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Unusual presentation of squamous cell carcinoma of the maxilla in an 8-year-old child

2016; Elsevier BV; Volume: 122; Issue: 5 Linguagem: Inglês

10.1016/j.oooo.2016.06.017

2212-4411

Marco Magalhaes, Gino R. Somers, Paula A. Sikorski, Vito Forte, Moustafa Abouzgia, Edward J. Barrett, Grace Bradley,

Head and Neck Cancer Studies

Oral squamous cell carcinoma (OSCC) is extremely rare in patients younger than 20 years of age. We present here a case of OSCC of the maxillary alveolar ridge in an otherwise healthy 8-year-old patient. The clinicohistologic presentation was not typical for mucosal SCC, and the possibility of an intrabony origin from the odontogenic epithelium was considered. The patient was treated with surgical resection, and treatment decisions were made with consideration of the need for eradication of tumor as well as tissue preservation to allow normal growth and development. A review of the literature indicated a preponderance of gingival–alveolar ridge as the site of OSCC in children with no known genetic predisposition to cancer. More studies of this rare subset of OSCC will help understand the underlying biology and guide treatment decisions. Oral squamous cell carcinoma (OSCC) is extremely rare in patients younger than 20 years of age. We present here a case of OSCC of the maxillary alveolar ridge in an otherwise healthy 8-year-old patient. The clinicohistologic presentation was not typical for mucosal SCC, and the possibility of an intrabony origin from the odontogenic epithelium was considered. The patient was treated with surgical resection, and treatment decisions were made with consideration of the need for eradication of tumor as well as tissue preservation to allow normal growth and development. A review of the literature indicated a preponderance of gingival–alveolar ridge as the site of OSCC in children with no known genetic predisposition to cancer. More studies of this rare subset of OSCC will help understand the underlying biology and guide treatment decisions. Oral squamous cell carcinoma (OSCC) predominantly affects individuals over 40 years of age and is extremely rare in patients younger than 20 years of age.1Bhanuprasad V. Mallick S. Bhasker S. Mohanti B.K. Pediatric head and neck squamous cell carcinoma: report of 12 cases and illustrated review of literature.Int J Pediatr Otorhinolaryngol. 2015; 79: 1279-1282Abstract Full Text Full Text PDF Scopus (13) Google Scholar, 2Bodner L. Manor E. Friger M.D. van der Waal I. Oral squamous cell carcinoma in patients twenty years of age or younger—review and analysis of 186 reported cases.Oral Oncol. 2014; 50: 84-89Abstract Full Text Full Text PDF PubMed Scopus (50) Google Scholar The diagnosis of OSCC in pediatric patients may present a significant challenge to the clinician, because it may be mistaken for a reactive lesion, which may cause a delay in diagnosis.3Bill T.J. Reddy V.R. Ries K.L. Gampper T.J. Hoard M.A. Adolescent gingival squamous cell carcinoma: report of a case and review of the literature.Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2001; 91: 682-685Abstract Full Text Full Text PDF PubMed Scopus (30) Google Scholar, 4Woo V.L. Kelsch R.D. Su L. Kim T. Zegarelli D.J. Gingival squamous cell carcinoma in adolescence.Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2009; 107: 92-99Abstract Full Text Full Text PDF PubMed Scopus (13) Google Scholar Other important considerations include careful treatment planning and prognosis and the possible long-term impact on the development of the facial structures, including teeth and jaws.5Hutton A. McKaig S. Bardsley P. Monaghan A. Parmar S. Oral carcinoma cuniculatum in a young child.J Clin Pediatr Dent. 2010; 35: 89-94Crossref PubMed Scopus (21) Google Scholar Because of the limited number of reports of OSCC in pediatric patients, the risk factors and clinical behavior of this rare disease are poorly understood.2Bodner L. Manor E. Friger M.D. van der Waal I. Oral squamous cell carcinoma in patients twenty years of age or younger—review and analysis of 186 reported cases.Oral Oncol. 2014; 50: 84-89Abstract Full Text Full Text PDF PubMed Scopus (50) Google Scholar Here, we present a case of OSCC of the maxilla in an 8-year-old patient and provide a brief review of the current literature. The clinical, radiographic, and histologic findings in this patient were distinctive, and there were challenges in the diagnosis and treatment. Follow-up examination of the patient showed a favorable clinical course, which supported the choice of treatment for the patient. A healthy 8-year-old male presented with a 2-week history of left facial swelling that was gradually increasing in size. Intraoral examination revealed a pink-red, oval soft tissue mass, measuring 4 cm in maximum diameter, located in the left maxillary ridge. The lesion extended from the deciduous molars to the distal aspect of the first molar, which was mobile. The clinical differential diagnosis included peripheral giant cell granuloma, fibrosarcoma, and malignant fibrous histiocytoma. Cone-beam computed tomography (CBCT) examination revealed an osteolytic lesion on the buccal aspect of the left maxillary alveolar ridge in the region of the first molar, with destruction of the buccal cortex (Figure 1A and B ). The floor of the maxillary sinus was displaced superiorly, and there was a thin, smooth layer of periosteal new bone over the tumor mass (Figure 1 and Figure 4A). Inflammatory mucosal thickening was present in the left maxillary sinus but later absent on the multidetector CT, suggesting a transient mucositis. The roots of the first molar were medially displaced, but there was no evidence of root resorption. The CBCT findings were originally interpreted as a benign non-odontogenic lesion. It was unclear if the lesion was intraosseous or peripheral in origin, but the relationship of the tumor mass to the buccal cortex at the anterior aspect of the lesion was suggestive of an intraosseous lesion that had extended into the overlying mucosa.Fig. 2Axial multidetector computed tomography (CT) image showing the extent of the left maxillary tumor (posterior extent indicated by black arrows) and displacement of the facial artery and buccal fat pad laterally (white arrow). A, Bone window. B, Soft tissue window. The multi-detector CT study is available for viewing as DICOM data.View Large Image Figure ViewerDownload (PPT) An incisional biopsy was performed and revealed carcinoma extending from an irregular, ulcerated mucosal surface into the connective tissue of the entire biopsy (Figure 3A). Most of the tumor consisted of sheets of basaloid cells with focal areas of abrupt squamous differentiation (Figure 3B) and some of the tumor islands had a ragged periphery, with small cords and single tumor cells invading into the connective tissue stroma (Figure 3C). The basaloid cells had oval, vesicular nuclei without marked pleomorphism and numerous mitotic figures (Figure 3D). Basaloid SCC was considered, but the lack of peripheral palisading, comedonecrosis, and deposition of hyaline material within the tumor islands were not consistent with that diagnosis.6Gnepp D.R. Diagnostic Surgical Pathology of the Head and Neck. Saunders, St. Louis, MO2009Google Scholar The biopsy result was carcinoma with squamous differentiation that could have arisen from mucosal epithelium or odontogenic epithelium. The patient was referred to the Pediatric Head and Neck Oncology service. Clinical evaluation showed the patient to be otherwise healthy, with no history of genetic cancer predisposition syndromes or previous chemotherapy or radiation therapy for malignant disease. There was no history of injury to the jaws or exposure to tobacco. A multidetector CT scan of head and neck area was obtained to evaluate the left maxillary lesion in greater detail, and it revealed a large tumor mass centered at the buccal surface of the left maxilla at the first molar region, displacing the facial artery and the buccal fat plane laterally (Figure 2A and B ). The bony crypts of the developing second premolar and the second molar were mostly intact, except for defects adjacent to the lesion, and the follicular spaces of these teeth appeared uniform (Figure 4A and B). Clinical examination of the neck showed no lymphadenopathy; CT and magnetic resonance imaging did not show evidence of disease in the regional lymph nodes. Investigations for distant metastasis were not performed, given the low incidence of distant metastasis of this type of tumor. Therefore, the clinical stage for this tumor of the gingival–alveolar ridge was T3 N0 M0.7American Joint Committee on Cancer Cancer Staging Manual.6th ed. Springer-Verlag, New York2002Google Scholar The patient underwent left partial maxillectomy and the defect was closed with local advancement flaps. The surgical resection included the deciduous second molar, permanent first molar, and the developing second premolar and second molar. The intraoperative margins were negative. The resected tumor showed carcinoma with basaloid and squamous morphology, similar to the incisional biopsy. The surface mucosa showed a focal area of dysplasia, supporting an origin of the carcinoma from mucosal epithelium (Figure 5A and B ). The tumor was p16 positive, with strong but patchy staining of the tumor islands (Figure 5C). There was diffuse, strong nuclear positivity for p53 in the tumor cells (Figure 5D) and in the deep aspect of the adjacent mucosal epithelium. In situ hybridization for Epstein–Barr virus-encoded small RNAs (EBER) was negative. Tissue specimens from the gingiva at the medial aspect of the resection and from the deciduous second molar socket showed nests of basaloid epithelium with focal squamous differentiation and focal atypia. These areas of the epithelium were interpreted as being malignant rather than reactive odontogenic (Figure 6). Specimens from the floor of the left maxillary sinus were negative for carcinoma.Fig. 6Photomicrograph of one of the margins (deciduous second molar and periodontal tissue), showing nests of atypical squamous epithelium with central keratinization (hematoxylin and eosin, original magnification ×400). A high-resolution version of this slide for use with the Virtual Microscope is available as eSlide: .View Large Image Figure ViewerDownload (PPT) The final diagnosis was SCC of the left maxillary alveolus with positive margins. A decision was made not to perform repeat surgical excision of the margins because the defect was easy to monitor visually in the postoperative period. Radiation therapy was considered but ultimately not provided because of possible adverse effects on growth and development. The patient has been examined at 6-month intervals at the Head and Neck Oncology clinic; at 24 months following surgical resection, the surgical site was observed to have healed completely. There was no oral antral fistula and no evidence of recurrence. The patient also attends the dentistry and orthodontics clinics, where the growth and development of his dentition and jaws are monitored. He is functioning well, and there is no plan to restore the region prosthetically at this time because of his young age. Pediatric OSCC is a rare disease and may present a diagnostic and treatment challenge to the health care team. A literature search from 1976 to the present was made for reports of OSCC in patients younger than 20 years of age with no known predisposition to cancer, and 42 cases were found. Only reports including specific information on the age and gender of the patient and the site of the carcinoma were included (Table I).1Bhanuprasad V. Mallick S. Bhasker S. Mohanti B.K. Pediatric head and neck squamous cell carcinoma: report of 12 cases and illustrated review of literature.Int J Pediatr Otorhinolaryngol. 2015; 79: 1279-1282Abstract Full Text Full Text PDF Scopus (13) Google Scholar, 3Bill T.J. Reddy V.R. Ries K.L. Gampper T.J. Hoard M.A. Adolescent gingival squamous cell carcinoma: report of a case and review of the literature.Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2001; 91: 682-685Abstract Full Text Full Text PDF PubMed Scopus (30) Google Scholar, 4Woo V.L. Kelsch R.D. Su L. Kim T. Zegarelli D.J. Gingival squamous cell carcinoma in adolescence.Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2009; 107: 92-99Abstract Full Text Full Text PDF PubMed Scopus (13) Google Scholar, 5Hutton A. McKaig S. Bardsley P. Monaghan A. Parmar S. Oral carcinoma cuniculatum in a young child.J Clin Pediatr Dent. 2010; 35: 89-94Crossref PubMed Scopus (21) Google Scholar, 8Amichetti M. Squamous cell carcinoma of the oral tongue in patients less than fifteen years of age. Report of a case and review of the literature.J Craniomaxillofac Surg. 1989; 17: 75-77Abstract Full Text PDF PubMed Scopus (20) Google Scholar, 9Atula S. Grenman R. Laippala P. Syrjanen S. Cancer of the tongue in patients younger than 40 years. A distinct entity?.Arch Otolaryngol Head Neck Surg. 1996; 122: 1313-1319Crossref PubMed Scopus (101) Google Scholar, 10Binahmed A. Charles M. Campisi P. Forte V. Carmichael R.P. Sandor G.K. Primary squamous cell carcinoma of the maxillary alveolus in a 10-year-old girl.J Can Dent Assoc. 2007; 73: 715-718PubMed Google Scholar, 11de Carvalho M.B. Sobrinho Jde A. Rapoport A. et al.Head and neck squamous cell carcinoma in childhood.Med Pediatr Oncol. 1998; 31: 96-99Crossref PubMed Scopus (22) Google Scholar, 12Earle A.S. Park C.H. Vlastou C. Oral squamous cell carcinoma in children.Ann Plast Surg. 1988; 20: 148-152Crossref PubMed Scopus (23) Google Scholar, 13Fadoo Z. Naz F. Husen Y. Pervez S. Hasan N.U. Squamous cell carcinoma of tongue in an 11-year-old girl.J Pediatr Hematol Oncol. 2010; 32: e199-e201Crossref PubMed Scopus (6) Google Scholar, 14Jin Y.T. Myers J. Tsai S.T. Goepfert H. Batsakis J.G. el-Naggar A.K. Genetic alterations in oral squamous cell carcinoma of young adults.Oral Oncol. 1999; 35: 251-256Abstract Full Text Full Text PDF PubMed Scopus (46) Google Scholar, 15Mehanna P. Patel J. Bailey B.M. Mandibular SCC in a 10 year old child—a clinical rarity.Br J Oral Maxillofac Surg. 2009; 47: 148-150Abstract Full Text Full Text PDF PubMed Scopus (9) Google Scholar, 16Patel D.D. Dave R.I. Carcinoma of the anterior tongue in adolescence.Cancer. 1976; 37: 917-921Crossref PubMed Scopus (23) Google Scholar, 17Randhawa T. Shameena P. Sudha S. Nair R. Squamous cell carcinoma of tongue in a 19-year-old female.Indian J Cancer. 2008; 45: 128-130Crossref PubMed Scopus (20) Google Scholar, 18Ribeiro C.M. Gueiros L.A. Leon J.E. do Carmo Abreu e Lima M. de Almeida O.P. Leao J.C. Oral squamous cell carcinoma in a 7-year-old Brazilian boy.Int J Oral Maxillofac Surg. 2011; 40: 994-997Abstract Full Text Full Text PDF PubMed Scopus (10) Google Scholar, 19Sacks H.G. Holly R. Blum B. Joy E.D. Case 58: maxillary alveolar mass in a 13-year-old boy.J Oral Maxillofac Surg. 1985; 43: 958-963Abstract Full Text PDF PubMed Scopus (15) Google Scholar, 20Sidell D. Nabili V. Lai C. Cheung G. Kirsch C. Abemayor E. Pediatric squamous cell carcinoma: case report and literature review.Laryngoscope. 2009; 119: 1538-1541Crossref PubMed Scopus (20) Google Scholar, 21Smith A. Petersen D. Samant S. Ver Halen J.P. Pediatric mandibular reconstruction following resection of oral squamous cell carcinoma: a case report.Am J Otolaryngol. 2014; 35: 826-828Abstract Full Text Full Text PDF PubMed Scopus (9) Google Scholar, 22Solanki M.C. Gandhi S. Koshy G. Mathew G.C. Squamous cell carcinoma of the maxilla in 10 year-old boy: a rare case report.Int J Pediatr Otorhinolaryngol Extra. 2012; 7: 33-35Abstract Full Text Full Text PDF Scopus (5) Google Scholar, 23Son Y.H. Kapp D.S. Oral cavity and oropharyngeal cancer in a younger population. Review of literature and experience at Yale.Cancer. 1985; 55: 441-444Crossref PubMed Scopus (122) Google Scholar, 24Soni N.K. Chatterji P. Carcinoma of the tongue in a young child of eleven years.Indian Pediatr. 1982; 19: 187-189PubMed Google Scholar, 25Stolk-Liefferink S.A. Dumans A.G. van der Meij E.H. Knegt P.P. van der Wal K.G. Oral squamous cell carcinoma in children: review of an unusual entity.Int J Pediatr Otorhinolaryngol. 2008; 72: 127-131Abstract Full Text Full Text PDF PubMed Scopus (29) Google Scholar, 26Sturgis E.M. Moore B.A. Glisson B.S. Kies M.S. Shin D.M. Byers R.M. Neoadjuvant chemotherapy for squamous cell carcinoma of the oral tongue in young adults: a case series.Head Neck. 2005; 27: 748-756Crossref PubMed Scopus (22) Google Scholar, 27Torossian J.M. Beziat J.L. Philip T. Bejui F.T. Squamous cell carcinoma of the tongue in a 13-year-old boy.J Oral Maxillofac Surg. 2000; 58: 1407-1410Abstract Full Text Full Text PDF PubMed Scopus (12) Google Scholar, 28Usenius T. Karja J. Collan Y. Squamous cell carcinoma of the tongue in children.Cancer. 1987; 60: 236-239Crossref PubMed Scopus (14) Google Scholar The ages of the patients varied between 6 and 19 years. There were 29 males and 13 females. The most common sites were the gingiva and the tongue, with 21 cases and 18 cases, respectively. Twelve of the 42 patients were 10 years old or younger, and all had been diagnosed with carcinoma of the gingiva. The present report added one more case of a patient younger than 10 years of age with carcinoma of the gingival–alveolar ridge.Table ISummary of reported cases of oral squamous cell carcinoma in patients younger than 20 years of ageAuthorYearAgeGenderSiteHistologic gradeOutcomePatel and Dave16Patel D.D. Dave R.I. Carcinoma of the anterior tongue in adolescence.Cancer. 1976; 37: 917-921Crossref PubMed Scopus (23) Google Scholar197616MTonguen.s.DOD 1 year17MTonguen.s.NED16MTonguen.s.DOD 1 year11MTonguen.s.DOD 1 year11MTonguen.s.DOD 1 yearSoni and Chatterji24Soni N.K. Chatterji P. Carcinoma of the tongue in a young child of eleven years.Indian Pediatr. 1982; 19: 187-189PubMed Google Scholar198211MTonguen.s.n.s.Son and Kapp23Son Y.H. Kapp D.S. Oral cavity and oropharyngeal cancer in a younger population. Review of literature and experience at Yale.Cancer. 1985; 55: 441-444Crossref PubMed Scopus (122) Google Scholar198510MMx gingivaWellNED 274 m17FBuccalWellDOD 19 m18MTongueWellDOD 9 m19MTongueWellDOD 15 mSacks et al.19Sacks H.G. Holly R. Blum B. Joy E.D. Case 58: maxillary alveolar mass in a 13-year-old boy.J Oral Maxillofac Surg. 1985; 43: 958-963Abstract Full Text PDF PubMed Scopus (15) Google Scholar198513MMx gingivaWellNED 24 mUsenius et al.28Usenius T. Karja J. Collan Y. Squamous cell carcinoma of the tongue in children.Cancer. 1987; 60: 236-239Crossref PubMed Scopus (14) Google Scholar198714MTongueKeratinizingDOD 8 mEarle et al.12Earle A.S. Park C.H. Vlastou C. Oral squamous cell carcinoma in children.Ann Plast Surg. 1988; 20: 148-152Crossref PubMed Scopus (23) Google Scholar19887MMx gingivaModerateNED 30 mAmichetti.8Amichetti M. Squamous cell carcinoma of the oral tongue in patients less than fifteen years of age. Report of a case and review of the literature.J Craniomaxillofac Surg. 1989; 17: 75-77Abstract Full Text PDF PubMed Scopus (20) Google Scholar198914FTongueModerateDOD 5 mAtula et al.9Atula S. Grenman R. Laippala P. Syrjanen S. Cancer of the tongue in patients younger than 40 years. A distinct entity?.Arch Otolaryngol Head Neck Surg. 1996; 122: 1313-1319Crossref PubMed Scopus (101) Google Scholar199619FTongueWellDOD 22 m19MTongueWellNED 6 yearsde Carvalho et al.11de Carvalho M.B. Sobrinho Jde A. Rapoport A. et al.Head and neck squamous cell carcinoma in childhood.Med Pediatr Oncol. 1998; 31: 96-99Crossref PubMed Scopus (22) Google Scholar19986FMx gingivan.s.NED 130 m14MMx gingivan.s.DOD 6 mJin et al.14Jin Y.T. Myers J. Tsai S.T. Goepfert H. Batsakis J.G. el-Naggar A.K. Genetic alterations in oral squamous cell carcinoma of young adults.Oral Oncol. 1999; 35: 251-256Abstract Full Text Full Text PDF PubMed Scopus (46) Google Scholar19996.5FGingivaWellNED 6.2 yTorossian et al.27Torossian J.M. Beziat J.L. Philip T. Bejui F.T. Squamous cell carcinoma of the tongue in a 13-year-old boy.J Oral Maxillofac Surg. 2000; 58: 1407-1410Abstract Full Text Full Text PDF PubMed Scopus (12) Google Scholar200013MTongueWellNED 2 yearsBill et al.3Bill T.J. Reddy V.R. Ries K.L. Gampper T.J. Hoard M.A. Adolescent gingival squamous cell carcinoma: report of a case and review of the literature.Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2001; 91: 682-685Abstract Full Text Full Text PDF PubMed Scopus (30) Google Scholar200114MMd gingivaWellNED 36 mSturgis et al.26Sturgis E.M. Moore B.A. Glisson B.S. Kies M.S. Shin D.M. Byers R.M. Neoadjuvant chemotherapy for squamous cell carcinoma of the oral tongue in young adults: a case series.Head Neck. 2005; 27: 748-756Crossref PubMed Scopus (22) Google Scholar200518MTonguen.s.NED 1 m16FTongueWellNED 51 mBinahmed et al.10Binahmed A. Charles M. Campisi P. Forte V. Carmichael R.P. Sandor G.K. Primary squamous cell carcinoma of the maxillary alveolus in a 10-year-old girl.J Can Dent Assoc. 2007; 73: 715-718PubMed Google Scholar200710FMx gingivaWellNED 2 yearsStolk-Liefferink et al.25Stolk-Liefferink S.A. Dumans A.G. van der Meij E.H. Knegt P.P. van der Wal K.G. Oral squamous cell carcinoma in children: review of an unusual entity.Int J Pediatr Otorhinolaryngol. 2008; 72: 127-131Abstract Full Text Full Text PDF PubMed Scopus (29) Google Scholar200811MMx gingivaWelln.s.Randhawa et al.17Randhawa T. Shameena P. Sudha S. Nair R. Squamous cell carcinoma of tongue in a 19-year-old female.Indian J Cancer. 2008; 45: 128-130Crossref PubMed Scopus (20) Google Scholar200819FTongueModerateDOD 8 mMehanna et al.15Mehanna P. Patel J. Bailey B.M. Mandibular SCC in a 10 year old child—a clinical rarity.Br J Oral Maxillofac Surg. 2009; 47: 148-150Abstract Full Text Full Text PDF PubMed Scopus (9) Google Scholar200910MMd gingivaWellNED 1 yearSidell et al.20Sidell D. Nabili V. Lai C. Cheung G. Kirsch C. Abemayor E. Pediatric squamous cell carcinoma: case report and literature review.Laryngoscope. 2009; 119: 1538-1541Crossref PubMed Scopus (20) Google Scholar20096MMx gingivaWelln.s.Woo et al.4Woo V.L. Kelsch R.D. Su L. Kim T. Zegarelli D.J. Gingival squamous cell carcinoma in adolescence.Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2009; 107: 92-99Abstract Full Text Full Text PDF PubMed Scopus (13) Google Scholar200918FMd gingivaWellNED 51 m17MMx gingivaWellNED 46 m11FMx gingivaWellNED 6 m11FMd gingivaWellNED 24 mHutton et al.5Hutton A. McKaig S. Bardsley P. Monaghan A. Parmar S. Oral carcinoma cuniculatum in a young child.J Clin Pediatr Dent. 2010; 35: 89-94Crossref PubMed Scopus (21) Google Scholar20107FMx gingivaWellNED 2 yearsFadoo et al.13Fadoo Z. Naz F. Husen Y. Pervez S. Hasan N.U. Squamous cell carcinoma of tongue in an 11-year-old girl.J Pediatr Hematol Oncol. 2010; 32: e199-e201Crossref PubMed Scopus (6) Google Scholar201011FTonguen.s.No treatmentRibeiro et al.18Ribeiro C.M. Gueiros L.A. Leon J.E. do Carmo Abreu e Lima M. de Almeida O.P. Leao J.C. Oral squamous cell carcinoma in a 7-year-old Brazilian boy.Int J Oral Maxillofac Surg. 2011; 40: 994-997Abstract Full Text Full Text PDF PubMed Scopus (10) Google Scholar20117MMx gingivaWellNED 18 mSolanki et al.22Solanki M.C. Gandhi S. Koshy G. Mathew G.C. Squamous cell carcinoma of the maxilla in 10 year-old boy: a rare case report.Int J Pediatr Otorhinolaryngol Extra. 2012; 7: 33-35Abstract Full Text Full Text PDF Scopus (5) Google Scholar201210MMx gingivaWellNED 1 yearSmith et al.21Smith A. Petersen D. Samant S. Ver Halen J.P. Pediatric mandibular reconstruction following resection of oral squamous cell carcinoma: a case report.Am J Otolaryngol. 2014; 35: 826-828Abstract Full Text Full Text PDF PubMed Scopus (9) Google Scholar20146MMd gingivaModeraten.s.Bhanuprasad et al.1Bhanuprasad V. Mallick S. Bhasker S. Mohanti B.K. Pediatric head and neck squamous cell carcinoma: report of 12 cases and illustrated review of literature.Int J Pediatr Otorhinolaryngol. 2015; 79: 1279-1282Abstract Full Text Full Text PDF Scopus (13) Google Scholar201510MGingivaModerateLost to follow-up15MGingivaModerateLost to follow-up15MTongueModerateDOD17MHard palatePoorNED 1.5 year19MFloor of mouthModerateNED 2 yearsDOD, died of disease; Md, mandibular; Mx, maxillary; NED, no evidence of disease; n.s., not stated. Open table in a new tab DOD, died of disease; Md, mandibular; Mx, maxillary; NED, no evidence of disease; n.s., not stated. The etiology and pathogenesis of OSCC in the first 2 decades of life are thought to differ from OSCC in adult patients. Most of the patients have not had significant exposure to tobacco, alcohol, or betel nut, which are significant risk factors for OSCC in adults. However, genetic cancer predisposition syndromes have been reported to increase the risk of OSCC in young patients. Patients with Fanconi anemia, xeroderma pigmentosum, and dyskeratosis congenita are at increased risk of OSCC in the first 2 decades of life, with the majority of cases occurring in the tongue.2Bodner L. Manor E. Friger M.D. van der Waal I. Oral squamous cell carcinoma in patients twenty years of age or younger—review and analysis of 186 reported cases.Oral Oncol. 2014; 50: 84-89Abstract Full Text Full Text PDF PubMed Scopus (50) Google Scholar The predilection of oral carcinoma to develop in the gingiva and the alveolar ridge in young patients suggests an association with tooth development and eruption, but further studies are needed to support this hypothesis.2Bodner L. Manor E. Friger M.D. van der Waal I. Oral squamous cell carcinoma in patients twenty years of age or younger—review and analysis of 186 reported cases.Oral Oncol. 2014; 50: 84-89Abstract Full Text Full Text PDF PubMed Scopus (50) Google Scholar The case presented here showed unusual clinical and histologic features and led to an interesting discussion about etiologic factors, central vs peripheral origin, and treatment. Clinically, an enlarging swelling associated with teeth in a young patient may be mistaken for a reactive lesion or an odontogenic tumor.4Woo V.L. Kelsch R.D. Su L. Kim T. Zegarelli D.J. Gingival squamous cell carcinoma in adolescence.Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2009; 107: 92-99Abstract Full Text Full Text PDF PubMed Scopus (13) Google Scholar The initial CBCT findings of the present case suggested a benign lesion, which was in sharp contrast to the clinical appearance that was suspicious for malignant disease. An incisional biopsy provided the diagnosis of carcinoma and thus led to timely treatment. The microscopic findings of this case were also unusual. Histopathologic analysis revealed a predominantly basaloid morphology with focal areas of abrupt squamous differentiation, whereas the majority of OSCC cases in pediatric populations had been reported to have a well-differentiated appearance (Table I). There was no comedonecrosis, hyaline intercellular deposits, nuclear pleomorphism, or peripheral palisading of the tumor islands to indicate basaloid SCC, which is considered an aggressive tumor with frequent metastases.6Gnepp D.R. Diagnostic Surgical Pathology of the Head and Neck. Saunders, St. Louis, MO2009Google Scholar In the histopathologic analysis of the resected specimen, it was challenging to evaluate the surgical margins for carcinoma because the nests of basaloid epithelial cells that were seen could represent carcinoma or reactive odontogenic epithelium associated with developing teeth. A viral etiology of this carcinoma was considered, including Epstein-Barr virus and high-risk human papillomavirus (HPV). In situ hybridization for Epstein-Barr early RNA was negative. There was positive staining for p16 in a patchy pattern suggestive of HPV; however, further investigations for HPV by genotyping were not performed. Studies of OSCC have shown a low etiologic fraction for oncogenic HPV, and p16 immunohistochemical staining was found to have a poor positive predictive value for detection of HPV in oral sites.29Lingen M.W. Xiao W. Schmidt A. et al.Low etiologic fraction for high-risk human papillomavirus in oral cavity squamous cell carcinomas.Oral Oncol. 2013; 49: 1-8Abstract Full Text Full Text PDF PubMed Scopus (255) Google Scholar We have also considered an intraosseous vs a mucosal origin of this tumor. Although the clinical presentation and the histologic finding of dysplastic mucosal epithelium favored a mucosal origin, some of the radiographic features suggested an intraosseous origin. Overall, we concluded that this likely represented a mucosal OSCC rather than a rare intraosseous OSCC. Treatment decisions were made empirically in this case because of the unusual combination of clinical, radiographic, and histologic features. The patient was treated with a partial maxillectomy, without postoperative radiotherapy or chemotherapy. The final histopathologic diagnosis was SCC of the left maxillary alveolar ridge with tumor present in two of the margins. The decision was made not to perform repeat surgical excision of the margins because the defect was easy to monitor in the postoperative period, and there was concern about the adverse effects of more extensive surgery on the growth and development of the young patient. The patient has been monitored closely, and there has been no evidence of disease recurrence to date, 24 months after surgical treatment of the carcinoma. We present a rare case of OSCC in an otherwise healthy 8-year-old boy. The carcinoma was located in the gingival and alveolar ridge, which is the most common location for OSCC in this demographic group. The histologic features of this case were unusual in comparison with reported cases of pediatric OSCC and raised the possibility of having an odontogenic epithelium origin. The postoperative course was uneventful, and the patient remained disease free at 16 months after surgical resection, which has validated the treatment decisions that were made. More studies may help delineate a subset of OSCC in pediatric patients and guide treatment.