7
2. Barbieri M, Gentile R, Cordone MP, et al: Primitive malignant melanoma of the parotid gland. ORL J Otorhinolaryngol Relat Spec 64:297, 2002 3. O’Brien CJ, McNeil EB, McMahon JD, et al: Incidence of cervi- cal node involvement in metastatic cutaneous malignancy in- volving the parotid gland. Head Neck 23:744, 2001 4. Balm AJ, Kroon BB, Hilgers FJ, et al: Lymph node metastases in the neck and parotid gland from an unknown primary mela- noma. Clin Otolaryngol Allied Sci 19:161, 1994 5. Prayson RA, Sebek BA: Parotid gland malignant melanomas. Arch Pathol Lab Med 124:1780, 2000 6. Batsakis JG, Bautina E: Metastases to major salivary glands. Ann Otol Rhinol Laryngol 99:501, 1990 7. Santini H, Byers RM, Wolf PF: Melanoma metastatic to cervical and parotid nodes from an unknown primary site. Am J Surg 150:510, 1985 8. Greene GW, Bernier JL: Primary malignant melanomas of the parotid gland. Oral Surg Oral Med Oral Pathol 14:108, 1961 9. Takeda Y: Melanocytes in the human parotid gland. Pathol Int 47:581, 1997 10. Woodwards RT, Shepherd NA, Hensher R: Malignant mela- noma of the parotid gland: A case report and literature review. Br J Oral Maxillofac Surg 31:313, 1993 11. Bahar M, Anavi Y, Abraham A, et al: Primary malignant mela- noma in the parotid gland. Oral Surg Oral Med Oral Pathol 70:627, 1990 12. Vuong PN, Cassembon F, Houissa-Vuong S, et al: Primary ma- lignant melanoma of the parotid gland: Anatomico-clinical study: Apropos of a case and review of the medical literature. Ann Otolaryngol Chir Cervicofac 103:45, 1986 13. Martins AS, Souza AL, Souza LS, et al: Surgical procedures for primary, metastatic or adjacent parotid tumours. Int Surg 84: 318, 1999 14. Batsakis JG: Pathology consultation: Parotid gland and its lymph nodes as metastatic sites. Ann Otol Rhinol Laryngol 92:209, 1983 15. Bron LP, Traynor SJ, McNeil EB, et al: Primary and metastatic cancer of the parotid: Comparison of clinical behavior in 232 cases. Laryngoscope 113:1070, 2003 16. Ballo MT, Garden AS, Myers JN, et al: Melanoma metastatic to cervical lymph nodes: Can radiotherapy replace formal dissec- tion after local excision of nodal disease? Head Neck 27:718, 2005 17. Ang KK, Byers RM, Peters LJ, et al: Regional radiotherapy as adjuvant treatment for head and neck malignant melanoma: Preliminary results. Arch Otolaryngol Head Neck Surg 116:169, 1990 18. Lotem M, Peretz T, Drize O, et al: Autologous cell vaccine as a post operative adjuvant treatment for high-risk melanoma pa- tients (AJCC stages III and IV): The new American Joint Com- mittee on Cancer. Br J Cancer 86:1534, 2002 J Oral Maxillofac Surg 66:813-819, 2008 Primary Intraosseous Malignancy Originating in an Odontogenic Cyst in a Young Child Makepeace Charles, BSc, MEd, DDS,* Torin Barr, BSc, DDS,† Iona Leong, BDS, MSc, FRCDC,‡ Bo Yee Ngan, MD, PhD, FRCPC,§ Vito Forte, MD, FRCSC, and George K.B. Sándor, MD, DDS, PhD, FRCDC, FRCSC¶ Primary intraosseous squamous cell carcinomas (PIOSCCs) are uncommon jaw malignancies de- rived from odontogenic epithelial remnants. The World Health Organization’s classification of primary intraosseous carcinomas distinguishes among solid-type carcinomas, carcinomas originating from keratocystic odontogenic tumors (odontogenic keratocyst), and car- cinomas derived from odontogenic cysts other than ker- *Chief Resident, Division of Oral and Maxillofacial Surgery and Anaesthesia, Mount Sinai Hospital, University of Toronto, Toronto, Ontario, Canada. †Senior Resident, Division of Oral and Maxillofacial Surgery and Anaesthesia, University of Toronto, Toronto, Ontario, Can- ada. ‡Assistant Professor, Department of Oral Pathology, University of Toronto, Departments of Dentistry and Pathology and Laboratory Medicine, Mount Sinai Hospital, Toronto, Ontario, Canada. §Assistant Professor, Department of Pediatric Laboratory Medi- cine, Division of Pathology, University of Toronto and The Hospital for Sick Children, Toronto, Ontario, Canada. Otolaryngologist-in-Chief, The Hospital for Sick Children, Pro- fessor, University of Toronto, Toronto, Ontario, Canada. ¶Director, Graduate Program in Oral and Maxillofacial Surgery and Anesthesia, Mount Sinai Hospital; Coordinator, Pediatric Oral and Maxillofacial Surgery, The Hospital for Sick Children and Bloor- view Kids Rehab, Toronto, Ontario, Canada; Professor of Oral and Maxillofacial Surgery, University of Toronto, Toronto, Ontario, Can- ada; Professor, Regea Institute for Regenerative Medicine, Univer- sity of Tampere, Tampere, Finland; Docent in Oral and Maxillofacial Surgery, University of Oulu, Oulu, Finland. Address correspondence and reprint requests to Dr Sándor: The Hospital for Sick Children, S-525, 555 University Avenue, Toronto, Ontario, Canada M5G 1X8; e-mail: [email protected] © 2008 American Association of Oral and Maxillofacial Surgeons 0278-2391/08/6604-0031$34.00/0 doi:10.1016/j.joms.2007.10.005 CHARLES ET AL 813

Primary Intraosseous Malignancy Originating in an Odontogenic Cyst in a Young Child

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2. Barbieri M, Gentile R, Cordone MP, et al: Primitive malignantmelanoma of the parotid gland. ORL J Otorhinolaryngol RelatSpec 64:297, 2002

3. O’Brien CJ, McNeil EB, McMahon JD, et al: Incidence of cervi-cal node involvement in metastatic cutaneous malignancy in-volving the parotid gland. Head Neck 23:744, 2001

4. Balm AJ, Kroon BB, Hilgers FJ, et al: Lymph node metastases inthe neck and parotid gland from an unknown primary mela-noma. Clin Otolaryngol Allied Sci 19:161, 1994

5. Prayson RA, Sebek BA: Parotid gland malignant melanomas.Arch Pathol Lab Med 124:1780, 2000

6. Batsakis JG, Bautina E: Metastases to major salivary glands. AnnOtol Rhinol Laryngol 99:501, 1990

7. Santini H, Byers RM, Wolf PF: Melanoma metastatic to cervicaland parotid nodes from an unknown primary site. Am J Surg150:510, 1985

8. Greene GW, Bernier JL: Primary malignant melanomas of theparotid gland. Oral Surg Oral Med Oral Pathol 14:108, 1961

9. Takeda Y: Melanocytes in the human parotid gland. Pathol Int47:581, 1997

10. Woodwards RT, Shepherd NA, Hensher R: Malignant mela-noma of the parotid gland: A case report and literature review.Br J Oral Maxillofac Surg 31:313, 1993

11. Bahar M, Anavi Y, Abraham A, et al: Primary malignant mela-noma in the parotid gland. Oral Surg Oral Med Oral Pathol70:627, 1990

12. Vuong PN, Cassembon F, Houissa-Vuong S, et al: Primary ma-lignant melanoma of the parotid gland: Anatomico-clinicalstudy: Apropos of a case and review of the medical literature.Ann Otolaryngol Chir Cervicofac 103:45, 1986

13. Martins AS, Souza AL, Souza LS, et al: Surgical procedures forprimary, metastatic or adjacent parotid tumours. Int Surg 84:318, 1999

14. Batsakis JG: Pathology consultation: Parotid gland and itslymph nodes as metastatic sites. Ann Otol Rhinol Laryngol92:209, 1983

15. Bron LP, Traynor SJ, McNeil EB, et al: Primary and metastaticcancer of the parotid: Comparison of clinical behavior in 232cases. Laryngoscope 113:1070, 2003

16. Ballo MT, Garden AS, Myers JN, et al: Melanoma metastatic tocervical lymph nodes: Can radiotherapy replace formal dissec-tion after local excision of nodal disease? Head Neck 27:718,2005

17. Ang KK, Byers RM, Peters LJ, et al: Regional radiotherapy asadjuvant treatment for head and neck malignant melanoma:Preliminary results. Arch Otolaryngol Head Neck Surg 116:169,1990

18. Lotem M, Peretz T, Drize O, et al: Autologous cell vaccine as apost operative adjuvant treatment for high-risk melanoma pa-tients (AJCC stages III and IV): The new American Joint Com-mittee on Cancer. Br J Cancer 86:1534, 2002

J Oral Maxillofac Surg66:813-819, 2008

Primary Intraosseous MalignancyOriginating in an Odontogenic

Cyst in a Young ChildMakepeace Charles, BSc, MEd, DDS,* Torin Barr, BSc, DDS,†

Iona Leong, BDS, MSc, FRCDC,‡

Bo Yee Ngan, MD, PhD, FRCPC,§ Vito Forte, MD, FRCSC,�and George K.B. Sándor, MD, DDS, PhD, FRCDC, FRCSC¶

Primary intraosseous squamous cell carcinomas(PIOSCCs) are uncommon jaw malignancies de-rived from odontogenic epithelial remnants. TheWorld Health Organization’s classification of primary

intraosseous carcinomas distinguishes among solid-typecarcinomas, carcinomas originating from keratocysticodontogenic tumors (odontogenic keratocyst), and car-cinomas derived from odontogenic cysts other than ker-

*Chief Resident, Division of Oral and Maxillofacial Surgery and

Anaesthesia, Mount Sinai Hospital, University of Toronto, Toronto,

Ontario, Canada.

†Senior Resident, Division of Oral and Maxillofacial Surgery

and Anaesthesia, University of Toronto, Toronto, Ontario, Can-

ada.

‡Assistant Professor, Department of Oral Pathology, University of

Toronto, Departments of Dentistry and Pathology and Laboratory

Medicine, Mount Sinai Hospital, Toronto, Ontario, Canada.

§Assistant Professor, Department of Pediatric Laboratory Medi-

cine, Division of Pathology, University of Toronto and The Hospital

for Sick Children, Toronto, Ontario, Canada.

�Otolaryngologist-in-Chief, The Hospital for Sick Children, Pro-

fessor, University of Toronto, Toronto, Ontario, Canada.

¶Director, Graduate Program in Oral and Maxillofacial Surgery

and Anesthesia, Mount Sinai Hospital; Coordinator, Pediatric Oral

and Maxillofacial Surgery, The Hospital for Sick Children and Bloor-

view Kids Rehab, Toronto, Ontario, Canada; Professor of Oral and

Maxillofacial Surgery, University of Toronto, Toronto, Ontario, Can-

ada; Professor, Regea Institute for Regenerative Medicine, Univer-

sity of Tampere, Tampere, Finland; Docent in Oral and Maxillofacial

Surgery, University of Oulu, Oulu, Finland.

Address correspondence and reprint requests to Dr Sándor: The

Hospital for Sick Children, S-525, 555 University Avenue, Toronto,

Ontario, Canada M5G 1X8; e-mail: [email protected]

© 2008 American Association of Oral and Maxillofacial Surgeons

0278-2391/08/6604-0031$34.00/0

doi:10.1016/j.joms.2007.10.005

CHARLES ET AL 813

atocystic odontogenic tumors.1 Most PIOSCCs associ-ated with cysts are well-differentiated squamous cellcarcinomas arising from residual periapical cysts anddentigerous cysts; PIOSCCs originating from odonto-genic keratocysts and orthokeratinized odontogeniccysts are less common.1,2 Carcinomas arising from alltypes of odontogenic cysts occur more frequently inmen, and most patients are older adults,2-8 with a meanreported age of occurrence of 57 to 61 years.2

We present an unusual case of a tumor resemblinga primary intraosseous carcinoma arising from anodontogenic cyst in a 5-year-old girl, with 7 years offollow-up after treatment.

Report of a Case

In 1999, a 5-year-old Nigerian female immigrant to Can-ada presented to an oral and maxillofacial surgeon with aright mandibular swelling that had been present for 1 week.The swelling was centered around the primary first andsecond molars and was not associated with paresthesia,pain, tooth mobility, or systemic symptoms. The patient’smedical history was noncontributory; she was otherwisehealthy, achieving all developmental milestones. Extraorally,the right mandibular mass produced no facial asymmetryand was firm and nontender on palpation. All cranial nerveswere intact. No restriction of mouth opening was noted.The overlying skin was normal. The submandibular nodeswere firm, nontender, and palpable bilaterally.

Intraorally, the right mandibular alveolus was slightlyexpanded buccally into the vestibule in the area of thedeciduous first and second molars. The deciduous first mo-lar was mobile and had been displaced buccally. The over-lying mucosa was intact and nonerythematous. A panoramicradiograph and intraoral films revealed an enlarged crypt ofthe developing right mandibular first premolar (Figs 1, 2). The

crypt of the nearby right mandibular second premolar wasdisplaced posteriorly, and advanced resorption of the rootsof the right deciduous first and second molar teeth wasnoted. The radiographic features were suggestive of a moreaggressive lesion than an odontogenic cyst.

An incisional biopsy was done within 2 days of the initialpresentation, including removal of the first and seconddeciduous molars and the developing permanent first pre-molar tooth. The biopsy revealed that the lesional tissueconsisted grossly of tan-brown soft tissue, part of which wasattached to the cementoenamel junction of the developingpremolar tooth. Histological examination showed multiplefoci resembling poorly differentiated squamous cell carci-noma arising abruptly from a cyst lined by benign stratifiedsquamous nonkeratinized epithelium exhibiting neitherdysplasia nor ameloblastic differentiation (Fig 3A). The tu-mor infiltrated the fibrous tissue of the cyst wall and wascomposed primarily of islands, cords, and nests of cohesiveround cells (Fig 3B). Occasional small tumor islands dem-onstrated squamous differentiation (Fig 3C), whereas othersshowed microcyst formation and central necrosis. Most ofthe neoplastic cells were medium-sized, with scant cyto-plasm and large central nuclei containing finely stippledchromatin and occasional nucleoli. Numerous mitotic fig-ures were present (3 per high-power field; �400 originalmagnification). The malignant cells contained dot-like cyto-plasmic inclusions of periodic acid-Schiff–positive, diastase-resistant cytoplasmic granules, consistent with glycopro-tein. Larger melanin-laden cells, some polygonal and othersdendritic in shape, were located in close proximity to thetumor islands and within the tumor islands, both singly andin clusters (Fig 3D). The tissue stained positive for epithelialmembrane antigen (EMA) (Fig 3E). The surface mucosa wasnot seen in the sections. The cyst lining and most of thetumor cells were positive for pancytokeratin (Fig 3F) andlow molecular weight cytokeratin (Fig 3G). Focal tumor cellexpression of neuron-specific enolase and CD99 was seen(Fig 3H). The tumor cells did not express vimentin, HMB45,S100, desmin, actin, myogenin, neurofilaments, or chromo-granin.

Approximately 2 weeks after the initial presentation andbiopsy, the swelling had increased to 2 cm in diameter,associated with serosanguinous drainage from the posterioraspect of the lesion near the biopsy site. Panoramic radio-

FIGURE 1. Panoramic radiograph showing a lesion involving thedeveloping tooth bud of the mandibular right first premolar with ex-pansion of the contents of the crypt. Note the mesial displacement ofthe deciduous canine, the distal displacement of the developing sec-ond premolar tooth bud, and the advanced root resorption of both thefirst and second deciduous molars.

Charles et al. Primary Intraosseous Malignancy in an Odonto-genic Cyst. J Oral Maxillofac Surg 2008.

FIGURE 2. Occlusal radiograph of the right mandibular body andparasymphysis showing a large lytic lesion with displacement of theright first deciduous molar.

Charles et al. Primary Intraosseous Malignancy in an Odonto-genic Cyst. J Oral Maxillofac Surg 2008.

814 PRIMARY INTRAOSSEOUS MALIGNANCY IN AN ODONTOGENIC CYST

graph and intraoral films taken at that time revealed anill-defined radiolucency of the right mandible extendingfrom the deciduous canine to the socket of the deciduoussecond molar (Fig 4A). A mandibular periosteal responsewith reactive bone could be seen (Fig 4B).

Definitive treatment was delayed for 1 month due tofamily reasons. A computed tomography (CT) scan showedthe previous biopsy site and a wide transition zone betweenthe biopsy site and normal bone with the lesion extendingposteriorly beyond the developing molars (Fig 5). This wasbelieved to represent an infiltrative process of the mandible.The CT scan also revealed bilateral jugolodigastric nodeenlargement. The biopsy results were reviewed and a met-astatic workup was completed, which confirmed no pri-mary tumor elsewhere.

A subtotal right hemimandibulectomy was performed ex-tending from the contralateral left first premolar site to theramus of the right mandible with modified bilateral radicalneck dissection through a lip-splitting approach (Fig 6). Themandibular continuity was reestablished with a reconstruc-tion plate bridging the condylar stump to the remaining leftmandible (Figs 7A,B). The final specimen grossly appeared

as a segment of mandible containing all of the remainingright mandibular deciduous and permanent teeth up to theleft permanent canine. The mucosa covering the mandibleremained nonulcerated. There was no continuity of thetumor with the overlying skin or mucosa. The specimencontained no residual cyst, only tissue similar to the intraos-saeous carcinoma noted on the previous incisional biopsy.The hard and soft tissue margins were negative. Both thesubmandibular and jugulodigastric nodes were positive fortumor bilaterally. No necrosis of the involved lymph nodeswas noted.

The patient received a course of postoperative radiationtherapy comprising 60 Gy in 30 fractions to the head andupper cervical area. During frequent postoperative fol-low-up over the past 7 years, the patient has shown noclinical or radiographic signs of tumor recurrence. Herosseous reconstruction has been delayed to allow surveil-lance of her neck and mandibulectomy sites. The sole com-plication has been a nondisplaced fracture of the recon-struction plate, which the patient and her family havechosen to not replace at this time.

FIGURE 3. A, Cyst with carcinoma in the wall. (Hematoxylin and eosin; original magnification � 200.) B, Islands and infiltrative cords ofcarcinoma cells with cohesive round cells and early squamoid differentiation. (Hematoxylin and eosin; original magnification �100.) C, Carcinomawith focal squamous differentiation. (Hematoxylin and eosin; original magnification �400.) D, Multiple mitoses with view of nuclear detail andmelanin. (Hematoxylin and eosin; original magnification �400.) (Figure 3 continued on next page.)

Charles et al. Primary Intraosseous Malignancy in an Odontogenic Cyst. J Oral Maxillofac Surg 2008.

CHARLES ET AL 815

DiscussionTo date, fewer than 50 cases of PIOSCC originating

from an odontogenic cyst other than keratocysticodontogenic tumor have been reported.1 Our case isunusual in the following respects: young age of thepatient, poor differentiation of the tumor, and associ-ation of the tumor with melanocytes and melaninpigmentation. The cyst of origin was a dentigerouscyst, based on the finding of cyst attachment to thecementoenamel junction of the unerupted premolar.The diagnosis was based on the histopathologic find-ing of significant amounts of benign cyst lining fromwhich foci resembling poorly differentiated carci-noma were seen to originate, which excluded cysticdegeneration of a squamous cell carcinoma. Featuresof ameloblastic differentiation that would justify con-sideration of a diagnosis of ameloblastic carcinoma

were lacking. Origin from oral mucosa and overlyingskin and metastasis from a distant primary were clin-ically excluded.

Most cases of PIOSCC (55%) arise in residual cysts,with approximately 25% of cases originating fromdentigerous cysts.2,9 Fewer than 60 cases of squa-mous cell carcinoma arising in a dentigerous cyst havebeen reported over the last century, with a reportedage range of 37 to 90 years and an average age of 57to 64 years.7,8 Gulbranson et al4 reported the onlyother known childhood case, a 16-month-old girl.

Histopathologically, PIOSCCs are usually low-grade,well-differentiated keratinizing squamous cell carcino-mas.2,9 The etiologic factor stimulating the transitionfrom benign cyst to malignant tumor is poorly under-stood, but chronic inflammation and infection havebeen implicated.7,9 The chronic inflammatory the-

FIGURE 3 (cont’d). E, Carcinoma with nests of focal squamous differentiation showing strong EMA expression by immunohistochemical stainingfor EMA. (Original magnification �200.) F, Differentiated and some moderately differentiated tumor cells expressing pan-cytokeratin on immuno-histochemical staining for pan-cytokeratin. (Original magnification �100.) G, Tumor cells expressing low molecular weight cytokeratin onimmunohistochemical staining for low molecular weight cytokeratin. (Original magnification �200.) H, Focal tumor cell expression of CD99 seenon immunohistochemical staining for CD99. (Original magnification �400.)

Charles et al. Primary Intraosseous Malignancy in an Odontogenic Cyst. J Oral Maxillofac Surg 2008.

816 PRIMARY INTRAOSSEOUS MALIGNANCY IN AN ODONTOGENIC CYST

ory may help explain cyst and associated tumordevelopment at an advanced age at diagnosis formost patients, but clearly does not account for thecurrent pediatric case or that described by Gulbran-son et al.4

Pigmented odontogenic tumors and/or cysts arevery rare. In a review of pigmented odontogenic tu-mors, Takeda et al10 found only 30 cases reported inthe English literature, occurring in calcifying odonto-genic cysts, adenomatoid odontogenic tumors, odon-togenic keratocysts, and lateral periodontal cysts. Al-though melanocytes and melanin are found throughoutthe skin and oral mucosa, they are not normally presentin bone. This raises the question of a possible neurec-todermal origin of this neoplasm, such as a primitiveneurectodermal tumor. The presence of melanocytesin odontogenic lesions may reflect the dental laminaoriginating from the primitive oral lining or migrationof melanocytes through the mesenchyme; however,the origin of the melanocytes in odontogenic lesions

is merely speculative.10 With the exception of mel-anotic neuroectodermal tumor of infancy, racialpigmentation may be an important factor in theoccurrence of melanin-pigmented jaw lesions; infact, most patients with pigmented odontogeniclesions are Asians and blacks, as was the patient inthis case.10

Many reported cases of PIOSCC have occurred inthe mandible with symptoms of swelling, pain, andparesthesia or anesthesia of the lower lip; however,many patients are asymptomatic, with diagnosis ofcarcinoma made only after microscopic examinationof a presumed odontogenic cyst.2,11

PIOSCCs may exhibit a varying radiographic ap-pearance, presenting as unilocular or multilocular le-sions with ill-defined or well-defined but uncorticatedborders.11 In this particular case, the radiographicfeatures with indistinct borders and enlarged crypt ofthe developing first premolar and displacement of thedeciduous teeth were not typical of a dentigerouscyst.

FIGURE 5. Pretreatment CT scan showing bony infiltration from thebody of mandible toward the angle.

Charles et al. Primary Intraosseous Malignancy in an Odonto-genic Cyst. J Oral Maxillofac Surg 2008.

FIGURE 4. A, Radiograph at the time of referral after biopsy withremoval of the deciduous molars and permanent first premolar in theright mandible. The bone appears to be expanded with a periostealreaction. The developing second premolar is in an ectopic location.B, Occlusal radiograph at the time of referral with bony reaction andinfiltration with indistinct boundaries.

Charles et al. Primary Intraosseous Malignancy in an Odonto-genic Cyst. J Oral Maxillofac Surg 2008.

CHARLES ET AL 817

PIOSCCs are aggressive malignancies and may in-volve large portions of the jaws. The 2-year survivalrate is approximately 50%.12 Local recurrence hasbeen cited as the major problem in patients nottreated with radical excision.9 Metastastes to the re-gional lymph nodes have been reported but appear tobe uncommon. PIOSCCs have been reported to spreadalong the inferior alveolar canal and follow a perineuralpathway along the inferior alveolar nerve.8,13 Because ofthis infiltrative behavior, aggressive treatment, oftenhemimandibulectomy or maxillectomy, has been advo-cated.

The transition from benign cyst lining to malignanttumor may be evident only early in the developmentof the lesion and may be missed on biopsy.14 Manage-ment of odontogenic cysts may involve enucleation orpossibly en-bloc resection. This may result in under-treatment of an undiagnosed carcinoma arising in anodontogenic cyst.15 Considering the possible malig-nant nature of such cysts, we advocate biopsying any

lesion with an unusual presentation, obtaining multi-ple specimens of representative areas of the lesion.

References1. Barnes L, Eveson JW, Reichart P, et al (eds.): World Health

Organization Classification of Tumours: Pathology and Genet-ics of Head and Neck Tumours. Lyon, France, IARC Press,2005, p 290

2. Neville BW, Damm DD, Allen CM, et al: Oral and MaxillofacialPathology (ed 2). Philadelphia, Saunders, 2002, p 609

3. Norris LH, Baghaei-Rad M, Maloney PL, et al: Bilateral maxillarysquamous odontogenic tumors and the malignant transforma-tion of a mandibular radiolucent lesion. J Oral Maxillofac Surg42:827, 1984

4. Gulbranson SH, Wolfrey JD, Raines JM, et al: Squamous cellcarcinoma arising in a dentigerous cyst in a 16-month-old girl.Otolaryngol Head Neck Surg 127:463, 2002

5. Stoelinga PF, Bronkhorst FB: The incidence, multiple presen-tation and recurrence of aggressive cysts of the jaws. J Crani-omaxillofac Surg 16:184, 1987

6. Fanibunda K, Soames JV: Malignant and premalignant changesin odontogenic cysts. J Oral Maxillofac Surg 53:1469, 1995

7. Manganaro AM, Cross SE, Startzell JM: Carcinoma arising in adentigerous cyst with neck metastasis. Head Neck 19:436,1997

8. Yasuoka T, Yonemoto K, Kato Y, et al: Squamous cell carci-noma arising in a dentigerous cyst. J Oral Maxillofac Surg58:900, 2000

9. Waldron CA, Mustoe TA: Primary intraosseous carcinoma ofthe mandible with probable origin in an odontogenic cyst. OralSurg Oral Med Oral Pathol 67:716, 1989

FIGURE 7. A, Reconstruction plate bridging the gap between theright condylar stump and the remaining left mandible. B, Panoramicradiograph taken 3 years after subtotal hemimandibulectomy showingthe reconstruction plate in situ.

Charles et al. Primary Intraosseous Malignancy in an Odonto-genic Cyst. J Oral Maxillofac Surg 2008.

FIGURE 6. The mandible and neck exposed through a lip-splittingapproach to facilitate subtotal hemimandibulectomy and neckdissection.

Charles et al. Primary Intraosseous Malignancy in an Odonto-genic Cyst. J Oral Maxillofac Surg 2008.

818 PRIMARY INTRAOSSEOUS MALIGNANCY IN AN ODONTOGENIC CYST

10. Takeda Y, Sato H, Satoh M, et al: Pigmented ameloblasticfibrodentinoma: A novel melanin-pigmented intraosseousodontogenic lesion. Virchows Arch 427:454, 2000

11. Chaisuparat R, Coletti D, Kolokythas A, et al: Primary intraosse-ous odontogenic carcinoma arising in an odontogenic cyst orde novo: A clinicopathologic study of six new cases. Oral SurgOral Med Oral Pathol Oral Radiol Endod 101:194, 2006

12. Gardner AF: A survey of odontogenic cysts and their relationshipto squamous cell carcinoma. J Can Dent Assoc 41:161, 1975

13. Maxymiw WG, Wood RE: Carcinoma arising in a dentigerouscyst: A case report and review of the literature. J Oral Maxillo-fac Surg 49:639, 1991

14. Johnson LM, Sapp JP, McIntire DN: Squamous cell carcinomaarising in a dentigerous cyst. J Oral Maxillofac Surg 52:987,1994

15. Scheer M, Koch AM, Drebber U, et al: Primary intraosseouscarcinoma of the jaws arising from an odontogenic cyst: A casereport. J Craniomaxillofac Surg 32:166, 2004

J Oral Maxillofac Surg66:819-820, 2008

Mesiodens: An Unusual Cause ofa Facial Sinus

Arun Chandu, BDSc, MBBS(Hons), MDSc, FDSRCS(Eng), FRACDS(OMS),*

Mehmet Manisali, MSc, FFDRSCI, FRCS,† and

Nicholas Hyde, MS, FDSRCS, FRCS‡

Dental pathology is a common cause of chronic sinustracts in the oral and maxillofacial region.1 In mostcases, this is due to infection associated with nonvitalteeth or osteomyelitis. We report an interesting caseof a discharging facial sinus due to an unusual dentalcause: horizontally impacted mesiodens.

Report of a Case

A 19-year-old female patient of Indian extraction was re-ferred to the maxillofacial surgery department with a persis-tent, pigmented draining sinus in the philtrum region. This hadbeen causing intermittent pain for the last 6 months. She hadbeen referred to the same department 3 years before thispresentation for removal of a horizontally erupting mesiodens.As this was asymptomatic at the time, she declined treatment.Her medical history was unremarkable.

On examination, the patient had a pigmented, dischargingsinus in the philtrum region with some focal tenderness(Fig 1). A firm lump was palpable deep to this sinus via bothextraoral and intraoral examination. She had good alignmentof her upper central incisors with minimal diastema. Theremainder of the maxillofacial examination was unremarkable.

Inspection of the patient’s lateral cephalogram showed ahorizontally erupting mesiodens, just deep to the skin over

the philtrum (Fig 2). This finding was confirmed with or-thopantomogram examination. The lump palpated in herupper lip region was consistent with the erupting mesio-dens. The mesiodens was removed with the patient underlocal anesthesia. She is under long-term review to assesswhether secondary corrective surgery in the philtrum re-gion is required.

Discussion

The mesiodens is the most common of all supernu-merary teeth in humans2 and is a common cause ofmalocclusion in the anterior maxillary region.3 Al-though the most common position for a mesiodens ispalatal, usually with the crown in an inverted posi-

Received from the Maxillofacial Surgery Unit, St George’s Hospital,

London, England.

*Maxillofacial/Head and Neck Oncology Fellow.

†Consultant.

‡Lead Consultant.

Address correspondence and reprint requests to Dr Hyde: Maxillo-

facial Surgery Unit, Blackshaw Road, St George’s Hospital, Tooting,

London SW17 0QT, England; e-mail: [email protected]

© 2008 American Association of Oral and Maxillofacial Surgeons

0278-2391/08/6604-0032$34.00/0

doi:10.1016/j.joms.2007.04.032

FIGURE 1. Lateral cephalogram showing horizontal mesiodens. Theminimal overlying soft tissue shadow over the labial surface of themesiodens should be noted.

Chandu, Manisali, and Hyde. Mesiodens and a Facial Sinus.J Oral Maxillofac Surg 2008.

CHANDU, MANISALI, AND HYDE 819