cystic epidermoid carcinoma of the jaws. Acta Radio1 34:25, 1950 27. Szerlip L: Cystic mucoepidermoid tumor. Report of case. Oral Sure 9:584. 1956 28. Rzucidlo i?B, Rzeszotko-Adamiczka D: Uber einen mucoepidermoid tumors eines Unterkiefers. Zentralbl Allg Pathol 105:409, 1964 29. Attenson M, Kaufman M, Brown S: Mucoepidermoid carcinoma of the mandible: Report of a case. J Oral Surg 22:350, 1964 30. Lacour M, Reynaud J, Grappin G, et al: A case of mucoepidermoid tumors of the mandible. Bull Sot Med Afr Lang Fr 11:73, 1966 31. Karmarkar SR: Central mucoepidermoid tumors of the mandible. Indian J Surg 31: 154, 1969
Surg
1990
Perios teal Osteosarcoma
of the Maxilla:
A Case Report and Review of Literature LTC ADRIAN PATTERSON, DDS, DC, USA,* ROBERT 0. GREER JR, DDS, ScD,t AND DANIEL HOWARD, DDSS
Juxtacortical osteosarcoma was first fully described by Jaffee and Selin’ in 1951, although Geschickter and Copeland had described a similar lesion under the heading “parosteal osteoma” in 1949.*s3 Since these initial reports, juxtacortical osteosarcoma has been described using various names, including ossifying parosteal osteogenic sarcoma, and parosteal and periosteal ossifying fibrosarcoma.4T5 Juxtacortical osteogenic sarcoma occurs much less commonly than central medullary osteosarcoma, which has an incidence rate of 0.07 per 1,000 population. The clinicopathology of fewer than a dozen cases of juxtacortical osteosarcoma has been
* Chief, Surgery Branch, United States Institute of Dental Research, Department of the Army, Walter Reed Army Medical Center, Washington, DC. t Professor and Chairman, Division of Oral Pathology, University of Colorado School of Dentistry; Professor of Pathology, University of Colorado School of Medicine, Denver. $ In private practice, Washington, DC. The opinions or assertations contained herein are the private views of the authors and are not to be construed as official or as reflecting the view of the Department of the Army. Address correspondence and reprint requests to LTC Patterson: Surgery Branch, US Institute of Dental Research, Department of the Army, Walter Reed Army Medical Center, Washington, DC 20307-5300. 0 1990 American
Association
geons 0278-2391/90/4805-0018$3.00/O
of Oral
and Maxillofacial
Sur-
well documented in the English literature (Table l)? Recently, Zarbo et al’* suggested that juxtacortical osteosarcoma can be further subdivided into two clinicopathologic variants: parosteal osteosarcoma and periosteal osteosarcoma. The purpose of this article is to report a rare example of maxillary juxtacortical osteosarcoma of the periosteal type and to discuss the clinicopathologic nature of that disease process. Report of a Case A 65year-old black woman was referred by her general dentist for evaluation and treatment of a mass in the right maxillary vestibule and alveolus. The lesion had been present and slowly enlarging for approximately 1 year. It had been asymptomatic until about 3 weeks before her visit, when the patient began to have mild pain in the maxillary right quadrant. Physical examination revealed a 2.1 x 2.5 x 2 cm hard mass in the right maxillary vestibule (Fig 1). The lesion was lobular in appearance. The gingiva overlying the lesion showed increased vascularity, but no ulceration. The buccal vestibule was nearly obliterated by the lesion in the region of the premolar and canine teeth. There was neither paresthesia or anesthesia of the infraorbital nerve. A panoramic radiograph showed a slightly radiopaque shadow approximately 0.5 x 0.5 cm distal to the premolar (Fig 2), as well as a longitudinal fracture of this tooth. There was also a loss of lamina dura around the involved teeth. Neck examination was negative for tenderness and adenopathy. An excisional biopsy was performed under local anes-
PATTERSON,
523
GREER, AND HOWARD
Table 1. Cases of Juxtacortical Osteosarcoma Affecting the Jaws Sex/Age
Authors Som and Peimerh Rota et al’
M/45
Left mandible
F/17 Ml63
Midline of maxilla Right mandible
Solomon et al”
Ml38
Right mandible
Newland and AyalaY Banejee” Adekeye et al’ ’ Zarbo et al” Bras et alI3 Regezi et alI4
Ml25 F/47 F/17 M/57 M/40 F/13 Ml68
Left maxilla Left mandible Right mandible Left mandible Left mandible Posterior maxilla Posterior maxilla
Abbreviations:
Treatment
Site
Follow-up
Multiple local resections, followed by hemimandibulectomy Local and partial resection Several local resections, followed by right mandibulectomy Local excision, followed by hemimandibulectomy Local resection Enbloc resection Enucleation Hemimandibulectomy Hemimandibulectomy Excision Excision
7 yr, no recurrence 9 yr, no recurrence 6 yr. DOC, no recurrence 6 mo. no recurrence 6 mo, no recurrence 8 yr. no recurrence NA I yr. no recurrence 11yr, no recurrence 5 yr, no recurrence 2 yr. 2 recurrences; DOC
DOC. died of other causes; NA. not available
thesia. A full-thickness mucoperiosteal flap was raised exposing the lesion. The covering gingiva and mucoperiosteum separated easily from the lesion. A portion of the lesion was easily removed and submitted for histologic examination along with a piece of the buccal alveolar
bone. The premolar was extracted. The enced an uneventful recovery. Following a diagnosis of juxtacortical the patient was referred to Walter Reed Center for evaluation and definitive care. derwent a partial hemimaxillectomy and currence of disease after 1 year.
patient experiosteosarcoma, Army Medical The patient unhas had no re-
well-organized trabeculae of lamellar bone that blended with the cortical plate of the maxilla. The stroma was richly cellular and composed predominantly of spindle cells. Distributed throughout the stroma were atypical spindle-shaped and polyhedral cells with hyperchromatic nuclei consistent with malignant osteoblasts (Fig 3) and an abundant malignant chondroid matrix (Fig 4). Malignant tumor osteoid radiated between chondroid and osseous trabeculae. Mitotic figures were common within the chondroid matrix, two to three per high-power field. The excised material showed extension of tumor to the tissue margins. A diagnosis of juxtacortical (periosteal) osteosarcoma was made.
GROSS PATHOLOGY
The original excisional biopsy tissue sample consisted of several tooth fragments that measured 2.8 x 2 x 1 cm and glistening, granular, gritty, somewhat polypoid pieces of soft tissue that measured 2.7 x 2.5 x 2 cm.
Discussion
Microscopic examination of the short-term decalcified tissue specimen showed a tumor that was composed of
Primary osteosarcoma of the mandible and maxilla is exceedingly rare, both in terms of absolute incidence and the frequency of sarcomas in the entire skeleton. Juxtacortical osteosarcoma is even more unique (Table 1). Adekeye et al” reviewed a
FIGURE 1. Broad-based lobular tumor mass in the right maxillary vestibule.
FIGURE 2. premolar.
HISTOPATHOLOGY
Focal radiopacity distal to maxillary right second
524
PERIOSTEAL
OSTEOSARCOMA
OF THE MAXILLA
FIGURE 3. High-power photomicrograph showing malignant tumor osteoid (arrow) arising adjacent to pleomorphic osteoblasts. (Hematoxylin-eosin stain. Original magnification, X260.)
series of I5 primary and I metastatic osteosarcoma of the jaw and identified only one juxtacortical osteosarcoma, a parosteal variant that was treated by enucleation. Follow-up data on the patient were not presented. Regezi et a1’4 described the clinical, microscopic, and immunohistochemical characteristics of 17 osteosarcomas and chondrosarcomas in a 1987 review and reported only 2 juxtacortical tumors. Both neoplasms were parosteal. One patient
FIGURE 4. Predominantly cartilaginous neoplasm demonstrating spindle cell stroma and malignant chondroid matrix with atypical chondroid differentiation. (Hematoxylineosin stain. Original magnification, X260.)
was a 13-year-old girl who showed no signs or recurrence 5 years after complete surgical excision of her tumor. The second patient, a 68-year-old man, developed two recurrences in the 2 years following excision of his tumor and died tumor free 5 years later of other causes. Some investigators separate juxtacortical osteosarcoma into low-grade (parosteal) and highgrade (periosteal) variants (Table 2).15,16 Zarbo et
PATTERSON,
Table 2.
525
GREER, AND HOWARD
Distinguishing Features of Periosteal and Parosteal Osteosarcoma of the Jaws Clinical Features
Radiographic Features
Histologic Features
Male predominance
Lobular, with well-defined periphery
Cortex intact, with no marrow involvement
Male predominance
III defined; tends to overgrow base of origin
No radiographic continuity; may show medullary involvement
Predominantly composed of poorly differentiated malignant cartilaginous tissue Spindle cell stroma with mild cytologic atypia
Lesion
Age
Sex
Periosteal osteosarcoma
Peak occurrence at 20 yr
Parosteal osteosarcoma
Peak occurrence at 39 yr
al” have suggested that such a distinction is warranted because of the distinctive clinical, microscopic, and behavioral characteristics unique to each tumor. Periosteal osteosarcoma is the least common variant. de Santos et alI7 have reported a 2-to-1 male predominance in long bones and a peak occurrence at about age 20 years for the periosteal varient. None of the tumors reported by Regezi and associates’4 or in the African series of Adekeye et al” were periosteal tumors. Only 1 of 23 periosteal osteosarcomas in Dahlin’s series” involved the mandible. While parosteal osteosarcoma occurs over a broad age range that peaks at 39 years, l7 periosteal osteosarcoma occurs a decade or two later. Radiographic, gross morphologic, and histologic features serve to further separate the two variants of juxtacortical osteosarcoma. The cortex in periosteal osteosarcoma is radiographically intact and sometimes thickened, with no involvement of the underlying marrow cavity. Grossly, the tumor is most often lobulated and well defined, with little evidence of infiltration into the surrounding soft tissue. Unlike parosteal osteosarcoma, it is exceedingly rare for periosteal osteosarcoma to overgrow its base of origin. The histologic differences between periosteal and parosteal forms of the neoplasm are dramatic. Periosteal osteosarcoma is predominantly a cartilaginous neoplasm, whereas parosteal osteosarcoma is characterized by a spindle cell stroma with mild to moderate atypia and rare mitotic figures dispersed between irregular osseous trabeculae. Fine, lacelike osteoid can be found in the chondroid islands of periosteal osteosarcoma, thereby distinguishing it from juxtacortical chondrosarcoma. Unni et alI9 report that in long-bone sites the prognosis for periosteal osteosarcoma is considerably more favorable than for conventional central osseous osteosarcoma, but poorer than that for
Prognosis Capable of local recurrence and distant metastasis
Minimal metastatic potential
parosteal osteosarcoma. The small group of juxtacortical osteosarcomas of the jaws reported in the literature show a much better prognosis than their long-bone counterparts. However, because the number of cases reported is so small, the exact prognosis remains unclear. The bland microscopic appearance of parosteal osteosarcoma may lead to a microscopic diagnosis of osteoma, osteochondroma, heterotopic ossification, or myositis ossificans. Chondroma or chondroid choristoma must also be considered in the differential microscopic diagnosis of periosteal osteosarcoma, and a diagnosis of juxtacortical osteosarcoma requires complete synthesis of clinical, radiographic, and histologic features of the tumor. Local excision is considered to be an inadequate form of treatment. Surgical therapy requires al least an adequate margin of tissue, hemimandibulectomy, or wide maxillary resection. Summary
Juxtacortical osteosarcomas, including periosteal and parosteal subtypes, are uncommon neoplasms of the jaws. In long bones, the biologic behavior of the periosteal variant is reported to be more aggressive than the parosteal tumor. Juxtacortical osteosarcoma of the jaws appears to have a slightly more favorable prognosis than in long bones, although a larger series of cases needs to be evaluated before a definitive conclusion concerning the behavior of juxtacortical osteosarcoma of the jaws can be made. References 1. Jaffe HL, Selin G: Tumors of bone and joints. Bull NY Acad Med 27:165, 1951 2. Geschickter CF, Copeland MM: Tumors of Bone (ed 3). Philadelphia, PA, Lippincott, 1949, pp 367-384
526
MAXILLARY
3. Geschickter CF, Copeland MS: Parosteal osteoma of bone: A new entity. Ann Surg 133:790, 1951 4. Dwinnell LA, Dahlin DC, Ghormley RK: Parosteal (juxtacortical) osteogenic sarcoma. J Bone Joint Surg 36A:732, 1954 5. Lichtenstein L: Tumors of periosteal origin. Cancer 8: 1060, 1955 6. Som M, Peimer R: Juxtacortical osteogenic sarcoma of the mandible. Arch Otolaryngol 74:532, 1961 7. Rota AN, Smith JL, Jing B: Osteosarcoma and parosteal osteogenic sarcoma of the maxilla and mandible: Study of 20 cases. Am J Clin Path01 54:625, 1970 8. Solomon MP, Biemacki J, Slippen M, et al: Parosteal osteogenic sarcoma of the mandible. Arch Otolaryngol 101:754, 1975 9. Newland JR, Ayala AG: Parosteal osteosarcoma of the maxilla. Oral Surg 43:727, 1977 10. Banejee SC: Juxtacortical osteosarcoma of the mandible: Review of the literature and report of a case. J Oral Surg 39:535, 1981 11. Adekeye EO, Chau KK, Edwards MB, et al: Osteosarcoma
J Oral Maxillofac 46526-530.
12. 13.
14.
15. 16. 17.
18. 19.
AMELOBLASTIC
FIBRO-ODONTOMA
of the jaws-A series from Kaduna, Nigeria. J Oral Surg 16205, 1987 Zarbo RJ, Regezi JA, Baker SR: Periosteal osteogenic sarcoma of the mandible. Oral Surg 57643, 1984 Bras JM, Donner R, Van Der Kwast WAM, et al: Juxtacortical osteogenic sarcoma of the jaws. Review of the literature and report of a case. Oral Surg 50:535, 1980 Regezi JA, Zarbo RJ, McClatchey KD, et al: Osteosarcomas and chondrosarcomas of the jaws: Immunohistochemical correlations. Oral Surg 64:302, 1987 Dahlin DC, Unni KK: Osteosarcoma of bone and its important recognizable varieties. Am J Surg Path01 1:61, 1977 Huvos AG: Bone tumors: Diagnosis, treatment, and prognosis. Philadelphia, PA, Saunders, 1970 deSantos LA, Murray JA, Finklestein JB, et al: The radiographic spectrum of periosteal osteosarcoma. Radiology 127:123, 1978 Dahlin DC: Bone tumors: General aspects and data on 6,221 cases (ed 3). Springfield, IL, Thomas, 1978 Unni KK, Dahlin BC, Beabout JW, et al: Parosteal osteogenie sarcoma. Cancer 37:2455, 1976
Surg
1990
Massive Maxillary Ameloblastic Fibro-Odon toma: Case Report With Surgical Management E.M.G. PIETTE, MD, BDS,* H. TIDEMAN, DDS, MD, PHD,t AND P.C. WU, MD, FRCPATH, FRCPAS
The ameloblastic fibro-odontoma is a rare mixed odontogenic tumor, described under a variety of terms until Hooker’ separated it from ameloblastic odontomas and described it as a separate entity. This tumor has been classified as an ameloblasticectomesenchymal tumor by Reichart and Ries.’ Slootweg3 reviewed the international literature between 1908 and 1980 and found 55 cases consistent with the diagnosis, together with his own cases. The purpose of this report is to describe a new unusual Received from The Prince Philip Dental Hospital, University of Hong Kong, Hong Kong. * Senior Lecturer, Department of Oral Surgery and Oral Med-
icine . ? Professor and Head, Department of Oral Surgery and Oral Medicine. $ Reader, Department of Pathology, Oral Pathology Unit. Address correspondence and reprint requests to Dr Piette: Department of Oral Surgery and Oral Medicine, The Prince Philip Dental Hospital, University of Hong Kong, 34 Hospital Rd, Hong Kong. 0 1990 American Association of Oral and Maxillofacial geons 0278-2391/90/4805-0019$3.00/0
Sur-
case of voluminous odontoma.
maxillary
ameloblastic
fibro-
Report of a Case A 7-year-old Chinese boy was referred by an oral surgeon because of a swelling of the right maxilla first noticed 6 months previously and, according to the parents, slowly growing. The right cheek was grossly deformed, with normal overlying skin. There was no paresis or hypoesthesia in the affected area and no nasal discharge. The boy had a mixed dentition. The right maxilla was swollen on its buccal aspect from the first premolar to the region of the tuberosity; the palatal vault was not deformed. Standard radiographs showed a tumor approximately 7 cm in diameter, expanding the right maxilla and extending cranially into the orbital floor and dorsally into the pterygoid region. Small calcifications were scattered throughout what looked like a solid lesion. An incisional biopsy gave a picture consistent with a fibromyxoma. An axial and coronal computed tomography (CT) scan without contrast (Fig 1) was performed to assess the extent of the tumor. The radiographs showed a large soft tissue mass over the right maxilla, expanding into the maxillary antrum and bulging into the lower portion of the right infratemporal fossa. Destruction of the right maxil-
PERIOSTEAL
19. Schultz W, Whitten JB: Mucoepidermoid carcinoma in the mandible: Report of a case. J Oral Surg 27:337, 1969 20. Frederickson C, Cherrick HM: Central mucoepidermoid carcinoma of the jaws. J Oral Med 33:80, 1978 21. Unal T, Zeytinoglu B: Central mucoepidermoid carcinoma of the jaws. J Oral Med 40:202, 1985 22. Pincock JL, El-Mofty SK: Recurrence of cystic central mucoepidermoid tumor of the mandible. Int J Oral Surg 14:81, 1985 23. Stoch RB, Smith I: Mucoepidermoid carcinoma of the mandible: Report of case. J Oral Surg 3856, 1980 24. Lepp H, cited in Hertz J: Mucus secreting tumors of the jaws. Acta Chir Stand 103:276, 1952 25. Hertz J: Mucus secreting tumors of the jaws. Acta Chir Stand 103:276, 1952
J Oral Maxillofac 46:522-526,
OSTEOSARCOMA
OF THE MAXILLA
26. Sonesson A: Mucus-secreting and cystic epidermoid carcinoma of the jaws. Acta Radio1 34:25, 1950 27. Szerlip L: Cystic mucoepidermoid tumor. Report of case. Oral Sure 9:584. 1956 28. Rzucidlo i?B, Rzeszotko-Adamiczka D: Uber einen mucoepidermoid tumors eines Unterkiefers. Zentralbl Allg Pathol 105:409, 1964 29. Attenson M, Kaufman M, Brown S: Mucoepidermoid carcinoma of the mandible: Report of a case. J Oral Surg 22:350, 1964 30. Lacour M, Reynaud J, Grappin G, et al: A case of mucoepidermoid tumors of the mandible. Bull Sot Med Afr Lang Fr 11:73, 1966 31. Karmarkar SR: Central mucoepidermoid tumors of the mandible. Indian J Surg 31: 154, 1969
Surg
1990
Perios teal Osteosarcoma
of the Maxilla:
A Case Report and Review of Literature LTC ADRIAN PATTERSON, DDS, DC, USA,* ROBERT 0. GREER JR, DDS, ScD,t AND DANIEL HOWARD, DDSS
Juxtacortical osteosarcoma was first fully described by Jaffee and Selin’ in 1951, although Geschickter and Copeland had described a similar lesion under the heading “parosteal osteoma” in 1949.*s3 Since these initial reports, juxtacortical osteosarcoma has been described using various names, including ossifying parosteal osteogenic sarcoma, and parosteal and periosteal ossifying fibrosarcoma.4T5 Juxtacortical osteogenic sarcoma occurs much less commonly than central medullary osteosarcoma, which has an incidence rate of 0.07 per 1,000 population. The clinicopathology of fewer than a dozen cases of juxtacortical osteosarcoma has been
* Chief, Surgery Branch, United States Institute of Dental Research, Department of the Army, Walter Reed Army Medical Center, Washington, DC. t Professor and Chairman, Division of Oral Pathology, University of Colorado School of Dentistry; Professor of Pathology, University of Colorado School of Medicine, Denver. $ In private practice, Washington, DC. The opinions or assertations contained herein are the private views of the authors and are not to be construed as official or as reflecting the view of the Department of the Army. Address correspondence and reprint requests to LTC Patterson: Surgery Branch, US Institute of Dental Research, Department of the Army, Walter Reed Army Medical Center, Washington, DC 20307-5300. 0 1990 American
Association
geons 0278-2391/90/4805-0018$3.00/O
of Oral
and Maxillofacial
Sur-
well documented in the English literature (Table l)? Recently, Zarbo et al’* suggested that juxtacortical osteosarcoma can be further subdivided into two clinicopathologic variants: parosteal osteosarcoma and periosteal osteosarcoma. The purpose of this article is to report a rare example of maxillary juxtacortical osteosarcoma of the periosteal type and to discuss the clinicopathologic nature of that disease process. Report of a Case A 65year-old black woman was referred by her general dentist for evaluation and treatment of a mass in the right maxillary vestibule and alveolus. The lesion had been present and slowly enlarging for approximately 1 year. It had been asymptomatic until about 3 weeks before her visit, when the patient began to have mild pain in the maxillary right quadrant. Physical examination revealed a 2.1 x 2.5 x 2 cm hard mass in the right maxillary vestibule (Fig 1). The lesion was lobular in appearance. The gingiva overlying the lesion showed increased vascularity, but no ulceration. The buccal vestibule was nearly obliterated by the lesion in the region of the premolar and canine teeth. There was neither paresthesia or anesthesia of the infraorbital nerve. A panoramic radiograph showed a slightly radiopaque shadow approximately 0.5 x 0.5 cm distal to the premolar (Fig 2), as well as a longitudinal fracture of this tooth. There was also a loss of lamina dura around the involved teeth. Neck examination was negative for tenderness and adenopathy. An excisional biopsy was performed under local anes-
PATTERSON,
523
GREER, AND HOWARD
Table 1. Cases of Juxtacortical Osteosarcoma Affecting the Jaws Sex/Age
Authors Som and Peimerh Rota et al’
M/45
Left mandible
F/17 Ml63
Midline of maxilla Right mandible
Solomon et al”
Ml38
Right mandible
Newland and AyalaY Banejee” Adekeye et al’ ’ Zarbo et al” Bras et alI3 Regezi et alI4
Ml25 F/47 F/17 M/57 M/40 F/13 Ml68
Left maxilla Left mandible Right mandible Left mandible Left mandible Posterior maxilla Posterior maxilla
Abbreviations:
Treatment
Site
Follow-up
Multiple local resections, followed by hemimandibulectomy Local and partial resection Several local resections, followed by right mandibulectomy Local excision, followed by hemimandibulectomy Local resection Enbloc resection Enucleation Hemimandibulectomy Hemimandibulectomy Excision Excision
7 yr, no recurrence 9 yr, no recurrence 6 yr. DOC, no recurrence 6 mo. no recurrence 6 mo, no recurrence 8 yr. no recurrence NA I yr. no recurrence 11yr, no recurrence 5 yr, no recurrence 2 yr. 2 recurrences; DOC
DOC. died of other causes; NA. not available
thesia. A full-thickness mucoperiosteal flap was raised exposing the lesion. The covering gingiva and mucoperiosteum separated easily from the lesion. A portion of the lesion was easily removed and submitted for histologic examination along with a piece of the buccal alveolar
bone. The premolar was extracted. The enced an uneventful recovery. Following a diagnosis of juxtacortical the patient was referred to Walter Reed Center for evaluation and definitive care. derwent a partial hemimaxillectomy and currence of disease after 1 year.
patient experiosteosarcoma, Army Medical The patient unhas had no re-
well-organized trabeculae of lamellar bone that blended with the cortical plate of the maxilla. The stroma was richly cellular and composed predominantly of spindle cells. Distributed throughout the stroma were atypical spindle-shaped and polyhedral cells with hyperchromatic nuclei consistent with malignant osteoblasts (Fig 3) and an abundant malignant chondroid matrix (Fig 4). Malignant tumor osteoid radiated between chondroid and osseous trabeculae. Mitotic figures were common within the chondroid matrix, two to three per high-power field. The excised material showed extension of tumor to the tissue margins. A diagnosis of juxtacortical (periosteal) osteosarcoma was made.
GROSS PATHOLOGY
The original excisional biopsy tissue sample consisted of several tooth fragments that measured 2.8 x 2 x 1 cm and glistening, granular, gritty, somewhat polypoid pieces of soft tissue that measured 2.7 x 2.5 x 2 cm.
Discussion
Microscopic examination of the short-term decalcified tissue specimen showed a tumor that was composed of
Primary osteosarcoma of the mandible and maxilla is exceedingly rare, both in terms of absolute incidence and the frequency of sarcomas in the entire skeleton. Juxtacortical osteosarcoma is even more unique (Table 1). Adekeye et al” reviewed a
FIGURE 1. Broad-based lobular tumor mass in the right maxillary vestibule.
FIGURE 2. premolar.
HISTOPATHOLOGY
Focal radiopacity distal to maxillary right second
524
PERIOSTEAL
OSTEOSARCOMA
OF THE MAXILLA
FIGURE 3. High-power photomicrograph showing malignant tumor osteoid (arrow) arising adjacent to pleomorphic osteoblasts. (Hematoxylin-eosin stain. Original magnification, X260.)
series of I5 primary and I metastatic osteosarcoma of the jaw and identified only one juxtacortical osteosarcoma, a parosteal variant that was treated by enucleation. Follow-up data on the patient were not presented. Regezi et a1’4 described the clinical, microscopic, and immunohistochemical characteristics of 17 osteosarcomas and chondrosarcomas in a 1987 review and reported only 2 juxtacortical tumors. Both neoplasms were parosteal. One patient
FIGURE 4. Predominantly cartilaginous neoplasm demonstrating spindle cell stroma and malignant chondroid matrix with atypical chondroid differentiation. (Hematoxylineosin stain. Original magnification, X260.)
was a 13-year-old girl who showed no signs or recurrence 5 years after complete surgical excision of her tumor. The second patient, a 68-year-old man, developed two recurrences in the 2 years following excision of his tumor and died tumor free 5 years later of other causes. Some investigators separate juxtacortical osteosarcoma into low-grade (parosteal) and highgrade (periosteal) variants (Table 2).15,16 Zarbo et
PATTERSON,
Table 2.
525
GREER, AND HOWARD
Distinguishing Features of Periosteal and Parosteal Osteosarcoma of the Jaws Clinical Features
Radiographic Features
Histologic Features
Male predominance
Lobular, with well-defined periphery
Cortex intact, with no marrow involvement
Male predominance
III defined; tends to overgrow base of origin
No radiographic continuity; may show medullary involvement
Predominantly composed of poorly differentiated malignant cartilaginous tissue Spindle cell stroma with mild cytologic atypia
Lesion
Age
Sex
Periosteal osteosarcoma
Peak occurrence at 20 yr
Parosteal osteosarcoma
Peak occurrence at 39 yr
al” have suggested that such a distinction is warranted because of the distinctive clinical, microscopic, and behavioral characteristics unique to each tumor. Periosteal osteosarcoma is the least common variant. de Santos et alI7 have reported a 2-to-1 male predominance in long bones and a peak occurrence at about age 20 years for the periosteal varient. None of the tumors reported by Regezi and associates’4 or in the African series of Adekeye et al” were periosteal tumors. Only 1 of 23 periosteal osteosarcomas in Dahlin’s series” involved the mandible. While parosteal osteosarcoma occurs over a broad age range that peaks at 39 years, l7 periosteal osteosarcoma occurs a decade or two later. Radiographic, gross morphologic, and histologic features serve to further separate the two variants of juxtacortical osteosarcoma. The cortex in periosteal osteosarcoma is radiographically intact and sometimes thickened, with no involvement of the underlying marrow cavity. Grossly, the tumor is most often lobulated and well defined, with little evidence of infiltration into the surrounding soft tissue. Unlike parosteal osteosarcoma, it is exceedingly rare for periosteal osteosarcoma to overgrow its base of origin. The histologic differences between periosteal and parosteal forms of the neoplasm are dramatic. Periosteal osteosarcoma is predominantly a cartilaginous neoplasm, whereas parosteal osteosarcoma is characterized by a spindle cell stroma with mild to moderate atypia and rare mitotic figures dispersed between irregular osseous trabeculae. Fine, lacelike osteoid can be found in the chondroid islands of periosteal osteosarcoma, thereby distinguishing it from juxtacortical chondrosarcoma. Unni et alI9 report that in long-bone sites the prognosis for periosteal osteosarcoma is considerably more favorable than for conventional central osseous osteosarcoma, but poorer than that for
Prognosis Capable of local recurrence and distant metastasis
Minimal metastatic potential
parosteal osteosarcoma. The small group of juxtacortical osteosarcomas of the jaws reported in the literature show a much better prognosis than their long-bone counterparts. However, because the number of cases reported is so small, the exact prognosis remains unclear. The bland microscopic appearance of parosteal osteosarcoma may lead to a microscopic diagnosis of osteoma, osteochondroma, heterotopic ossification, or myositis ossificans. Chondroma or chondroid choristoma must also be considered in the differential microscopic diagnosis of periosteal osteosarcoma, and a diagnosis of juxtacortical osteosarcoma requires complete synthesis of clinical, radiographic, and histologic features of the tumor. Local excision is considered to be an inadequate form of treatment. Surgical therapy requires al least an adequate margin of tissue, hemimandibulectomy, or wide maxillary resection. Summary
Juxtacortical osteosarcomas, including periosteal and parosteal subtypes, are uncommon neoplasms of the jaws. In long bones, the biologic behavior of the periosteal variant is reported to be more aggressive than the parosteal tumor. Juxtacortical osteosarcoma of the jaws appears to have a slightly more favorable prognosis than in long bones, although a larger series of cases needs to be evaluated before a definitive conclusion concerning the behavior of juxtacortical osteosarcoma of the jaws can be made. References 1. Jaffe HL, Selin G: Tumors of bone and joints. Bull NY Acad Med 27:165, 1951 2. Geschickter CF, Copeland MM: Tumors of Bone (ed 3). Philadelphia, PA, Lippincott, 1949, pp 367-384
526
MAXILLARY
3. Geschickter CF, Copeland MS: Parosteal osteoma of bone: A new entity. Ann Surg 133:790, 1951 4. Dwinnell LA, Dahlin DC, Ghormley RK: Parosteal (juxtacortical) osteogenic sarcoma. J Bone Joint Surg 36A:732, 1954 5. Lichtenstein L: Tumors of periosteal origin. Cancer 8: 1060, 1955 6. Som M, Peimer R: Juxtacortical osteogenic sarcoma of the mandible. Arch Otolaryngol 74:532, 1961 7. Rota AN, Smith JL, Jing B: Osteosarcoma and parosteal osteogenic sarcoma of the maxilla and mandible: Study of 20 cases. Am J Clin Path01 54:625, 1970 8. Solomon MP, Biemacki J, Slippen M, et al: Parosteal osteogenic sarcoma of the mandible. Arch Otolaryngol 101:754, 1975 9. Newland JR, Ayala AG: Parosteal osteosarcoma of the maxilla. Oral Surg 43:727, 1977 10. Banejee SC: Juxtacortical osteosarcoma of the mandible: Review of the literature and report of a case. J Oral Surg 39:535, 1981 11. Adekeye EO, Chau KK, Edwards MB, et al: Osteosarcoma
J Oral Maxillofac 46526-530.
12. 13.
14.
15. 16. 17.
18. 19.
AMELOBLASTIC
FIBRO-ODONTOMA
of the jaws-A series from Kaduna, Nigeria. J Oral Surg 16205, 1987 Zarbo RJ, Regezi JA, Baker SR: Periosteal osteogenic sarcoma of the mandible. Oral Surg 57643, 1984 Bras JM, Donner R, Van Der Kwast WAM, et al: Juxtacortical osteogenic sarcoma of the jaws. Review of the literature and report of a case. Oral Surg 50:535, 1980 Regezi JA, Zarbo RJ, McClatchey KD, et al: Osteosarcomas and chondrosarcomas of the jaws: Immunohistochemical correlations. Oral Surg 64:302, 1987 Dahlin DC, Unni KK: Osteosarcoma of bone and its important recognizable varieties. Am J Surg Path01 1:61, 1977 Huvos AG: Bone tumors: Diagnosis, treatment, and prognosis. Philadelphia, PA, Saunders, 1970 deSantos LA, Murray JA, Finklestein JB, et al: The radiographic spectrum of periosteal osteosarcoma. Radiology 127:123, 1978 Dahlin DC: Bone tumors: General aspects and data on 6,221 cases (ed 3). Springfield, IL, Thomas, 1978 Unni KK, Dahlin BC, Beabout JW, et al: Parosteal osteogenie sarcoma. Cancer 37:2455, 1976
Surg
1990
Massive Maxillary Ameloblastic Fibro-Odon toma: Case Report With Surgical Management E.M.G. PIETTE, MD, BDS,* H. TIDEMAN, DDS, MD, PHD,t AND P.C. WU, MD, FRCPATH, FRCPAS
The ameloblastic fibro-odontoma is a rare mixed odontogenic tumor, described under a variety of terms until Hooker’ separated it from ameloblastic odontomas and described it as a separate entity. This tumor has been classified as an ameloblasticectomesenchymal tumor by Reichart and Ries.’ Slootweg3 reviewed the international literature between 1908 and 1980 and found 55 cases consistent with the diagnosis, together with his own cases. The purpose of this report is to describe a new unusual Received from The Prince Philip Dental Hospital, University of Hong Kong, Hong Kong. * Senior Lecturer, Department of Oral Surgery and Oral Med-
icine . ? Professor and Head, Department of Oral Surgery and Oral Medicine. $ Reader, Department of Pathology, Oral Pathology Unit. Address correspondence and reprint requests to Dr Piette: Department of Oral Surgery and Oral Medicine, The Prince Philip Dental Hospital, University of Hong Kong, 34 Hospital Rd, Hong Kong. 0 1990 American Association of Oral and Maxillofacial geons 0278-2391/90/4805-0019$3.00/0
Sur-
case of voluminous odontoma.
maxillary
ameloblastic
fibro-
Report of a Case A 7-year-old Chinese boy was referred by an oral surgeon because of a swelling of the right maxilla first noticed 6 months previously and, according to the parents, slowly growing. The right cheek was grossly deformed, with normal overlying skin. There was no paresis or hypoesthesia in the affected area and no nasal discharge. The boy had a mixed dentition. The right maxilla was swollen on its buccal aspect from the first premolar to the region of the tuberosity; the palatal vault was not deformed. Standard radiographs showed a tumor approximately 7 cm in diameter, expanding the right maxilla and extending cranially into the orbital floor and dorsally into the pterygoid region. Small calcifications were scattered throughout what looked like a solid lesion. An incisional biopsy gave a picture consistent with a fibromyxoma. An axial and coronal computed tomography (CT) scan without contrast (Fig 1) was performed to assess the extent of the tumor. The radiographs showed a large soft tissue mass over the right maxilla, expanding into the maxillary antrum and bulging into the lower portion of the right infratemporal fossa. Destruction of the right maxil-
