Oral squamous cell carcinoma in children;
review of an unusual entity
, A.G. Dumansa
, E.H. van der Meija,
* , P.P. Knegtb
, K.G.H. van der Wala
aDepartment of Oral and Maxillofacial Surgery, Erasmus University Medical Center, Rotterdam, The Netherlands
bDepartmant of Otorhinolaryngology and Head and Neck Surgery, Erasmus University Medical Center, Rotterdam, The Netherlands
Received 27 June 2007; received in revised form 28 August 2007; accepted 2 September 2007 Available online 29 October 2007
Squamous cell carcinoma (SCC) of the oral cavity is rare in patients of age 40 and younger, being pri- marily a disease that occurs in males in their sixth or seventh decade. Younger patients (aged less than 40 years) account for approximately 4% of all oral cancers . Only a small sample of this subgroup exists of pediatric patients (20 years), making oral SCC in the pediatric age group an extremely rare entity.
The purpose of the present report is to describe an 11-year-old boy who developed a SCC of the gingiva, and to review the literature of oral SCC in pediatric patients.
2. Case report
An 11-year-old boy was referred to the Department of Oral and Maxillofacial Surgery by his orthodontist because of a non-tender, progressive growing swel- ling of the gingiva of the upper front teeth, noticed by the patient since 6 weeks. The relevant medical history did not reveal any abnormalities. The patient did not use any medication at presentation.
Physical examination revealed a firm, non-ten- der, verrucous swelling with indurated borders and central ulceration on the buccal and palatal aspects
Squamous cell carcimoma;
Summary Most commonly, squamous cell carcinoma (SCC) of the oral cavity pre- sents during the fifth and sixth decades of life. Less than 4% of these cancers occur in patients younger than 40 years of age. Only a small sample of this subgroup exists of pediatric patients (20 years), making oral SCC in children an extremely rare entity.
An 11-year-old boy is presented who developed a SCC of the gingiva. The relevant literature of oral SCC in pediatric patients will be reviewed as well.
#2007 Elsevier Ireland Ltd. All rights reserved.
* Corresponding author at: Department of Oral and Maxillofa- cial Surgery, Erasmus University Medical Center, P.O. Box 2040, 3000 CA Rotterdam, The Netherlands. Tel.: +31 10 4634138;
fax: +31 10 4633098.
E-mail address:firstname.lastname@example.org (E.H. van der Meij).
0165-5876/$ — see front matter # 2007 Elsevier Ireland Ltd. All rights reserved.
of the left upper front teeth measuring 2.5 cm by 2.0 cm (Fig. 1). The left upper incisors were mobile but tested positive on vitality tests. Multiple, non- tender, mobile submandibular lymph nodes were palpated bilaterally. On additional radiographic examination erosion, possibly infiltration of several millimeters of the alveolar bone between the two left upper incisors was observed. Histopathological examination of an incisional biopsy of the swelling showed a well-differentiated SCC. Because of the age of the patient there was some doubt about the diagnosis; a verruciform xanthoma with character- istics of pseudoepitheliomatous hyperplasia (PEH) was therefore considered as well (Fig. 2a).
A second biopsy specimen showed on microscopic examination again characteristics of a well-differ- entiated SCC (Fig. 2b). Based on fine needle aspira- tion cytology of the submandibular lymph nodes as well as a radiograph of the chest there were no signs of regional and/or distant metastasis. An additional computed tomogram revealed invasion of the buccal cortex of the left upper incisors (Fig. 3). Nineteen days after the initial presentation the tumor was diagnosed as a T4aN0M0 SCC of the gingiva of the left maxilla. The tumor was obviously grown in this short period (Fig. 4).
A partial maxillectomy was performed. The former diagnosis of a well-differentiated SCC was
confirmed on histopathological examination of the surgical specimen. The margins seemed to be free of tumor.
Most commonly, SCC of the oral cavity presents during the fifth and sixth decades of life. Less than 4% of these cancers occur in patients younger than 40 years of age . Only a small sample of this subgroup exists of pediatric patients (20 years), making oral SCC in children an extremely rare entity. The results of a literature review on oral SCC in pediatric patients from the period 1970—2005 have been summarized inTable 1 [1—5,7,8,10—18].
A total of 65 cases were found. There was no gender predilection. The majority of tumors occurred on the tongue, the lip or the gingiva. Patients’ ages ranged from 2 to 20 years with only four cases being
128 S.A.H. Stolk-Liefferink et al.
Fig. 2 (a) A biopsy specimen from the swelling shows histopathological features of a well differentiated SCC. A verruciform xanthoma with characteristics of pseudoe- pitheliomatous hyperplasia (PEH) was considered as well (haematoxylin—eosin, 10). (b) A second biopsy specimen confirming the former diagnosis; a well differentiated SCC (haematoxylin—eosin, 10).
Fig. 1 A firm, non-tender, verrucous swelling with indu- rated borders and central ulceration on the buccal and palatal aspects of the left upper front teeth measuring 2.5 cm by 2.0 cm (a + b).
Table 1 Summary of studies and case reports reporting oral squamous cell carcinoma in pediatric patients (20 years) (1970—2005)
Authors Year Country No. of
Age (years) Site Histology (differentiation)
Byers 1975 USA 4 Unknown 17, 19, 19, 19 Tongue, n = 4 Well, n = 1; moderate,
n = 1; poor, n = 1
Krolls and Hoffman 1976 USA 19 Unknown 14, n = 3;
15—19, n = 16
Harper and Copeman 1981 UK 1 1/0 18 Tongue Poor
Yagi K et al. 1981 Sudan 1 0/1 10 Tongue Well
McGregor et al. 1983 Canada 1 0/1 18 Tongue Unknown
Newman et al. 1983 USA 4 Unknown 14, 16, 18, 18 Tongue, n = 4 Unknown
Son and Kapp 1985 USA 4 3/1 10, 17, 18, 19 Tongue, n = 2; cheek,
n = 1; gingiva, n = 1
Well, n = 4
Sacks et al. 1985 USA 1 1/0 13 Gingival Well
Earle et al. 1988 USA 1 1/0 7 Gingiva Moderate
Keukens et al. 1989 The Netherlands 1 1/0 9 Tongue Well
Lund and Howard 1990 UK 1 Unknown 20 Tongue Unknown
Tsukuda et al. 1993 Japan 4 2/2 14—19, n = 4 Unknown Unknown
Sarkaria and Harari 1994 USA 1 1/0 17 Tongue Unknown
Atula et al. 1996 Finland 1 1/0 19 Tongue Unknown
Thompson et al. 1999 USA 20 10/10 2—20 Tongue n = 9; lip n = 6; unknown n = 5 Well—poor
Bill et al. 2001 USA 1 1/0 14 Gingiva Well
younger than 12 years, making our case of an oral SCC in an 11-year-old boy unique.
Recently it has been observed that there is an increasing incidence of oral SCC in the younger population of several countries . The role of traditional risk factors such as tobacco, alcohol, betel quid chewing, and low consumption of fruits and vegetables is unclear in this age group. Some studies have suggested that these patients may exhibit a predisposition to genetic instability.
There is a general trend in reported studies for SCC of the oral cavity in young patients to be particularly aggressive and carry a poorer prognosis than older patients. A poorer prognosis in young patients could be due to (1) a differing disease etiology and tumor behavior or (2) delay in presen- tation and/or diagnosis (patients’ and/or doctors’
delay), the latter obviously playing a role in the currently presented case. The presentational and diagnostic delays may arise because of the reduced expectation of cancer in the young patient.
As inflammatory lesions in pediatric patients can become highly proliferative and assume neoplasm- like characteristics, the histopathologic diagnosis of SCC may present difficulties in this specific patient group. Pseudoepitheliomatous hyperplasia (PEH), a reactive process, may then be difficult to distinguish from SCC. Although differentiation between PEH and SCC in the adult patient group is generally not difficult for an experienced pathologist, PEH in pediatric patients may exhibit cellular atypia and irregular growth to such extend that this dis- tinction may be less obvious. The difficulties that were encountered in our patient in attempting to histopathologically distinguish between PEH and SCC are comparable with the difficulties seen in several previously reported case of SCC in pediatric patients[4,13].
Although rare, SCC does occur in pediatric patients and should therefore be included in the differential diagnosis of inflammatory-like, rapid growing lesions of the oral cavity. PEH may be difficult to histopathologically distinguish from SCC in this specific patient group. Close collabora- tion between clinician and pathologist is therefore from utmost importance to achieve the assessment of a proper diagnosis.
 S. Atula, R. Gre´nman, P. Laippala, S. Syrja¨nen, Cancer of the tongue in patients younger than 40 years, Arch. Laryngol.
Head Neck Surg. 122 (1996) 1313—1319.
 T.J. Bill, V.R. Reddy, K.L. Ries, T.J. Gampper, M.A. Hoard, Adolescent gingival squamous cell carcinoma: report of a case and review of the literature, Oral Surg. Oral Med. Oral Pathol. Oral Radiol. Endod. 91 (2001) 682—685.
 R.M. Byers, Squamous cell carcinoma of the oral tongue in patients less than thirty years of age, Am. J. Surg. 130 (1975) 475—478.
 A.S. Earle, C.H. Park, C. Vlastou, Oral squamous cell carci- noma in children, Ann. Plast. Surg. 20 (1988) 148—152.
 J.I. Harper, P.W. Copeman, Carcinoma of the tongue in a boy with xeroderma pigmentosum, Clin. Exp. Dermatol. 6 (1981) 601—604.
 A. Iamaroon, K. Pattanaporn, S. Pongsiriwet, S. Wanachan- tararak, S. prapayasatok, S. Jittidecharaks, et al., Analysis of 587 cases of oral squamous cell carcinoma in northern Thailand with a focus on young people, Int. J. Oral Max- illofac. Surg. 33 (2004) 84—88.
 F. Keukens, P.C. van Voorst Vader, A.K. Panders, S. Vinks, J.W. Oosterhuis, W.J. Kleijer, Xeroderma pigmentosum:
squamous cell carcinoma of the tongue, Acta Derm. Vener- eol. 69 (1989) 530—531.
 S.O. Krolls, S. Hoffman, Squamous cell carcinoma of the oral soft tissues: a statistical analysis of 14,253 cases by age, sex, and race of patients, J. Am. Dent. Assoc. 92 (1976) 571—574.
 C.D. Llewellyn, N.W. Johnson, S. Warnakulasuriya, Factors associated with delay in presentation among younger
130 S.A.H. Stolk-Liefferink et al.
Fig. 3 A computed tomogram revealed invasion of the buccal cortex of the left upper incisors.
Fig. 4 Nineteen days after the initial presentation. The tumor was obviously grown in this short period.
patients with oral cancer, Oral Surg. Oral Pathol. Oral Radiol.
Endod. 97 (2004) 707—713.
 V.J. Lund, D.J. Howard, Head and neck cancer in the young:
a prognostic conundrum? J. Laryngol. Otol. 104 (1990) 544—
 G.I. McGregor, N. Davis, R.E. Robins, Squamous cell carcinoma of the tongue and lower oral cavity in patients under 40 years of age, Am. J. Surg. 146 (1983) 88—92.
 A.N. Newman, D.H. Rice, R.H. Ossoff, G.A. Sisson, Carci- noma of the tongue in persons younger than 30 years of age, Arch. Otolaryngol. 109 (1983) 302—304.
 H.G. Sacks, R. Holly, B. Blum, E.D. Joy, Case 58: maxillary alveolar mass in a 13-year-old boy, J. Oral Maxillofac. Surg.
43 (1985) 958—963.
 J.N. Sarkaria, P.M. Harari, Oral tongue cancer in young adults less than 40 years of age: rationale for aggressive therapy, Head Neck 16 (1994) 107—111.
 Y.H. Son, D.S. Kapp, Oral cavity and oropharyngeal cancer in a younger population, Cancer 55 (1985) 441—444.
 L. Thompson, J. Castle, D.K. Heffner, Oral squamous cell carcinoma in pediatric patients: a clinicopathologic study of 20 cases [abstract], Oral Surg. Oral Med. Oral Pathol. Oral Radiol. Endod. 88 (1999) 204.
 M. Tsukuda, K. Ooishi, I. Mochimatsu, H. Sato, Head and neck carcinomas in patients under the age of forty years, Jpn. J.
Cancer Res. 84 (1993) 748—752.
 K. Yagi, A.E.L.-G. Ali, K.E.L.-D. Abbas, S.R. Prabhu, Carci- noma of the tongue in a patient with xeroderma pigmento- sum, Int. J. Oral Surg. 10 (1981) 73—76.
Available online at www.sciencedirect.com